DIAGNOSTIC IMAGING HEAD & NECK - Rick Harnsberger PDF

IMAG!:N POR RESON.
\NCIA
MAGNtTICA, SA DE C.V.
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IAGNOSTIC IMAGING
DIAGNOSTICIMAGING
HEAD AND NECK
H. Ric Harnsberger, MD H. Christian Davidson, MD
Professor of Radiol ogy/ Neu ro rad io logy Associa te Professo r o f Radi o logy
It C. W illey Ch air in Neuro rad io logy Vice Cha irma n, Depar t m en t o f Radiol ogy
Un ivers ity o f Uta h School of Medi cine Un iversity o f Uta h School of Med icine
Richard H. Wiggins III, MD Andre J. Macdonald, MBChB

Assi stant Professor of Radiology Atte nd in g Radio log ist, VA Salt Lake City Il ealth Ca re Syste m
Head a nd Nec k Ima gin g Ad ju nct Assista n t Pro fessor, Radi ology
Un ive rsity o f Uta h Sch oo l of Medi cine Un ive rsity o f Uta h Sch ool o f Medi cin e
Patricia A. Hudgins, MD Christine M. Glastonbury, MBBS

Professor o f Radi ol ogy/ Neuroradiology Assistant Clinical l'ro fcssor
Head and Neck Imaging San Franci sco G ene ral Ilospit al
Emo ry Un ivers ity Hospital University of California San Francisco
Michelle A. Michel, MD Joel K. Cure, MD

Associat e Pro fessor o f Radi ology and O to laryngo logy Associa te Professor of Radiology
Froedtert Memorial Lutheran Hospital U n iversit y o f Alaba ma M edi cal Ce n ter
C h ief, H ead and l':cd: Ne uroradio logy
M edical College of w tscon srn
Barton Branstetter I~ MD
Joel Swartz, MD Associ at e Pro fessor of Radi ol ogy a n d Ot ol aryngo logy
Uni versity o f Pittsbu rgh
l'resident, Germantown Imaging Associates Directorof Head and Neck Imaging
Gladwyne, Pennsylvania Associate Di rector o f Informatics
Uni ver... it)' o f Pittsburgh M edical Cen ter
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To j ungle j , (we' re d oin g pr ett y good Norm, 7ol?), m y rock a n d love o f m y life, a nd
m y :l so ns, BW-Da ve, Do o Da h I'< Dy l-Man , a ll m y reason s fo r bein g . Also , to m y
par ent s, Doris &: Hu tch, who ga ve m e m ore th an eno ug h love to m ak e it th ro ugh.
To To ny Man cu so , w ho turn ed m e o n to H I'< N im a g i n g . To j eff Hall er I'<

C lo ug h She lto n, t h a n ks for mak in g it fu n . To m y co lle ag ues wh o h ave
s t ru gg le d a lo ng w ith m e t oward m y Am irsys d ream , es pec ia lly Pau l Sc ho ltes
wh o rig h te d th e shi p wh en it tri ed to s in k. T h an ks Anni e O . &: t he "Hom e
Team " , I wo u ld n eve r have fini sh ed thi s c razy ph as e with ou t yo u g uys .
v
CONTRIBUTORS
Below are li sted the intrepid group o f ph ysician s wh o took the ti me

to help find the case material necessary to fill the ex tensive image
galleries of Dlag nostic Im agin g: Head £< eck, Withou t t he ir
willi ngu ess to "share the wealt h ", th is book woul d have been far
less rich an o ffering.
Thanks to yo u all. No hook of th is na t ure could have been don e

alo ne!
II. Ric Harn sbe rger, MD
An il T. Ah u ja, MO; H on g Kong , Ch ina

Susa n I. Hlnscr, MlJ; Toronto , Canada
lIar ton F. Bra nst e tt er. MD; l'ittsbu rgh , I'A
Phi lip Ch a p ma n, MD; Spoka ne , WA
j oe l K. Cur e, MD, Birm ingha m , AL
H. Ch ristia n Davidso n , MD; Salt Lake City, VT
Lawrence E. Ginsberg, MD; Hou ston, TX
C h rist in e M. Glasto nbury, MIlBS; San Fran cisco, CA
H. Ric Ha rn sbergcr, MD; Salt Lake City, VT
Gary L. Hed lun d , DO; Salt Lake Cit y, VT
Pat ricia A. Hudgin s, ~m ; Atla n ta , GA
Bernadett e Koch , M D; Cincin nati , O H
Andre Macd ona ld , MIlCh ll; Salt Lake C it y, VT
Mich ell e A. Mich el , MD; Milwaukee, WI
C. Do uglas Ph illip>, MD; Ch a rlo tt esville, VA
Anth o ny j . Scude ri, MD;jo hnst own , I'A
joe l D. Swa rtz, MD; Phil ad el ph ia, I'A
Ro be rt Wallace, MD; Ph oe n ix, AZ
Rich a rd II. Wiggins III. ~ m ; Salt Lak e Ci ty, VT
VII
DIAGNOSTIC IMAGING: HEAD AND NECK
\Vith the success o f hi s hi g hl y-acclnim cd Ha ndbook of Ilt:ad and Nt.·ck Imag in g, th e d iagn osti c rad iol ogy a nd
otolaryngology surgery communi ties ha ve wait ed fo r a new co mp rehensive text by Ric Harn sbc rge r. \Ve at Amirsys
a nd Elsevie r a rc proud to presen t a preceden t-set ting, image- and grap hics-packed sertes that co n tin ues with a brand-
new wor k by II. Ric Harn sbe rger and co lleagues . Th is spl end id wor k is tru ly a textb oo k o f th e twent y-first ce ntu ry:
No t yO U T o ld-fas h ioned . den se prose exposit io n wi th co m paratively few im ages. The uni q ue bu llet ed fo rm al of the
ViaSl1mtic Imasi"s books allow s o u r a utho rs to present a pproxima tely twice the in fo rmatio n and fou r times t he
im ages per d iagnosis, co m pa red to the o ld- fash lo ne d trad it ion al pro se tex tb ook.
Ikg ill ui ng with David Sto ller's I2.liW o ostic Im ag in g- O rt ho paedi cs fo llo wed by m y ll.ri!in volume, th ese ric h ly
illu strat ed hook s (over all ma jor hotl y areas. All th e Dis fo llo w a sim ila r fo rm at. The sa me in format ion is in the sa me
place: Eve ry tim e! An in no vation o ur readers have fo un d part icul arly welc ome is th e new visual differen tial
d iagnosis "th um bna il" tha t provides at -a-glan ce lo ok s at en tities that can mi mi c th e diagn osis in qu estion . "Key Fact s"
bo xes provide a succi nct sum mary for qu ick, ea sy review, In sho rt, this is a product designed with yo u, th e read er, in
mind. 'Io day's typ ical practi ce setti n gs demand efficien cy in hoth image in terp retatio n and lea rn tng. \Ve th ink yo u' ll
find til l' I JiaSl1o."i tic Im asillS format a highl y efficie nt and wo nderfully rich reso urc e. Enjo y!
Ann e G. Os bo rn. M D
Ed ito r-in- C h ie f, Am lrsys In c .
IX
FOREWORD
Th e rad iol ogy co m mu n ity h as a no the r " Hurnsbcrger" winner to add to it s co llectio n . w orl d f C110 W Il t'd Un iversit y
o f Ut ah EN'!' rad io logi st, Dr . Ric Harn sbcrgcr h as o u tdo ne himself wit h hi s l at est work of art , D i" s"mtic Imas;,zs: Hcud
011I1 Neck. p ub lish ed by Arn irsys, In c. Ric, assisted hy hi s ENT radi ol ogist co lleagues Drs. \Vigg in s, I ludgin s, Mich el,
Swa rtz, Davidson , Macd onald , Glasto nbury . Cure &. Bra nste tt er, has rai sed th e bar for h ead and neck radi ology texts,
p rod u ci n g a n info rm atl o u -d cnsc. d nta b ase-gcn cm tcd . d tagn o sts-bascd 1000 pa ge text 0 11 h ead and n ec k im ag in g. As
rad iol ogi sts art' n o w faced w it h less d iscre tion a ry (loadin g tim e and m ore work th an eve r befo re, th e n eed fo r co n cise.
qu ick so u rces o f referen ce info rm ati o n is critica l. V I: Head and Neck deli vers a so lutio n to th is prob lem. Thi s user-
designed textbook will fulfill the n eed for poin t of service an swe rs to H &. 0: pro b lem s th at co me up du rin g dail y
readout s.
V I: Head tl lld N eck: Is easy to navigat e. Like h is 1Ian dbook; o{l tnut & Neck Jl1Iasil1S, eac h sectio n hegin s wi t h An ato my
&. Imaging Issues cha pt er. J Iere crit ica l underlyin g an at o my and key H &. N im aging qu estio n s are presented . T he
remain de r of eac h sec t ion de li neates the key d iagnoses o f the a na to mic locat ion . Each diagn osis has a n ide nti ca l
or ga n izat ion with Te rmin ology, Imaging Findi ng s, Path o logy, Clin ical Issues and Diagn ost ic Che cklist. A key facts box
gives th e reade r th e o pt ion of a qui ck rev iew in stead of read ing th e en tire dia gn osis descr ipti o n . Th e style is repea ted
th rou gh t he book en ha n ced w it h ove r 3000 hi gh qua lity im ages and su pe rb co lo r illust rati o ns t hat de mo nst rate
var ia tio ns o f ima ging flnd in gs, an atom y a n d path ology.
()1: f kad und Neck: was crea ted fro m a ric h dep ository o f inf o rm ation integ rat ed in to a n XML d atabase of im ages
a nd text ga the red hy Ric ove r man y yea rs. I lis a tte n tio n to deta il a nd o rga niza tio na l ta len ts are eviden t th rou gh out
the book . Th e beaut y o f t hi s a ppro ach is t hat th e inf or mat ion ca n and wlll bc used to develop co rnplem cu ta ry
ed ucatio n a l prod ucts, such as I'D A program s and PACS reference data ba ses (STATdx ) for years to co m e. 1 loo k fo rwa rd
to havin g access to suc h offerings o n line at th e co m pute r wor ksta tio n, wh ich will en h ance clin ica l ca re, learn in g, a nd
teach in g .
It is my pleasure to welco me th is new o ffering fro m m y great frtc nd , Ric Harn sberger. I' m sure t hat it will qu ickl y
becom e a cla ssic in t he field fo r yea rs t o co me.
Will iam P. Dillon, i\.1 D

Professo r a nd v ice-C ha i r
Depa rtm ent of Radi ol ogy
Un iversity of Ca lifo rn ia, San iran ctsco
Xl
PREFACE
It ha s been my dr eam since finis hi ng th e Handbook of Head &. Nt;'ck Imagi ng to au th o r a textboo k in th is field .
l loweve r, I have never wanted to writ e a tr ad itio nal prose , embedded referen ce text. I find su ch bo ok s arc oft en
di fficult to naviga te, locate informati o n in and more important ly, m ust be re-written hom scrat ch with each new
edi tion . As th ere is usuall y d en se tex t in tr ad it ional textboo ks, th e n umber o f illustrat io ns iii ne cessa rily lim ited-
usuall y to clas sic imagi ng appea rances a nd only th e mo st co m mo n varia nts.
Diagnosti c Imaltin g: Head &: Keek (indeed. th e wh ole Diagn o st ic Imaging textboo k series tha t began w ith David
Sto ller 's Or t ho paedics an d An ne Os bor n's Brain ) is t he reali zation o f a IO-year jo urney. We have created a dat abase
system th at o utput s a n exc iti ng, br and-new typ e o f referen ce tex tbook in diag nostic rad iology.
An ile Os bor n a nd I had a vision whe n we started Amlrsys, We wa n ted to found an au t ho r-ce n t ric co mpany th at
wo uld be bot h market -facing a nd database-d riven, specializing in high ly in novati vc yet simple ways 0 1 pre scnting
co m plex co n lent. The cha nges we 've all ex perience d in our rad io logic pra ctice pattern s o ve r th e last decade manda te
th e need fo r new a pp roac he s. Our eve r-increasi ng case loa d makes ti me a luxur y mo st o f us d o n 't have. We need o u r
informati on in eas ily accevsihle forma t. Th e clowr to th e " po in t-o f-care" th e bett er!
The l.2ii!~ no\ t i c I m a ~ i.nJ,: series is slated to cover all majo r areas o f radiology . w ith th e publica tio n of 1)1: Head &
Ncrk, there are no w 3 o f t he 12 antic ipa ted vo lu me s avail able. The in itial two specta cular 0 1vo lu me'Sare David
Sto ller 's PI : Orthopaedics an d An ne Osborn 's 1>1' Brain . All th ree hooks use a su pe rabu ndanc e o f graph ics and hig h
in formatio n d en sity t hat is t he result of usin g bull et ed text (rat he r t ha n traditi onal prose) . You don 't ha ve time to read
extr a words th a t do n't ca rry essen tial informatio n-so we d o n 't write t hem ! \Vt..' added Key Facts h OXl' S to each
diagnosis for qui ck revi ew and have chose n selected references for yo u r furthe r d electa tion and delight. You will not e
that t here are man y 200"'" refer en ces incl ud ed-t he refere nce were upd ated to include key art tclcs publishe d withi n
two months o f t he hook's go ing to press.
PI · Head & Nt;'ck was produced (sta rt to fini sh ) in 9 months. To accom plish suc h a task in this sho rt time peri od
required th e undivided at tcn tlo n o f th e 9 o t he r accomplished Head & Neck radi ol ogists listed 0 11 t he bo ok Cove r.
Images ca me from ma n y generous case co n t rib u to rs, als o listed in th e front matter o f t he book. Man y o f th ese stellar
auth or s & case co n t ribut o rs are literally househ o ld nam es in Head & Nec k radiology- and th e o t he rs arc all
pr omisin g new yo u ng acad em ics. The templat ed ap proac h we took and til l' use o f bulleted infor mation elimi na te s
nearly a ll th e st ylisu« differences tha t have pla gued multi-author textbooks in th e pa st. \Vit ho ut looking at th e Table
of Co n ten ts, you probably ca n't tell wh o wrote what. \Ve feel th e sac rifice o f individua l creativity for th e sake o f
u n ifo rm ity in depth and qua lity is w('11 worth th is sac n ficc.
I believe yo u will find Dr: Head & Neck provides accessible ans we r" to yo u r Head &: Nec k q uestions qu ick ly and
easily where you nee d t hem , at t he " po in t o f ca re". Enjoy!
II. Hie Ha m sberger, Mil
XII I
ACKNOWLEDGMENTS
Illustrations
L11ll' R. Ben nion. ~1 S
Richard <:00 1111>:-;, '''is
ja mes A. Cooper, MD
Art Direction and Design

L lII l..' R. Bcu n hm , MS
Ric-b a rd Coombs. \ fS
Image/Text Editing
An gie D, Masrarcnaz
Kacrl l M ain
Medical Text Editing

An ne G. Osborn, M D
Kare n 1.. Salzm an, Ml)
Richa rd II. W iggin s III. M l)
Andre Ma cdo n ald , MO
Case Management
Cass ie L Deart h
David Har u sberger
Ro t h LaFleu r
Production Lead
xv
SECTIONS
PART I
Temporal Bone and Skull Base
CPA-lAC [I]
Temporal Bone ~
Skull Base ~
PART II
Orbit, Nose and Sinuses
Orbit [I]
Nose and Sinus ~
PART III
Suprahyoid & Infrahyoid Neck
Introduction and Overview rnJ
Pharyngeal Mucosal Space (PMS) [I]
Lymph Node Diseases ~
Hypopharynx, Larynx & Cervical Trachea ~
Oral Cavity [I]
Mandible/Maxilla ~
Masticator Space (MS) ~
Parotid Space (PS) [l]
Carotid Space (CS) lID
Retropharyngeal Space (RPS) [2]
Perivertebral Space (PVS) 1 01
Visceral Space (VS) 1111
Posterior Cervical Space (PCS) [1 21
PART IV
Pediatric & Trans-spatial Lesions
Pediatric Lesions [I]
Other Trans-spatial Lesions ~
XV II
TABLE OF CONTENTS
Supe rficia l Siderosis, C P,\ -IAC . -1-42
PART. II. Ric HlI f m bt.''.,\('r. :\I I)
Vascu la r Loop Co m pression , C PA-lAC
Temporal Bone and Skull Base II. Ric lIam d Jt",.l:C'f. .\ 10
SECTION 1 SECTION 2
CPA-lAC Temporal Bone
Introdu ctio n and Overview Introducti on and Over vi ew
CPA-lAC Ana tom y and Im aging Issues 1- 1-2 Te m po ral Bone Ana tom y and Imaging Issues 1-2-2
H. Ric IJaf m bcr..v,cr, All) H . Jar /lam~ / l{'rg('f. .\ In
Co nge nita l External Auditory Cana l

Epidermoid Cyst, C PA-lAC 1- 1-6 EAC Atresia 1-2-6
II. Rk lIaf m !Ja X('I. :\II) l 'lIlrid" A. Illldsil n , Mil
Arach noid Cyst, C PA-lAC 1- 1- 10 Necro tizing Ext erna l O ti tis 1-2· 10
H. Ric Illl rlHbt 'rSt'/~ ;\IlJ Rlct und 11. H '(,{l,'im III, ,\ 11 )
Lipoma, CPA-lAC 1- 1- 14 EAC Cho lesteato m a 1-2-14
If. Ric Jlarm[Ja gcr. su: Rictmrd 11. Wi.'i:..~ifl.~ III• .\IV
NF2, CPA· IAC I- I- Ill EAC Keratosi s Obtu ra n s 1-2- 16
I/. Ric flllfm l lf.'r:<er, ,\IV Richurd II. l\ ·i.'(~itl.\ III, ,\ fV
EAC Med ial Ca na l Fibrosi s 1-2- 18
In fection & Inflamm ati on Ric/IlI,d 11. \\'i.'(~il/.\ III, AllJ
ka m say lIu nt Syndrom e 1- 1-20 EAC Exo stoses 1-2-22

II. Ric Htu m ! l(".'.:(" , ;\IV Richard H . t\'i,,{liim III, ;\IlJ
Sarco idosis, C PA-lAC 1-1-24 F.AC Os teo ma . -2-24

H. Ric lIarm bo ;ga , .\IV Richard H. '\ 'i."~ ;1l5 III. MV
EAC Squamous Cell Ca rcino ma . -2-26
Ben ign Tum ors Rictturd II. a 'i,t.:,l,'ill.'i III. s tn
Acousti c Schwa n noma 1- 1-26

II. Ric 11"(11\/":' '.,\('" .\IV Middle Ear-M astoid, Congen ita l
M en in gioma , CPA-lAC 1- 1-30 Co nge n ita l Cholestea to ma, Middl e Ear 1-2-30
H. Ric 1I11rn \/lt''.o.; tT, stn Patriciv A . I lud.~;m. ,\ 10
faci al Ne rve Sch wa n n om a, C PA-lAC 1- 1-34 Ova l Window Atresia 1-2-34
II. Ric Ham \hf '..\wr, MI) !lH." V . Swar u , ,\ ILJ
Late ralized In te rn a l Ca ro tid Arte ry 1-2-36
M ali gn ant Tumo rs 1)lIt,icia A. IIl1tts i m , .\IV
Metastases, C PA-lAC 1- 1-36 Aberrant In tern a l Caro tid Arte ry 1-2-38

II. Ric If,,m,\ I't'I;ga , .... ,lJ Barton I·: Bran stetter 1\ : ;\I/)
Per sistent Sta ped ial Arter y . -2-42
1\'i,'{~ i ll.'i
Vascul ar Uk /lllrd 11. III. '\11 )
Ane ury sm , C PA-lAC 1- 1-40

H. Ric I1c Ifl 1\befsa , ;\tV
XVIII
Middle Ea r-M asto id, In fection & Labyrint h in e Ossifica ns 1-2- 118
H. Ric Hamsberger, AtD
In fl amm ati on
AO M W ith Coalescent O tomastoidi tis 1-2-44 Inner Ear, Tum ors
Joel D. Swartz, ,\}lJ
In tralab yrinthin e Schwa n noma . -2- 122
AOM W ith Abscess 1-2-48 fl . Ric l l arnsbctger, ,""tD
Barton E Bran stetter IV; ," V
En do lym ph at ic 5ac Tumor . -2- 126
CO M W ith O ssic u lar Erosio ns 1-2-52 H. Ric HlIrII.'i[Jerger, ,",,11)
Joel V . Sm UlZ, .\ IV
CO M With Tym panosclerosls 1-2-56 Inner Ear, Miscell an eou s

I ud D. SWtlftL, .\IV
Coch lear Im p la n t 1-2- 130
Acquired C ho lestea to m a, Pars Flaccida 1-2-60 Barton F. Brans tetter n: ~\lV
Patricia A . lI /idSil1'i, ,\IV
In t ra lab yri n t h in e Hem orrhage 1-2- 134
Acq u ired Ch o lestea to m a, Pa rs Ten sa 1-2-64 fol'! V . Swartr, MI>
Patricia A. JludSil1s, .\ 11 )
Sem ici rcu lar Ca na l Dehiscen ce 1-2- 136
Acq u ired Ch o lestea to ma . Mura l 1-2-68 If. Ric IItlrll.'1!J" Q;a, MI>
l orl D. SU'llf tz, MD
Fenestra l Otosclerosis 1-2- 13H
Cho leste ro l G ra n u lo m a, Middle Ea r 1-2-70 I f. Ric I llIrw/Ja .'\(',; M lJ
Berton r. Hmmtetta I\~ .\ IV
Coc h lea r Otosclerosis 1-2- 142
H . Ric Hli rm/J"r.'o:er, MIJ
Middl e Ear -Mastoid, Tumo rs
Midd le Ea r Ade no ma 1-2-74 Petrous Apex
loci D. Swartz, MLJ
Asy mm et ric Mar ro w, Petro us Apex 1-2- 146
Midd le Ear Schwannorna 1-2-76 I I. Ric I filfllS!J('r.";C:I; MLJ
Joel D. Swartz, ,\IV
Subarcuate Art ery Pscudo lcslo n 1-2- 148
M idd le Ear M eningtom a 1-2-78 H. Ric Hl1f1ls!w ga, MV
Barton F. Bran stetter n: ut» Ch olest eato m a, Petrou s Ape x 1-2-150
Glom us Ty mpanicum Paragangl iom a 1-2-8 2 Patricia ,1. 1 1l 11 (~ il1s, AlI>
H. Ric Haf/1sbcrga , un
Pet ro us Apex Ce p ha locele 1-2- 15 4
Mid d le Ear Rh abd o m yosa rcoma 1-2-86 Tay/or Reichel; ,\ -IV & If. Ric l hunsbereer, MlJ
Patricia A. HlldSill s, .\ IV
Apica l Pet rosit is 1-2- 158
1/. Ric Harnsberger, ~\;/V
Middl e Ear -Masto id, Miscell an eous Tra pped Fluid, Petrous Apex 1-2-162
Post-O per ati ve Cep ha loce le. Mid d le Ea r 1-2-90 II. Ric Hamsberger. :\If)
H. Ric Harnsbetger, ,\If)
Ch o leste ro l Gra n u lo ma, Petr ou s Apex 1-2-166
Ossicular Prosthesis 1-2-92 II. Ric lIamsbcIser, MI)
toe! D. Swartz, ,\IV
ICA Ane u rysm , Pet ro us Apex 1· 2· 170
H. Ric Hurnsbetgrr, MV
Inner Ea r, Congenita l Petr ous Apex Meta stasis 1-2- 174
Labyrin t hi ne Ap lasia 1-2-96 f ad V . Swart z, ,\'l lJ
Patricia A . Hll t/sim , ,\ IV
Coch lea r Aplasia , In n er Ear . -2-98 In trat emporal Faci al N erve, Variants
Patricia A . Hllds i m , ,\ [I>
f acial Nerve Enh an ceme n t , lnt rate m po ral 1-2- J 76
Co m m o n Cavity, In n er Ear 1-2- 100 If. We II1llll ,'i!JeQwr, AJL>
I' litricill A . IIl1dx ius, ,\ IV
Pro la psing Facia l Ne rve , Midd le Ear 1·2· 178
Cyst ic Co ch leovest ib u la r Anoma ly 1-2- 102 f ad D. Swart z, ."-.11 )
Pat ri cia A. H ll ds i m , ,\I V
Se mi cir cu lar Ca n a l Dyspla sia 1-2- 10 4 Intrat emporal Facial N erve, In fection
Patricia A. H li dSill'i, MV
lIeli Palsy 1-2- I!\O
La rge Endo lymp hatic Sac Anoma ly 1-2- 108 fl . Ric Ham .'i1Jerger, Mf)
II. Ril l1cllm/'t.'rSt'r, ,\IJ)
In trat emporal Faci al N erve, Tum ors

Inn er Ear, In fect ion & Inflamm at ion
Facial Nerve Schwa n no m a , T-Bone 1-2· 18 4
Lab yrin thitis . -2- 112 H . Ric Hamsberger, MV
' (1('/ n . Swuru, MI>
Facial Ne rve Hem an giom a, 'l- Bo ne 1-2- 188
Otosyp hi lis 1-2- 116 H. Ric Ham .'11Jer.~er, AJD
l od V . Swur tr , MV
XIX
Perineu ral Parotid Ma lign an cy, 'l -Bo ne 1-2- 192 Di ffu se Skull Base Di sease
H. Ric f/,m lsbt'Qwr, All>
Gia nt Cell Tum or, Sku ll Base 1·3-50
lod K. Cure, MD
T-Bon e Lesion s W ithout Spe cific
Plasmacyt oma, Sku ll Base 1· 3·5 4
Anato m ic Location lot.'! K. Cure:, AID
Tem poral Bone Fractures 1-2- 19 6 Cho nd rosarcom a, Skull Base 1-3-5 8
loel D. Swartz, MV ' 0('/ K. Cure, 1"'0
CSF Leak , T-Ilone 1-2-200 Lange rh an s Ilisto cyt osis, Sku ll Base 1-3 -62
loci D. Swartz, MD Patric ia A. [ [IIt('{i l1s, AIV
Fibrous Dysp lasia, T-Bone 1-2-20 2 Fibrou s Dyspla sia, Skull Base 1-3 -66
Iud D . Swartz, MI) lod K. O m', .'viJ)
Paget Disease, T-Bo nc 1-2-20 6
I/ld I ). Swvru, su:
Osteo petrosis, T-Bon e
lod V . Swurtr, ,\(lJ
1-2-2 10
PART II
Postirrad iated T..Bo ne 1-2-21 2 Orbit, Nose & Sinuses
101'1 D. Swartr, AlD
SECTION 3 SECTION 1
_ _
Skull Base -------.J1 1 - - - Orbit
Introdu cti on and Overview Introdu cti on and Overview
Sku ll Base Ana tomy a nd Imaging Issues 1-3-2 Orbit Anatom y a nd Ima gin g Issues 11- 1-2
AlI/lTf /. .\ IllCdoll d ld . MU CIIU & H. Ric Hams!Ja s c'T, .\tD AI/tin! I . ;\fan IOllllld . MUeh n & I I. Ric Hamst xr gcr, .\IV
Clivu s Co nge nita l

Meningioma, Clivus 1-3-6 Coloboma 11- 1-6
If , Ric lJ amsba s er, MD H . Chrlstiun V lI l'id.\ 0I1, M V
Gian t Pituitary Macroad cn oma, Clivus 1-3 -8 Persistent Hyp erp last ic Primary Vit reous 11 - 1- 10
II. Ric Harmllt.'Ts eT. I\ W H. Christian VlIl'it1.\OII, ."v fl)
Cho rdom a, Clivus 1-3- 10 Dermoid and Epidermoid , O rbit 11 -1- 12

Ak.~ JI"r Gupta, AID & Jl. Ric HllTm bcrxl'f, .\tV II. Ctui.ctian Dnvidsun, t\.'f[)
Neu rofibro ma tos is Typ e I, O rh it 11 - 1- 16
Jugul ar Forame n 1/. Christian Davidson, un
j ug ula r Bu lb Pseudo lesion 1-3- 14 Lym pha ng ioma , O rb it 11 · 1-20
II. Ric Harnsbe rger, .'viI)
II. Christ ian V" I'id.WII, ;\If)
Dch iscent Jugu lar Bulb 1-3- 18 Cave rnous Heman gioma, Orhit 11 41-24
11. Uk Hllrm "t'r,gcr, ,\If)
II. Christian Duvidwn, MV
j ugula r Bulb Divert icul um 1-3-22

II. Ric Iliu m /Jelser, A41J Infecti on & Inflammati on
Glo m us jugular e Paragan gliom a 1-:l -2 6 Id iopat hi c Orbita l Infl a m m atory Disease 11 -1-2 8
H . Ric Hams ber ger, AID H. Christian Davidso n. ," IV
jugu lar Foram en Schwa n noma 1-3-30 Sarco idosis, O rbit 11- 1-32
Tm y M d rlow, AID & 1/. Ric Hart/ s/Ji.'fSf'r, AllJ 11. Cll rh tid /1 t savidwn. MV
Hypog lossa l Sch wa n no ma 1-3-3 4 Optic Neurit is 11 -1-34

II. Ric lJafl/,\bt'rS('f, M D II. Cllrh tilil/ Davidson, :\tD
Jugu lar Fora men Menin gio ma 1-3-3 6 Subpe riostea l Abscess, O rbit 11 - 1-38
H. Ric Hanls!Ja s('r, J'-'ILJ II. Chr istian Davktwn, ,\ I D
Du ral Sinuses Neoplasm, Beni gn Tum ors

Arachno id Gra n ulatio ns, Skull Base 1-3 -40 Ca pillary Hema ngiom a, Orbit 11 -1-4 2
H. Ric l1t1f1ls!Jt'(.l(f'r, uu 11. Christi an LJm,jdwlI, AID
Dural Sin us Thrombosis, Skull Base 1-3-42 Optic Nerve Sh eath Menin giom a 11 - 1-46
Barton E Branstetter 1\: .\II) 11. Ctmstian JJal'i d\otJ, Al l>
Dur al A-V Fistul a, Sku ll Base 1-3 -4 6 Benig n Mixed Tumor, La crimal II - I -SO
11. Ric H amsbcrger, ,\ ,fIJ H. Christian Dnvidsan, ,\11 )
xx
Ne oplasm, Malign an t Tum o rs Muco cele, Sinon asal 11 -2-56
Michdh' A. Mid/d , .\-IV
Retin oblastoma 11- 1-52
If . Christian Davids on, MV Wege ner Gra n u lo ma to sis, Sino nasal 11-2-60
M ie/ldle A. M ichel, MV
Oc u lar Melan oma 11- 1-56
I I. Christian Davidson, MD
Ne oplasm, Ben ign Tum ors
Optic Pathway Gliom a 11- 1-60
H. Christian Davidson, AJD Juven ile Ang iofib roma 11-2-64
M icltelf e A. Mi chel, All)
Ade noi d Cystic Ca rcinoma , Lacri mal 11 - 1-64
H. Christian Davidson, A-Ill In ve rted Papill om a, Sinon asa l 11 -2-68
.\-licltdle A. A.fielll'I, U D
Lymphoprolife rativ e Lesions, Orbit 11- 1-66
1/. Cttrlstian Davidson, MLJ Heman giom a, Sino nasa l 11 -2- 72
M k lt('/Ic A. Mk/ld, MD
Miscell an e ous Osteoma, Sin us 11-2-74

,1vl ichell t' A..\ -fic/lel, MV
Th yroi d Associated Orb ito pat hy 11 - 1-70
I-l. Christlan Davidson, MD Oss ifying Fibrom a, Sin us 11-2-78
M id/elle A. M ichel, MO
Ve no us Va rix, Orb it 11 -1-74
fl. Christian Davidson, MI )
Ne oplasm, Ma ligna nt Tum ors
Sq ua mo us Cell Ca rcino ma, Sino nas al 11 ·2·82
SECTION 2 ,\ fi cltd fe 1\. Michel, MI )
Esthcs io ne u roblasto ma 11 -2-86
Nose and Sinus M ic/lelle A. M iehd , .\ IV
Adenocarcino ma , Sinonasal 11 -2-90
Int rodu ction and O verview Michcitc A. Mi chel, MD
Nose and Sinu s Anat omy a n d Imaging Issues 11 -2-2 Melano ma , Sino n asal 11 -2-92
A ndre f. Ma cdonukt, MHClII l & H. Ric Harnsberger, M O
Michell e A. ,'-liellel, A·IV
No n- Hodg kin Lym ph o ma, Sino nas al 11 -2-9 4
Co nge nita l M ichd le A . M iel/d , MD
Naso lacrima l Duct Dac rocyst ocele 11-2-6
M ichd h' A . M ichel, AID
Miscell an e ous
Choa n al Atresia, Nasa l 11 -2- 10 Fib rou s Dysplasia, Sinonasa l 11 -2-98
,\ ti cJld h' A . Michel , MlJ
M ichell e A. Mi cltd , MLJ
Nasa l Glio ma 11-2-14
,\fic/wlle A . Michl'l, .\ID
Fron toethmoida l Cep h aloce le 11-2-18
Mid/t·lI" A. M icll d , I\-t f)
Nasal Derma l Sin us 11-2-22
PART III
If. Ric Hilrw;/)erxa. MI) Suprahyoid & Infrahyoid Neck
Infe cti on & Inflammat ion Introducti on a nd Overview
Acu te Rh ino sinusit is 11 -2-26 Global Im agin g Ana to my of th e Neck 111-1-2
M ielldh' A. AJicl,d , A,fD H . Ric 11amsb erger, /" 'V
Ch ron ic Rhi nosin usitls 11 -2-30
Michel/l' A . M ichel, ut:
Rhi n osin usitis, Co m plica tio ns 11 -2-34 SECTION 1
M ichd lt' A . M icJld, MLJ
Pharyngeal Mucosal Space (PMS)
Fu nga l Sin usitis, Myceto ma 11 -2-38
,\ fi rhd lt' A . M icltel, M lJ
Introdu ction and Overview
Invasive Fun gal Sin usitis 11-2-40
M ichelle A. M ichel, M i)
Mucosal Space Ana to my and Im agin g Issues 111-1 -2
H. Ric Hamsherger. MI)
Fu ngal Sinusiti s, Allergic 11-2-44
M ichelle A. M ichel, MD
Conge nita l
Sinona sal Pol yposis 11-2-46
Mi d tclte A. M ichel, M l) Torn wald t Cyst 111- 1-4
Patricia A. ! l lldXi llS, MD
Sino nasa l Solita ry Po lyps 11-2-50
Miel lelle' A. Midld , MD
Silent Sin us Syn d rom e 11-2-54
.\ fi clld le A . ,\ Iiehd , M LJ
XXI
In fection & Inflammation Degen erati ve, Ac q ui red
To nsi llar Abscess 111- 1· 6 Laryn go cele 111 -3-6
Pat ricia , i. 1I1I1I:-:;IIS, All) Joel Cure, J\ l D
Reten tio n Cy st (l'!\IS) 111- 1- 10
Pattid a A. 1I111{1;;IIS, AIL> Traum a
La ryn x Trau ma 1IJ-:l -lO
Neopl asm Iwl O m" AlV
Beni~ n Mixed Tu mor (l':vIS) 111 - 1- 12 Trachea l Stenosis, Acq uired 111-3 -14
I'l1tridll ~ \. IIl1 dSill~ , un 101'1 Cll rI', sn»
Squa mo us Cell Carci no ma, Naso pha rynx 111 - 1- 16
Patr icia A . HliliSilh, tvtLJ Neo p lasm, Malignant Tum ors
Lin gual Tonsi l SCCa 111 - 1-20 SCCa, Larynx , Su praglott ic 111 -3- IB
Pntricia iL 1I11l(" ilJ.\, ,\ lLJ Richard H. Wi gS;"s III, ui:
fa uc ia l To nsil SCCa 111 -1-2 4 SCCa, Lary nx, G lottic 111 -3-22
Patricia A. HI/II"ill\, All} Uidlitrd H. U' iSSill s W , u t:
Minor Salivary Gland Malig nancy (PMS) 1II- 1-2B se ca, Larynx, Su bglott ic 111-3-24
Patricia A. Hudxim , Ml> Richard 11. l Vi,l;.l;im Ill, MV
No n-Ho dgkin Lym pho ma (l'MS) 111-1-30 Cho nd ro sarco ma , I.aryn x 1II-:l -2B
I'tl lridd ,L lIlIdSi lu, Mf) joel Om;, .\IIJ
SCCa , Hyp op ha ryn geal 111-3-32
jocl Clln>, Ml)
SECTION 2
Lymph Node Diseases Treat m ent-Related Lesion s
Radiated La ryn x 1II-:l -:l 6
Int ro ducti on and O vervi ew Illd Om>, AllJ
Lym ph Node Anatom y and Imaging Issues 111-2-2 Vocal Cor d Para lysi s 111-3 -40
I J. Ric I larl1.\ l)t'Q; e/~ ,\II> j ad Cure', AIL>
In fecti on & In fl amm ati on

Reactive l.yrn ph Nod es 111- 2-4
SECTION 4
Ch ris t ine ,\ 1. C !m toll /)lIry. MB HS Oral Cavity
Sup pu rat ive Lymp h No d es 111-2-B
Christine ,\ I. (j/ll-\lcllll"" y , MBRS Int rodu ctio n and Overvi ew
Cas tlem a n Disease 111-2 -12 O ral Cavity Ana to my and Imaging Issues 1I1 --!-2
Chri sti ne M . ( ;rll .\ to" IJll 1)~ .\ IRRS 1\ l1dn; t. Ma cdol1ald, ~tH ChU N JJ. Uic JJtlm \I,,'r.~('f, ,\If)
Kim ura Disease 111-2 -16

Christine M . G /d .\ to l/ l' /I r) ~ ,\fHUS Co nge nita l
Lingu al Thyroid III A -6
Neopl asm Ridlilrd H. U'igg im Ill, M D
No n-I lodg kin Lymp homa No des 111 -2-20 Accessor y Salivary Tissue (SMS) II H -B
Patricia I\. 11I11(I;ill.\, ,\:tV Riehl",1 II. U'i,I;.\'im IJI, .\ I V
Hod gkin Lym ph o ma Nodes 111-2-2 4 Dermoid and Epider moid, Oral Cavity 111-4- 10
Chr istine lvl. (J 1"~ I (lIIhllr)', ,\IUHS Uid ll1rtl li. U 'i,I;.~illS Ill, wn
Sq uamous Cell Carci no ma Nodes 111 -2-2B Lymp han gioma. O ral Cavity 111-4- 14
Christin e ,\ 1. Gtastonbury; MIWS Rid/llnill . U' i,<.:.\,il1S W , MV
Syste m ic Metastases, Neck Nodes 111 -2-32
Patricia -l . HIII(~i/ls, ,\If) In fecti on & Inflammati on
Abscess, Ora l Cavity 111-4- 16
l<id/ llrd 11. U'igs im 111, MV
SECTION 3 Sialocc le, Or al Cavity 1IJ-4-20
Hypopharynx, Larynx & Cervical Richa rd II . \\'(l;.l;im Ill, ,\ If)
Trachea Sub ma nd ib ular Sialad end iti s 111-4-22
Richurd tt. \\ 'iggim 111, ,\ If)
Introdu cti on and Overview Ra n ula 111-4-26

Rictnmt t l . \\'iggi m 111, ,\ 11 )
Il ypo p haryn x-Laryn x An ato my- Imag lng Issues 111 ·3· 2
Kt/r('11 L. Satannn, 1\4V &: fI. Ric Ilt11"1/\ he/xcr, ,\ tV
XXII
Neoplasm, Benign Tum ors No rma l Vari ants & Pseudolesion s
BMT, Su b ma n d ib ula r G la nd 111 -4-30 Pterygo id Venous Plex us Asymmetry 111-6 -4
Uid wfl/ II. Wt\:S'in'i W, AID Christine AJ. Glustnnbury, .\Il mS
lIen ign Masticator Mu scle Hypert ro phy 111 -6 -6
N eoplasm, M ali gn ant Tum ors Jod Cure, ,\I V
SCCa, O ra l Tongu e 111 -4-3 4 Mo to r Dener vati on eNS 111-6 -8

Ridumt H. W(~i m 1J1, Mf) Chri sti ne .\ 1. GIII_
'itol1bf/ry, M /JHS
SCC a, Mand ibul ar Alveo la r Rid ge 111 -4-38

Richard Jl. W iss im I II , AID In fecti on
Min or Saliva ry Gla nd Ma lig na ncy, Ora l 111-4-40 Mast icato r Spa ce Absce ss 111-6 - 12
Richard II. l\'i,~~il1S Ill , MV II. Ric IItlrm /){'rs",~ M D
Su blingu la r Gla nd Ca rcin o ma 111-4-42

Richard II. i Viss im 1/1, ,\If) N eoplasm, Benign Tum ors
Su b ma nd ib ula r G land Ca rci no m a 111-4-44 Sch wa n noma , V3 (MS) 111-6 -16
Richard If . l\'iss im III. MLJ /01.'1 O m', ,\I I )
No da l Me ta sta ses, SCCa (SMS) 111 -4-46
Richard H. U' ;:'is im l ll, Mi> N eoplasm, M ali gn ant Tumors
No da l Nun -I lod gki n Ly mp ho m a (SMS) 111-4-48 Cho nd rosarco ma, Masticator Space 111 -6-20
Richunt H. Wi.~~ i"s Ill, ,\ 11 ) Bur ton F. Branstetter I\~ ,\ IV
Sarco ma , O ther, Ma stica to r Spa ce 111-6-24

Richdni ll. H'i,l:sim' IJI, .\ -IV
SECTION 5 Pe rin eu ra l Mali g nan cy, V3 (MS) 111-6-28
Mandible/Maxilla Barton F. Branst etter IV, AI/)
Introdu cti on and Ove rview

Ma nd ibl e, Maxi lla Anato m y-Im agin g Issues 111· 5 -2 SECTION 7
II. Ric Harnsbetger, u n Parotid Space (PS)
Tra u ma Introdu cti on and Overview
Man dibu lar Fract u re 111-5 -6 Pa rotid Space Ana to m y-Ima g ing Issues 111 -7-2
Ric/w nl fl . Wi:.:.~ im 1/1, ,\ IV II. Ric lIarm /Jt',:,; ('r, AI D
Neop lasm Infection & In fl ammatio n

Ameloblasto ma , Ma nd ib le-M axilla 111-5- 10 Pa rot iti s, Acut e 111 -7-4
Richard I/. W;:~s;m Ill, M LJ Ch ri!ltill(, M. Glll stoll b"ry, .\ IIUlS
O steos a rco m a, Man d ible-Ma xilla 111-5- 14 Ben ig n Ly mp h oepithcl la l Lesinns- HIV 111-7-8
Rich ard 11 . Wi.'{~ ills 111, u t: V ",'M Litrvu, M V & II. Ric 1I11111.\1'''Q:a , "' LJ
Bur kitt Lymph oma, Ma nd ible-Max illa 111-5- 16 Sjogren Synd rome, Parotid 111 -7- 12
Ricll ar d H . Wi.~.\ i 1lS 11/, ,\ 11 ) 11. Uk Jlarll\!JI.'I:ga , MV
Miscellaneou s Neoplasm, Benign Tum ors

Basal Cell Nev us Synd ro m e 111-5-20 Ben ign Mixed Tu mor , Parotid III · 7-16
Richard 1/. W i,I:s i m 1II, .\IV II. Ric IIll nlsba s a , ;\f{)
Den tigerous Cyst, Man d ibl e-Maxill a 111-5-2 2 Wart hi n Tu mor 111-7-20
Rktu mt II. Wig,gim 111, M D II. Ric lIil m .\ !JeT.'.:('T, .\IV
Odo nt og eni c Ke rato cyst, Man d- Maxi lla 111-5 -24
~\li.l;,li i /1.\
Richa rd II. W , MlJ N eoplasm, M ali gnant Tum ors
Mucoep idermo id Carcinoma, Parotid 111 · 7-24
Barton F. Urml.\ tctt a I\~ MV
SECTION 6 Adenoi d Cystic Carcino ma, Parotid 111 -7-28
Masticator Space (MS) Chr ist ine At ( i la stol1lmry, AW US
Metastatic Disease, Noda l, Pa rot id 111-7-30

In trodu cti on and Overvi ew Barton F. Branstetter I\ ~ ,\I L>
Masticat o r Space Ana to my and Imaging Issues 111-6-2 No n -Ho dg kin Ly mp homa , Parotid 111 -7-3 2
1/. Ric Hamsixrger, ,\ 11 ) II. Ric HdTm lJl.'Ts a , ,\II)
Skin Mali g nan cy In Paro tid Nodes 111 -7-3 6

H. Ric H ll m s" erscr, :\1V
XX II I
SECTION 8 SECTION 10
Carotid Space (CS) Perivertebral Space (PVS)
Introdu cti on and Overview Introdu ction and Overview
Ca rot id Space Anat o m y-Imag in g Issues III- H-2 Periver teb ral Space Anat omy-Imaging Issues 111 -10 -2
11. lac /I"I"/a /J",:gcr, ,\IV fl . Ric lIarmh 'rgel; ,\[V
Normal Variants & Pseudolesion s Normal Variants & Pseudolesions

Ecta tic Ca rot id Artery, Nec k 111-8 -4 Levat or Scap u lae Muscle Hypertroph y 111- 10-4
H. Ric 11",.l/\b('(.'\('(, "I V A ll tl n' l , :H acdoll ald, MRCIIH
Vascular Infection & Inflammation

Jugu lar Vei n Thrombosis, Neck III -H-6 Perivert ebral Space In fect ion 111 -10 -6
51£'/,'11111 Thiede, Al l) & H. Ric i larnsbergcr, J\ 1[) Audn' [ . M aedot l"ld, MIlC/,n
Carotid Arte ry Pseudoan eu rysm . Neck III -H- IO Lon gu s Co lli Ten do n itis 111- 10 -10
H. Ric f ldrusbt't;-':t" , MLJ A ndr e f. Man /ol/altl , ,\ W ChH
Ca rotid Arte ry Dissect ion, Nec k III -H- t 2

11. Ric Hamsberger, M V Vascular
Verteb ral Artery Dissectio n , Neck 111 - 10- 12
Neoplasm, Benign Tumors A I/tire f. MtJedOllll JcI, ,\ I/U":hIJ
Glom us Vagale Paraganglio ma 111·8- 16

If. Ric lI"msl'C' ,x(" , MV Neoplasm
Ca rotid Bod y Pa ragan glioma 111 -8-20 Brachi a l Plexu s Sch wan n o rna, PVS 111 - 10- 14
II . Ric H, ,,, ,~berst'r• .\1 LJ AI/dre f. Ma cdUlwld, ,\1RCIIU
Schwa n n om a, Caro tid Space III -H-2 4 Ch o rdom a, PVS 111- 10 - 16
H. Ric Har usbe rgcr, M J> Andre I . Ma cdollald, MUCI1U
Neu rofibro ma, Ca rotid Space 111-8-2 H Verte bral Body Metastasis, I'VS III - I O- I H
II. Ric Hamsbetgct, ,\ID Andre]. Macdol/ ald, MBChU
Meningio ma, Ca rotid Space 111-8 -3 2
fl. Ric Hamsberger, M lJ
SECTION 11
Visceral Space (VS)
SECTION 9
Retropharyngeal Space (RPS) Introdu ction and Overview
Visce ral Space Ana to m y-Imag ing Issues 111· 11· 2
Introduction and Overview H . We H tl msIJ£'rs('f, .'00
Retropha ryngeal Ana to my-Ima gi ng Issues 111 -9-2
JI. IHe H,mlsbrrsa , M /) Inflam mation
Hashimoto Thyroid itis 111- 11-4
Normal Variants & Pseudolesions Christine M. G/"s toI/ IJllr)~ M llBS
NOll-abscess fluid , RI'S 111-9 -4
Christine M . GJd5 to"JJUr;~ ;\/URS Toxic-Metabolic, Acquired
Mult inod olar Go iter 111 -11 · 8
Infection & Inflammation Amid f. Mucdonatd, A1HC/IH
Su p p u ra tive Adenopa thy, RPS 111-9-8
Chris t ine M . Glvs tonbury, lvfHBS Degenerative, Acquired
Ret ro p h a ryn gea l Space Abscess 111 ·9 -10 Zen ker Diverticu lu m 111- 1 1- 12
Cll r;'\l h lt' .\ 1. GI" .Hu lllm r}~ :\ fRRS Christine A1. G/a.\to//bllry, M UllS
Reactive Ad eno pa t hy, RI'S 111 -9 - t 4
Christ ine M. Gtustont ntry, ,"vIlJUS
Neoplasm, Ben ign Tumors
Thyro id Aden oma 111- 1 1- 16
N eoplasm, Metastatic Tumors Christine M . Gtastonbury, M I WS
Noda l Metasta ses, sees, Rf'S 111-9 - 16 Pa rath yroid Ade no ma, Viscera l Space 111 -1 1-20
A ndr t' , . .\fll cdmldld, MHC1IU I\ I/d rc' I . M ucdanatd, M BCIIU
Nod al No n-Hodgkin Lym ph o m a, IU'S 111 -9 - 18
Andre f. ,\ l a( £Iul1l1ld, MRO IH
XX IV
Neoplasm , Malign ant Tum ors Th yroglossa l Duct Cyst IV-I -22
Patricia A. lIudgills, MLJ
Differ en t iated Thy ro id Carci no m a 111 -11 -24
Andre I. A4acdol/tlld, A-w e li R Th ym ic Cyst IV- I-26
Patricia A . Hudgins, ,\ 1f)
Med ullary Th yro id Carcinom a 111-1 1· 28
Ctnistlnc ,\4. Glastonbury, MR ES Lym p ha n gioma IV-I -:lO
Patricia A . t iudgi ns, MD
Ana plast ic Thyroi d Ca rcin o m a 111-11-32
A ndre } . Milcc/O/wld, :\ mChH Ven o us Vascular Malfo rm ation IV- I-34
Joel Cure, MlJ
Cervica l Esoph ageal Carcinom a 11I-11-36
Christine M. Glast onbury, MBBS Neu ro fibro ma tosis Typ e 1 IV- l -:18
Alldn' I, ,\:fan/of/old , iv1BChB
Thyroid Non -Hodgkin Lym ph o m a 11I -11- 40
AI/dn' / . Mucdonatd, M ll CIIH
Traum a
Miscell an eous Fib ro mat os is Co lli IV-I -42
Joel Cure, MlJ
Parat hy roi d Cyst , Visceral Spa ce 11I- 11-44
Cnnstine ."-'1. Glastonbury, M EBS
Ne oplasm, Benign Tum ors
In fan ti le Hem angio m a IV- I-46
SECTION 12 Jod CII" ', A1L>
Posterior Cervical Space (PCS) Ne oplasm, Maligant Tum ors

Introduction and Overview Rhabdom yosa rco ma IV- I-SO
t'atricia iL H uds ins, ,\ -(lJ
Post erior Ce rvica l Anato my- Imagin g Issues 11I - 12-2
H. Ric Harl/s}JersC'r, ,""I /J
SECTION 2
Neo plas m, Benign Tum ors
Schw an no m a, Post erior Cervical Space 111- 12-4
Other Trans-spatial Lesions
Christine M . Gtastontnsry, MJWS
Aggressive Fib ro ma tosis IV-2-2
Neoplasm , Metastati c Tumors H . Ric H amsbere cr, M V
SCCa , Spina l Accessory Node 11I -12-8 Lipoma IV-2-6

,l l/ dn' [ , M acdol/ald, M HCIIH
Andre t. Man/ol/ ald, AIHC/I B
11I -12- 10 Liposarco m a IV-2- 10

N III" Spin a l Accesso ry Node
All/Ire I . Macdonald, ,\ !HClllJ
AI/dn' t. Ma cdoJ/ald, MHCJiH
PART IV
Pediatric & Trans-spatial Lesions
SECTION 1
Pediatric Lesions
Introdu ction and Overview
Co n gen it al Lesions of t h e Neck IV- I·2
Bern adette Koch, MlJ & H. Ric llar m berst'f, ,\IV
Congenital
l st Bran ch ia l Cleft Cyst IV-I -6
Patricia 1\. Hlldgills, J\ JO
2nd Branch ia l Cleft Cyst IV- l -IO
Patricia 1\. Hudgins, MV
:lrd Bra nchial Cleft Cyst IV- I-14
Joel Om" M V
-lt h Bran chial Ano ma ly IV- I-18
[ oct Om', M lJ
xxv
ABBREVIATIONS
\1 i \ c{'ll an l'OUS ,\ h b re \' i.t t iOIl\ PMS: Pharyngeal mu cosa l "pa n ,'
Ca: Ca rci nom a PPS: Parapha ryn gea l SI>ilH'
CS F: Ce re bros p ina l flu id 1'5: Parotid 'ip,](:e
Fat-sa t : Fat sa tu ra tion (\ IRI RPS: Ret ro ph aryn geal "pan '
Rile : Red bl ood ce ll I'V5 : Periver teb ral span .'
Sa: Sar co ma SZr-. IS: Supre zygo m a tl c mastica tor span '
W BC: White b lood ce ll VS: Viscera l "pace
XIU : Radiation th era py Oral Ca vilr (0<:)
Ccrebe lto p o n tl nv 'In g le (e llA) and In t er n a l audito ry r O ~ I : Floor o f m outh
ca nal (IA(;) ()~ IS: O ral m uco sal 'ipaCl'
Al e A: An teri o r inferior cerebel lar ;,U l eTY ROT: Root lit to ng lil'
C II I.: Co nd uct ive hea rin g lo ss SMG : Su b ma n di bul a r g la nd
F : Facia! nerve leN 7) 51$ : SUblin gua l SPilC('
NF2: Ne u rofi brom utosls type 2 :-l ~ (5 : Suhmandlbular vpace
PIC\: t'o stcrto r In ferio r cerebell ar arter y Vl's..c ls : Ar teries No Vei ns
SN I I L: Sensorlncurul h eari n g lo \''i CCI\: Co m m o n ca rotid a rte ry
Vest lbuloc oc hl ea r ner ve (eNS) ECA: Exn-ru al (..u o li ll a rte ry
Tem p o ra l bon e le A: ln tcm al ca ro t ld a rterv
CI II.: Con d uc tive hea rin g loss Ij V: In tern al jugu lar w i n '
E..\ C: Exte rna l au d itory conn! ),\ CA : Ant erior In tcrt or cerebellar artery
L'iC: Latera l semici rcu la r ca n al PICA: Po ste rio r lnf c n o r ce re bell ar arw ry
P/\ : l'etrou s apex C ra n ta l Nerves
sec: Semici rcul a r ca n al e N I : O lfac to ry nerve
S ~ lI l. : Sen so rin eu ral he aring Ims CN2: Opt lc n er ve
T\ (: Ty m pa nic memb rane e N]: Oculo m ot or n erve
'r-bo nc: Tem pora l bon e C N4: Trochlear n er ve
Skull n a sc C1'\5: Trtgcmtnat n crve
C1'\: C ra n ia l n e rve C1'\\' I: O p h t h al m ic branch . C ~ 5
(; WS: Grea te r win g of sph e no id C=,/ V2: ~Iil x i ll a r y branch. e NS
jF : jugu la r fora m en CNV3: Mandibular bran ch , Cl'\5
IOF: In ferior orbi tal a ssure CN 6: Abd uce ns n e rve
l WS: lesser \..'in K of sp he noid C1'\7: f aci al nerve
S8: Sku ll base C ~ R : Ves t lb ul ocochl ear n e rve
SOF: Su per ior o rbi tal fissu re C ~9 : Glossop h aryngea l ne rve
O rbit C:"l IO: Vagus n erve
O:"l': Optic n erve ( C~2) C r-\l l : Spin al ac..·c e ~ so l)· ner ve
' Fl: x cor c ttbrcmarc sts type 1 C1'\12: Hypoglossal n er ve
1'\o..e « Sim as Tumo rs, Ge nera l
:" I.D: Nasolacrimal duct ACCa: Aden o id cysuc ca rcl uo ma
O MU: Os tlomea tal unit Area: Anaplavn c thyr oid carci n o m a
ppr : Pterygo pala ti n e fossa B ~ I T: Ben lgu m ixed tu mor
Glo ha l Hea d N Nec k D'FCa: Dlffcrcn t tat ed th yroid ca rcinom a
li N K: Head and ned E1A"iT: En do lym phatic ...ac tu m or
JH ~ : In fra hyo id n eck E1'\ U: Esthcsto nc u robtastoma
SH1'\: Su p ra hyoid n ed Gj l' : G lo m us jug ula re paraga ugl loma
DI.-DC F: Deep la yer, deep ce rvic a l fascia GT Il: Glomu... tympa nicum pa rnga ngflom a
Ml · DC F: Mid d le la ye r, d eep ce rvica l fascia G VP: G lo m us va gal e parag an gli o m a
SI.-DC F: Superfici al layer, deep ce rvica l ~!EC a : Mucocpidcnn oid ca rci no m a
fascia M~G : Multin od u la r go i ter
SC ~ I : Ste rnocle idom as to id m uscle :--J tl l.: No n- Ho dgk i n ly m ph o ma
TM: Trapezius m uscl e =,/ PCa : Nasopha ryngea l carcinom a
Su p ra hyo id N In fr a h yui d Nec k Spa ces I'NT: Perin eural tum or
ACS: An terio r ce rvica l space Se Ca : Sq uamous (l' 1I ca rci noma
CS: Caroti d space
DS: Danger spac..'e
MS: Masti ca tor space
rcs: Po s terlor ce rvica l space
XXV II
DIAGNOSTICIMAGING
HEAD AND EeK
XXIX
PART I 3
Temporal Bone and Skull Base
CPA-lAC [I]
Temporal Bone rn
Skull Base rn
SECTION 1: CPA-lAC
Introdu ction and O verview

CPA-lAC Anatom y and Imaging Issues 1-1-2
Co ngenita l
Epide rmo id Cyst, CPA-lAC 1-1-6
Arachnoid Cyst, CPA-lAC 1-1-10
Lipom a, CPA-lAC 1-1-14
NF2, CPA-lAC 1-1-18
Infection & Inflammation

Ram say Hun t Syndro me 1-1-20
Sarco idos is, CPA-lAC 1-1-24
Benign Tum or s
Acou stic Schwa nnoma 1-1-26
Meningioma, CPA-lAC 1-1-30
Facial Nerve Schwa nno ma, CPA-lAC 1-1-34
M alignant Tum ors

Met asta ses, CPA-lAC 1-1-36
Vascular
Aneur ysm, CPA-lAC 1-1-40
Supe rficia l Sidero sis, CPA-lAC 1-1-42
Vascular Loop Com pressio n, CPA-lAC 1-1-46
CPA-lAC ANATOMY AND IMAGING ISSUES
1
2
Axial graphic shows normal dorsal (arrow) & ventral Axial T2Wf M R through inferio{ lAC shows normal
(open arrO\\') cochlear nuclei in laceral inferior inferior L'erebeJlar peduncle-cochlear nuck·j (arrovv),
cert'beflilf /x.'<1und e margin. No ll' cocbtcer nerve coc hlear ncvve (opon arrow) & int'i:',ior vestibular nerve
(curved arrow) in anterior CPA cistern. (curved arrow).
• Leaves spiral ga nglio n o f coc h lea as aud itory

ITERM INO l O GY axo ns
Abbreviati ons a nd Syno nyms • Trav els as coc h lea r nerve in antenor -lnfer ior
• Cerebe llo po ntine a ngle (CPA) qu adran t of lAC
• Int ern al audi tor y canal (lAC) • Joi ns SVN & IVN at porus acusticus (o pen ing to
• Cran ia l nerve ? & 8 (CN? & CN8) lAC) to become vestib ulocochl ear nerve bu nd le in
• Sup erior vest ibu lar nerve (SVN) CPA cistern
• Inferio r vesti bular nerve (lVN) • Crosses CPA ciste rn as post eri o r nerve bu ndle
• Ant er ior in ferior cerebella r artery (AICA) (facial n erve is an terior) to ente r bral nstem at
• eN8 = vestibu loco chlea r nerve, aco ustic ner ve junctio n of med ulla & pons
• En te ring nerve fibers pierce b rain stem, bifurcat e,
Definiti ons maki ng syna pses with both do rsal & ven tral
• CPA-lAC cistern : CSF flu id space in cerebe llo po n tine coc h lear nu clei
angle & internal aud itory canal co n taining CN? & • Nerves o rien ta ti on in lAC ciste rn
CN8 a nd AICA loop o "Seven-u p, co ke down " descri bes situ ation best
• lAC fund us: Lateral cap of lAC ciste rn filled with dista l o CN? : Ant erosup erior in lAC
cran ial nerves & CSF o Coch lea r n erve: Ant eroinferior in lAC
• Coch lea r aperture: Bony o peni ng bel ween lAC fundus a Superi or vestib ular nerve: Poste ros u pe rio r in lAC
& coc h lea o In ferio r vest ibu lar n erve: Postero in ferio r in lAC
• Modi olus: Hub of of coc h lea mad e up of spo ngy bo ne, • Arteries: CPA-lAC cistern
spiral ganglia a nd proximal nerve fibers of coc h lea r o Ante rior in ferio r cerebellar art er y (AICA loop)
nerve • Arises from basilar artery, rises su pe ro late rally into
lAC
• Co n tinues in lAC as in ter nal auditory ar ter y
IIMAGING ANATOMY • May mi m ic cran ial nerve o n hi gh-reso lut io n 1'2
• Supp lies inn er ear incl ud ing co ch lea, flocculu s of
Internal Struc tures -C ritica l Co nte nts cerebellum, an terolate ral po ns in area of CN
• Vestib ulococh lear n erve (CN8): CPA-lAC cistern n uclei for CNs 5, ? & 8
o Compo nents • Oth er struc tures in CPA cistern
• Vestibula r portion (balan ce) o Flocculus: Lobule of ce rebe llum that projects int o
• Coc h lea r portio n (hea ring) posterolat eral CPA
o Coc h lear n uclei o Choroid plexu s: May no rm ally pass from 4th
• Dorsal & ven tral cochlear nuclei found o n lateral vent ricle thoug h fora me n of Lusch ka into CPA
surf ace of in ferio r cerebellar ped uncle (restifo rm cistern
bod y) • Other struc t ures in lAC cistern
• The ir locatio n can be accur ately determ ined by o Crista falciform is: Ho rizon tal bo n y projecti o n from
look ing at hi gh-r esoluti o n T2 axial im ages & lAC fun dus
iden tifying inferior cerebe llar ped uncle co nto ur • Separates CN?-S VN above fro m CNfl-IVN below
• Th ese 2 n uclei receive axo ns from neu ro ns wit h o Bill's bar: Vert ical bo ny ridge in superior por tio n lAC
cell bodi es in spiral gan glion in cochlear mod iol us fundus
o Coch lear nerve portion, CN8 co urse • Separates CN? from SVN
_a !W'J
DIFFERENTIAL DIAGNOSIS
Pseudolesio ns • Intracranial pseudotumor 3
• Asymmetric cerebe lla r flocculus Vascular
• Asymmetric choro id plexus • Aneurysm (verte b robas ilar, PICA, AICA)
• Marrow foci aro u n d lAC • Arteri ovenous malforma tion
Congen ital Benign tumor
• Epidermoid cyst • Acoustic sch wan n o m a
• Arach noid cys t • Me n ing io ma
• Lipo ma • Facia l nerve sch wa n n oma
• Neurofibromatosis type 2 • C horoid p lexu s papilloma
Infectiou s M alignant tumor
• Meningitis • Metasta sis, syst emic o r suba rach n oid sp read
• Cys ticerco sis • Brainstem glioma, pe dunculated
Inflamm atory • Ependym oma
• Sarco idosis • Melanotic sch wa n n oma
• lo t see n n o rm all y o n cr o r MR o f th is a rea o Membra n ous labyrin th area o f inner ea r: Look for
o Coch lear ape rtu re: SmaIl lAC o u tle t of CS F at base o f in tralabyr inthine schwannoma &. coch lear
coch lea otosclerosis
o Meatal foramen: O pen ing from fu n dus for CN ?;
lead s to lab yrinthin e seg me n t CN? Imaging Pitfalls
o Macul a cr ibrosa: Per forat ed bone between lAC &. • No rma l va rian ts in CPA-lAC
vestibu le of inner ea r o No rm al struc tures, wh en unusu ally p romin ent,
tro ub le rad io logist evaluating C PA· lAC
o AICA loo p flo w vo id o n hi g h-r eso lution T2 MR
IANATOMY-BAS ED IMAGING ISSUES I • Will n ot p romin ently en h an ce o n Tl C+ MR
• Sub tle en h ancem en t in lAC o n TI C+ MR may be
Imaging Approaches m ista ken for sm a ll acoustic sch wan n oma
• Vestibu lar portion, CN S o Ch o ro id plex us p rot rud ing t hrough latera l recess of
o Seld om provides impetus for imaging CN 8 4th ve n tricle
o \Vh en vertigo , di zziness , or im balance im ag ed , MR • T1 C+ MR sh o ws en h ancing bilateral tear-sha ped
usually n ormal masses o f CPA cist ern
• Coch lea r portion, CN S • Sym metry &. ch a rac te rist ic appea rance make
o Prindpal im petus for imagi ng CN8 diagno sis
o Globa l choice o f imagin g tool in h earin g lo ss (C f vs o Ce rebe llar floccu lus is a lobul e o f cerebe llu m
MR) projecti ng in to posterolatera l aspect of CPA cistern
• Bone c r used in t rauma, o toscleros is & Paget • Signa l foll ows in tensit y of ce rebe llu m o n all MR
disease seq uen ces
• MR used fo r all o th er indi cati ons o Mar ro w spa ce foc i in wall s o f lAC ca n mimic lAC
• MR im aging ap proach to UNCO MPLICATED unilateral tumor o n T l C+ MR im ages
sens o rineu ral h earin g lo ss (SNHL) • Co rrelate locatio n of foci wit h lAC cis te rn
o Scree n ing MR in vo lves h lgh -re so lutt o n th in -sect io n • Bone c r of T-bone m ay be n ecessary to identify
T2 MR im agin g th rough C PA-lAC thi s norm al va rian t
• MR im aging ap proac h to COMPLEX SNHL (u n ila tera l
SNI IL + o ther sym p to ms )
o wh ole b rain &. po sterior fo ssa seq ue nces ICLTN ICA L IMPLICATIONS
• Begi n with wh o le b rai n axi al T2 ± FLAIR
seque nces Functi on- Dysfunct ion
• Co n clu de with axial &. co ro n al T l thin-section C+ • C PA-lAC lesions most com mo nl y present wit h SNHL
MR o f po steri or fos sa & C PA-lAC o Un co m plicated unilateral SNHL: Patien t otherwise
• Remember to visually interrogate foll owi ng a reas for h ealth y & pr esents with unila teral SNH L
lesions o Co m plicated SNHL: Patien t h as additional signs &.
o Restiform body of m edulla (area of coc h lea r nuclei): sym p to ms in add itio n to unilateral SNHL
Lo ok for st ro ke, tumor, cave rn o m a &. mu ltiple • Including o th er cranial neu ropathy, long tra ct
scleros is signs &. headache
o CN8 in C PA-lAC cistern : Look fo r acoustic • Coch lea r nerve in jur y
sc h wan n o rna. facial ner ve sch wan n oma, o Hea rin g lo ss &. ti n n itu s p rimary sym ptom s
m eningioma, epiderm o id &. a neu rysm
..
Axial graphic show'S fundJl cocbiesr nerve (arro, v ) is AVoiJl 11WI MR sha.vs cochk'ar Ot'f\'(' in lAC heading to
made up of spird! ganglion axon.~ (open dlTows ) in the iundU5 (arrCAv) w here it goes through cochlear
modiolus of cochlea.Spiralganglion a/so5ffids exons Co aperture on way to modiolus . Os'iCOUS spiral lamina of
Organ of Corti (w n eel arrow) . the nxhle.l (open iU1(1tV) .
o If unilat eral SNII L present, injury oc cu rred be twee n

coc h lea r m embrano us lab yrinth &. coch lear nuclei o f
IC USTO M DIFFEREN.TIAl DIAGNOSIS I
in ferior cerebe lla r peduncle of brainstem Cys tic CPA lesions
• Facia l nerve in ju ry. C PA-lAC po rtio n • Co ngen it al
o Peripheral facia l neuropathy: In clu d in g lacrim ati on, o Ep ide rmoid cyst
staped ia l reflex, a n te rio r 2/3 tongue taste loss & o Arach no id cyst
co m p lete loss o f mu scles of facia l exp ressio n o n side • In fecti ous
of lesion o Cys t ice rcos is
• CN7 rar ely inj ured by lesion in CPA-lAC • Vascu lar
• If lesion in C PA-lAC a nd CN7 is o ut, conside r o Ane urysm (vertcbro bas rla r, PICA, AICA)
n on -aco ust ic schwa n nom a ca uses suc h as facia l o Ven ous varix with d ura l AVF
n erve sch wa n no ma o r m et astat ic d isease • Ben ign tumor
o Ifem iracial spas m resu lts fro m vascu lar loop o Cystic aco usti c sch wa n norna
co m p ress ion o f root exit zo ne o f eN? o Acoustic sch wa n n om a + a rach n o id cys t
• Rarely C PA mass ca n ca use this sympto m o Cystic m en ingio m a (ra re)
• AICA th rom bosis • Malign ant tumor
o Un ilateral SNHL, vestibular dis tu rba nces, a taxia, o Nec ro tic m et astasis, syste m ic
ip silateral facial wea kness &. facia l a ne st hesia o Cystic epe ndym oma
IEMBRYOLOGY I.?EJECTED RE,FERENCES "

Em b ryo log ic Events I. Kochartan A et al: Hybrid ph ased array for imp roved
• lAC forms separate ly from in ner ea r & ex te rn al ea r int erna l auditory canal imaging at 3.0-T MR. J Magn Reso n
Imaging. 16(3):300-4, 2002
• Forms in respon se to mi grat ion of CN ? No CN8
2. Sartorcttt-Schcfer S et al: Spatial relation sh ip between
through th is area vestibular weighted fast spin-echo MR images. AJNR Am J
Pract ical Im pl ications Neu ro rad tol. 21(5):810-6, 2000
3. Daniels Rl et al: Causes of unilateral sensorineural hearing
• lAC m ay be ab se n t or present in de pe n den t o f in ne r, loss screened by h igh-resolution fast spin echo magnetic
m iddle or exte rna l ea r develo pm ental sta tus resona nce imaging: review of 1,070 consecutive cases. Am J
• lAC size depends o n n u mber of nerve bundles Oto l. 2 1(2): t 73-80 . 2()(X)
m igrating th rough thi s area at ti me of lAC forma tion 4. Sch malbrock I' ct al: Assessment of inte rna l a uditory canal
o Fewe r n erves, sma lle r lAC size tum ors: a comparison of co ntrast-enhanced Tl -weight ed
o An y o f -l nerves in lAC ma y be m issin g and steady-state TZ-wcigh tl'ti gradien t-echo MR imaging .
o Wh en o n ly o ne n erve is see n , it is usuall y facial AJN R Am) Neuroradiol . 20( 7):1207 -13 , 1999
5. Naganawa S et al: High-resolution ~tR cisternog raphy or
n erve I< lAC is very sma ll th e cerebellopo nttne angle, obtain ed with a
thr ee-dimensional fast asymmet ric spin-echo seq uence in a
0.35-T ope n MR imagi ng un it. AJNR Am J Ncuroradiol .
20 (6): 114:1·7. 1999
IIM AGE GALlERY

[ ;a;:; .....
1
5
Normal
(Lef t) Axial T2WI MR
thro ugh cephalad Me
u;'\'L'J ls normal facial ner ve
(black arrow) 8 supe rior
vestib ular nerve (open
arrow ). Cur ved s n o w: AICA
rOOf) . White arrow: Normal
cere be llar flocculus . (Rig },l)
Axia l bone CT through
superior lAC shows
labyrint hine segll )('nt o f facial
nerve canal exiting lAC
(arro w) S superior vestib utsr
nerve canal cOll n ecting lAC
to ante rior \'estibuJe (open
arrow ).
Normal
(I.ef l) Axial TlWI MR
th rough inferior lAC show s
co cti tear ner ve (arro w ) .'{
inferior vestib ular ner ve
(op en arrow) co urse through
high signal CSF. N otic e
margin o f interior coreb etter
peduncle (cu rved arrow ).
(Right) Axial bone CT
(in ferior lAO . Arrow:
Cochlear aperture. Open
arrow: tnkn ior vestibular
ner ve conat teeves fundu s.
Cur ved arro w : Singular
toresnen w ith po slerior
b ranch inferio r vestib ular
nerv e.
No rmal
(/£JI) Grap hic of fundus of
lAC sho w s all oJ nerves.
Anterio r supe rior is facial
ner ve (arro w) . An terior
inferior is cochlear ner ve
(op en arr ow). Superior
vestibular & in ferior
vestibula r ner ves also seen .
(Right) Sagittal o bliflUf-' TlWf
MR th rough mid-lAC shows
all four nor mal ner ves.
A rro w ; Facial nerve. Open
arrow ; Coch lear nerve.
Curved arro w ; Inferio r
vestibu lar nerve. Superior
vestib ul ar nerve not Idbeled.
EPIDERMOID CYST, CPA-lAC
1
(,
Axi.l! gr.lphic shows large CPA epklPrmoid C)'5t uli/hin Axial T2WI MR s!lO\vs the irregular margins (arrows) of
I)'pical -/x v:! of peads- a1J1xw ance. Notice 5th (open a CPA ('p ickrmoic/ cyst. No tice also the 7th cranial
arrO\v) and 7th & 8th cranial nerves (arrow ) are nerve engulfed in the anU.'fOsu/X'rior margin of the
( hiJfilCl('fi.\tically t>ngulti'Ci. lesion (open arrow ).
o Spread vecto r is cephalad int o medi a l m idd le cran ial

ITE RM INO LO GY fossa
Abbrevia tions and Synonyms • Size
• Epidermoid cyst of CPA ciste rn (EpC-CPA) o Wide range o f sizes reported ; 2-8 crn m axim u m
• Syno n ym s: Ep ide rmo id tumor, prima ry cholesteato ma diamete r
or epit he lia l incl usion cyst o Sign ificantly larger tha n acousti c schw an no ma at
presen tat io n
Defi nitio ns • Morphology
• Co nge n ita l in tra du ral lesion a rising from in clu siun of o Mass in sinuates into cis te rns, e ngulfs cra n ia l nerves
ectode rm al e pit he lia l eleme nts dur in g neural tub e & vessels
closur e o Ma rgins usua lly scallo ped o r irreg ular
• "Wh ite epidermoid" • Ca uliflower-llke m argin s possible
o Epid ermo id cyst with h igh protein co nte n t ca using o Wh en large m ay in vade ad jace nt brainstem ±
h igh signal o n Tl , lower sign al on T2 MR seque nces ce rebellum
o Rare Epe-CPA va ria nt
CT Find ings
• NECf
o Resembles cerebral sp in al fluid (CSf) on NECT
IIM AGING FINDINGS o Calcificatio n in 20 (}I'1 usually alo ng EpC-C PA margin s
Ge ne ral Featu res o Pressure erosio n of adjacent T-bone may occur
• Best diagnost ic clu e • CECT
o CPA ciste rna l insinuati n g m ass wit h h igh signal on o No en h ancem en t is rule
dif fusio n MR • Somet imes ma rgin o f cyst min im ally en h ances
• Engulfs cra nia l nerves (7t h &: 8t h), vessels (ArCA, • If nodular en h an cem ent seen , co ns ider rar e
vert ebr al a rtery ) squamo us cell ca rcin oma ar ising from EpC-CPA
• Location o "Dense" epiderm oid
o Posterior fossa most co m mon site • Rare h igh d ens ity EpC-C PA va ria nt
• CPA 75%, 4th ven tricle 25%
DDx: CPA Cyst ic Mass
Arach no id Cyst Ne urenteric Cyst Cystic Acou stic

Key Facts
1
Termin ology • Neu rente ric cyst 7
• Syn o nyms: Epide rmo id tu mo r, prim ar y • Cystic neop lasm
cholesteato ma or epithe lial inclusion cyst Pathology
• Conge n ita l intradural lesion arising from in clusion of • 3rd most commo n CPA ma ss
ectode rmal epithe lial eleme nts during neur al tu be • liM, of all int racran ial tum ors
closure • Pearl y whi te mass in CPA
Imaging Findings Clinical Issu es
• CPA cistern al in sinuating m ass wit h hi gh signa l on • Clinical profile: 40 year old pati ent with minor
diffusion MR sym pto ms ha s a la rge EpC-CPA di scov ered in CPA
• Eng u lfs crani al ne rves (7th & Sth), vessels (AICA, cistern o n MR
vert ebr al ar tery)
• Lack of any atte n uation o r "incom plete atte n uatio n" Diagn ostic Checklist
o n FLA IR is suggestive of EpC-CI'A • Diffu sion MH ca n a lso be used effective ly to diagn ose
recurr en t EpC-CPA
Top Differ ential Diagnoses
• Arachnoid cyst
MR Findin gs IDI FFERENTIAL DIAGNOSIS

• TlWI Arachn oid cyst
o Iso in tcnse o r slightly h yperint ense to CSF sign al
o Wh en sligh tly h yp erintense, term "d irty CSF" ha s • Displaces, does not eng ulf ad jacen t structures
been app lied • Does not insinuate
o "Wh ite epide rmo id": High '1'1 signal • T 1 & T2 signal foll ows CSF signa l
o May be higher signal on T2 becau se of n o CSF
• T2W I
o lsolnt cnsc to hyperin ten se com pared to CSF pu lsati on s
o "Wh ite e pide rmo id": Low '1'2 signal • Fully att enuates on FLAIR seque nce (low signa l)
• FLAIR
• Shows no rest rictio n on diffu sion weigh ted imaging
o EpC-CPA does not null (atte n ua te) (low signal)
• Lack of any at te nuatio n o r "incom plete Neurenteri c cyst
attenuation " on FLAIR is suggestive of EpC-CPA • Most commo n pre-ponti ne ciste rn in locat ion
• "Inco m plete atten uatio n" refers to a mixed signa l • Tl high signa l (migh t m imic "white epid erm oid")
lesion o n FLAIR wh ere part of lesion atten uates, • T2 signa l often low
pa rt do es not atten uat e
• DW I Cystic neoplasm
o High signa l on diffusion scans makes diagn osis • Cystic menin gioma and schwa n noma bo th rare
o High signal o n DvVI indicates restric ted diffusion is • Epend ymoma and astrocyto ma ped un culate from
present hrainstem and -lth ven tricle respectively
o Foci in su rgical bed indi cates recurrence • Will sho w some areas of en hanceme nt o n T l C+ MR
• T l C+
o No en ha nce me n t is rule
o Mild peripheral en ha nce me nt occurs in IPATHOLOGY
ap proxima tely 25 1)'h o f cases
• MRA General Features
o Vessels o f CPA may be displaced o r engul fed by • Etiology
EpC-CI'A o From inclu sion o f ectoderma l eleme nts d uring
o Artery wall dim en sion no t affected neura l tub e clo sure
• 3rd-St h week of em bryoge nesis
Imaging Re commendation s' • Resu lts in migrati on abnor ma lities o f epiblastic
• Best ima ging tool: Brain ~fR with FLAIR, DWI & ce lls
en ha nced Tl seq uences • Ep h..lemio logy
• Protocol advice o 3rd most commo n CPA mass
o Begin with routi ne en ha nced MR imagin g o }lyh of all int racranial t umors
o FLA IR & d iffusio n seque nces added to confir m
d iagn osis Gross Pathol ogic & Sur gical Featu res
o Follow-up study look ing for recurr en ce mu st inclu de • Pearly wh ite mass in CPA
FLAIR & diffusion seq uences • Surgeo ns refer to it as "the beautifu l tum or"
• Lobu lated , cauliflower-sha ped su rface featu res
• Insin uati ng growt h pattern in cistern s
o Engulfs ciste rn al vessels &. nerves
1 • May become ad he ren t
• May ca use hyp eractive dy sfun ctio n of cran ial
o Insin uati ng CPA ciste rna l lesion sign al is Iow a n T l ,
high on T2 (sim ilar to, but not iden tica l to CSJ=)
8 nerve (eN) 5 or 7 o In complet e atte n uation (mi xed signa l) o n FI.AIR
o Rest ricted diffu sion (h igh signa l) on DWI is present
Microscopi c Features
• Cyst wall: Sim ple st rat ified cubo ida l sq uamo us Image Interpret ation Pearls
epithe liu m Diffusion MR im agin g seq ue nce is t he key to co rrect
• Cyst co nte nts: Solid crystalline cho lesterol, diagn osis
kcra tln acco us d eb ris • Diffusio n MR ca n also be used effectively to d iagn ose
o Does not co ntain hai r follicles, sebaceo us gla nds o r recur ren t EpC-CPA
fat (i n cont rast to derm oid)
• Grow s in success ive laye rs hy desq uamatio n from cyst
wall ISELECTED REFERENCES
L Haml at A et al : Malign an t transfo rm ation o f in tracranial
epidermoid cyst with lepto m en in geal ca rcinoma tosi s: case
ICLINI CAL ISSUES re por t. Acta Ncurol Belg. 103(4):221 -4, 2003
2, Lakhda r A ct al : Ep ide rmoid cyst o f th e cerebellopo nt ine
Prese ntation ang le. A su rgica l scn cs o f 10 cases and review of t he
• Most co m mo n signs/s ym pto ms literature. Neuroc h lrurgte. 49(1):13-24 , 2003
o Princi pal presen ti ng sympto m: Dizziness :l. Koba ta II ct al: Ccrcbcllo pou tinc a ng le c pid en n oids
o Ot he r symptoms: Depend on locatio n, growth presen ting wit h crani al n N W hyperacti ve d ysfun cti o n:
pat hogen esis and long-term surg ica l resu lts in 30 pat ien ts.
pattern Neu rosu rgery, 51):27h-8S, 2lXJ2
• Trigem inal neuralgia (tic dou lou reux) 4. Du tt SN et al : Radlolo glc di fferen ti atio n o f intracran ial
• Sen sor ineural hearing loss e pide rmoids fro m a rach noid cysts. Oto l NCUfotOI.
• Facial neu ralgia (he m ifacial spasm) 23:84-92 , 2lXJ2
• Headach e 5. Decha mb rc S vt al : Diffusion-weigh ted MRI posto perat ive
o Sym ptom s usuall y present for > .. yea rs befor e assessme nt o f an ep idermo id tu mo ur in the
Er e- CPA is di agn osed cerebellopon t inc angle. I'\cu ro radi o logy. 4 1:829-3 1, 1999
• Clin ical profil e: 40 year o ld pa tient wit h mi nor 6. Oeh i M ct al: Unusual c r an d MR appea ran ce of an
e pider moid tum or of th e cc rcbcllo pon t lnc angle. AlNR.
sympto ms has a large EpC-CPA dis covered in CPA
19:11 ]]-5, 1998
cistern on MR 7. Talaechi A ct al: Assessm ent an d su rgical managem ent o f
posterior fossa ep ider mo id tu m ors: repor t of 28 cast's.
Dem ographics Neuro surge ry. 42 :242-51, 1998
• Age 8. Timmer F,\ ct al: Ch em ical analysis of an e pide rmo id cyst
o Alt hough co nge n ita l, presen ts in adult life w il h un usual C'T a nd MR cha racter istics . AjNR. 19 :1 111-2,
o Broad p resentati on from 20 to 60 years 1998
• Peak age = 40 yea rs 9. Ikushima I et al: MR of e pid en noi d s w ith a variety o f pu lse
seq uencl's. AlNR. 18:1359-63, 1997
Natural Histor y & Prognosis 10. Mohan ty A et al : Experie nce with ccrebellopontl ne angle
• Slow growi ng congen ital lesions that rem ain s cp lde rmoids . Neurosurgery. 40 :24 -9,1 99 7
cl inically silen t for m any years I I. Kuzma ct a l: Epiderm o id or ara ch noi d cys t? Su rg Neu rol.
• Smaller cistern al lesions a re readil y cured wit h su rgery 4 7:395-6, 199 7
12. Kallmes OF et al: Typica l and at ypi cal ~fR imagin g feat ures
• Larger lesions where up ward supra te n tor ial herniation
o f in tracrania l ep id ermoid tu m ors. AJR. 169:883 ·7, 199 7
has occurred a re more difficult to com pletely remove 13. Tlcn RO cr al: Variable band wid th steady-s ta te
o La rger lesions ten d to have 1110 re sign ifica nt surgi ca l free-precession MR im aging: a techni que for improving
co m plicat ions chara ctc rtzatlo n of ep ider m oid tu m or and arach noid cyst.
AJ IL 1640689-92 , 1995
Treatment I ~. Gao PY ct <11 : Radlologlc-pat hologlc cor re latio n.
• Co m plete su rgical rem oval is goa l Epiderm o id tumor of th e ccrcb cllopo nt ln c angle. M Nlt
o To tal rem ova l po ssible in < SO'){, U o86:l-72 , 1992
o Near-to tal remov al ofte n bett er surgical choice IS. Altschu ler EM et al: Op erative t reat ment o f in tracran ial
• Aggressive tot al remova l may cause sign ifican t cplde nuold cysts and cholestero l gran ulom as: re port o f 2 1
cases. Neuros urgery. 26 :606-13 , 1990
cra n ial neu rop ath y
16. Dexouzu CE et il l : Cercbcllo pon tin c angle epi de rmoid ey sh:
• Used when EpC-C PA ca psu le is ad herent to a re por t o n 30 cases. J Neurol Neu ros urg Psych iatr y.
brai n stem & cran ial nerves 52:986-90, 1989
• If recurs, ta kes man y yea rs to gro w 17. Tam pie rl D el al : r..m imaging o f c pldc rmold cysts. t\jN R.
o Diffusion MR sequence is the key in assessing for 10 :351-6, 1989
recu rren ce 18. Yam akawa K et 3 1; Clin ical co urse and surg ica l p rogn o sis of
:B cases o f in tracranial ep ide rmoi d tu mor s. Neu rosurgery,
24:568-73 , 1989
IDIAGNOSTIC CHECKLIST
Conside r
• MR diagn oses EpC·CPA whe n
IIM AGE GALLERY 1
'J
(/.e/I) Jh i.l l TlWI u« shows

.J high s;XflJI wilh d O
/(' .\;cm
;"egul.1fbore/, ·, . l/on~ ih
mstgin with the b,.lin,tt 'm
..Iu d n'(('bellum (, " 'OW \ ) .
,\ (il(/ penPlr.ttkm or lilt' Me
j, W('n tope n .1ff()W ' . (Hi/:h"
A~j.)' HAIR.\IR of IpC·CPA
rt>\ 'f:'. l/s · ;ncompletf·- l1uid
suenustion. NOli n - (h.I! thl'
JIJ/{ '(;()( portion h.h n o IJuid
.1lh 'tJudt;o n (,U fO "" 1\ hift'
IX}_~l e,i()r portion show .,
p.lrli,l/ .llt(·nfM tion (o/ )('n
,I" O W }.
Variant
(1.,(1" A,i,ll T2 WI AIR sho ws
J.Jfgt. I " C O 'A th,l l iIIus tr .llp.5
till' ;11\', 1,;\ '( ' 'loI/ufl' of '('sioll .
Nrl/i( "" /t'!oilm h. I.o; im'iJded
Cl'f('lJ ..llu lll ,lltlllfi .1 /U(),U/
fronl ( ,ufCII\'SJ. l \leA loop
clis,lfJlw, lI\ into m .15.5 (open
arrow ). (RiKItI) fJWI AIR
im,j~l' of I.Jr~tl, invssi ve
lpC-CI't\ /( '~'( 'd/, 11I.,.:hlr
c h.l r.J( · t1 ·fi~ l ir Ilrii:ht <;i~na '
(J ", )\ \' ~J 11I(!I { ·.ltiflR (/ilill\;On
restriction. I his
consp;cuot/\/r hiRh sl~ "JI
!<ign.ll uf(, I••h i/)' cJlli"('f('tJli.ltt->\
lpC-CI }\ in,'" ,u, lC'hno ir1
cyst .m d v /II(·, Cflt\ 11 · ~ i()fl~ .
Oth er
-~ (/.t'!O Sagilt .J1 gt .lp hlc of tht->
b, .1;nslcm .\ /,o,,'s .1 l p(_--CPA
,h.l t has im"O/\ '1'(/ the
pH 'Ix.m'ine ci..tt->m. ttwro II
('"1-:u1fs the b.hil.1I ,1Ilt'fy
(. lfH )\ \ ') . (Rig!lt) CrCJ.\~
p .ltllo/og y of 1('!i('C l(>(/
fp C-CPA Close-up \-;('\\"
~h()ws the lohul.lll-d./X.'.II /y
~1Jt ':1(,(' 0'- fhe l"} '!<l.
ARACHNOID CYST, CPA-lAC
1
10
Ih i.1f gr.1phic or arachnoid ("}'Sf in em .~ fJ(}',~ its thin. Axj,ll f 2WI A1R dt It'lIt,l of me low em ~h<)I'VS J high
If.1fb/t/(pn! lVaf!. N otic e i/5 "pus1Jing ~ rt'!dfiOllship to 71h ~ igf)alovoid mass (opon arrow ) tl.lllcning the adjacent
,~ lJIh Cf,m i.l f IJ('IV( >S (arro w ) Jnd braifJ51('m-cMx,lIum cerebf.'l/,If h('mi~p!)(>re. Notice Iligh ~igna l of arachnoid
(0 "'(' 11 .lrrmvs ). cy~t OTJ T2W! similar /0 CSF.
• May spread into lAC ( 1StJ.' iJ)

[TE RM INO LO GY • Size
Abb revia tions and Synonyms o Broad size ran ge expected
• Abbr eviation : Arach noid cyst (AC) o May be very la rge hut asym pt o ma t ic
• Syno nym s: Primary AC o r co ngen ita l AC o Wh en large, w ill exert mass effect o n adjacen t
br ainstem & ce rebe llum
Definition s • Mo rphology
• Definition : Arachnoid o r co llagen -ltned cavities th at o Sha rply dema rca ted lesio n wit h br oad a rch ing
do 110t co m m u n icate di rectl y wit h ven tricula r syste m m argins
o r subarach noid spac e a Displ aces, does 110t eng ulf sur ro u nd ing st ructur es
o Foca l lesio n th at pu sh es ciste rna l str uctures bu t does
not in sinuate (cf e piderm oid cyst )
[IM AGING FINDING S CT Findin gs
Gene ral Features • NECT
• Best d iagn o stic clue o Den sity sa me as csr-
o Cyst ic ciste rn a l mass w ith impercep tible wa lls wit h o Rare h igh den sity fro m hemo rrh age or
CSF d en sity (CT) or in ten sity (MR) pro tein aceou s fluid
o Lesion signal pa rallels signal of CSF o n a ll Mk a Bo n e ch a nges: Rarely causes pressur e eros ion of
seq uences adj acen t hone
• Co m plete flu id att en uatio n o n FLAIR MR im aging • CECT: No en ha nc emen t of cavity o r wall
• No diff usion restr icti on on DW I MR ima ging MR Findin gs
• I.o cati on
• Tl W I: l.o w signa l les io n Isointcn se to CSF
o 33 1M I o f a ll AC occur in posterior fossa
• CPA = most co m mo n in fratc n to ria l site
• T2 W I
a High sign al lesio n Isotn tcn sc to CSF
o Sp read patte rn s
• May have bri ghter signa l th an CSF
• Most rem a in co n fine d to CPA (601).'fJ)
• Fluid w ith in cyst lacks CSF pu lsa tio n s th at
• May sp read dor sal to hrain stem (251).(,)
decrease CSF signa l
DDx: Cystic C PA Mass
Epidermoid Cyst Neurenteric Cyst Cystic Acoustic

Key Facts
Termino logy • Neuren teric cys t
• Abbreviation: Arachnoid cyst (AC) • Cyst ic acoustic schwan noma
• Synonyms: Primary AC or co ngenital AC Pathology
• Definition: Arach noid or co llagen-lined cav ities th at • Embryonic meni nges fail to m erge
do not comm unicate di rect ly wit h ven tricu lar system • Noncommunicating fluid compa rtme nt surroun ded
o r suba rach noid space by arachn oid is formed that contains CSF
Imaging Findings Clinical Issues
• Cystic cist ernal mass with impercep tible walls with • Clin ica l profile: Adul t undergoing brain MR for
CSf density (Cl') or int ens ity (MR) unrelated symptoms
• Lesion signal parallels signal of CSF o n a ll MR • Most cases require no treatment
sequences
• 33% of all AC occu r in posterior fossa Diagn ostic Checklist
• CPA = m ost com mon in fra ten to rial site • Differentiate AC from epidermoid cyst
• AC lacks restricted diff usion (hig h signal) o n DWI MR
Top Differen tial Diagn oses = best cl ue
• Epidermoid cys t
o Well-cirru mscr lbed, push ing lesion co mp resses • Dura l ta ils, asymmetry to lAC still present with mi xed
adjacen t bra ins tem &. cere bellum whe n large en ha ncemen t on T l C+ MR
• Ilyd roceph alus seen with only la rger CPA AC
• Very rare associa ted find in g Cystic e pendy mo ma o r astrocytoma
• f lA IR: Suppresses co m p lete ly (low sign al) with FLA IR • Ependy mo ma ped uncu lates from 4th ventricle via
• OW l: No restriction (low signa l) on d iffusio n MR fo rame n of Luschk a
• Astrocyto ma pcdu nculates from brainstcm
• T I C+
o No en hance men t seen
o AC wa ll im perce ptible eve n o n co n tras t-en ha nce d
MR seq uen ces IPATH OLOGY
Imaging Recom me ndations Ge ne ra l Feat ures
• Best imagin g tool : Whol e bra in MR imaging • Genera l path co m m ents
• Protocol advice o Split a rach noi d con ta ins CSF
o O nce AC is suspected o n b rain MR, add fo llowing o In t racra n ia l AC
sequences • Most common locati o n for intracran ial AC is
• H .t\ 1R wi ll sho w AC as low signal mid d le cranial fossa (50%)
• Diffusion seq uence will show low signa l • Posterior fossa AC second most co m mon locati on
• Focused T l C+ 1\IR will show no en hancemen t (33%)
with im pe rceptible cyst wall • Sup rasellar ( l ()t)h) and othe r spo rad ic intracranial
locations (7%)
• Etio logy
ID IFFE RENTIA l D IAGNOSIS o Embryon ic meninges fail to merge
o Noncom m un icat in g fluid co m pa rt me n t sur rounded
Epide rmo id cyst by arach no id is form ed that co n tain s CSF
• M ajor lesion of differential co nce rn in setting of AC • Epide m iology: Accounts for PH, of intracranial ma sses
• flA IR MR: Incomplete att en uati o n (m ixed sign al) • Associated ab no rm alities: Acou sti c schwan n om a has
• Diffusio n MR: Restricti on (h igh signal) AC associated in 0 .5%
• Morpho logy: In stn u ates ad jacen t CSF spaces &
vessels-crania l nerves Gross Patho logic & Surgica l Features
• Fluid-co ntaining cyst with translucent membrane
Neuren teri c cyst • May displace but do es not engulf adj acent vessels or
• Very rare lesio n cra nial nerves
• Usually prc -po nttn c cistern n ear midline
• Often con tains proteinaceous flu id (high signal o n Tl Micro scopic Features
MR sequences) • Th in wa ll o f flatte ned but normal arac h no id cells
• No glial lim it ing membrane o r epit he lial lining is
Cystic aco ustic schwa nno ma prese nt in AC wall
• In t ramu ral cysts see n in larger aco ustic schwa n no ma • No infl a mmation or n eoplastic ch ange
• Foci of en h anc ing tu mo r always p rese n t o n T1 C+ MR
Cystic men ingioma
• Rare meni ngiom a varian t
1 ICLINICAL ISSUES • If an y n od ular en ha nceme n t, co ns ide r alt ernative
d iagn osis
12 Presentat ion
• Most com mo n signs/sy mpto ms
o Sma ll AC: Asym pto ma tic, in cid en tal find ing o n MR ISE LECTE D REFE RENCES
o Large AC: Symptoms from d irect co mpressio n &/oe I. Sinha S et al: Familial posterior fos sa arachnoid cyst. Chi ld s
raised in tracra nia l pressu re Nerv Sys t. 20 (2 ): 100 -3, 200 -1
• Cli n ical profile: Ad ult u nd ergoing brai n M R fo r 2. Chcmov MF ct al : Double-endoscopic a pp roach for
un related sym pto ms ma nag em ent o f co nvcxtry arach noi d cyst: case report. Surg
Neu rol. 6 1(5):-IK3-6; d iscussion 486 -7, 200-1
• O the r sym pto ms: Defined by locati on &. size
3. O'reilly RC et al: Posterior fossa a rach no id cysts ca n m im ic
o Vague, nonspecific sym pto ms co m mon Meniere's d isease . Am J Oto tary ngol. 24(6):420-5 , 2003
o Headache 4. Blaich cr \V et a l: Magn et ic reso n ance imaging and
o Dizzi nes s, tin n itus an d /o r sensorine u ral hearin g loss ul trasou nd in the assessm ent o f till' feta l cent ral nerv ous
(SNHL) system. J Perin at Mt"t.l. 31(6): -159-68, 2(XB ;3 1(6):459- 68.
o Hem ifacia l spasm or trigeminal neuralgia 5. McRrid e LA et al: Cystovcn tr lcular sh un ting of intracrania l
ara chnoid cysts. Pedlatr Neu ros urg, :~9 ( 6 ) : 3 23 · 9 , 2003
Demograph ics 6. Dutt S='I et al: Radiol ogi c d iffcren tiation o f in tracr an ial
• Age epidc n n oids fro m arach n oid cy sts. Otol Ncu rot o t.
o May 1>< first seen at any age 23( 1):84-92 , 2002
7. O ttavian i Fetal: Arach noi d cyst o f the cran ial po ster ior
• 751M) of AC occ ur in ch ild ren Iossa ca using sensorineura l he ar ing I O\~ and tin n itu s: a cast'
• Ge nde r: M:I' = 3: I report . Eur Arch Otor hino lary ng ol. 259 (6):306·8, 200 2
Nat ural History & Prog nosis X. Boltvhauser E ct al: Outco me in ch ild ren .....it h
space-occupying po sterior fossa arachnoid cvst s
• Most AC do not en large ove r ti me Neuropc diat rlcs. :\3 (3): 118-2 1, 2002
o In frequ entl y en large via CSf pu lsat io n th rou gh 9. Bon neville F N al: Un usual les io ns of t he ccrcbcllopo n tt nc
ball -valve o pe n ing int o AC an gle: a segmen tal a p proach . Radl ogra phlcs. 2 I(2):4 19-3K,
o I fe mor rha ge w ith subseq ue n t decre ase in size has 200 1
been rep or ted 10. Gangcm t M ct al : End o scopi c surge ry for large po sterior
• If su rgery is limi ted to AC where sym pto ms a re clearly fossa arach no id cysts. Min im In vasive Neu ros urg.
rela ted , prog nosis is exce lle n t 44(1 ):2 1-4, 200 1
II. Ucar T et al: uuewret ccrcncuoponune angle ar achno id
• Radical cyst rem o va l may result in cran ial neuropath y cys ts: case report. Neu rosurg ery. (4):966-8, 200n
and /or va scular co m prom ise 12. Sam ii ~1 ct at Arachno id cysts o f th e po sterior fossa. Surg
Ne u rol. 5 1(4):376-R2, 1999
Treat men t 13. Taka no S et .11: Facial spasm and paroxysma l ti nn itu s
• Most cases req uir e 110 trea tmen t associa ted with an arachnoid cyst o f th e ce rebcl lo po n tinc
• Su rgical int er vent ion is h ighly select ive process angle-case report v; e u ro l Mcd Ch ir. 3K ( 2): H Kk~, 199K
o Reserved for case s wh ere clear sym pto ms ca n he 14. Cho i JU et al: Pathogenes is o f ara chnoid cys t: co ng enital or
d irect ly lin ked to AC a nat o m ic locatio n . trau mat ic? Pedia tr Neu ro surg. 29 :260-6, 1998
o Endoscop ic cys t deco mpression via fen estrat ion 15. Shu kla R et al : Posterio r fossa arachnoid cyst p resen t in g as
• Least in vasive in itial approach hig h cervical co rd co m pressio n . IIr J Neuros urg.
12(:l):2 71-3, 1998
• Suboccipi ta l ret rosigrno id approa ch pre ferred
16. j ane G I et al: Arach no id cysts of ti ll' cerebello po n tm e
an gle: diagnosis and su rge ry. Neur osurgery, 40( 1):3 1· 7,
199 7
IDIAGNO STIC CHECKLIST 17. Hodmark 0: Ncu roradiology o f select ed d iso rders of t he
meninges, ca lvarium and ven om sin uses. AJNlt 13:483-9 1,
Conside r 199 2
• Differen tiat e AC from epi de rmoid cyst 18. Higashi S et al: Hem ifacial spas m associa ted .....ith a
o AC lacks restrict ed d iffusion (h igh sig nal) on DW I cerebcllopon tlnc an gle a rach no id cys t in a young adult.
M R = best clue Surg Neurol. 3 7(41:2X9-92 , 1992
19 . Ba bu R et al: Arachnoid cys t o f th e cerebel lopcn ttne ang le
• Det e rmine if sy mptoms match loca tio n of AC befor e
m ani fest in g as co nt rala tera l trigem in al n eur algia: cas e
co n sidering surgical treat men t report. Neurosurgery. 2X(6):886·7, 1991
• Progressivel y less su rgica l treatm ent is trend 20 , weiner SN ct al: MR im aging o f in t racra nia l arac hnoid
cysts. JCAT. 11:2:16-41 , 198 7
Image Interp ret ation Pearls
• AC sign al para llels CSF o n a ll MR seq ue nces = key to
rad io logic di ag nosis
o Remember T2 signal may be high er than CSF fro m
lack of CSF pu lsa tion
• D\VI MR seq ue nc e will show AC as low signal (no
di ffusion restricti on ) lesion
• Fl.AIR MR sequence will sho w AC as a low signal (f lui d
atte n uat ed ) lesio n
• No enh an cem en t of AC, in cludin g wall, is ex pected
IIM AG E GALLERY 1
Typical
(Left) Axial T2WI MR. ff.-' W dls
a med ium 5;7(' high s;gn,11
arachn oid cy st in the low
right Cl Vl cistern. T1Jis /esiofJ
can be 5('('0 (/i\ pl,K ing tht'
~th cranial ne rVI'
anreromedially ( ilrfOW) . Such
"p ushing " dispJ.lcPlJlent i.~
th e rule ill arach noid c yst.
(RighI) A xial fLll fI~ MR
image de monst ralt'S
comp totc fluid atte nu atio n of
this medium siLt' Jrar hnoid
c yst (arrow ), leaving this
lesion devoid of signal. The
9th cranial ne rve can be
S(' P Il pusht'd anlt'HJnwdi<,lIy
(opf.·n arrow ) by lil t' lesion.
Variant
-~.... (I,{'fl) Large· .1far hnoid cyst of
pos terior CPA cisu-m seen in
an axial 72 MR image sho ws
.1smoo th, pu.~h ing margin
(arro ws) in il s int erface wil h
rill' subiocon t cer ebcttum.
The high signal fluid within
th e cyst par<l!!efs the signcl f of
CSF. (Riglt l ) ;\xiJI D WllVIR
rev(',l/s no ovkicn ce (or
restrict ed diffusion l.1r8(' low
sign<l' arectmoid cyst
(arr o w s). A b wnce of
tesuiction on O Wl MR
se qu ence di fferen tiate s
arachnoid cyst from
epiderm oid cysl or CPA.
Other
Coronal gr,'phic o(
(I.e/I)
CPA arach noid cy.\t shows
typical translucen ! CYSl wall.
CN ? & tJ are push ed b y cyst
("' rro w) wi thou t being
t'n gulfed b y it. In vp icietmoici
cyst e Ns arc UWJI/ y
('ngulfed. (Higltl) Corona l
gross p<Hho/ogy specimen
views elf! elf,1Chnoid cyst ill
thc' left CPA cistern fro m
1)~ 'low (arrow s). Notic e the
gossamer-ihin, trenslucetu
walls of the cyst itself
(Courtesy f . T.
J {cd/ey-Whylt\ MO J.
LIPOMA, CPA-lAC
1
14
AJ(;'lJ graphic oi ,1 CPA lipom,1 (arrow ) illtl'itr,lll 'S the 7rh A'l:i.ll TIWI AIR C1Y\ lipoma is a~!>OC;a lcd with elf}
& 8th cranial nt:>(VC'S as "it4! as the 1\10\ 'o1:!S~d (open ;ntra\'(.:-slibular lipoma (open am:M.v). Nexico 8th a .1O;.11
.lffo\-Y) p.min,; through tht'lipoma on too'
way into rht.· oC'm:' (~Irro. v) 'lim ing through tbe em Ii, XXlJ.I on ib
lAC way to IhC'infernal alJ(lilory canal.
• Linear a long co urse of cran ial nerv es 7 &. 8 in CPA

ITE RM IN O LOGY • Ovo id with C PA ciste rn
Abbrevi ation s a nd Syno ny ms o Lar ge lesio n s
• l lumarto rnatou s lipoma of cerebellopo n ti nc a ngle • Broad-b ased hem ispherica l sha pe adherent to
(CPA) ± internal audito ry can al (lAC) late ra l m argin o f pon s
Defi nitions CT Find ings

• Lipoma, CPA-l AC : Benign , co ngen ita l fatt y lesion o f • N ECT
CPA ± lAC o Low den sity CPA mass
o Facial N vesn bulococh lcar nerves pass th ro ugh o Measure mass lI ounsfield un it (HU) if un certain
lesio n o n way to lAC • l lo un sfield un it range: -20 to -60 HU
• CEC r: Lesio n does not en ha nce
MR Findings
IIM AGING FINDINGS • TlWI
Gene ral Feat ures o High signal CPA m ass (para llels subcutan eous &
m arro w fat -Intensity )
• Best diagnostic clu e: Focal be nign-appear in g CPA m ass
• Inne r ea r noncontiguou s seco nd fatt y lesion may
which fo llo ws fat density ICT) &: in tensity (MRJ
be present
• l.oca t ion • In n er ca r locati on = vestibule
o Primary loca tion = CPA ciste rn
• T2W I
• lAC a lone rarel y seen
o Int ermed iate "fat-in ten sity" lesio n
• Co ncurren t Intravestlbular deposit ma y be see n in
o l oses signal in parallel with subcutaneous a nd
associati on with CPA or lAC prima ry lipoma
m arrow fat
• Size o Che m ica l shift artifa ct a long frequ ency e ncod ing
o 1-5 em in maximum diameter
directi on
• May 1)(: as small as few millimeters
• STIR: l esion "disappea rs" du e to STIR in herent
• Morphol ogy Iat- saturat lon
o Small lesions
• FLAIR: Fatty lesio n rem ain s high signal
DD x: C PA Hyp erinten se Mass
Wh itt' [p ic/('rmo id Neuren teric Cyst I iemon Ac oustic Nilpture d Oonnotd

LIPOMA, CPA-lAC I
Key Facts
1
Termi nology Path ology 15
• Lipoma, CPA-lAC: Ben ign , co ngen ital fatt y lesion o f • Lipo mas occu r less freq uen tly in CPA t ha n
CPA ± lAC epide rmoid & ara chnoid cysts
• Facial & vestibulocochlear n erv es pa ss th rou gh lesion • CPA lipoma is l()l,l(, of all intracrania l Hpomas
o n way to lAC • Associa ted abn ormalities: Co nc u rren t seco nd fatt y
lesio n may occ u r in inner car vestibule
Imaging Find ings
• Best d iagnostic clue: Foca l benig n-appea ring CPA Clinica l Issu es
mass wh ich follows fat de nsity (CT) & in tensity (MR) • Clin ical pro file: You ng ad ult presenti ng wit h slowly
p rogressive unilate ral se nso rine u ral hearing loss
Top Differential Diagn oses • No treatment is be st treat me n t
• "Wh ite" epi dermoid cyst • Surgical removal is no longer recommended in most
• Neu ren te ric cyst cases
• Ruptu red dermoid cyst
• Acoustic sch wan norna, h em orrhagic Diagn ostic Checklist
• Aneurysm • On ce high signa l lesion is seen in C PA on T1 C- MR,
use fat-satu rati on sequen ces to confi rm di agnosis
• T1 C+ Ruptured dermoid cyst

o Lesion is already high signa l o n Tl preco nt rast
ima ges • Ectodermal inclusion cyst
o Use fat-satu rated T1 C+ seq ue nce • Or igina l locat io n usually midline
• I.esion "disap pears" seco nda ry to fat- saturation • Ruptur e spreads fat d ropl et s through ou t suba rac h no id
aspec t o f thi s MR seq ue nce space
• No e n ha nce me n t in region of lesio n is prese n t • Rupture may lead to che mical meningitis
Imaging Recommendati on s Aco ustic schwa n noma, hemo rrhagic

• Rest im aging too l • Rare manifestati on o f co m mo n lesio n
o MR is l st stu dy o rde red wh en sym pto ms sugges t • Pat ch y intra paren ch ym al stgna l o n T1 C· MR
possihility o f C PA mass • High signa l a reas persist eve n wit h fat-saturat ed
o cr ca n eas ily co nfi rm d iagn oses by measur in g seq ue nces
Hounsfield u nits if some confusio n on ~f R images Ane urysm
pe rsists • Ovoid C PA mass wit h calcified rim (en & complex
• Protoco l advice layered signa l (MR)
o Whe n TI C+ MR focuse d to C PA a rea is a n tici pated,
• MR signa l co mplex with high signal areas fro m
need at leas t one preco ntra st '1' 1 seq ue nce methem oglobin in aneurysm lumen o r wall
• Th is T1 C- seq uence help s di stingu ish fatty &
• Docs n ot ente r lAC
hem orrhagic lesions from en h anci ng lesions
• CPA aneu rysm s fro m PICA > VA > AICA
• Fatty lesions incl ude lipoma &- de rmoid
• Hem orrhagic lesions wit h meth em oglobin high
signal include ane u rysm &- venous varix
o O nce hig h signal is see n on T1 C- seq uen ces,
!PATHO l O GY
fat-saturate d seq ue nce s d isting u ish fat from Ge ne ral Features
h em o rrhage • Ge ne ral pat h com me n ts
• Th is approach avoids mis tak ing lipoma for o Co nge n ital lesion
"en ha nci ng CPA ma ss" o Tenden cy to infiltrat e & sp lay a pa rt CPA cra n ial
ner ves (7, 8 )
o Embr yol ogy-an at omy
IDIFFERENTIAL DIAGNOSIS • Lipomas may ra rely he found wit hi n lAC or
"W hite" epide rmo id cyst membranou s labyrinth (vestibule)
• Etiol ogy
• Rar e imagi ng prese n ta tio n of more co m mo n lesion o Best cu rren t hypot hesis for lesion for ma tion
• High T t signa l prob abl y seco nda ry to h igh p rotein • Maldevelo pm en t of meni ngea l precu rso r tissue
co n te n t int ernal flui d (me ni nx pr imitiva)
• Sh ow s restr icti on (h igh signa l) o n d iffusion MR • Hyp erpl asra of fat ce lls normally wi th in pia
• Insinuates ad jace n t CSF spaces & st ruc tures • Maldiffercnriation o f mesod erm in to Iipocytes
Neure nteric cyst rath er t han arac h noi d al ce lls
• Most co m mo n in prepontine cister n • Epidem iolo gy
• Co n ta ins pro tein aceou s fluid (h igh signal o n '1'1 e- o Lipomas occur less frequently in C PA than
MR) ep idermo id & ara chnoid cysts
o Epidermo id cyst > a rach noid cys t > > lipo ma
LIPOMA, CPA-lAC
1 o CPA lipo m a is 10 1"11 of all int racrania l lipomas
• In terhe m ispheric H SIU,), q uadrigemina l/s upe rio r
• Lipom a m ost co m mon (dermo id rare)
a Hemo rrh agic lesio n
16 cerebel lar (25(MI), suprascl tar/ tn terpedu ncutar • Ane ury sm lumen clo t o r clotted ve no us va rix
(15%», sylvlan cisterns (5 %) (du ral ar terio ven ou s fistula )
• Associ at ed abno rmalities: Co ncu rre n t seco nd fatt y • Rare hem orrhagic aco usti c sch wan noma
lesion ma y occu r in in ner ca r vestibule o Highl y prot einaceo us flu id
• Neu ren te ric cyst (usually in prepo n tine cistern)
Gross Path ologic & Surg ical Features • Rar e "white e pidermoid"
• Soft, yellow ish mass
• May in corpor at e cra n ia l ner ves 7 &. 8 wit h den se Image Interpretati on Pearl s
adhesion s • O nce hi gh sig nal lesion is see n in CPA o n T I C- MR,
• May be adh erent to lateral br ain stem use fat -sa turat ion seq ue nces to co n firm d iagnosis
Micro scopic Features
• Highly va scu lar ized lipo m ato us ti ssu e
• Matu re lipocytcs
ISELECTED REFERENCES
1. Gas kin C~ ( ct a l: Lipo m as, lipo ma va ria n ts, a n d
well -d lffcrcn tla tcd lipo sa rco mas (atypicall ipoma s): Result s
of MRJ evalua tio ns of 126 consecutive fatt y m asses. AJR.
IClI N ICA l l SSU ES 1H2: 7:U-9, 2004
2. Tan kcre F ct a l: Cc rcbel lopon tl ne a ngle li po m as: report o f
Presentation four cases an d review of the lit e rat u re. Ne uros urge ry.
• Most co mmo n sig ns/s ympto m s: Mild , u nil at eral S()(:I) :62 6-:1I ,2002
senso rineura l hearing loss (60 l MI) Da hl en RT ct al: CT and l\.W im agin g cha ract eristics o f
• Clin ica l p rofile: Young ad ult presen ti ng with slow ly iu trcvcstlb ula r lipoma , AJNR. 23(8):1413·7, 2002
progressive unilateral sens or ineu ral hearing loss 4. Ruggieri RM ct al : Tbera peut lc considerations in
cer ebellopontlnc an gle lipo mas in d ucin g hemlfaclal spasm .
• O ther Signs/s ym pto ms
Ncurol Sci. 2 1(5):329·3 1, 200 1
o May be fo und inci denta lly o n brain CT or MR Bigelow DC ct al: Lipomas of the internal auditory canal
5.
co mp leted for unrelated reaso ns a nd cerebcllo po n ttnc an gle. Laryng osco pe.
a Co m pressio n of cran ial ner ve R: Tinnitus (40 1}h), 108(10):1459-69, 1998
vertigo (45 1}1,) 6. Alleyne CH jr ct a l: l.tpoma tou s glio ne urocytoma of till'
o Co m pressio n of t rigem in al nerve roo t en try zo ne: posteri or fossa wit h div e rge nt dif ferentiatio n: case re port.
Trigem ina l neuralgia (15% ) Neu ros u rgery. 42(3):639·43, 1998
o Co m press io n of facia l nerve root exit lone : 7. Singh SP ct al: Li pom as of the internal auditory canal. Arch
Hemifacia l spasm , facia l ner ve weakness Pat hoi Lab Med . 120(7):681-3, 1996
8. Kato T ct al: Trige mi n al neura lgia ca used b)' a
Demographi cs ce rebclloponti n c-angle lipoma: case re port. Surg Neural.
44(1) :33-5, 1995
• Age: Rang e at presentati on : 10-40 years
9 . Truwit CL ct al: Pathogenesis of int racranial lipo ma: an MR
• Gen de r: No gender specificity study in -t 2 patients. AJIC 155(4):855-64, 1990
Natural History & Prognosis 10. Nish tza wa S ct al : Lipoma in the cc rcbcllopo n tl n c
angle-case re port. Neuro l Med Chir. 30(2):137-42, 1990
• Usua lly dot's not g row ove r tim e 11. Yos hii K ct al: Cc re bcllo po n t in e angle lipoma wit h
• Sta bility co nfirme d wit h follow- up exa mi na tio ns abnormal bony st ructu res-cease report. Ncuro l Mcd Chir.
• Attempts at co m p lete excisio n of CPA lipom as may 29(1):48-51. 1989
result in in jury to crania l nerv es 7 & H 12. Maiu ri r: ct 1:11: In tracraniallipomas. Diagn o stic a nd
• Co nse rva t ive sym pto m-based treatment yields th e rapeuti c considerations. J Neuros urg Sci. :~ 2 ( 4 ) : 1 61 ~ 7 ,
exce llen t p rogn osis 1988
13. Levin J:Vl et ill: Hemifacial spasm due to cerebellopontine
Treatm ent angle lipoma: case repor t. Neurology. 37 (2)::U 7-9, 1987
• No trea tm ent is best treatm ent 14. Dalley RW et al: Computed tomography of a
ce rebellopo n tlnc a ngle lipom a. J Ccm pu t Assist To m agr.
• Surgical rem o val is no lo nger reco m mended in most 1O(4):704.6, 19R6
cases I S. I'c nsa k Ml . cr al : Cere bellopo n ttne an gle lipo ma s, Arch
o "Cure was often wo rse tha n d isease" beca use of Otolaryn gol Head Nec k Su rg. 112(1):99-101, 1986
en twine d cran ial n erve s 7 &. 8 16. Stch nlc I ~ ct al: Lipoma in the cerelicllopontinc angle. Surg
o Histor icall y, 70% of patient s o pe rated suffered new Neurol. 240 ):73-6, 1985
postoperat ive defici ts 17. Rosen bloom SBct al: Cerebellopontinc angle lipoma. Surg
• Su rgica l in terventio n only if cran ial ner ve Neurol. 23(2): 134-8.1985
decom pression need ed 1H. Leib roc k LG et al: Cc rcbcllopon tt ne angle lipoma: a review.
Neuros u rgery. 12(6):697-9, 1 9 ~n
Co nside r
• When a high sign al lesion is see n in CPA o n TI
unenhan ced MR, 3 explanat io ns to ro nside r
o Fatty lesion
LIPOMA, CPA-lAC
IIM AGE GALLERY 1
17
(Left ) Axial T! W I MR shows

an o void high signal CPA
lip o ma (arrow). If an
enhanced T1 MR i.~ done-
without tst -s-nuretion when
lipo ma is present, it is
poss ible to mis take this
lesion for acoustic
schwannom a. (R ighi) Axial
tz: GRE MR shows the
lipoma as J dark o void m ass
(arrow). CPA- lA C lip oma
can he isolated to the CPA as
in this es se. JI is also p o.ssihle
for the lesio n to in volve both
the lA C & CPA or thL' lA C
a/om'.
Typical
(/.£}O Coro nal Tl WI M R
d em onstrate s a foea/ lipoma
in rhe fundus of the internal
auditor y canal (arrow).
There is no CPA o r in ner car
component in this ceso.
(Right) Corona l T2WI MR
sho ws the internal auditory
ca nal fund,,1lipo ma (arrow).
It is cr itical fo r Ihe radiologi.~1
to observe the black lines
along the medial an d lateral
e dge of the lip o ma to avoid
calling thi:.. lesion an acoustic
sch wannoma.
Variant
(Left ) Axial TlWI MR shows
aty p ical CPA lip oma with
CPA (arrow), post erior
petro us ap ex (op en arrow ) &
inner ear-vestibule (cu rved
arro w) components. Notice
direct connectio n of CPA.
inner ear p ortions. (Right)
Axial T2 WI MR reveals ,111 3
componen ts of a complex
CPA lipoma lh al a l.~o
involves th e p ctrous ape x
(op en arrow ). in ner ear
(curved arrow). Rlack lin('
alo ng CPA compone nt is
ch emical sh ift (arro w).
NF2, CPA-lAC
1
18
I h ial gr.lfJIJi{" sho ws hila/Nal CPA-lAC mass in Nf2. A:o.;al T1 C+ MN in p.ltienr with N F2 (t'W d /S bi/.llera/
No /ir e ti lt" IJ' h'e V('s tibufaf K hwannoma on right O n enh.lIl cing CPA-lAC scoustic scbwsnncxnes. N otice
/pit Ihl'ftl is ool h c1 facial schwannoma (a rrow) S d also ,('(/ tri~wmi/},)/ ~chwannoma (arrow ) t'x(f:'ruling
Vl'.~ Ii} Jtlf.J r 5c1JIVJ IlI )Om a (upt 'n arrow). d /on~ Vl·tix Clnll'11 ro (UtJdWll (O/X'1l arro w ) .
ITERM INO LO GY CT Findin gs

• N ECf: Bo n e images may sho w lAC flaring
Abb re viations a nd Syno nyms
• Ne u ro fibro ma tosis type 2 (NF2) MR Findin gs
• Schwa n noma tos is, ce n t ra l neurofibro matosis • T2W I
o Bila teral CPA-lAC masses wit h in h igh sig na l CSF
De finiti ons o High -reso lu tio n T2 may d ist ingu ish acousti c fro m
• N F2 = in herited synd rome wit h mu lt ip le faci al sch wa n no m a
sc hwan no ma s, m en in gio mas &. ep end ym o m as
• Tt C+
o Sm all: En han ci ng ma sses in lAC
o La rge: Enha nci ng masses in lAC &. C PA
IIM AGING FINDINGS
Imaging Reco mm endatio ns
Ge ne ra l Feat ures • NF2 screeni n g MR ima ging : 1'1 C+ MR of brain & spin e
• Hest d iagn ost ic clue : T l C+ MR: Bila teral en han cing o Screen pa tients w it h mu ltip le sch wa n no m as
CPA-l AC masses • Hig h-reso luti o n '1'2 o f CPA used to follo w CPA t um or s
• Locati o n: CPA-lAC, o th er cran ia l ner ves
• Size : Range from m illimet ers to ce n time ters
• Mor p ho logy: Ov oid wh en sma ll; "in ' crea m o n co ne" ID IFFE RENTIAL DIAGNOSIS
whe n la rge enoug h to fill lAC &: CPA
• Associ at ed im aging findin gs Sar coidosis, CPA-lAC
o CNS • M ult iple foca l e n h an cin g m en in geal ma sses
• Ca lcifica tio ns : Ch o ro id plexu s, ce rebe lla r
hem isp heres & ce rebra l co rtex Metasta se s, CPA-lAC
• Ot h er men ingio m as &. schwa n no ma (CN3 · 12) • Bilateral lAC e n ha nci ng masses
• Epend ym o m as > > glio ma s
o Spin e
• Meningio m as, sch wa n no mas & epe ndy mo mas
DDx: Bilateral CPA-lAC Masses
Sarcoid CPA 1 Sarcoid CPA 2 lAC M etosisses

NF2, CPA-lAC
Key Facts
1
Imaging Fi ndings Clinical Issu es 19
• Best diagnostic clue : T l C+ MR: Bilat eral enhancing • Resectio n \....ith heari ng preservation as possible
CPA-lAC masses • Genetic cou nseling esse n tial
• NF2 screen ing MR imagin g: T1 C+ MR of brain &
spine Diagn ost ic Checklist
• NF2 C PA-lAC masses may be acous t ic o r fac ial
Top Differ ential Diagn oses schw an nom a
• Sarcoido sis, CPA-lAC • If d iagn osis o f NF2 mad e in ad ult, co nside r
• Metastases, CPA-lAC alterna tive diagnosis o f met astases o f CPA-lAC
IPATHO LO GY I DIAGNOSTIC CHECKLIST

Ge ne ral Features Image Inte rpr et ation Pearls
• General path co mmen ts • NF2 CPA-lAC masses may be acoustic or facial
o Sepa rate d isor der from NFl schwannoma
o Bewa re! lAC tu mor s may be facial schwannom as • If diagnosis of NF2 made in adult, co ns ide r a lte rna tive
• Gene tics diagnosis of meta sta ses of CPA-lAC
o Autoso mal domina nt disorder
o Mutation NF2 gen e located on long arm of
chromosome 22 ISELECTED REFERENCES
o S()lJof, result from new dominan t gene mu tatio n 1. Moffat DA ct al: Management strategies in
• Etio logy : Mut at lon of NF2 gene (tumor su pp ressor) neuro fi bromatosi s typ e 2. Eur Arch O torh l noiuryn gol.
crea te s e n viro n men t for mult ip le tu m o r gro w th 260( I ): 12-8. 20m
• Epide m io logy: I per - 3S,()()(); I I freq uent th an NFl 2. Brackm ann DE ct al: Earl y proact ive management o f
• Associated ab no rmalities: Meni ngioma s & vest ibular schwan no mas in ncuroflbrom atosls type 2,
ependy momas Neurosurgery. 49(2):274·XO: discussio n 280-], 200 1
3. Gillesp ie JE: Imaging in neurofibro ma to sis type 2;
Gross Pathologic & Surgica l Features scree n ing using magnetic resonan ce imaging. Em No se
• Fusiform tu mo r no t primaril y involvi ng paren t nerve Throat J. 78(2) : 102-:1, 106. 10 8-9. 1999
4. Kirogl u MM et al: Bilateral aco ustic ncurofibrom utusls wit h
Microsco pic Features bilateral multicentric facial schwannomas. Eur AKh
O torhinolaryn gol. 253 (4-5):30S·X, 1996
• Schwann cells in Antoni A N R patterns
s. Akeson P et "II: Radiological In vest iga tion of
ne u ro fib ro ma tosis type 2 . Neu ro radlology, 36(2 ):10 7- 10,
19 94
IClI N ICA L ISSU ES
Prese nta tion
• Most co m mon signs/sy m pto ms
IIM AG E GALLERY
o Un ilate ral sensorine u ral hearing loss (SNIlL)
o Othe r sympto ms: Tin ni tus, vert igo, VII pa ralysis
• Stro ng fam ily histo ry of NI=2 may not be present since
5( 1)(, cases de novo
• Skin sch wan no mas ap pear as "skin tags"
De mographi cs
• Age: Mea n age at diag nosis ~ 25 yea rs
Natura l History & Progno sis
• CPA tumor growth results in profound SNHL
• Signifi ca nt morbidi ty I'< I lifespan associated with NI:2
Treatme nt (ILf l) Axial 11 C+ M R shows lAC masses. Kigh/ lAC mJSS is scoustic
• Resection with hearing preservation as po ssible schwannom.J. Lei/lAC mas..,: atyp ical shape ~ uggesls facial (.lIfOW' &.
o Progressive growth o ften makes hear ing preservation superior \'f"~ t ihul.lf nerve (open •.IffOW ) tosions. Curved .l rrow :
outcome temporary Meningioma . (Highl} Axial T2 high-resolution MR shows lim 11'it lAC
• Genetic co un seling essen tial lesions in Nfl p.JtictJl. Arltprior facidl sc/I\Yimnom.l (arr o w) is
separatC'd from posterior superior vestib ular scbwennoms (op en
arrow) by CSf clrit,
RAMSAY HUNT SYNDROME
20
CliniC<ll phologriJph in d pdtK.'f11 with .K tA't' RI IS tt"\'l ·.lf~ Ih i..ll TI C+ AIR. in th~ p.uiffit with RJ/5 &. dctiK'
distinctive hemorrhagic VC'SiclIlar r<l.~h of extern." m f vtOSidt.'5 on rig ht l OXtf'ffl..l1 fW JKJ',\'S l'fIhJ rJ('('f'f'K'lll in lh<>
(<1fTo.'o') Ih,lt i.~ .'if>f>n with acute on,'>4:'1 7th •.;. 11th CfJlli.11 ri~hl lAC (,lff(J',V) and J('liw in flJmnMIJon of the right
neurup"t1w· t'xtt'ffl. ,1t'.lf (orX'fI arro w s).
• w hen m inimally invo lved , linear or no

ITERM INO LOGY en ha n ceme n t see n
Abbreviatio ns a nd Synonyms • Mo rp ho logy: Linear o r fusiform lAC en ha nc eme n t is
• Abbrev iation: Ramsay Hun t synd rome (RHS) ru le
• Syno ny m : Herpes zoster otic us CT Findings
Definitions • NF.CT: Negative for hon e cha nges or o ther findi ng \
• Varicella zos ter virus in fectio n involving sen so ry fibe rs • CECT: Negative for co n trast-en ha nce men t in lAC
of cra n ial n erves 7, 8 &. po rt ion of exte rn al car MR Find ings
su pplied by auricu lotempo ral nerve • '1'1WI: Line r int er med ia te signa l see n in lAC fundus
(represen ts in flamed cranial nerves)
• T2WI
IIM AGIN G FINDINGS o Th ick-secti o n T2 (2:. 4 mm ) usually normal
Gen e ral Feat u res o High -resolut ion '1'2
• Fundal, 7t h &. 8t h cra nia l n erves th ickened
• Rest diagnost ic cl ue: Pathol ogic en hanceme n t o n TI
• STIR: High signal in soft tissues o f extern a l ea r
c+ MR of cran ial nerves 7 ± 8 in lAC fundus a long
• FLAIR
with a ll o r pa rt of membrano us labyrinth
o Paren ch ym al brain n ormal
• l.ocati on o l ligh signa l in soft ti ssues of ex tern al ear
o Sth cra n ial n erve affected in fundal Ir\C
a 7th cran ial n erve affected in fundal lAC &. with in • T I C+
o Extern al ca r
temporal bo ne
• Fat-satu rated TI C+ im ages m ay show
a Membranous lab yrinth also affected
en h ance men t o f exte rna l ca r vesicles &: assoc iated
• Size infl ammat ion
o Onl y size variatio n relates to degree of cra n ial nerve
o In ter nal audit or y ca na l
invol vem ent in lAC
• Linear to fusiform en ha nce men t in lAC fundus
• \Vh en th is area signi fican tly involved , ma y mim ic
(7th f< Hth cra ni al nerves co nt ribute)
mass lesion .
DDx: Linea r CPA- lAC Meningeal Enhancem ent

~~ 'M "J- '
, ~
~ .\ --
. . .....
" ...~. ...'
\'
•
8e" Palsy Sarcoid, CPA Met.15t<lSis, lAC
Key Facts
1
Te rminology • Sarcoidosis 21
• Synony m: He rpes zoste r o ticus • Me ni ngeal m eta stasis
• Varicella zos ter virus in fecti on in volvin g sensory Path ology
fibers o f cra nia l nerves 7, 8 & port ion o f exte rn al ea r • Infla m mat o ry infi ltra tes of lymphocytes & plasm a
supplied by a uricu lote m po ral n erve ce lls
Imaging Find ings • Foun d in ge nicu late gang lio n, 7t h & 8th cra n ial
• Best diagnostic clue: Pat ho logic en hance men t all T I n erves an d mem bra nous labyrin t h
C+ MR of cra nia l n erv es 7 ± 8 in lAC fundus along Clinica l Issu es
with all o r par t o f memb ranous lab yrinth • Mo st co m mon signs/sympto ms: Facial pa lsy
• If ex terna l ear vesicu lar rash is clinica lly appare n t, no associa ted wit h ex te rna l ea r ves icles
imaging is necessar y to in vestigate associa ted 7t h &
8th n erve palsy Diagn ostic Checklist
• Co m bi na tion of linear en ha nceme n t of fu nda l lAC,
Top Differe ntia l Diagn oses mem b ra no us labyrinth & in t ra tem po ral facial nerve
• Bell palsy sugges t lUIS
• Menin git is
• lAC en ha nce me n t n ot alwa ys present even wit h • Includ e axial &. corona l '1' 1 C+ fat-saturat ed
sensorine u ral hear in g loss ± vert igo th in-secti on (3 m m ) seq ue nces th rough lAC &
• En ha nceme n t of facial ner ve wit h in tem poral tempo ral bo ne
bone (laby rin t h ine. tympani c, masto id segme n ts
all possible)
• En ha n cem ent of part or all o f mem branou s IDIFFERENTIAL DIAGNOSIS
labyrint h (coch lear porti on e n ha nces mo st
co m mon ly) Bell palsy
o lnt ra te m por al facial n erve • En h a nceme n t o f 7th cra n ial nerve hut n ot
• En tirc lntrat cmpor al 7t h cra nia l n erve membranou s lab yrinth o r lith cra n ial n erve
e n ha nceme n t typi cal • Fundal 7th cra n ial ner ve en ha nci ng "t uft"
• Laby rl nthin c segme n t invol vement di stinctiv e • lAC en ha nce ment usually less int en se t han Hi lS
• Gen icu late ga ng lio n ofte n also en h ances
• Rest o f in tra tem poral 7t h c ran ial n erve less
Menin gitis
freq ue nt ly e n hances • Th icken ed, diffu sely e n h a nci ng me ni nges
o Membran ous labyr in th • CSF analysis may be reveali ng
• Pathologic en h anceme n t often acco m pa n ies lAC Sa rco idos is
&. facia l n erve e n h anceme n t • Multifocal meningeal e n ha nc ing foci
• Flu id spaces o f coc h lea, vestibu le & semic ircu lar • Spa res in ner ea r & fu nd us o f lAC
canals may all be variably affect ed • Increased eryth rocyte sed ime n tation rate (ESR) &.
• Me m branou s lab yrint h en ha nceme n t may not be seru m a ng iotensin co nve rti ng enzyme (ACE)
p resen t even whe n hea rin g loss &. vertigo presen t
o Brain stcm Me ningea l metastasis
• Facial n ucleus in b rains te m e n ha nces in frequently • Mul t ifocal meni ngeal e n ha nci ng foci
in RIIS • Inuapa ren ch ymal lesio ns may also be presen t
• Pro babl y secondary to sp read from lAC disease
Imaging Re commendati ons
• Best im agin g tool
IPATHOLOGY
o Wh ole b rain '1'2 MR with e n ha nced seq ue nces Ge nera l Features
focused o n C PA-lAC & tem poral bo ne • Ge ne ral path co m me n ts
o Findings best see n o n fat -satura ted '1'1 C+ MR im ages o Varice lla zoste r virus can be cultu red from vesicles or
o No role for cr in d iagn osing RHS from saliva
• Protoco l adv ice o Increased vascu lar pe rmea bility allo ws co nt rast to
o If exte rna l ca r vesicular rash is clinically a ppa ren t, pass th rou gh blood -n erve ba rrier
no imagin g is necessary to in vestiga te associat ed 7t h • Etiol ogy
& 8t h nerv e palsy o Classic h ypo th esis: Virus remains do rman t wit hin
o If clinica l prese n ta t ion is at ypical , MR imaging geni culate ga nglio n wit h pe riod ic reactivat io n
o rde red o Recen t h ypothesis: Prim ar y po lyn eu ritis wit h
• In clude whole brain FLAIR seq ue nce to exclude in fecti on of 7th & 8t h cra n ial nerv e trun ks wit h
int ra-axi al ca use of cra n ial n eu rop ath y spread via in tern eural co n ncc u ous
1 Gross Patho logic & Surgical Features Image Inte rpretation Pea rls
• Edem atou s. hyperemi c 7t h &. St h cran ial nerves seen • C o m b in a t io n o f linear e n h a n cem en t o f fun d a l lAC ,
22
in fu nd us of lAC m e mb ranous la b yrint h & in t ra t e m po ral facia l n er v e
suggest RHS
Microscopic Feat ures
• Inflammator y infil t rat es of lym phocytes & plasma
ce lls
• Found in gen icu late ganglion , 7th &. 8th cran ial nerves
I- Hu S c t al: Acyclovir respo nsive b rai n ste m disease after
and mem bran ou s labyrin th
Ramsay Hunt sy nd ro me. J Neu ro l Sci. 2 17:111-3, 2004
• Similar sym ptoms &; MR findings have been described 2. Lu YC et al: Vertigo for m h erpes zoste r o ticus: supe rior or
wit h hu m an herpes virus 1 (HHV- l ) in fecti on in ferior ves tibu lar ne rve o rigin? Laryn gosco pe .
I n(2):30 7- 11, 200 3
3. Grose C e t al: Ch icken pox and th e gen icu late ga ng lion :
!C Ll N ICA L ISSUES facial n erve pa lsy, Ramsay Hun t syn d rome and acyclovir
trea tmen t. Pedi a tr Infect Dis ]. 2 1(7):6 15-7, 2002
Prese ntation 4. Ku h welde R ct al: Ramsay Hun t synd rome:
• Most co m mon signs/sym ptom s: Facial palsy associated patho p hysiology o f coc hleovesti bular sym pto ms. J
Laryn go l O tol. 116(10):844-8, 2002
wit h exte rnal ear vesicles
5. Suzu ki F e t al: Herpes virus rcactl vatlon and
• Oth er sign s/sym ptom s gadoli n iu m-en ha nced magnet ic reso nance im agin g in
o Deep , hurn ing pain in ea r patie n ts with facial palsy. O w l Neu ro tol . 22( 4 ):549-53,
o Facial pa ralysis mo re severe than wit h Bell palsy 200 1
o Painful eryt he ma tous vesicular rash of extern al ca r 6. Sween ey CJ et al: Ramsay Ilu n t syn drome. J Neuro l
o Sensorine ural hearing loss (SNI-IL), tinn itu s & Neurosurg Psych iatry. 71(2): H 9·S-l, 2001
vertigo du e to Hth cranial nerve invol vem en t 7. Lavl ES lot a l: En ha nce me n t o f th e eigh th cran ial nerve an d
• Interneu ral co n nectio ns from 7t h cra n ial n e rve lahyrint h o n ~ R imaging in sud den sensori ne ural h earing
loss associa ted wit h h um an Herpesvirus 1 in fection : Case
o Invo lv e m ent o f o t her cra n ia l n e rv es, es peci a lly 5t h
report. AJNR. 22:1380·2 , 200 1
crania l nerve po ssib le (oph t h a lm ic d tv iston )
8. Sa rto rettl-Sch efer S e t al: Ram say Hunt synd rome
o Ve rti go develops a fte r o n se t o f pa in a n d e it her associa ted with brain stem en hance me n t. A]NR.
be fore o r af ter ve sicu la r e ru p t io n 20 (2):27H-HO, 1999
o N a usea & v o m it ing po ssib le 9. Stein er I ct al: Bell's palsy and herpes viruses: to {acyclojvi r
or n ot to (acyclo)v ir? J Neu ra l Sci. 15; 170(1):19-23, 1999
Nat ural Histo ry & Prog nosis 10_ Ber ret ti n l S et nl: He rpes zos te r oticus: cor rela tio ns be twee n
• Ear p a in fo llowed in - 7 d a ys b y e ry t h e m at o u s cli nical and MRI find ings. Eur Nc u rol . 39 (1):26-3 1, 1998
ve sic u lar ras h o f ex te rna l ea r 11. Brand le P ct a l: Co rrelatio n o f Mill, clin ica l, and
• C ra n ia l n e u ropat hi e s a ppea r after onse t o f ea r pa in clect ron curo nogra phic fin di ngs in acut e facial ne rve pal sy.
o Ap pe a r bef ore or a fter v esic u la r eru p tio n Am J Otol. 17(1):154-61 , 1996
12. Kuo MJ et al: Early d iagn osis an d treatment o f Ram say
o Wile n b e fore , imag ing m a y be d on e lo o kin g fo r
Ilun t syn d rom e: th e role o f magn et ic reso nan ce im agin g. J
e t io lo gy o f 7t h cra n ia l n er v e pal sy Laryngol O tol. 109(8):777-80, 1995
• M u ltip le cra n ia l n erv e pa lsies & o ld er ag e bo th a rc 13 . Jo nsso n L e t al : Cd -DlY rA e nh anced M RI in Hel l's palsy and
n eg a t ive pro gn o stic ind ica t ors he rpes zos te r ot icus: an overview and lm pll ca tions for
• Facia l pal sy n atura l h ist o ry fu ture studies. Acta Otol ary ng ol. 115(5):5 77-84, 1995
o Ma jor da m a ge to 7t h c ra n ia l n e rv e n ot until 2-3 l -t. Adou r KK: Oto logical co m plications of h erpes zoste r. Ann
weeks afte r o n se t Neuro l. :{S Supp l:S62·4, IlJ94
o C o m pa re to Bell Pa lsy w here d a m age pea ks a t 10 IS. Tada Y et al: Gd· DTPA en hanced MRI in Ramsa y Hu nt
syndrome. Acta Oto lary ngoL Sup pI5 11: t7 0-4, 1994
days 16. Snrtore tti-Sche fer S et ill: Id iopat hic, herp eti c, a nd
Treatment Hlv -assoclated facial n erve palsies: abn o rm al ~m
en h ancement patterns. Aj='JR. IS(3 ):479-HS, 1994
• C o n se rva ti ve m a n agem ent firs t 17. Down ie AC ct ill: Case repor t: prolonged co n t rast
o Wa r m com p resses en ha ncem en t of th e in ner car o n mag n et ic reso nance
a Ana lgesic s imaging in Ram say I lun t synd rome. Br J Rad lol.
o Co rn ea ca re for faci a l paralysis 67(HOO):8 19-2 1, 1994
• Ph a rm acol o g ic t re a tm e nt 18. Korzcc K et ul: Gadoli nium-e n ha nced magn et ic resonance
a C ort lco st ero ld s imagin g of the facial n erve in herpes zoster otlcus and
o Acyclovir re d uces pai n &. is h elpful in im p ro v in g Bell's palsy: cllmcal im plicatio ns. Am J Oto!' 12(3 ):163·8,
fac ia l fu ncti o n 199 3
19. Yanagida M et al: En han ced MRI in pati en ts wit h
Ramsay-I h ull 's syn d rome . Acta Oto laryn gol. Suppl
500:58·6 1, 1993
ID IAGNO STIC CH ECKLIST 20. Rovira Can ellas A et al: Ramsay-Hunt syn d rome and
h igh -reso lut io n 3 f)J·"!,MRI. J Co m pu t Assist Tom og r.
Consider 17(3):495-7, 199:1
• MR im ag in g sho u ld o n ly be d one w he n cl in ica l 2 1- Osumi Act al: MR fin d ings in a patien t wit h Ramsay-Hunt
p re se nt a t ion is a ty p ica l syn dro me . ] Co mp ut Assist Tom ogr. 1 4 ( 6) : 9 9 1 ':~ , 1990
• If im ag in g su gg ests RHS, co n tact re fe rr in g cli ni c ia n for
h ist o ry of ex te rna l e a r ve sicu la r ra sh
IIMAGE GAL LERY 1
23
(1.R11) Coronal f l WI.\IN.

sholVs typical /\4R
appeara llc e of 1~ IfS ,15 J low
.~ jgn.l / .lf(',1 in righ t inteffJ.11
auditory canal (arrow). Ib is
"fill ing detect " l~ secondary
to e d{'IJJ.Jtou." 7 th and 8th
cranial nerve.... (RighI)
Coronet T 1 C+ AIR in palient
with RJIS reveet« Iypica l ,\ I R
appearan ce .h mixture of
linear (x /Joelul.1f
enhancem('nt in 1,1((-'(.11
inlt'rnaf auditory cana l
( d ffOW) 0 11 right.
Va riant
(1.£! I ) Axi.1I " C+ MR
m ,,~nifir 'd to righ t ( ',If fl'w"l...
typical M I< OlPP(',lr.lIlcC' of
Rt lS a .~ linear t'n hallcemeflf
wilh/Il II\C (d{{( WV)
iJccomp anipd hy more
unusual enhatKf:'nll 'nl oi
coc hlear labyrinth (opm
,mow). (R igJd) Corollal I 1
C+ MR ma~f1j(i('d /0 right f'd f
_..h ows entumcemcru in lAC
(arro w) and more lat f:'rafly
en!JJnn'menl of inne I:'dr
(on on arrow' . This
combination of lAC ,\: inner
car entuncenwnt is a well
know variant MR
app ('.Jrance jn RJIS.
Va riant
(I.efO A\ial TI C+ M R
demollSlra tes fo cal. nodular
('nhancemenl ill the fUlldus
o f thL~ lA C on /h(' It41
(arrow). For flll Jiltpfy, thi s
tumeioctive ap pearance is
rare as an M R m.mitcs ta/i()/}
of RIIS. (Rig ht) Coronet T1
C+ M R in pmicnt w ith
extern al es t vesicles an d
acu te onse t o f 7th .'\ 8 th
('«m ial f)eum[J<llhiL'S (Rf IS)
shows solid en bsncemcnt of
lA C fun dus (arro\\') in
asso ciation lVith inner Cdr
C'nha nCflmf'n l (ope n arrow) .
SARCOIDOSIS, CPA-lAC
1
24
A\i.11 T2WIM Rshows the norma! high sign.J1C5f ;0 th..• Axial TI C + lIAR s.1rcoidos is P.ltient IDO\VS m eni ngm l
lAC 11.15 bt-en (('p lan>d by bv signal t;S5(J(' (am Av) . thickening (CUM Y/ ,JffOl,vs), pt-'fint>ura/ tim lf' (arra.v) &
5J.rcoidr,.;is Well. diagnosed but intrJc<1Il.l/icul.lf positiw l'illiJ / codve s (C'.I(JffI .l1TU'tv }. Tymp.:mic CN7
mminnioml WeB <J/so d possibility. mhafJct'5 pathologically
o En plaque, nod ula r or lin ear me ningeal

ITE RM INO LO GY en ha nce me nt
Abbre viatio ns and Syno nyms o Spreads via Virch ow- Robin spaces into bra in
• Neu rosa rco ldos ts o Cra nia l n erve en ha nce me n t
Definitions Imaging Recommendations

• Systemic d isord er wit h noncascating gra n ulomas of • Mit with n AIR & T I C+ ima ges m odality o f cho ice
multiple o rga n syste ms • '1' 1 C+ MR thi n-sect ion im ages throu gh CPA· IAC
included
IIM AGING FIND INGS

IDIFFERENTIAL DIAGNOSIS
Ge ne ral Features
• Best diagnosti c cl ue: Multifocal enha ncing meni ngeal
Infecti ous meningitis
masses • T I C+ MR may m imic sa rco idosi s espec ially in fu ngal
o r tu be rcu lou s meningitis
• Location
o Meningea l invol vement (15% of all patie nt s) • Clin ical: Disease course more fulminant
o Ot he r in tracran ia l local ion s Invasive idiopathi c pseud o turn or
• Cra n ial nerves, pituitary gland, 3ed ventricle &: • Usua lly unifocal en ha ncing m eni ngeal mass
hypothal am us • Unde rlying bone may show erosions
• Mo rph ology: En plaque or nod u la r meningeal foci • Very rare di sease
CT Findings Meningiomatosis
• C ECf: Foca l en ha ncing meningea l ma sses • Mult ifocal e n ha nci ng ma sses
MR Find ings • Hyperostosis of underlying bone possible
• T2W l: Ilypo in tcn se m eningeal foci • Clin ical: Absen t systemic manifestations o f sarcoidosis
• T l C+ . Men ingeal metastases
• Nod ula r men ing eal meta stases < diffuse
DDx: Focal Meningeal Thickening CPA- lAC Area

rr- ,..,-,""r:-r.....
M eningitis Mening iomtl l ()si.~ Pseucknutn or Me tastasis, CPA

SARCOIDOSIS, CPA-lAC
Key Facts
Term inology Top Differential Diagn oses
• Systemic disord er with non caseatin g granulomas o f • Infectious meningitis
mul tip le organ syste ms • In vasive idiopath ic pseud otumor
• Meningiomatosis
Imaging Findings • Meningeal metastases
• T2WI: Hypoint ense men ingeal foci
• En plaq ue, nodul ar or linear meningeal en hancemen t Diagnostic Checklist
• When iden tify m ultiple "meningio mas" in patien t
with systemic disease, thi n k sarcoidos is
• When nodular, MR appearance similar to sarcoidosis

• CSF cellular ana lysis usually provtd es diagnosis Treatme nt
• Clinical: Prim ary neopl asm known • Prom pt ad ministration of steroids
• Othe r steroid spa ring medication s
a Azath iopr ine, cyclospo rin, chloroq uine
IPATH O LOGY • Low do se radiation t herapy if steroid refractory
Gen era l Features

• General path co mmen ts IDI AGN O STIC CHECK LIST
o Noncaseating gran ulomas are charac ter istic
o Diagnosis ofte n mad e after blops y o f skin lesion s Image -Interpretation Pear ls
o In ne urosarcoid osis 2 pattern s seen • When identify mult ip le "meningiomas" in pati ent
• Gran ulom at ou s men ingit is with systemic disease, th in k sarco idosis
• Coa lescen t nod ules form brain ma sses
• Etio logy: Path oph ysiology un known
• Epidemiology: Intracran ial sarco idosis evide n t in 5% ISEl ECT ED REFER ENCES
• Associated ab norma lities: Sinonasal disease (- 20')fJ) 1. Krcuzberg B ct al: Th e co nt rib utio n of MRI to th e dia gn osis
o f diffu se rnenlngcal lesio ns. Neu ro radlol ogy.
Gro ss Pathologic & Surgica l Features 46(3): 19&-204 _2004
• Meni nges appear inflamed « thickened 2, Kidd D et al: The neurological co mplicatio ns o f syste m ic
sa rcoidosi s. Sarcoid osis Vase Diffuse lung Dis. 20(2) :85-94,
Micro scopi c Features 2003
• Noncaseating granu lomas 3, Ch rlstofon dis GA ct al: MR of CNS sarcoido sis: correlati on
• Lange rha ns gian t cells mi xed with in epithelioid cells o f imag ing features to clinica l symptoms and response 10
o Thin rim o f lymph ocyt es rings indivi dua l tu bercles treatm en t. AJ~R Am J Neu rorudiol . 20{.J);6SS·69 , 1999
4, l.exa FJ et al: MR of sarcoidosis in th e h ead and sp ine:
• Increased h yalini zatio n wit h even tual fibrosis
spec trum o f manifestation s an d radiograph ic respo nse to
• Often mi x o f acu te, subacu te & chro nic lesion s stero id th erapy. AJNR Am J Neu rorad lol. 15(5):9 73-82,
1994
ICLlN ICA L ISSUES

Prese nta tio n IIMAG E GALLERY
• Most com mon signs/symptoms
I :".
f
o Systemic sarcoidos is: Pulmon ary sympto ms .::f! ~
'J' , ~~'"",. A· ~ ' "

o CNS sarcoidosis: Visual loss & d iabetes insipidu s :t ..... - -;J ,;
o CPA-lAC: Unilateral SNIIL ± facial neu ropath y

• Clinical profile: Adult with visual loss, cen tral diabet es
Insipidus & unilateral SNHL
..
.. . .-'
I : -7" ~
) (""
~- '\:.
",:""
.
J\I~ , .
.j
"
• Laborator y find ings arc co nfirma tory
o CSF: lIigh protein & leuk ocyte coun t; lymphocytosis
o Increased CSF angiotensin co nverting enzyme (ACE) , "
\~ .!:- ' : i
i:I
I '~ <,
Dem ograp hics
• Age: Most co mmon ly seen in 20- ·W year a ids
\ , .:-- : /
/
• Et h nicity: Afrlcan-Amertcans > > ot he r et h nicities
(iLJI) A xial T1 C+ AIR revee ts meningeal sarcoidosis (arrow ). Unear
Nat ural History & Pro gnosis a rea s of c>nhancf:'ment represent channels of brain invasion (op fon
• 2/3 have self-limited mon ophasic illness arrows). Invol vement of medial lAC (curved arro w ) also evident.
o Remai nde r have relapsing or ch ronic course (Right) Axial T2 WI M R shows characteristic low signal mL'n ingeal
depo sits of sarcoidosis (arro ws)_ The posteri or lesion has invo lved
• > 50% recover without significan t morbi d ity
adj acent brachium ponlis (open arrow) dml con tiguous cereboltsr
• Progn osis worse if brain ± spinal lesions present hemisph£>re.
ACOUSTIC SCHWAN NOMA
1
26
Axial graphic shows SfTldlJ intracanalicular scosuc Ava! T2WI MR sh()'l'VS 5 mm intracanalicu lar ocouek:
schwannoma (op< 'fl <1rrow) from superior \~tibul.1f .\t h W<I/l flOm a (open .lfrO\ lI ) . Cnchlmr apt>rl ulP is sparp<1
norvo. No (in' «J(h!P,1f ilp('rtuft' i~ UnitlVOfvlV./ (.l lfo. V' . (Jrro..",J. A -I mm -fundal CiJp · « csr is pre.wnt.
• Scree ni ng MR done ea rlie r

ITE RM IN O LO GY o Larger lesio ns: Up to 5 em in ma ximum d iamete r
Ab breviatio ns a nd Syno nyms • Morpho logy
o Whe n sma ll &. ln traca nallcula r, loo ks like ovo id to
• Co m mo n synony ms : Aco ustic schwan noma (AS),
aco ustic neu roma, aco usti c tumo r, vesti bula r cylind rical mass
o Large r lesio n s look like "icc crea m (C PA) o n co ne
schwan no ma
(lAC)"
• Unco m mon : Neu rinoma , neurilemm oma
o Seldo m herniate cepha lad Into m idd le crania l fossa
• Vesti bu lar sch wan noma is m ost an-urate te rm
o Since most lesions arise o n th e vesti bula r portio n o f (cf CPA me n ingio ma )
Hth cra n ial ne rve (CN) CT Findings
Definitio ns • Cl.Cl'
• Acou stic sch wan no ma = ben ign tu mor arising from o \Vell-de lineated , e n ha ncing mass o f C PA~ I AC ciste rn
o Ca lcificat ion no t present (c f CPA me n ingioma )
Sc hwann cells th a t wra p ves tlbu lococh lear ne rve in
o May fla re lAC when larger
C PA-lAC
o Sma ller tn t racana licu lar lesio ns « 6 nun ) may be
mi ssed wit h CEC r
IIMAGING FINDINGS MR Find ings
Gene ra l Features • TlWI
o Int er m edi a te signa l most comm on
• Best d iagn osti c clue: Avidly en ha nci ng cy lind rica l
o High signal foc i if rar e he mo rrh agic lesion pres en t
(lAC) or "ice cre am o n co ne" (C PA-lAC) m ass
(0 .5% )
• Locati o n
o Small lesions: In tracan alicu la r • T2WI
o lli gh -reso luti o n 1'2 MR: "Fillin g defect" in h igh
o Large lesio n s: In traca nalicula r wit h C PA cistern
signa' CSF o f CPA-lAC ciste rn
exte nsion • Sm a ll lesio n: Ovoid filling defect in h igh signa l
• Size CSI' of lAC
o Sma ll lesions: 2· 10 m rn
DD x: CPA Ma ss
A rachnoid Cyst Epidermoid Cyst Meningioma. CPA CN ? Schwe nnome

ACOUSTIC SCHWANNOMA
Key Facts
1
Termino logy • Meningioma 27
• Acoustic schwanno ma = benig n tumor a rising from • Facial nerve sch wa n no ma
Schwann cells th at wrap vestibulocochlea r nerve in Pathology
CPA-lAC • Most co m mon CPA-lAC ma ss (85-90%)
Imaging Find ings • Second most common extra-axia l neo plasm in ad ults
• BeS! diagn osti c cl ue : AVidly en hancing cy lind rical Clinical Issu es
(lAC) or "ice crea m o n cone" (C PA-lAC) mas s
• Most co m m on signs/s ympt oms: Adults with
• High -reso luti on T2 MR: "Fillin g defect" in h igh signa l unilat eral SNIIL
CSF o f C PA-lAC ciste rn
• Focal, en han ci ng mass o f C PA-lAC cistern centered Diagn ostic Checklist
on porus acusticus • Un ila te ra l well -circumscribed lAC or CPA-lAC m ass
• Best imagi ng tool : Go ld sta ndard is full brain T2 MR sh o u ld be co ns idered AS until proven ot he rwise
with a xia l I< co ro na l T1 C+ MR imaging o f C PA-lAC • Com me n t o n AS involvement o f coc h lea r a pe rture
a nd/o r lAC fundus in radi olog ic rep ort
Top Differen tial Diagnoses
• Epidermo id cyst
• Large lesion : "lee cream o n cone sha ped fillin g • l.ook for labyrinth ine segment "ta il" to differenti ate
defect in CPA-lAC
• T l C+
Metastasis & lymphom a
o Foca l, en ha nc ing mass o f C PA-lAC ciste rn ce n te red • May be hil ateral ; m u lli foca l menl ngea t invo lvem ent
on porus acus u cus • Beware o f "NF2" di agn osis in adu lt
o 100% en hance strongly Aneurysm
o 151)06 wit h intramural cys ts (low signa l fnci)
• Ovoid to fus ifo rm com plex sign a l mass at C PA
• Ot her MR find in gs
o O.SIMI associated arach no id cyst
o Dura l "ta ils" a rc rar ely presen t (cf meningioma)
!PATHO LO GY
Imaging Re commen dation s
• Rest ima ging too l: Go ld sta nda rd is full brain '1'2 MR
Gen eral Features
with ax ial I< co ro n al T1 C+ MR im aging of CPA-lAC • General pat h com me nts: Vestibula r d ivision o f CN8
• Pro toc o l advi ce far m ore com m on with AS than coc h lea r n erv e
o High -resolu tio n T2 MR im aging o f CPA-lAC is o n ly • Geneti cs
scree n ing exam for AS o Inacti vating mutati on s of NF2 tum or suppresso r
• Used for un co m plica ted un ila teral sen so rine ural ge ne in 6Q<){. of spo rad ic AS
h ea ring loss (SNI1 L) in adult o Lo ss of ch ro m osome 22q also seen
o Multiple or bilat eral sch wa n no mas = NF2
• Etiology: Benign tumor a rising fro m vesti bular porti on
IDIFFERENTIAL DIAGNOSIS o f CN8 at glial-Schwann cell junction
• Epid emio logy
Epidermoi d cyst o Most co m mon lesion in pati ents with unilateral
• May mimic ra re cystic AS SNHL (> 90%)
• Insinuating morp hology o Most co m mo n CPA-lAC m ass (85 -9(1*,)
• 1'1 C+ MR: No ne n ha nci ng CPA mass o Second most co m mon extra-a xial neoplasm in
• Fl.AIR: Pa rtial o r abse n t a tte n uation adults
• DWI: Diffusion restricti on (h igh signal) • Associa ted abnormaliti es: Arachn oid cyst (D.ScX)
Arac hno id cyst Gross Pathologic & Surgical Features

• Pushin g CPA lesion th at doe s not en te r lAC • Tan, round-ovoid , encapsulated mass
• Foll ow s CSF sig na l on all MR seq uences • Arises ecce n t rically from eNS at glial-Schwann cell
• 1'1 C+ shows n o e n han cem ent ju ncti on
o Glial-Schwann cell junction m ost co m mo n ly near
Me ningioma porus acu sticus
• In tracan alicula r men ing ioma ma y mim ic AS (ra re)
• CECf: Ca lcified dural -based mass eccen tric to porus Microscopic Features
ac usti cu s • Differentiated n eoplast ic Schwann cells in a
• T1 C+ MR: Broad dura l-ba se wit h associated du ral co llage no us m at rix
"ta ils" • Areas of co m pac t, elo ngat ed ce lls = Ant oni A
o Most AS co m pr ised m ostl y of An to n i A cells
Facial nerve sc hwa nnoma • Areas less de nsely cellula r wit h tu mor loose ly
• When confi ne d to CPA-lAC, m ay exactly mi m ic AS a rranged, +/- clu sters of lipid -lade n cells = An to n i B
ACOUSTIC SCHWAN NOMA
1 • Stro ng, diffuse expr ession of 5-100 protein
Image Interpre tati on Pearl s
• No necrosis but may have intram ural cysts; rarely
28 hem orrhagic • Un ilate ral well-circu mscribed lAC o r C PA· IAC mass
should be co nside red AS u nt il proven o the rwise
Stag ing, Grading or Classifica tion Crite ria • Co mmen t on AS invo lvement o f coc h lea r ap erture
• WHO grad e I lesion and/or lAC fundu s in rad iologic report
• Always make sure th ere is n o "labyrin t h ine tail " o n all
AS to avoid mi sdiagn osing a facial nerve schwa n noma
IC LI N ICA L ISSU ES
Prese nt ati on
• Most co mmo n signs /sy m pto ms: Adult s with unilateral
ISELECTED REFER EN C ES
I. Darrou zet V et al : Vesti bul ar sch wan uoma ~ urge ry
SNIl l. o utco mes : o u r multidi sciplina ry ex perience in 4lX> cases
• Clin ical profile o ver 17 yea rs. Laryngoscope. 114(4):681 -8, 2004
o Patient com plains of slowly prog ressive SNHL 2. Yates I)D et al: Is it wo rth while to attem pt hear ing
o Labor ato ry p reservation in larger aco ustic neur omas? O tol Neu ro tol .
• Ilrainstem electric response audi ometry (HERA) 24 (3 ):460-4, 2003
most sen sitiv e pre-im agin g test for AS 3. Rupa V et al: Cost-effec.tl ve in itia l screen i ng fo r vestibular
• HERA may be u n n ec essa ry if ea rly, scree ni n g Mit is scbwa n noma: aud itory bra lnst ern response or m agn etic
resona nce im ag ing? O to laryngol I lead Nec k Surg.
em ployed t 28(6):823-8, 2003
• Oth er sympto ms 4. Kobayash i M ct al: Dista nce from acoustic neu ro ma to
o Small AS: Tin n it us (ringing in ear); disequ ilibriu m fundus and a pcstc perut lve facial palsy. La ryn gosco pe .
o Large AS: Trigemi nal and/o r facia l neu ropath y 112 (1): 168-71, 2002
Spickler E~ I ct al: The vesttbulococ hlear nerve. Scml n
De mogra phi cs Ult rasound c r MR. 23 (3):2 18-37, 2002
• Age 6. Nakashima K et 31: Thr ee-di men sional fa..t recovery fast
o Adults (rare in ch ild ren u n less NF2) spin -echo imag in g of the inner ear and the
o Peak age = 40-60 years vestibulococ hlcar nerv e. Eur Radiol . 12(11):2776-S0, 2002
o Age range = 30-70 yea rs 7. Na tik SL ('I al: Determ inants o f tu mor size an d growt h in
• Ge n der: No gen de r specificity vestibular sch wa n nomas. J Neurusu rg. 94:922-6, 200 1
H. Komatsuznkl A: Ne rve origin o f t he acoust ic neu ro m a . J
Natural History & Progn osis Laryng ol O to !. 115 :3 76-9, 200 1
9. Somers T ct al: Prognostic va lue o f magn eti c rCMm\.l'KC
• 7S l}h o f AS arc slo w growing; will grow a t a gradua l
ima ging fin d in gs in hear ing p reservati on ~ u rgl'TY for
pace if left untrea ted vestibu lar sch wan noma . Owl Nc u ro tol. 22 :8 7-94, ZOOI
• 10% of ASgrow rapidly (;:, 1 ern per year) 10. Salzm an KL et al: Dumbbell ..ch wan nomas o f th e int erna l
• 15% o f AS grow ver y slowly and can he left alone in au d itor y canal. AJNR Am J Ncu ror adiol . 22(7) :1368·76,
o lder pati ents 2001
• If hearin g is absen t o r very poor, successful surg ical II , Ho istaJ DL et al: Upd ate o n co nser vative managem en t o f
rem oval o f AS will not rest ore any hearing already lost acoustic neu ro m a. Oto l Neu rotol . 22(S) :6K2-5, 200 1
• Negative progn ost ic imaging findings for hearing 12. Sclesn ick SH et a l: In ter na l aud itor y ca na l in vo lvement o f
acoustic n euromas: su rgical co rrelates to magn et ic
preservati on resonance imagi ng find ing s. Ot ol Neurot ol. 22( 6):9 12-6,
o Size > 2 em 200 l
o I\S invo lves lAC fundus and /o r coc h lear a pert ure 13. Zealley fA c t: MRI screen ing for acou stic neuro ma: a
co mparison o f fast sp in ec ho and co n tras t e n hanced
Treatment imaging in 1233 pa tien ts. IIr J Radiol. 73:242·7, 2000
• Tran slabyrinth ine resecti on if n o hearing preservation 14. Gillespie JE: ~I RI scree ni n g fo r aco ustic neu roma. Br J
possible Radio\. 7:1: 1129 -30 , 20m
• Middl e cran ial fossa approach for intracanalicul ar AS, I S. O'Reilly B et al: The co nserva tive managemen t o f acoust ic
especially lateral lAC location neuroma : a review o f forty-four pa t ien ts wit h magnet ic
• Retrosigm oid a pp roach when C PA or medi al lAC resonance ima ging. Clin Otola ryngol . 25:9 3-7, 2000
component present 16 . Nakam u ra II e t al: Serial foll o w-u p l\.IR imaging afte r
gam ma kn ife rad iosur gery for vest ibul ar sch wan noma.
• Radiation t he rapy Aj I'I!. 2 1: 1540 -46, 2llOO
o Gam ma knife: Low dose, sha rply collima ted, foc used 17, Rose nberg 51: Natur al hi sto ry o f aco ustic ne uromas.
cobalt-en treatmen t La ryngo sco pe. 110:-19 7-508 , 2(XJ()
o Used wh en medi cal co nt rain dications to surgery & 18. Du brulle F et al: Coc h lea r fossa en ha nc eme n t at M R
residual post-o perative AS eva luatio n o f vest ibul ar sch wan noma : correlatio n with
success at hear ing-preserv ati on su rgery. Radio logy.
2 15:458-2, 2llOO
IDIAGNOSTIC CHECKLIST 19. Allen RW ct al: Lo w-co st hi gh-r eso luti on fast spin-ec ho ~ l R
o f acou st ic schwannoma: a n a lterna tive to enhanced
Co nsider co nv en tio na l spin -echo MR? AJNR. 17:1205-10, 1996
20 . j ackler RKct al: Selectio n o f su rgical approach to aco ustic
• Co nside r using high-resolution T2 unenhan ced ax ial ne u roma. O tola ryngo l Clin North Am . 25 :36 1-87, 1992
& co ro nal MR as "screen ing" for AS
• Th in- secti on , T I C+ axia l & coronal MR is go ld
stan dard imaging approach
ACOUSTIC SCHWANNOMA
IIM AG E GALLE RY 1
29
(I.eft) Co ro nal T2WI MR

d em o nstrates a sr1lJfI,
iotrsceneticutsr-tundst
acoustic schwdnnom a
(arrow) that b ow s tbo crista
(,l lci(ormi_~ ceph alad (vppo
,,,,mv), indic ating its Ofigi n
from the interior vp:;t ihul,Jf
nerve. (Right) A x;al T 1 C+
MR sho ws an avidly
enhancing J.lfge CPA-JAC
aco ustic 5ch wan noma. In
this caw Ihe lumor has
compIL,tcly fill ed the
co chlear s ponuro (.l rr o w) .
Surgical (emoval w ill most
likely (eSUfl in no rt'sid lhll
boe ring.
Variant
(Lef t ) Ax ial T 1 C+ M R shows
large enhancing Jco ustic
scbwe nncun a with only
minimal pcneus uo n o f lAC
(arro w ). Inl r.1fnUfal cysts
(ope n iI/TO WS) and J large
essocieted .1rachnoid cy.~ t
(cu rved arm w) e vident.
(Ri~ "l) II XI.ll T2WI M R
reveels larw' Cf~ acoustic
schw an nom a fJa_~
inhomoueneous sign al. The
4th wn tricte (arro lV) can be
di_~t illg uish ('d from
.l.\s()c;att'd arachnoid {y~ t
(c urved arrolV) m ort' rm dily
lVi/h l l imaging.
Other
(l.eft ) Axial graphic of a large
acoustic schlVannom a
reveets the typical nee
Cfmm on cooe " CPA ·IAC
morphology. M.m ('ffIX! on
middlf' cerehellar pt'dutlcle
(.If(o w s) is evide nt. ( Rig /II)
A xial TI WI MR sho ws CPA
ma ss w ith central low &
p er ip heral high signal
(arro w ). An extra -axial strip
o f high signa' (op en arrow)
ex/en ds .l/ong poster ior
p otrou s bone. A t sur8£,ry.
hemorrhagic AS found.
MENINGIOMA, CPA-lAC
1
30
Axial graphic <11 k-.'t'/ of lAC shows lijrge CPA A),i.1! gross pathologic *Ction viev..rotnxn beIcM'shows
m('f)ingiomJ cJw ng lJldSS t.,iioll on brdinsJ.('1l1 & a I.ug(· em meningioma with a broad dutill-b.be
cC'f"C'lx>llum. Nooct, broJd dU'iJ/~ creates the shape compressing the cerPbellum. The spec;fTlf!('I
of a mu~hroom hc.1d. Af ro,,"': Dura/ -ltli'-, ck'fflOl1"" <lt~ c~r·va5Cula( ckaft (a rrONS) .
• Ovoid mass mim ics aco ustic schwan noma (5%)

ITE RM INO LO GY o CSF-vascular "deft" between mass &. brain
Abb re viat ions and Syno nyms o Frequently (5lYX,) herni ates cepha lad int o med ial
• Posterior fossa meningioma midd le cran ial fossa
• Aco ustic sch wa n noma rarely shows this vector of
Definitions spread
• Benig n , unenca psu lated neop lasm arising fro m
meningot helia l arachnoid cells of C PA-lAC dura CT Findin gs
• NEc r
o 3lYlV isodense: 70% h yperdense
IIM AGING FINDINGS o 25% calcified; 2 types seen
• Ho mogeneou s, sand-like
Ge ne ral Features • Den se ch un ks sprin kled th rou gh out mass
• Best dia gn ostic clue: CPA dural-based en hanc ing mass o Bo ne wind ow findings
eccen t ric to lAC poru s acusticus • Hype rostoti c or per mea tive-sclerotic bo ne cha nges
• Location possible (en plaq ue type)
o 10% occu r in posterior fossa • lAC flarin g rare (cf acous tic schwa nnoma)
o Wh en in C PA, asymme tric to lAC porus acusticus • CECf: 90% stro ng . un iform en hancemen t; l OC){)
• Size inhomogeneou s
o Broad ran ge; may be large but most in 1-8 em range MR Findings
a Gen erally significant ly larger th an aco ustic
schwan noma at presentation • TlWI
o Isoin tense o r m ini ma lly hyperin tense to gray ma tter
• Morp ho logy o Whe n tumor has calcifications or is high ly fibro us,
o Three d istin ct mo rpho logie s
h ypol n ren se areas are visible
• "Mush roo m cap" (hem isphe rical) wit h broa d-base
towards posterior petro us wall (75%) • T2W I
o Wide range of po ssible signals on T2W I
• Plaqu e-like (en plaqu e), - l - bone inva sion with
hy perostosis (20%) • lso tntcn se or hyp olnten se CPA mass (com pa red to
gray matt er) most likely meningioma
DDx: CPA Mass
Acoustic Schwanno ma Epidermoid Cys t Arachn oid Cyst Facial Schwannoma

MENINGIOMA, CPA-lAC
Key Facts
1
Te rmin ology Path olo gy 31
• Benign, unencapsu lated neoplasm arising fro m • Most co m mo n pri mary non glial tumo r
mcn ingothcllal a rach nold cells o f CPA-lAC d ura • 2nd m ost co mmo n CPA-lAC mass (acou stic
schwan no ma = Ist most co m mo n)
Imaging Find ings • Sha rply circumscribed, u nen capsul at ed
• Rest diagnostic clue: CPA dural-hased en hanci ng ma ss
eccent ric to lAC po ru s acusttcus Clinica l Issues
• Enhanc ing d ural-ba sed ma ss with dura l "tails" • Adult fem ale unde rgo ing brain MR for unrelated
cente red along posterio r petrou s wall i n dicat io n
Top Diffe re nt ial Diagnoses Diagn ostic Checklist

• Sarcoidos is • Foca l or d iffuse h ypoint en sity o n T2W I in CPA mass
• Acoustic schwa n noma (AS) suggests men ingio ma
• Primar y men ingeal lym phoma • Dura l "ta il" in lAC suggests meningioma wit h d ura l
• Intracran ial pseudo tumor reactio n, not acoustic schwa nno ma
• Foca l o r d iffuse parench ymal low signa l seen if o Axial &. co ro nal thi n -secti on T 1 C+ ?\.tR best fo r
ca lcified o r h ighl y fibro us men ingio ma
o Cxt-vascu lar cleft
• Pial blood vessels seen as sur face flo w vo ids
betwe en tumor &. brain IDIFFERENTIAL DIAGNOSIS
o Arterial feede rs to tumor seen as arbor iZing flow
vo ids Sarco idos is
o High signal in ad jacen t brainstem or cerebellum • Often m ultifocal, dura l-based foci
• Repr esent s per ttum or al brain ede ma • Loo k for infu ndi hular sta lk invo lve ment
• Co rrelates wit h pia l blood supply
Aco ustic schwa nno ma (AS)
• Its presen ce signa ls pro blem s wit h sa fe removal &.
early recur rence • lntracan a ltcu la r first, th en C PA extension
• T2- GRE: May "bloo m" pa ren ch ymal lo w signal • Int racanalicular men ingiom a may m im ic AS (rare)
• T l C+ O the r schwa nno ma
o Enha nci ng dural-based mass with du ra l "tails" • Trigeminal, facial ner ves
cen tered alo ng posterior petrous wall • Ro un d > flat dural -based
• 9Sl MI en hance stro ng ly
• Heterogen eo us en hance me nt co m mo n especi ally Le ptom en ingeal metastasis
in larger lesio ns • May he bilateral in CPA· lAC area
o Dur al thi ckenin g ("ta W) in 60% • Multifocal meningeal involv em ent
• Repr esen ts reactive ruther tha n neoplastic change
in most cases Primar y meningeal lymphoma
• Whe n extends into lAC may mim ic lAC • Rare int racranial lymph o ma
co m po ne nt of aco ustic schwa n no ma • Foca l area en hanc ing. t h ickened m en inges
Angiogra phic Fi nd ings Intr acran ial pseu dotu mor

• Co nven tion al • Rare diffuse me n ingea l t h icken ing &. en ha nceme n t
o Dural vessels supply tumor ce nte r, pial vessels Nonneoplastic cyst
supply tum o r rim
• Epiderm oid cyst
o "Sunbu rst" patt ern of en larged d ural feede rs
• Arach noid cyst
co m mo n
o Prolonged vascular "stain" in to ven o us phase
o Arte rio venous sh un tin g may occu r
IPATHOLOGY
Imaging Recomm end ati ons
• Best imagin g tool f.
o Brain MR wit h f(K used posterior fossa imagi ng best
Ge ne ral Feat ures
• Gene ra l path co m me nts: Multi ple meni n giomas occ ur
approach in 10% of spo rad ic cases
o Bone o n ly axial &. co ronal cr recommended if bone • Gcnet lcs
reaction or invasion suspec ted o n MH o Lo ng-arm de letio ns of ch ro moso me 22 co m mon
• Protoco l advice o NF2 gene inacti vated in 60% of spo radic cases
o Full br ain T2 and /o r Fl.AIR used to loo k for o Angiogen ic fact or s (FGF-2, VEGF, in tegrin s)
parenchyma l hra in ede ma expressed
MENINGIOMA, CPA-lAC
1 o Ma y have progesterone, prolact in receptors; may
express growth ho rmone Natura l Histo ry & Prognosis
32 • Etiology: Arises fro m mcni ngo th elia l arach noid cells • Slow growing tum or th at engulfs but does no t
(arach noid ' cap' cells), not d ura typ ically destroy cra nial nerves it en co unt ers
o Epidem iology • Negative progn ostic findings o n MR
o Accou n t for - 20% o f primary int racrania l tumor s o Pcritum ora l edema in ad jacen t b rainstem
• Most com mo n prima ry nongUal t u mo r o Lo ss of CSF-vascular cleft betwe en tum or &.
o 1-1. 5911pr evalen ce at autopsy or imagin g brainstem
o HN~ l multiple (NF2; multiple meningiomat osis) o Signifi cant subjace n t hon e invasion
o 2nd most co mmo n CPA-lAC ma ss (aco ustic Treatme nt
schwa nno ma = lst most common )
• Surgica l removal if medi cally safe
• Associated ahnormalit ies: Men ing iom a + schwa n no rna o Co mplete su rgical removal possible in 9S'.l11wh en
= NF2
tum or does not in vade skull base
Gross Path ologic & Surgical Feat ures • Radiat ion t herap y
• "Mush room cap" (he mispherical) morpho logy most o Adj u nctive th erapy when su rgical rem oval near
co m mo n (75%) co m plete
o En plaque morphology (20%) also see n in CPA
o Alternative th erapy when skull base inva sion is
• Sharply circu mscribed, u nen ca psulated ext ensive
• Dist inct CSF-vascu lar "cleft" between mass & adj acen t
bra in
o Adjace n t d ural thicken ing (collar o r ' tail') is usually IDI AG N O STIC CH EC KLI ST
reactive, not neo plastic
Co nside r
Microscopic Features • Meningioma when im aging sho ws hemi spherical,
o Subty pes (wide range of histology with littl e bearing dura l-based en han cing CPA ma ss wit h dural "tails"
on imaging appea ran ce or clinical outcome) • Men ingioma whe n CPA mass is large bu t relat ively
o Men ing othclial (lobules of rnen ingoth elial cells) asympto matic
o Fihro us (par allel, in ter lacing fascicles of
spindle-shaped cells)
Image Interpr etation Pea rls
o Transit ional (m ixed form ; "onio n-bulb" who rls and • Focal o r diffuse h ypointen sity o n T2W I in C PA 111 ,ISS
lobu les) suggests meningioma
o Psam momat ou s (n u me rous small calcifications) Dural "tail" in lAC suggests men ingioma with dural
o Angiomatous (abundan t vascul ar cha n ne ls), not reaction, not aco ustic schwa n noma
eq uated w it h obso lete te rm "angiohlastic
m enin gioma"
o Miscellaneo us forms (rn tcrocys ttc, c hocdoid , clea r ISE LECTED REFER EN C ES
cell, secretor y, Iym phoplasmocytc-rich , etc) 1. Wh ittl e IR ct a1: Men ingi o mas. Lan cet. 36J(9-4 20) : 1535--4 J ,
2lXH
Staging, Grading or Classificat ion Crite ria 2. Nakam u ra M et al: ~ h.-n tng tomas o f the in terna l aud itor y
• WHO grad ing classificatio n can al. Ne u ros u rgery 55: 119 -128, 2004
o Meningiom a (classic, ben ign ) == 90 c,li) 3. Asao ka K et al: ln tracanall cular m eningiom a mimi ckin g
o Atypi cal m en ingi oma = 9% vestibular sch wa n noma . AJNR. 23: 1-493-6, 2002
-4 . Roberti F c t al: Post eri or fossa menin gio ma s: surgical
o Ana plastic (ma ligna n t) meningiom a = ]lMI
exper ience in 161 cases. Surg Neu rol. 56:X·20, 200 I
5. Filippi CG et al: Ap peara n ce of m enin gioma s 0 11
diff usi on-weight ed images: correlati ng d iffus io n constants
ICLIN ICA L ISSUES wit h histopath ol ogic findin gs. A.J NR. 22:65-72 , 200 1
6. Ku rutsu J ct al: ln cldc n cc an d clin ical fea tu res o f
Presentat io n asym pto m ati c m enin giom as. J Neu ros urg . 92 :766 -70 , 20Cl0
• Most co m mo n signs/symptoms: Most co m mo n to find 7. lld an F et a l: Correlation of th e relation sh ips o f
inciden tally b rain-t umor in terfaces , m ag netic reso na nce im agin g, a nd
angrographi c find ings to p red ict cleavage o f m eni ngio m as.
• Clin ical profile
D Ad ull fem ale undergoing brain MR far unr elated
J Neu ros u rg. 9 1:38-4·90, 1999
K. Yo shi oka II ct al: Per it u mora l brain edem a associ ated wit h
i n dication m en in gioma: in fluence o f vasc u la r e nd o thelial grow th
o Minimal sym pto ms often presen t eve n wit h larger, factor exp ressio n and vas cu lar blood su p pl y. Ca nce r,
m ore in vasive lesio ns 8S:936-H, J 999
9. Hau gh t K et al: En ti rely intracanali cul ar m enin gio m a :
Demograph ics co n trast -en h anced MR find ings in a rare en tit y. AJNR.
• Age 19:1831-3,1 998
o Midd le-aged, elderly pati en ts; peak age = 60 years 10. Lalwan i AK et al: Preoperative d ifferen tiatio n be tween
o If fou nd in child ren, co nsider po ssibilit y of NF2 m en in gioma o f t he ccrc be llo po n ti nc angle and aco ust ic
o Gender: M:F = 1:3 n eu rom a using ?>.1R1. Oto laryn go l Head Neck Su rg.
109:88-95, 1993
MENINGIOMA, CPA-lAC
jlM AGE GALLE RY 1
33
(/.('!I) ;\\i.l l 11 C+ AIR show~

a sm.llll" C1 ~\ meningioma
<15 an tmh.mdng. dur,l!·ba...ed
ma ss (al ro w ) ,by mmetrically
o,;enrC'd to /)()(U5 scusticu «
of lAC (opt·" .m ow}. Itwre
is minimal but d"llnile
pent1tf.llion into mf'flidllAC
(c uf\,t'( /.m o w} . I X;1:" l )
Coronal T, C+ ,\IR tt'\'e.a/s d
.~ mJ IJ('r CPA nU'n ing ;omJ
(arrow) ,It 1(·\'('/ of l A C.
Tum o r pt'tWIt.U I' S potu_,
,1CUQ ;c us (CUf \ 't 'fl .urol\' ) .
~ 1t>1I ra/C' - ritual -t.lI1" (opt>n
JIfOlV ) hd p... flMl..e the
lUt'ningiom .l diJgnmi.s.
Variant
(/£ / 1) fm laled
hltf scanaticutsr nwningio/llcJ
is .t r.1((' /('5;00 to encounter.
I his ,oronal cr 'hrough lAC
show s cI iOt '<11 ,1( (, ,1 oi
/111 '111111::;0 111,1 .l5 .~OC;.l h>( /
h}'f)( 'roS/( h ;S (.m ow) OW ,JI
,i'n l R'.1Il1 I ' may ')uI-:1.;I'."
m lm "',1of lAC. (HiK" l)
COI'OIJ.l1 11 C+ ,\-l R
d t'lII o,,\tra le... 'he ,Ul'.1 of
hyf>('w !'olol ic hom' (arrow )
;le/jan-n t to tht' ('nhmcing
int rdCdfl J!ic'ular meningom.l
(of>(·n arrow). Black liflL'
w p .1f.lting llinC'.lr sress of
enbsncement (C"Uf\'ro
i/f(OW) i.~ cristJ iskiiomiis.
Vari ant
(J.~JO Ih idl TI C. AIR sho ws
.1lougt' CPA mt>ningiomd fi/1~
both CI~ (,m ow) , lAC
(ope n ,lfWW). Noticelesion
involvf'" middle (--', Ir (cur ved
drrow). M.I~to;d ,1u;d
enhsnces much le ss than
meningioms. (Rig" ' ) Axial 11
C+ M R sho ws /.lfgt' low CPA
meningioms (arro ws) with
pn plaq ue morphology.
NO/ict' occludf"fJ W[febral
,lftery (open JffowJ .~ ICA in
c arotid 5!Mn' (c u rvf'C!,JffOW)
,1ft ' ('11/:U/(('C/ b)' tumor.
FACIAL NERVE SCHWAN NOMA, CPA-lAC
1
34
Axial graphic of a larger f NS shows CJ~ (ice cream) & A xial T1 C+ M R in p.1lien{ with FN S shows CPA (arrow )
lAC (com» compo nonts mimic acoustic schwannoma . lAC (cur ved arrow) componorus. Notice the diagnostic
I ht' l.lbyrinthinf! ~t'gmenl or fadal ner ve involwrnt.,ll ',ILJyrinthint> wgm ent of (acial !1t'n'P Mla i/" (open arrow ).
(.m ow) m.lk~ di<lgno!lis.
ITERM INO LOGY MR Findin gs

• T2WI : FNS CPA-lAC is mass d isplacing h igh signa l CSF
Abbrevi ati ons and Synonyms • T l C+: CPA-lAC-laby rin th ine cana l en h anc ing m ass
• Facial nerve schw an nom a (FNS), CPA-lAC = facia l
nerve schwan nom a in cerebelloponrine angle-internal Imagin g Re commendati ons
audit or y ca na l • MR recommendation: Axial thin-secti on (3 m rn) T1 C+
• Facial neu rom a; facial neur ilemmoma MR in ax ial &; co rona l pla ne through CPA-lAC
Defin itions • cr recommen dation: Verify susp icio n of t
labyrin th in e FNC wit h bo ne CT
• Rare benign tum or o f Schwarm cells th at invest the
perip hera l FN
IIM AGIN G FINDINGS Bell pal sy (herpeti c facial paralysis)
• T I C+ MR: Prominent enha ncem ent o f int rat emporai
General Features facial nerve wit h lAC fundal "tuft"
• Best d iagnost ic clu e: CPA-lAC m ass with "tail" in
labyr in th ine FN cana l Acoustic sc hwan noma
• Location: CPA-lAC &. lab yrinthin e segme n t FN can al • T1 c+ MR: CPA-lAC enhancing mass without
(FNC) lab yrinthine can al "ta il"
• Size: Wid e range from mill im eters to cen t ime te rs
• M orphol ogy Meningioma, CPA-lAC
o Large: CPA-lAC "ice cream on ice cream CO OL'" with • T1 C+ MR: Dur al-based , eccent ric CPA enhancing m ass
co m ma -sha ped "ta il" with dural "ta il" proj ecti ng in to lAC
o Sm all: lAC ma ss curves in to lab yrinthine "tail"
CT Findin gs
• NECT: , Size labyrin t h ine FNC ± gen iculate fossa
DDx: CPA-lAC Mass
Bell Palsy Acoustic Schwannoma Meningioma CPA

FACIAL NERVE SCHWANNOMA, CPA-lAC
Key Facts
1
Termin ology Top Differ ential Diagn oses 35
• Ra re beni gn tumor of Sch wan n cells th at invest th e • Bell palsy (he rpetic facial paralysis)
periphe ral FN • Acoustic schwa n nom a
• Meningioma, CPA-lAC
Imaging Find ings
• Best diagn ostic clu e: CPA· IAC ma ss with "tail" in Path ology
labyrInthin e FN canal • Epide miology: Rare tum or (m tratemporal > >
CPA-lAC> intraparolid)
!PATHO LO GY IDIAGNOSTIC CHECKLIST

Gen eral Features Co nsider
• Genetics: Multiple schwan nomas = NF2 • Th in-section imaging shows labyrinthine "tail"
• Etiology: Schwarm cells investin g facial nerve
• Epide m iology: Rare tu mor (in t rate mpo ral > > CPA-lAC Image Interpretati on Pe arls
> intraparotid ) • FNS CPA8lAC exac tly mim ics aco ustic schwann om a if
• Associated abnormalities n o labyrin thin e "tail" presen t
o Neuro fibro ma tos is 2 • Laby rin th ine "tail" is key to imagi ng d iagnosis
• Bilat eral acous tic schwa nnoma
• Othe r schwannoma, meningiom as also seen
Gross Pathol ogic & Surgical Features l. Kim JC et al: Facial n erve sch wan n o ma. An n Otol Rhinal
• Tan , ovo id-tubular, en capsulated mass Laryn gol. 112(2):185-7, 200 3
• From outer nerve she ath layer, expand ing ecce nt rically 2. Liu R et al: Facial ne rve sch wan no ma: surgical excisio n
versus co nse rvat ive man agem ent. Ann Otol Rh inal
Microscopic Feature s l.aryng o l. 110(1 1):1025-9, 200 1
• Encapsulated tumo r com posed of bund les of 3. Salzma n KLet al: Dumbbell sch wan no mas of th e internal
spind le-sha ped Schwa rm cells forming who rled aud itory canal. AJNR Am J Neurorad ioi. 22(7):1368- 76,
patte rn 2001
• Cellular architectu re co nsists of den sely cellu lar 4. Yokot a N et al: Facial ner ve schwan noma in th e
(An ton i A) areas ± loose, myxomat ous (An to n i B) areas cerebe llopon tine ciste rn . Findi ngs on high resol u tion CT
and MR ciste rnography. Br J Neu rosu rg. 13(5):5 12·5, 1999
5. McMen omey SO et al: Facia l nerve neuromas presenti ng as
acou stic tumors. AmJ Ot ol. 15(3):30 7.12, 1994
Prese nta tio n
• Most co mmo n signs /sym ptoms IIM AG E GALLERY
o SNHL :::= facial ne rve paralysis
o Ot he r sym pto ms: Vertigo , hemifacial spasm
Dem ographics
• Age: Average age at presentati on :::= 40 years
• Gen der: No gen der predilection
Natural History & Progn osis
• FN paralysis takes years to develop
• Surgical cu re can be wo rse than d isease
Treatme nt
• Co nservative: Do noth ing until FN paralysis present
o Some do not grow (Left) Axial bone CT reveels enlarged labyrinthine segment of the
o Some n ever become sym ptomatic facial nerve canal (arrow ) in patient with facial nerve schwannoma of
• Surgery whe n FN paralysis + othe r sym ptoms evolving CPA-lAC. Notice there is erosion into subj acent cochlea. (Right) Axial
o Su rgical goal: Comp lete tumor rem oval with T1 C+ MR shows FNS (curved arrow ) with large arachnoid cySl
preservatio n of hearing & restoration of FN fun ction (arrows). Notice the labyrinthine -tail- (open arrow) in the
labyrinthine segment of the facial nerve canal.
METASTASES, CPA-lAC
1
/ h ial ~H),;.'i fl al/ldogie ~')l'cim l::'fl vil '\\ing ( "I ~ \ Jrl'a/TrJm J'.ltiel)/ Id/ h aeu/" {m~d 1111 L' 8th ner ve p ,l/jies. Ib id/
"/'(1\\'.<; /lfH!ul<1f IIlf'fl ingf'oll met,h l" _i." (d It OW)
, lh O I't' f / C+ i\ lf~ \IIo\\'5 nod ular md, I!>I. 15i~ ill right (/ ~\
plOlrudi/J~ (rom POfu ) ,If 1I5fic"th of in lf 'lIlal ,w ili/ory C('ll fe/f 'l.J anfe rio r to lAC !inp" , l'llh llU {'IJ)('nf "/oHM
c,m al ( C() lIflt'~y R. I te..vton. M f) I . IJl I·n ill/-.'l '.\ 0 ;//\< I H t·~f.-'/ I ' {, l/ fO IV}.
• Prima ry eN S tu mor seed ba sa l cisterns (drop

!TE RM INO LO GY metastases )
Abb re viations a nd Syno nyms o C PA an d /or lAC rn cnln ges
• Synony m s: t.c p to rn cn ln gcal carctno m atos ts. • Any ot he r m en in geal sur face m ay be con cu rren tly
meni ngea l carciuo ma tosrs, carcino mato us men tngitls af fected
o All o f these t l' 1'1I1 5 a re m lsuo me rs o Ot h er CPA loca tion
• Nco plavruv art' no t a lways ca rcino mas • Metastatic fo cu s in flo cc u lu s o f cere be ll um
• Pach ym eninges (d ur a ) &. le pro m e n tn gcs (p ia + p ro ject s in to CPA cistern
arac hno id) arc o ften bo th in vo lved o ~ Ia y ex te n d in to lACs &. resem b le bilat eral acousn c
• Usually does n o t co n ta in a n mtlnrn mat o ry t-it is) tu mors (N F2)
componen t • Size: O ften sm a ll « 1 em) : Gl USt' S sym p to m s ea rly
• Morphology: In filtra ting m a rg in s u su ally present
Defin itions
• Diffu se In flltrntlon o f lept omenin ges by m align ant
CT Find ings
cel ls mctastnstztng from syst e m ic n oop lasta • NECT: O fte n normal
• CECT
o Un ilate ral or b ila tera l pa thologic men ingeal
IIM AGING FINDING S e n ha ncemen t of m en in ges alo ng C PA N lAC
o CT sh ows lcston s o n ly w he n larger a nd /or m ultip le
G e ne ral Fe atures M R Find ings
• Best diagnostic clu...' ; Bila te ral li near o r n odu lar
• T1W I
m eu tngeal cn ha nce m en t ill CPA-l AC on T l C+ t-. m
o Focal m enin gea l th ic keni n g
• Lo cation o M c n tng ea! foci votn tenw to g ray m att er
o Co mmon sites o f or igin
• Prim ary tumo rs incl ude breast. lu ng &. mt'!'1Il 011l'1 • TZW I
o High-reso lu tion T2 Mk
• Mcn ingca l lymphop ro liferat lve rna hgnancy «
• Ttssuc-ln ten stty lin ea r m at e rial a lo ng
lym ph oma &. leukem ia
lepto men in ges
• 7t h &. Sth cra n ia l Il e [\-CS ma y he thic keu ed
DD x: Linear-Multinodular Lesions o f C PA- lAC

_ ..,-"c-.-...
.~•1,(. 'CI'' ' ;;::;
Meni ngitis RIfS Sarcoid, CPA

METASTASES, CPA-lAC
Key Facts
Terminology Top Differential Diagnoses 3
• Synony ms : Lept o men ingeal ca rcino ma tos is, • Meningitis
men ingeal ca rcinomatosis, ca rcino mato us meni ngitis • Ramsay Hu n t syn drome (RHS)
• Diffuse in filtrat io n of leptomen inges by malignan t • Sarco idosis, CPA· lAC
cells metastasizing from syste mic neo plasia • Bilatera l aco ustic sch wa n no ma (NF2)
Imaging Findin gs Path ology
• Best d iagn o stic clu e: Bilateral linear o r nod ula r • Most co mmo n ly resu lts when extrac ran ial neop lasm
men in geal en h ance men t in C PA-lAC o n 1'1 C+ MR seeds meni nges
• Primary tu mo rs incl ude b reast, lun g &. melano ma
• Men ingea l lymph op rol iferative malign an cy = Clinica l Issu es
lym p ho ma & leu kem ia • Most co mmo n signs /sy m pto ms : Rap idly prog ressive
• Prima ry eNS tum o r seed basa l ciste rns (d rop unil at eral or bila teral 7th & Bth cran ial nerve palsies
metastases) • No cu rative treatmen ts available
• Tl C+ MR shows dif fuse thicken ing &. en hance me n t • Thera pies aim ed at preserving neuro logic fu ncti on
o f lepto men inges (p ia + arachnoid) and im p roving qua lity o f life
• FLAIR Sarco idosis, C PA-lAC

o Larger lesio ns ma y cause h igh signa l in ad jacen t
brains tcm and/or ce rebellum • T l C+ MR ma y be identical whe n multifocat
o Associated parenc hy ma l brain metastases seen as men ingea l type
multip le h igh signal areas • Loo k for in fundibular sta lk in vol vemen t
• In creased eryth rocy te sed ime n ta tio n rate (ES R) &
• Tl C+
o '1'1 C+ MR sho ws d iffuse t h ickeni ng &. en ha nce m en t seru m ang iotensin co nve rting enzy me (ACE)
of leptomen inges (pia + arachnoid) Bilateral aco ustic sc hwa nnom a (NF2)
o En ha nci ng mass ce n te red in floccu lus o f cerebe llum • Younger patients without h istory of malign ancy
o Meni ngea l metastasis • Focal C PA-lAC nodular masses
• Pat h o logic en hance men t of meni nges • Remai n ing men inges spa red
• Process typically d iffuse &. linear
• Nodular masses have been de scribed
• Un ilat eral o r bilate ral
• Associat ed ot he r men ingeal foci com mo n
IPATHOLOGY
• Mult iple en h anci ng paren ch yma l bra in met astases Gen eral Fe ature s
may be asso ciated • Gener al path co mme nts
o Floccular metastasis o Lepto men ingeal disease ma y arise de novo
• Enhan cin g mass ce nte red in floccu lus en larging • Prima ry tu mor may be undi agn osed o r neve r
into CPA cistern fou nd
Imagin g Re commend ati ons o Most com mo n ly resu lts when cxt racran ial neo plasm
• Rest imaging too l: '1'1 C+ MR t hroug h posteri or fossa is seeds men in ges
best ima gin g too l &. seq ue nce o Key anato my : Men inges has ] d iscrete layers
• Du ra (pachy me n inges): Den se co n nec tive tissue
• Proto col advi ce
att ac hed to calvarium
o Axial &: coron al plane s recommend ed
o Look for lAC en h an ceme n t bu t also eva luate • Pia: Clear mem bran e firmly attach es to su rface of
men inges of the su pra- &. infr at entorial brain brai n; extends deepl y in to sulci
• Arach n oid: In terposed bet ween pia &: dur a; pia +
arach n o id = lept omeni nges
ID IFFERENTIAL DIAGNOSIS • Etio logy
o Met astatic tu mor involves lepto men in ges of CPA
Meningitis and/o r lAC
• Bacteri al, funga l o r tubercu lou s meningi tis o Leptomenin ges follow 7th & 8t h crania l nerves int o
• T1 c+ MR may be ide ntical to CPA-lAC metasta ses lAC
• Clin ical in formati on & cerebros pina l fluid (CSF) o Any lep tom en ingeal d isease may man ifest with in
evaluatio n keys lAC
o Frequen t association of CPA-lAC lesio ns with ot he r
Ramsay Hunt synd ro me (RHS) menin geal lesions suggests following pa thwa ys o f
• Extern al ea r vesicu lar rash spread
• Tl c+ MH shows en hancement in lAC &: in ner ear ± • Cere brospina l flu id d issem ination
7th cra n ial nerve • Leptomen ingeal exte ns ion
• Epldcm io logy
METASTASES, CPA-lAC
1 o In creasingly more commo n neuro logic co mplication
of systemic cancer Image Interpret ation Pearl s
38 • Due to inc rease in survival rat e of cancer patients • If suspect CPA-lAC met astasis from T I C+ MR
• Associa ted abno rmalities appea ra nce or h istory of kn own malign an cy, make
a Multip le other lept om en ingeal foci arou nd brain sure to review the followi ng
o Parench ymal bra in metastases ma y also be present o Extracranial and calvarial structu res for ot he r lesions
to confirm diagnosis
Gro ss Pathologic & Surgical Fea tur es o Look for involvem ent of ot her men ingeal sites such
• Diffuse, nodu lar &/o r discrete as pa raselIar, ot her basal meninges
o Paren chymal brain fo r ab normal FLAIR high signal
Micro scopi c Features an d/or en hancing lesions on T1 C+ sequen ces
• Com mo n tissue types found
o So lid tumo rs = breast, lun g & melan om a
• All invo lve bo th leptomenin ges & pachymen inges ISELECTED REFERENCES
o Lymph oproliferative malign an cy = lym phom a &
I. Soyu er S ct al: Intracranial menin geal
leuke mi a
heman giopericytoma: the role of radiot herapy: repo rt of 29
• Involve both leptomen inges & pach ymeninges cases and review of th e literature. Cancer. 100(7):149 1-7,
o "Drop" metastases from eNS tum ors: 200 4
Medu llob lastoma, ependy mo ma, gliob lastoma 2. Siomin VE et a l: Posterior fossa meta stases: risk of
mul tiform e leptom en ingeal disease when treated with stereotactic
rad iosurgery com pa red to surgery. J Neurooncol.
67(1-2): 1t 5-2 t , 200 4
!C LI N ICA L ISSUES 3. Kesa ri S et al: Leptom en ingeal met astases. Neurol Clin .
2 1(1)25·66,2003
Presentation 4. Kralnlk A ct al: MRI of unusual lesions in th e in tern al
auditor y cana l. Neuro radiology. 43:52-7, 200 1
• Most co mmon signs/sym ptoms: Rapidly progressive 5. Schick R et al: Magnetic reson an ce imaging in patien ts
unilat eral or bilat eral 7th & 8t h cranial nerve pa lsies with sudden hearing loss, tinnitu s and vertigo. Oto l
• Clinical profile: Patien t wit h past histo ry of treat ed Neurotol. 22:808· 12, 200 1
malignan cy 6. Wh in ney D et al: Primary ma ligna nt melanoma of the
• Other symptoms cerebellopontine angle. Otol Neurotol. 22:218·22, 200 1
o Vertigo & po lycranial neuropat hy 7. Cha ST ct al: Cerebello ponttne ang le metastasis from
pa pillary carcinoma of the thy roid: case repor t and
Demographi cs literat ure review. Surg Neurol. 54(4):320·6, 2000
• Age: Old er adults Shen TY et al: Meningeal carcino matosis man ifested as
• Gende r: No gende r specificity bilateral progressive sens orine ural hearing loss. Am J Otol.
2 1:5 10-2, 2000
Natural History & Prognosis 9. lamanl AA: Cerebellopcn tine angle tumors: role of
magn etic resonance imagin g. Top Magn Resort Imaging.
• Men ingeal meta stases usually a late stage finding 11(2):98-107, 2000
• Poor prognosi s as patien ts have advanc ed, incurable 10. 1.ewanski CR et al: Bi lateral cerebellc pc ntlne metastases in
d isease by defin ition a patient with an un known prima ry. Clio Oncol.
11(4):2 72-3, t 999
Treatm ent 11. Swartz JD: Meningeal metastases. Am J Otol. 20:683-5,
• No curative t reat me nt s available 1999
• Therapies aimed at preserving neurologic function and 12. Arriaga MA et al: Metastat ic melanoma to th e
im proving qu ality of life cerebellopontlne angle. Clinical and Imaging
• Treatments are same as for underlying neop lasm characteristics. Arch Otol aryngol Head Neck Sorgo
o Radiotherapy ± che mo therapy depend ing on tissue 12 1(9): t052·6,1 995
type 13. Kin gdom TT et al: Isolated metasta tic melan oma of the
cercbelloponttne angle: case repo rt. Neuros urgery.
• Surgery rarely will play a role at this stage 33(1 ):142 -4, 1993
o Solita ry melano ma met may be exception 14. Mark AS et al: Sensorineural hearing loss: mor e th an meets
• If any question of diagno sis, excisional biopsy th e ey e? Aj NR. 14:3 7-45, 1993
n ecessary 15. Yuh wr et al: Metastatic lesions Involving the
cerebellopontine angle. AJNR. 14:99-106, 1993
16. Lee YY et al: Loculated int racran ial leptom enin geal
IDIAGNOSTIC CHECKLIST metastases: cr and MR cha racteristics. AJR. 154(2):35 1·9,
1990
Co nside r 17. Maiur i F et aJ: Cerebellar metastasis from prostatic
carcino ma sim ulati ng, on cr-scan, a cerebelloponttne
• Bilateral "acous tic sch wan nom a" in ad ult, consider an gle tumor. Case repor t. Acta Neural. 11(1):2 1-4, 1989
metas tati c tum or, not NF2 18. Gent ry LR et al: Cerebellopontine an gle-petromastoid mass
• Rapid ly progressive 7th cranial nerve palsy + CPA mass lesions: com parative st udy of diagnosis with MR imaging
suggests metastati c focus and CT. Radiology. 162(2):51 3-20, 1987
METASTASES, CPA-lAC
IIM AG E GALLERY 1
39
(Lef t) Axial T1 C+ MR
revea ls bifal f'fa/ f:'nhandng
"ptugs" of tissue filling both
interna l auditor y can als
(arro ws). O n 1st glance the
diagnos is of NF2 might be
considere d. Howe ver, this
otdcr pa tient has bilateral
lA C me tastases. (Rig llt) Ao;iaJ
T1 C+ MR shows en plaque
meningeal thick ening with
enhancement (arro w s). Inn er
ea r (op en arrows)
enhancement bilaterally
help s wggf:'sts diag nosis of
multiple me tastatic foci.
(u JI) A xial T2WI MR

demonstrates coc hlear &
vesti b ular nerves (arro w) in
the lA C fundus thickened hy
pia-arachnoid mctesunic
di sease, 7h(' m odiolus is a l.~()
enlargpd (op en ,,,,my) .IS <I
result of metastatic
itwotvotnont. (NiXIl I) AX;.l / T I
C+ A1Rshows pia·arachnoid
metastatic di5l',Jse ('oa ling
the crani.)! nprv{'s in ,hr'
distal II$ (arrow). Spread
through IIw cochlear
aperltJre in to the cochlear
l.lhyrirJfh h.H . ,,~O oc curre d
(op en ,UI()I V) .
(lAt!n l$(i.11 T1 C+ MR in
pa tient wit h unilateral SNI 1L
show,~ (ocal enhancing
m eta stases centered in left
iloc cu ius (open arrow ). Low
signal w ithin mJSS S irregular
margins .w BgC'st this
diagnos is. Arr ow. Norma l
op pos ite flo cc ulus. (Right)
Axial T2Wf M R acquired
while screening a patient
with un ilateral SN HL re veals
a low signal metastases
within lefe tlcc culus (open
arrow ). Adja cent middle
cere bellar peduncle &
cerebellar hemisphere shows
high signal edema (arrow s).
ANEURYSM, CPA-lAC
1
40
('-Oronal r 1 C+ MR .~ 1Jow.~ J IJfg(' ba!)i!ar artery Antffopmtl'fior Vl'rtel)lal ,mgiogram ShOINS if fusiform
aneurysm (arrow ) projecting into the riglll CI'l\ cistern. lJelsil.Jr.JrtlY Y ant'ur)!5m (a" o lVs). The true dimension of
The b.lsilar ar!f:" y can be 5('('11 inferiorly entering the the aneurysm cannot be spprecistcd on angiography.
ancurysm ( ¥){'n .1f(( )lV' .
• Co m plex ma ss with cen tra l or ecce n tric

!TERM INO LOGY enha ncing lumen , n onenhancing mura l th ro mbus
Ab b rev iatio ns a nd Syno nyms • Ofte n ha s ca lcified rim
• Post eri or inferior cerebe llar artery (PICA) a Com pletely thrombosed a ne u rysm
• Anterior inferi or cerebella r ar tery (AICA) • No en ha nc ing lum en
• CfA: Approac h ing co nven t ional angiogram resolution
Definition s
• Focal ballooning of wall o f PICA. vert eb ral artery (VA)
MR Findings
o r AICA in CPA-lAC ciste rn • T1WI: Suba cute lum inal clot is high signal on T l MR
images secondary to meth em oglobin Tl shorte n ing
• TZWI
IIM AGING FINDINGS o Pha se arti fact acro ss from pat ent an eurysm co m m on
o Signal varies from hyp otntcn sc "flow void" to
Ge nera l Features com plex mi xed sign al ap pea rance
• Best dia gn ostic clue: CPA mass with calcified rim (en • Varies with flow rat e & age of lum inal th rombu s
or layered co m plex signal in wa ll (MR) • T2* G RE: Ca lcified wall & luminal clo t may "bloo m "
• Location: CPA aneu rysm s from PICA > VA > AI CA • '1'1 C+: Aneurysm lum en en ha nce s if slow flow pre sen t
• Size: Millim et ers to cen time te rs • MRA: Delineates relationsh ip o f lesion to parent vessel
• Morphology Angio graphic Findings
o Round to ovo id m ass
• Visib le lu men ma y be sma lle r th an overall a neu rysm if
o May be fusiform
clot is presen t
CT Findings o Angiogram ma y underestimate a neurysm size
• CliCl' • Angiogra ph y delin eates precise vascul ar rela tionships
o Patent a ne u rysm Ima gin g Re comm endati ons
• we ll-dehn eatcd iso- to h yperden sc extra-ax ial
• On ce Cf o r MR suggests aneu rysm , co n firm with MRA
mass with strong, uniform en hancemen t
or a ng iogra phy
a Partiall y th rombosed aneurysm
• Most surgeo ns do angiogr ap hy prior to clipping
DDx : Vascular l esions of CPA- lAC
AVF + Venous Varix

ANEURYSM, CPA-lAC
Key Facts
1
Terminology Top Differential Diagn oses 41
• Foca l ha lloon ing o f wall o f PICA, vertebra l a rtery (VA ) • Vertebrobasilar do lich oectasia
or AlCA in C PA-lAC cistern • Ve nous va rix + du ra l AVF
• Arterioven ous malform ation (AV~l )
Imaging Findi ngs
• Best diagnostic cl ue: CPA m ass wit h ca lcified rim (C l') Diagn ost ic Chec klist
o r layer ed co m p lex sign a l in wa ll (MR) • CT: Rim calci ficatio n in CPA mass sugg ests a ne urysm
• MR: Co m p lex sign a l in CPA mass suggests aneurysm
• More su rgeo ns are oper ati ng o n MRA alon e

• < 1 em ru pture < t h an large a neurysm s
IDIFFERENTIAL DIAGNOSIS • I.e ft un cli p ped , aneu rys m ru pt ur e "gro wing" po ssibility
Ve rteb robasilar dolich oect asia Trea tme nt

• l\ IRA repro ject ions o r so u rce im ages sh ow no
• En dovascu la r co ili ng vs surgica l cl ippi ng
aneu rys m
Ven ou s varix + dural AVF IDIAGNOSTIC CHECKLI ST
• Angio delineat es best
• MR ve n ogra ph y m ay he lp de lin eat e Image Interpre ta tion Pearls
• C l: Rim ca lcifica t io n in CPA m ass suggests aneu rys m
Arte riove no us ma lforma tio n (AVM) • MR: Co m p le x sign a l in C PA m ass sugges ts a n eur ysm
• Large feedi ng arte ries + nidu s
IPATH O LO GY 1. Bo nn evill e F ct ul: Un usuallesion s of th e cc rcbcllo po n ti nc
angle: a segment al a pproac h. Radiog raphies. 2 1(2 ):4 19·3 H,
Ge ne ral Features 2001
• Genetics: Aneurysm propensity has hereditary d river 2. Mizush im a H et al: An eurysm o f t he distal an terio r in fer ior
• Etio logy: Inhe rit ed fac to rs + he m od yn am ic-ind uced ce rebell a r a rte ry at th e m edia l b ra n ch : a ca se repo rt an d
de ge n erat ive ch a nges in vessel wa ll o ften co m bine to review o f t h e litera ture. Su rg Neurol . S2 (2 ): 13 7-42, 199 9
form aneurysm 3. Kin ney we et .11: Rare lesio n s o f th e poste rio r fossa wit h
• Epi demi o logy in it ia l retro coch lca r auditor y an d vesti bula r com pla int s.
Am J 0 101. 18(3):3 73-80 , 199 7
o C PA a neu rys m s acco u n t for 5 I eM) C PA m asses
Mor ris D(1 et al: Th rom bosed pos terior-i nferio r ce rebellar
o 10% in tracra n ia l a neu rys m s ve rte hro has ila r
art ery a n eu rysm : a rar e ccrcbctlopo nu nc a ng fc tu mour. J
Gross Path olo gic & Surgical Features l.ary ngo l O tol . 109 (5 ):429-30 , 199 5
• Sa ccu lar : Berry-like o ut po uc h ing o f a rter y wa ll

s. Fu ku ya T et a l: Ane ury sms of t h e perip he ral por tio n o f t h e
a n te rior in fer io r ce rebella r arte ry; report o f tw o ca ses.
• Fu sifo rm : Enlarged, ec ta tic a t heroscle ro tic a rte ry Neu ro radi ology, 29(5):-l9 3-6, 1987
Microscopic Features
• Lac ks interna l e lastic la min a, sm oo t h mu scle layer s
• Dege nera tiv e ch a nges in pa re n t vesse l co m m o n
IIM AGE GALLERY
• Th ro m b us, a therosclerosis a re co m mo n
ICLIN ICA L ISSUES

Presentati on
• Most co m m o n signs/s ym p to ms
o Un ila tera l SN HL (70% )
o Ot her signs/sy m p to ms
• Head ach e from su barach noid he m orrhage (SO(},6)
• Tinnltus. ve rtigo
• IIcmifacia l spasm or facial n er ve pa lsy
• C lin ica l pro file: Mid dl e-aged patie n t, un ila tera l SNHL
(I.efl) Ih ial N Ee ] reveals a rig hl CPA high tlt>flSit y mass (arro w ) th J I
De mogra phics turn ed oUl to be a p Jrtiall y thromb osed .1Ilt' ur y.mJ. (Highl) A xial T2 WJ
1'-'I R .~ llo\V.~ <l right CPA mass (arrow) wi ll) comp lex .signal in its wall.
• Age: 40-60 yea rs
This p,utidlfy th rombos ed aneurysm IMs cak itication ill its waif U11 CT
as wcil as differefll age clot in irs iunwn .
SUPERFICIAL SIDEROSIS, CPA-lAC
1
42
AXI:l! graphic shoo.vs darker /)(0'''''0hefno!;k!f.,in st.lining Adl l Tr CRE MR ff;"\ 't'J/~ 5upf.-,rr,d.l1 5i<k'msi~ .IS d.lr~
<1/1 surfaces of the brain, nJcni,'h't'S and cr.mi,,1 0('( \ '("5. hcrtxxK.hin staining in (oIi., oi C(-'tebf'//um (a"o\V~) . In
Notice cranial ocoes 7 ~I;, 8 in the CPA-lAC are addition , the 7th & Bfh a .mi.11 f}(-'f\'l."S in the CPA-lAC
! )"Jrticul.lrly iJffec:1t'd. .l ff' black (01'('0 arfOI.VS ).
• Relatively in sen sit ive to presen ce o f hemosiderin

ITE RM INO LO GY on CNS surfaces
Abbrev iati ons and Syno nyms • CEC1'
• Sy no ny ms: Cen tral nervou s system siderosis, siderosis o Brain atro phy o nly
o Especially marked in posterio r fossa
Definitions • Ce rebellar sulci o ften d ispropo rtio nately large
• Recu rren t subarach no id hem orrhage (SAH) causes
hemosiderin dep ositi on 0 11 su rface o f brain, hralnstem
MR Findings
& cran ial nerve lepto meninges • T IWI: Hyperinten se signal may be seen on e N S
su rfaces
· 1'2Wl
IIM AGING FINDINGS o lli gh -resolu tio n, th in -section 1'2 MR 01CPA-lAC
• Cranial nerves 7 I< II appear da rker I< thi cker than
Ge nera l Featu res nor mal
• Rest d iag nostic clu e: Co n to urs o f b rain « cra n ial • Adjacen t cerebellar structu res &. brainstem show
nerves o utlined by h ypo int en sc rim o n 1'2 o r 1'2* GRE low signa l su rfaces
MR imag es • Less easily seen th an o n '1'2* GHE images
• Locati o n: Cerebral hemispheres, cerebe llu m, • flA IR: Dark bo rde r on lncal su rface 01 brain ,
b rainstcm, crania l nerves & spinal co rd may all he hrai nstem , cerebe llu m &. crania l nerves
affected • 1'2. GRE
• Size: Linea r low signa l along e NS su rfaces varies in o Most sensitive to hem osiderin deposition o n CNS
th ickness but usuall y s 2 mm surfaces
• Morph ol ogy : Cu rvilinear dark lines on e NS su rfaces o "Blooms" dark signal; makes it ap pea r mor e
co nspicuous, th icker
CT Fi ndi ngs • Tl C+: Surface 01 CNS d oes not en hance
• NECT
o Cerebral I< cerebellar atro p hy Ima ging Re comm en d atio ns
o Sligh tly h yperdense rim over brain su rface • Best imaging tool
o Brain MR with T2* seq uence
DD x: Low Signa l o n Brain Surface
Sup erficia l Vein Ne uro C M elanosis Meningoangiomatosis

Key Facts 1
Termino logy • Men ingoa ngo matosis 43
• Synonyms : Cen tral nervous system sideros is, siderosis Path ology
• Recurrent subarach no id hemorrhage tSAH) causes • Repeated SAH d eposits hem osiderin o n meningea l
hemosid erin deposition o n su rface o f brain, lining o f CNS
brain stem &. cranial nerv e lepto men inges • Hemoside rin is cyto to xic to neu rons
Imaging Find ings • Ca uses o f recurrent SAH found in - 509')
• Best diagnostic clue: Co n to u rs of brain &. cranial • CS ~ cav ity lesion (surgical cavity) with fragile
nerves o utlined by h ypointense rim on T2 o r TZ' GRE neov ascularity most co m mon
MR images • Bleeding neopl asm s (35%)
• Locati on: Ce rebral hemispheres, ce rebe llu m, • Vascular abno rmalities (18 % )
brainstem , cranial nerves & spinal co rd may all be Clinical Issues
affected , Classic pre sentat ion is ad ult pati ent with bilatera l
Top Differ e nt ial Diagn oses SNIIL & ataxi a
• MR sequ ence artifact • Pre-symptomatic phase averages 15 years
• Neu rocutaneo us melan osis • Treat source o f bleeding
• Once diagnos is of superficial side rosis is made, o Xant hochrom ic CSF

search for cause o f recurrent SAH must co mmenc e • Etiology
• Wh ole bra in MR with MRA & co nt rast 1st o Rep eated SAH deposits hem osiderin o n meningeal
• Tota l spine MR Znd if b rain negative for lin ing o f CNS
underlying lesion • Affect s brain, bratnstem , ce rebe llu m, cra nia l
• Protocol ad vice nerves & spina l co rd
o Brain MR o Hem osiderin is cy to toxic to neu ron s
• Unenhan ced MR wit h ~LAI R in itially • "Free" iron with excess prod uctio n o f h yd roxyl
• If suspect su pe rficial siderosis, add TZ' GRE radi cals is best cu rren t h ypothesis explalnl ng
seq ue nc es to co nfirm cytotox icity
o 8th cra n ial nerve is exte nsively line d with e NS
myelin which is su ppo rted hy hem osiderin-sen sitive
IDIFFERENTIAl DIAGNOSIS m icroglia
• Increased ex pos u re in CPA ciste rn
MR seq ue nce art ifact o MR findi ngs do not co rrelate with severity of di sease
• Variab ly thick & prominent low signal o n sur face o f • Epidemiology
brain o Rare ch ro nic progressive disorder
• Imaging clue = not presen t o n all seq uences o 0.1 S ~l o f pati ents undergo ing MR imagin g
Brain surface vesse ls • Associat ed abn o rmaliti es
o Causes o f recurrent SAH path ologies include
• Normal o r ab no rmal su rface veins • Trau matic nerve root avul sion, bleed ing CNS
• Linear, foc al area of low signa l o n su rface of brain neopl asm , vascular malfo rmatio ns & ane urysm
Neuroc utaneous melanosis Gross Patho logic & Surgical Features
• Co ngen ital synd rome • Dark brown discoloratio n o f lept om eninges,
• Large o r multiple congenital melanocytic nevi epe ndy ma I< subpial tissue
• Ben ign or malignan t pigm ent cell tum ors of th e • Causes o f recurr ent SAH fo u nd in - 5()g6
lept omeninges may he low signal on surface o f b rain o Dural pathology (47%)
, Tl h igh signal diffus ely in pia • CSF cavity lesion (su rgical caviry) with fragile
• TZ low sign al diff usely in pia neovascu larity most co mmon
Meningoa ngo ma tosis • Traumatic cervical nerve root avulsion
• Hama rtom at ous proliferati on o f men ingeal cells via o Bleedi ng neoplasms (35%)
Int rapa rcnch ymal blood vessels into cerebral co rtex • Epe ndy moma, o ligode ndroglio ma & astrocytoma
• Lep tomeninges are th ick & infiltrated with fibrou s o Vascular abnormalities (18 1M,)
tissue, may be calc ified • Arteri ove nous malfo rm ati on (AVM) o r an eurysm
• Multiple caverno us mal fo rmat ion s near brai n
su rface
IPATHOLOGY o Id io pathi c (46%)
Ge ne ral Features Microscopi c Features

• Hemosiderin sta ining of men inges and subpia l tissues
• Gene ral path co m men ts
o Hem oside rin stain ing o f meninges to 3 m m depth
• l lem oslder in is cytotoxic to u nderlying tissues • Thi ckened lept o men inges
1 • Ce rebellar fo lia: Loss o f Pu rkinj e ce lls a nd Berg ma n n
glio sis
ISELECTE D REFERENC ES
44 I. Mcssori A et al : Th e importance o f su specting supe rficial
side ro sis o f th e cen tra l n ervous system in cli n ical practice. J
Neu rc l : l..'u rosurg Psych iat ry. 75( 2): 188 -90, 2() ()-I
ICLINICAL ISSUES 2. xtcc arr on M O et al: Su pe rficial siderosis o f th e cent ral
Prese ntation nervo us system m any yea rs aft er neurosurgical pr ocedu res .
J Ncu rol Ncu rosu rg Psychiatry, 7-1(9):1326·8, 2CXU
• Most co m mo n s ig n s /sy m p to m~ : Bilatera l sensorineu ral J. Ka le SU et al: Su pcr ftcla l sidero sis of th e m eni n ges and its
h ea ring loss (SNHL) pres en t in 9S'.K1o f case s o tol aryn gologlc co n n ect io n: II series o f five pati ents. O tol
• Clin ical profil e Neu ro tol . 2-1 (1 ):90·5 , 2003
o Classic presentat io n is ad ult patient w it h b ilate ral 4. l.cussi n k VI et . 1 1: Su pe rflclal sid ero sis o f t he ce n t ra l
SNHL N atax ia nervou s system: path ogcncnc Ill..tcrogcu ctty and
o Seen less co m m o n ly as lat e co m plicatio n of treat ed th erap eut ic a pp roac h es. Ana Ncuro l Sca nd . I0 7( I ):54·6 I ,
ch ildhood cerebella r tu mor 21KJ:!
5. Dhoo gc JJ et al: Coc hl ear im plan ta tio n in a pati ent w it h
• Labo ratory su pe rficial sid eros is of th e ce n tral n ervous system . 0 101
o CSF from lumbar puncture Ncur o tol . 23( 4) :-168·72. 2(X12
• High prot ein (1( 0)( 1) 6. I.i I\W et al: Su pe rflclal sldc rosls assoc lan..-d with multiple
• Xa n th oc h ro mic (7S'KI) cavernous m alforma tio n s: re port of three cas es.
• Othe r sym pto ms Neurosurgery. 48 (5 ): I 1-17· SO. 200 1
o At axia (8S-X,) 7. Iannacco ne S e t al: Ce n tra l ne rvous sys tem su pe rflcia l
o Bilat eral h em lparesis siderosis. headache, and e p tlcp..y. Headache. 39(9):666 .9 ,
o Hyperreflexia, bladder d istu rban ce, an osmi a, t 999
8. Manfred i ~J et al: Superfici al side ros is o f th e ce n tra l
dem en tia & head ach e nervou s syste m and anticoagulan t thera py: a case repo rt.
o An osmi a: O lfacto ry nerve pa rticu larly sensiti ve to Ita l J Ne uro l Sci. 20( 41:2-17·9. 1999
hemosiderin d cposttton 9. weller ~1 ct al: Elevated C.~ F lncto ferrin in superficial
o Pre-sym ptomatic ph ase averages IS years side rosis of the cen tral nervou s system. J Neurol.
2-1 6( 10 ):94 3· 5, 1999
Demograp hics 10. Hsu WC ct al: Su pe rflcla l siderosis of th e eNS associa ted
• Age: Broad age range : 14-77 year s wit h m u lt ip le caver nous malfo rmat ions . A.INlt
• Gen de r: M :F = :1: 1 20( 7):1245 -8, 199 9
1I, An derso n ~ E ct al : Superflcl a l siderosis o f t he ce n t ra l
Natura l History & Prognosis n ervous system: a late co mplication o f cerebellar tumor s.
• Profound bil atera l SNl{L &. a ta xia wit h in )5 yea rs of Neurology, 1;52( 1): 163-9, 1999
o nse t 12. Schievi n k WI c t ..11: Surgical treat m ent o f su perficia l
• Deafness a lmost ce rta in if unrecogn ized side rosis associated with iI spin al a rte rio venous
m alform atio n. Case rep o rt. J Neuros urg. 89 (6):10 29 ·3 1,
• Z5% be come bed- bo und in years follo wing l st
1995
sym pto m 13. Lcmmer ttn g M ct al: Sec onda ry superficial sidl..'Josis o f th e
o Result of ce rebella r ataxia, m yelopath ic syn d ro me o r cen tral n ervous syste m in a patien t pr es ent in g with
both 0 -0 se nsorineu ra l h eari n g loss . Neu ror adiolo gy, -10(5 ):31 2·-1.
1995
Treatm ent 14, Matsu m oto S et al: Sp ina l m en ingeal m clan ocytorn a
• Trea t so urce of bleed ing pre..e nu ng wit h superficial si d N~i s o f th e cen t ral nervo us
• Surgi ca lly remov e so urce o f bleeding (su rgical cavity, sys tem . Case report and revie w of the lite ratu re. J
tumo r) Neu ro su rg. 88 (5):890·-1, 1998
• End o vascul a r t he ra py for AVM N. ane ur ysm IS. Castelli ML ct al: Su pe rficial sideros is o f th e ce nt ral ne rvo us
system: an u n de restim at ed ca use o f hear ing los s, J Laryng o l
• Coch lear im plan tatio n fo r SN lH.
0101. I I 1(1 »so-z. 199 7
16 . Irving RM et a l: Coc h lear im planta tio n in supcrnc ta !
sid cro vis. J Lary ngol Oto l. I 10(12):115 1·3, 199 6
IDIAGNOSTI C CHECKLIST 17. Tapscot t SJ ct al : Surgical m a na gement o f supe rficia l
sidl'cosis following cervical nerve roo t avuls io n . Ann
Conside r Ncurol. -10 (6 ):936·-10 , 1996
• Rem embe r t hat superficlalslde rosis is an effect, n o t a IX. Mau rizi CP: Superficial sid cros ts o f th e brai n: roles for
ca use cerebrospi na l flu id ci rcul atio n, iro n an d th e h ydroxyl
• Look fo r so u rce o f recurrent SAl{ so mewhe re in spine rad ical. Med Hyp o th eses. -1 7(4 ):261·4,1 996
I t). Offcnbacher H et ill: Su pe rficial siderosis o f th e cen t ral
or brain
n er vous system : !\IRI fin di ngs a nd cli nical signif ica nce.
• MR findings d o not co rrelate w ith th e sever ity of th e Neuro rad iol ogy 38 Suppl 1:SS1·6, 199 6
pa tien t's sy m p to ms 20 . Feamley J ~ I ct al: Supe rficial siderosis of th e ce n t ra l
o MR dt agn osts ma y be mad e in ab sence o f sym pto ms nervo us syste m . Brain. I IX: 105 1·66, 1995
Image Inte rpre tatio n Pearls
• e NS su rfaces incl ud ing cra n ial n erves appear "o utlin ed
i ll black " o n '1'2 & TZ'" MR images
IIMAGE GALLERY 1
45
(I.ef t) ;\ x;.11 T;r G Rr A1R ill ,

patien t with dlax ia end
bila/eral SN I IL shows inten se
JIf.'mo.~idf 'rin $caining of 111('
.~ tJrfiJ n· of tho n'w/}el/um (I ~
hyp(Jin/( 'f1R'. bllHJming
Slfl;WS (arr o ws) . (R iKh /J
Coronal rl · C Rt ,\ I R in
pat icilt lVith ~upe-rfida l
side rosis sh mvs low signal
alon g all d ural ami ce rt'hel/a r
sudan 's in Ihe posterior (OS .~.1
(.u ro w s), lh is reslJll _~ trotn
h ( 'mo.~hJ('rin staining
wU)fl d,)ry to chronic
suberecbnokt he morrhage.
Va riant
(/.£JI) Axial T2WI MR show»
juvenile pilocvtic
,Htrocytoma (arro w) Gwsinu
ch ron ic hefllOrrh l};<.' "
.~ up( ' dic·id f .~ id('rm i5 . O, )!>n
.u rnlV: /v fid hrain staining.
Curved arm w: m edial
temporal lobe staining .
(RiK" n AX;,ll T1WI MR in a
palimlwich j m -('ni /('
pi!ocytic d.5 /rocytom,1 .~ h { ) \v.~
sup<·rfici.l l ~ id('f( J.~;.~ tm m
chronic ~tJ/)clr,]('h noic/
he m orrh .lgc a.s . 1 dar/.. li/l(' on
the su rface oi the high
n >rvica/ ..p inal cord (arrow ).
Va riant
(/..eJI) A xial12WI AfR shows
large b" ..i/c1r lip c11lt'u ry sm
(,m o w) cau sing 5upl'did .11
sidorost« w /lw mmidl' rin ,,/ain
Oil POIl\ (or.wn amnv) (x
ac/jaee/lt tentorium cerebotti
(cur ved arrow) (Co lJl tes}' R.
Willian', MOJ. (Right) Axial
12 ~ VI AIR show s sic/efO, i." or
'i.LI/ }(' r ;fJf (('rf-> / )('II.l r .H l f l;l ("e s
(.UfOW). !ft>m o sidl>rin stain
d/~o marks ~ uda n~ o r
m idbrain ,H hypointenw limo
(ope n arrow). Patient hcl5
hasilclr lip clllC'urysm.
VASCULAR LOOP COMPRESSION, CPA-lAC
1
46
a \Iffy
Axial T2 WI MR shows vertebral artery (arrow ) in C(JI"()n,JI T2WI MR in J patient w ith left hemifacial
tortuous course impinging on deep cerelx'l!opontine spasm R'VCals the Jeft V(¥tebral artery (arrow)
angle in ared of fOo l exit zone of facial nerve . Symp tom : compressing the proxima! facia' nerve (open arrow) al
H em ifacial spasm. the fool exit zone .
• May impinge o n prox ima l 7t h eN or RExZ

ITERM INO LOGY (cau sing HFS)
Abb reviations a nd Syno nyms • Location
• Trigem ina l n eura lgia (TN), tic d ou loureu x, trig eminal o Vascular loop is high , an te rior CPA cistern alo ng
ner ve vascu la r loop synd rome , trigeminal nerve a rea of proxim al preganglionic segme n t of Sth eN
h yperacti ve dysfunction synd rome or at ItEnZ (TN)
• Hemifacial spasm (HFS), facia l n erve vascula r loop o Vascular loop is in mid- CPA cister n alo ng proxima l
syn d rome, facial n erve hyp eractive dy sfun cti on 71h CN or at ItExZ (HFS)
syn dro me • Size
o Offend ing vessels ran ge from tiny to fusiform
De finit ions ane u rysmal dilat ation o f vertebroba silar system
• Vascular loop co m pressing cra nial nerves (eN ) within o Very sma ll vessels may be sub -rad iologic in size
CPA-lAC cistern causing spas mod ic h yp erfuncti on • Morphol ogy
o Vascular loop comp resses 5t h eN at roo t en try zone o Asym me t ric vascu lar loop flatt en s proxim al e N S o r
(REnZ) results in tr igemi na l neuralgia 7
o Vascu lar loop co mpresses 7t h eN at roo t exit zone o Wh e n offen ding vessel is atherosclerot ic, a ppea rs as
(RExZ) results in h emi facia l spasm serpig inous, irregular vessel
• Offen d ing vessels
o Trigeminal neu ralgia
IIM AG IN G FINDINGS • Superio r cerebellar a rtery > posterior inferior
cerebellar a rte ry > ve rte brobasila r syste m
Ge ne ra l Feature s o Hemifacial spasm
• Best dia gn ostic clu e • An te rio r inferior cerebe lla r arte ry > posteri or
o High-resolution 1'2 MR shows serp igin ous inferi o r cere bellar artery > verteb ral artery
asym me t ric signal vo id (vessel) in CPA
• May im p inge on prox ima l prega ng lio ni c segme n t CT Findings
or REnZ of 51h CN (causing TN) • N EG '
o Co m mon ly n ormal
DDx: Vascular l esions of CPA
AVM , CPA Veno us Angi oma A neurysm, CPA

Key Facts
1
Te rmin ology • Arterioveno us m a lformatio n , C PA 47
• Vascular loo p com presses 5t h eN at roo t en try zone • Ven o us a ngioma , posteri or fossa
(REnZ) res u lts in t rigem in al n eura lgia • Aneu rys m , C PA
• Vascular lo o p com presses 7th eN at ro ot exit zo ne Clinica l Issu e s
(RExZ) resu lts in hem ifacial spas m • TN: Epi sodic la n cin ati n g pain fo llow in g V2 &'/o r V3
Imagin g Find ings d istribu tions
• High- reso lution T2 MR shows serp igin o us asy m me t ric • HFS: Un ila tera l in volun tary faci al spa sm s
sign al void (vessel) in C PA • Ne gati ve MR d o es n ot pr ecl ude ex p lo ra to ry surge ry
• Vascul ar lo o p is h igh , a nt e rio r C PA ciste rn al ong ar ea Diagn osti c Chec klist
o f pro xim al p rega ng lio n ic seg m en t o f St h eN o r a t • Th ere ar e m an y n o rm al vessels in d iffer ent par ts o f
REn Z (TN) C PA cist ern
• Vascu la r lo o p is in mid-C PA cis tern a lo ng p ro xima l • Close co rre la tio n be tw een sy m pto m atic CN &.
7th CN o r at RExZ (HFS) a sym metric vas cu la r lo o p im perative
Top Differential Diagn o ses
• Verte brobas ila r d oll choect asia (VBD)
o Vertebrobasilar loo p with high d ens ity, ca lcified o Begi n w ith w ho le b ra in T2 o r FLAIR seq ue nce to
vessels possibl e excl ud e m ultiple scleros is
• CECT o Fo llo w wi th axial &. coro na l Tl C+ im agin g o f
o Co m mo n ly n ormal br a instem, C PA ciste rn in cluding d eep face
o Vertebrobasila r lo op w it h in travascu lar • Look for asym m etric ven o us ca use
enhancement • Also loo k fo r cr an ial neurit is, peri neu ra l tu mor
a nd cist ernal tumor
MR Findin gs o High-reso lu ti o n t h in -sec t ion im ag ing o f brai n stc m
• TlWI Sr C PA cistern nex t
o Vessel usually not seen although contrast • Best seq ue nce to lo o k fo r o ffe n d ing a rte ry
enhancement may hel p o MRA fo cused t o poste rior foss a
o Visibility de pend ent o n size o f ve sse l &. n ow rate • Remem ber to view so u rce images be fo re
• T2WI reprojection im ag es
o High -reso lu t ion T2 o r T2 images o n 3'1' MR
• Vessel best see n as lo w sign a l tube cour sing
th roug h high sig n al CSF IDIFFERENTIAL DIAGNOSIS
• FLAIR
o Adjacen t br ai n m ost co m mon ly no rma l Vertebrobasilar d olich o e ct asia (VBD)
o Mul tip le scle ros is plaque in REnZ o r RExZ m ay • Com mon ath ero sclerot ic find in g in o ld pat ten t
p resen t with TN or II I'S • Tortuo u s, dil atatcd vcrt cb robaslla r syste m
• DWI • Rarel y ca uses vascu lar lo o p syn d ro me
o High sign al (rest ricted d iffu sion ) in C PA ciste rn
ma kes d iagn osis o f e pid ermoid Arteri ovenous malformation, C PA
• Cistern a l m asses may ca us e TN o r HFS • Muc h la rger vessels (art eries &. vei n s) wi t h n id u s
• Tl C + • Rar e in po sterio r fo ssa
o May e luci d ate ra re ve nous ca use o f tri ge m ina l
Veno us a ngio ma , post eri or fossa
neuralgia
o En h a ncing m en ing ioma o r sc h wan n o m a possib le • La rger vessel s (ve in s)
• Poste rio r fossa lo cati o n co m mo n b ut C PA rar e as
• Men in gea l m a ss or ad jacent sc h wa n n o ma may
drai n age ro ute
cau se TN o r ff FS
• Rarely ca n ca use ve nous com press io n wi th II FS or TN
• MRA resulti n g
o So u rce im ages m ost h elp ful
o Repro ject ed im ages h elpfu l for larger vessels Aneurysm , C PA
Ang iog ra p hic Fi nd ings • PICA o r vertebra l a rtery a n eur ysm
• Oval com plex -sig na l m ass
• Co n ve n t io n a l: Not h elp ful ; ca n not assess re lati o nsh ip
o f vessel to 5 th o r 7t h CN
Imaging Re commend ati ons I PATHOLOGY
• Best im ag in g too l
o T h in -sectio n h igh-reso lu tion T2 MR o f C PA a llows Gene ral Feature s
best vas cu lar loop visu alizat ion • Ge ne ra l pa th com men ts
o 3T MR w ill be be st too l fo r th is ima ging p ro blem o 5t h o r 7t h eN bu n dl e expe rie nce s "irr itat io n " fro m
• Prot o co l ad vice vessel
1 o Braln stcm nuclei seco nda rily affected
• Abn ormal hrainstem resp on se (ABR)
IDIAGNOSTIC CHE CKLI ST
48 • Etiology Con side r
o Multiple sclerosis h as been reported to cause TN or • Th er e a re man y n o rma l vessel s in differe nt parts o f
HFS CPA cistern
o Cist ern al ma sses such as ep idermoid or meni ngi oma • Close correlat io n bet ween sym pto m a tic eN «
ma y cause TN or HFS asym me tric vascular loo p imper ative
o "Kin d ling th eo ry"
• Vesse l co n tact resu lts in ecto pic excitatio n Image Interpretat io n Pearls
• Antid romi c impulses t ravel back to nucleu s • First look for ciste rn al mass lesio n s such as
• Reorganizati o n wit h in nu cleu s results in increased e pide rmo id, m eningioma o r sch wa n noma
d isch arge • Ne xt det ermine if so urce im ages for MRA o r
• Ilyperactivity tr avels o rth od ro m ica lly down 7th h igh-resolution T2 im ages ide n tify o ffen d ing vessel
nerve • Follow affected n erv e (Sth or 7t h eN) di sta lly into
• Epidem io logy: TN > IIFS; TN in cid en ce 1 per 100 ,000 deep face to excl ude neuriti s & perineural tumo r
• Negati ve MR does n ot p reclude su rg ica l ex plo rat io n in
Gro ss Path ologic & Surgical Feat ures clea r cut TN o r IIFS sell ing
• Offending vessel co mp resses REnZ (TN) or RExZ (HFS)
• Myelin co ver O il affect ed cran ial nerve is breac hed ISELECTED RE FERENC ES
1. Polo G ct a t: Bralnst e m auditory evoked potential
monitorin g during microvascular decompr ession for
IC LIN ICA L ISSU ES hemifacial spasm. Neurosurg. 54:9 7- 106, 2CXl4
2. Yoshino N et al: Trigeminal neuralgia: Evaluation of
Prese ntat ion neuralgic manifestations am i site of neur ovascul ar
co mpression with 3 D C1SS MR imaging and MR
• Most co m mo n sign s/sym pto ms angiography. Radiology. 22 8:53 9· 45, 2003
o TN: Episodi c la ncin ating pain fo llowin g V2 &/or V3 :1. Miwa H et al: familial hemifacial spasm: re port of cases
d istribution s and review of literatu re. J Nc u ro l Sci . I S; 193(2):9 7- 102,
o HI;S: Un ila te ral involuntar y facial spa sm s 2002
• O the r sign s/ sym pto m s 4. Tan NC et al: Hemifacial spasm and involuntary facial
o TN: Pa in spo n ta neo us or occ urs in resp onse to movements. QjM . 95(8):49 3· 500, 2(XJ2
ge n ti le tactile stim u la tio n of trigger point 5, Zakrzcwska J M: Diagnosis and differential dla gn osis o f
o Hem ifacial spa sm trigeminal neuralgia. Clin J Pain. 18(1):14-21, 2002
6. Just W H ct a l: Bot u lin um toxin: e vid ence-based med icine
• Begins with or bicu laris o cu li spas m s crite ria in blepharospasm and hemifacial spasm. J Neural.
• Ton ic-clonic bursts whi ch becom e co n sta n t o ver 248 1:2 1-4, 200 1
tim ~ 7. Moller AR: Vascular com pression of cranial nerves: II:
pathophysiology. Ncu ro l Res. 2 1(5):--439·43, 1999
Demographi cs 8. Herzog JA ct al: Vascular loops of the internal auditory
• Age: Older pati e nt s (usually greate r th an 65 yea rs) canal: a diagnostic dilemma. Am JO Wl. 18(1):26· 31 , 1997
• Ge nder : No ge n der spec ificity 9. Illingworth HI) et J I: He mifacial spasm: a pr ospecti ve
lo ng-term follow up of 83 cases treat ed by microvascular
Nat ural History & Prognosis decom p ress io n at two neurosurgical ce n tres in the United
• Pro gnosis Kingdom. ] Neura l Nc u ro su rg Psychiatry. 60(1):72-7, 1996
o Trigeminal n euralgia 10. Majoie e ll ct al: Trigemina l neu ropathy: evaluatio n with
• 70 1.11, pa in -free o n no m ed icati ons 10 yea rs aft er NtH imaging. Radiograp hies. I S(4):79S·KI I , 1995
microvascu lar decompression (MVU) 11. Ohashi Net al: Vascular c ross -co mpressi on of the VlIth
and Vllith cran ial nerves. J Laryngol Oto l. 106(5):43 6·9,
• If recurren t TN, h appens in 1st 2 yea rs afte r MVD
199 2
• 11M, have per manen t post-MVD co m plica t io n 12 . Darl o w LA ct a l: Magnetic resonance imaging in the
(un ila te ra l deafness mo st co m mo n ) diagnosis of trigem in a l neuralgia. .l Oral Maxillofac Sur g.
o f lem ifacial spasm 50(6 ):621·6, 1992
• 90% ac h ieve > 5 y~a r sym pto m reli ef with MVD 13. Parnes L\ et al: Vascular relationships of the
• 10% have perman ent post-oper at ive co m plica tio n vestl bulocochl car nerve on magnetic resonance imagi ng .
(u n ilate ra l dea fness) Am ] 01 0 1. 11(4):2 78-81 , 1990
14. Esfahani r et al: Air C f cisternography in the diagnosis of
Treatme nt vascular loop causing vestibular ne rve dysfunctio n. AjNR.
• So me pati ents ca n be managed co n servatively w ith 10(5):1045-9, 1989
I S. Haberman RS e t al: Fa lse-positi ve ~f RI and CT fincJings of
drug t her apy
an acoustic neuro ma. Am] Otol. 10(4):301·3, 1989
• MVD used wh en sym pto m s are di sahling in spite of
drug ther ap y
o Ra rely ot her causes of TN & HFS are identified
• Th ese include mu ltiple scle ro sis, ciste rn tu mor ,
n eu ritis « perineura l tu mo r
• Negati ve MR d oes n ot pr eclude exp lo rato ry surgery
IIM AGE GALLERY 1
49
(I.el" Axi.II MRA !oh(,., ,,~

asymmetrically I,JTge " 'CA
loop (arrow) knuckling into
rlw .J1t>d of the f oot (·.... it rone
0; the [ac;al ner ve. A t >ul ge, y
this loop \\'') 5 {('It to be the
cause of patient 's hem ifacial
sPJsm. (RiI: lrl) M RA
fl'projlXlion few.l/ s .m
.,symmetrically I.]fge AIO\
loop (arrow) thaI hilS if sh arp
bend at .lpproximaldy the
.m·,) of the C1~ . Source
imdge:.showt"d the '0'('55('/5
impinged on the root exit
zone of ,hf' facial tlt'T W .
Typical
(/A~ft) A'I(;,ll MRA sour ce
im ag<' in / MtierH with figh t
I fFS sho lVs a /or tuou> fight
VA (arrow ) & 'JSsoci.llt-d
PICA (r )/ )('o ,mflw) p ush ing
on root entr y Z O f}L' of f.leia'
(lpn'I' , CUf vt·c/ arrow: r.le/al
rlf>fW in CPA cistom. (RighI)
An lt'mpmtt'rior MRA
f"/HOj('Ct;oll sho ws how .J
(,,(/linus righ t VA (J r (() w}
Cdn d t'Vdtt' PICA loop (opt'n
Jfftnv ) in CPA cistern, In thi!>
c.m> VII -PICA comple'(
ColUS('C!symp tom of
hemifJd J/ ·\I )"l5m .
(Lef t) Axial T2Wf AIR in this

p.1tit>nt with right trigcm ind!
neur algi;, shol.'o's tht· sUfX·rior
cetebeltsr drter y (arrow)
co mpressing the
pn_·g.mglion ic segment of tbe
"igt'minJI ner ve (open
Jrrm'o'). (Rig" ') Coronal
fl Wf AIR sho ws flattened
eN5 root entry 700(' (open
.m ow) . Pst iem 's trigem inal
neuralgia was w condary 10
comp ressing combina tio n of
sup erio r cc-cbctler artery
(a rrow ) & PICII (cu r ved
arrow).
II;;::: L
SECTION 2: Temporal Bone

Introdu ction and Overview Inn er Ear, Infection & Inflammati on
Tem poral Bo ne Ana tomy an d Imaging Issues I-Z-Z Labyrin th itis 1-2- 112
Otosy p h ilis 1-2-116
Exte rna l Auditory Canal Labyrin th in e Osslfica ns I-Z-118
EAC Atresia I-Z-6
Necrotiz ing Ext ern al Oti t is I-Z-IO Inner Ear, Tumors
EAC Cholesteatoma I-Z-14 In tralabyrin thi n c Schwa n nom a 1-2-122
EAC Keratosis O bt uran s I-Z-16 En dc lym pha tic Sac Tu mo r 1-2-126
EAC Medi al Can al Fibrosis I-Z-18
EAC Exostoses I-Z-ZZ Inn er Ear, Miscellaneous
EAC Osteo ma I-Z-Z4 Coc h lear Im pla n t 1-2- 130
EAC Squa mous Cell Ca rci no ma I-Z-Z6 In tralabyrin th in e Hem or rh age 1-2-134
Sem icircu la r Ca na l Dehiscen ce 1-2- 136
Middle Ear-Mastoid, Con genital Fen estr al Oto sclero sis 1-2-138
Con ge n ita l Ch olestea toma , Midd le Ear I-Z-30 Coch lear Oto sclero sis I-Z-14Z
Oval Window Atresia I-Z-34
Lateralized Intern al Ca rotid Artery I-Z-36 Petrou s Apex
Aberr ant Internal Carotid Art ery 1-2-38 Asym m et ric Marrow, Pet rou s Apex 1-2-146
Persistent Staped ial Arte ry I-Z-4Z Suba rcuate Artery Pseudoleslo n 1-2-148
Ch o lesteatom a, Pet rous Ape x 1-2-150
M iddle Ear-M ast oid, Infection & Pet rou s Apex Ce ph aloc ele 1-2-154
Inflammation Apical Petrosit is 1-2-158
AOM Wit h Coa lescen t Otom asto iditis I-Z-44 Trapped Fluid, Pet ro us Apex 1-2- 162
AOM Wit h Abscess I-Z-48 Ch oleste rol Gran ulom a, Petrous Apex 1-2-166
COM Wit h Ossicular Erosio n s I-Z-5Z ICA Ane u rysm, Petrous Apex 1-2-170
COM Wit h Tym pan oscle rosis I-Z-56 Pet rou s Apex Metastasis 1-2-174
Acqu ired Cho lesteato ma, Pars Haccida I-Z-60
Acqu ired Ch o lestea to ma, Pars Tensa I-Z-64
Intratemporal Facial Nerve, Variants
Acqui red Ch olestea to ma, Mur al I-Z-68 Facial Nerve En han cem ent, Intrat empo ral 1-2- 176
Cho leste rol Gra nu lo ma, Middl e Ear I-Z-70 Prolap sing Facial Nerve, Middl e Ear 1·2-178
Middle Ea r-Mas toid, Tum ors Intratemporal Facial Nerve, Infecti on

Middl e Ear Aden om a I-Z-74 Bell Palsy 1-2-180
Midd le Ear Sch wan no mu 1-2-76
Midd le Ear Men in gioma 1-2-7R Intratemporal Facial Nerve, Tum ors
Glom us Tympa n icu m Paraga n gliom a 1-2-R2 Facia l Nerve Sch wa n no m a, T-Bon e 1-2- 1H4
Midd le F.1r Rhabdo m yosa rcoma I-Z-86 Facia l Nerve Hem a ngio ma, T-Bone 1-2- 18R
Per in eur al Pa rotid Malign an cy, T-Bone 1-2- 192
Middle Ear-Masto id, Miscell aneou s
Post-O pera tive Ce pha locele, Middle Ear I-Z-90 T-Bon e Lesion s Without Spe cific
Ossicular Prost hesis 1-2-92 An atomic Loc ati on
Tempo ral Bo ne f ractures 1-2- 196
Inner Ea r, Co ngenita l CSF Leak, T-Ilo ne 1-2-200
Lab yrinthi ne Ap lasia I-Z-96 Fib rou s Dysp lasia, T-Bon e 1-2-202
Co ch lea r Ap lasia, Inner Ea r I-Z-98 Paget Disease, T-Bone 1-2-Z06
Co m mo n Cavity, In ne r Ea r I-Z- 100 Osteo pet rosis, T- Bone 1-2-ZIO
Cys t!c Coc h leoves t ibula r Anom aly I-Z- I02 Post irradiated 'l-Bone 1-2-Z12
Sem icircu lar Can a l Dysplasia I-Z- 104
Large Endolymphat ic Sac Anoma ly 1-2- 108
TEMPORAL BONE ANATOMY AND IMAGING ISSUES
2
2
Axial graphic through low lAC (arrow) shows coch lear Axial l-eone O ' through low mcsotympanum shows
ner ve anteriorly & inferior vestib ular ner ve fXJsteriorl y. no rmal manubrium of ma fleu.~ (arrow ) &
Descending eN7 (open arrow ) & horizontal petrotJs incudosta,x>dial snkute tioo (open arrow), Basal tum of
10\ (cur ved arrow) a/50 seen. cochlea end at roun d w imkJlv (cur ved arrow ),
o Mad e up of tym panic bo ne m ed ially, fib rocartilage

ITE RM INO LO GY laterally
Abbreviations and Synonyms a Media l bord er EAC: For med by tym panic memb rane
• Abbr evia tion : Temporal bon e (T-bone ) th at attaches to scu tu m superio rly & tymp an ic
• Syno nym : Pet rou s temporal bo ne an n ulus infe riorly
o Nod al d rainage of EAC 8< ad jacen t scaip is to pa rotid
Definitions nod es
• T-bo ne: Paired lat eral bo ne s of skull base primarily • Middle car-mas toid
ma de up of pet rou s pyram id & mastoid co m plex o Epitympa n um (att ic): Tegmen tym pan i is roof wit h
floo r defin ed hy lin e bet ween scut u m & tympanic
segmen t of facial nerve
IIM AGING ANATOMY • Prussak space = lateral epit ympan ic recess
• Tegmen tympan i (I.. for "roof of cavity"): Thin
Anatomic Relationships bo n y roof betwe en epitym pan um 8< middle
• Int ern al organi zation cran ial fossa du ra
o 5 bo n y pa rts to T-bone o Mesotym pa n u m: Midd le ear area between line
• Squamo us: Form s lateral wall of middle cran ial between scutu m & tympani c segme n t o f facial nerve
fossa abov e and lin e between t ym panic annu lus & base of
• Mastoid: Postn atal develo pment of postero in ferior coc hlear pro mont o ry belo w
ma stoid • Posterior wall} mesotym panum h as 3 impo rtant
• Pet rou s: Con ta ins in n er ear, lAC, pet rous ap ex st ructures: Facial nerve recess, pyram ida l
• Tym panic: V-sha ped bo ne forming most of bony eminen ce & sin us tym pani
extern al ea r • Med ial wa ll: Lateral sem icircular canal, tympa nic
• Styloid : Form s styloid process after birt h segme n t facial nerve, ova l & round wind ow found
o Majo r co mponen ts of 'l-bo ne here
• Exte rna l aud itory ca na l (EAC) o Hypo tym panu m : Sha ilow tro ugh in floor of midd le
• Middle ear -masto id (ME-M) ear cav ity
• Inner ear o Mastoid sinus: 3 impo rtan t st ruc t ures
• Petrous apex • Ad itus ad ant rum (L. for "en t rance to cave") :
• Facia l ne rve Co n ne cts epity mpanu m o f mid dle ear to mastoid
• Adjacen t anatomy-bou nd aries antrum
o Sp he no id bone an te rior • Mastoid an t ru m (L. fo r "cave"): Large, cen tral
o Occipital bone poster ior & media l mastoid air cell
o Parietal bone su perio r & lat eral • Korner septu m : Part of petrosq uamosal sutu re
ru nn ing postero lateral ly t hrough mastoid ai r cells
Location • Inner ear
• Located in posterolat eral floor of m iddle cranial fossa o Membranou s lab yrinth ; fluid spac es within bony
Inte rnal Structu res -C ritica l Co nte nts labyrinth
• Externa l audito ry canal • Fluid & so ft tissues wit hin vestibule (utricle &
saccule), sem icircular canals & coch lea
• Endolympha tic duc t 8< sac: Con tains end olymp h
DIFFERE NTI AL DIAGNOSIS

M iddle ear-masto id Pet ro us apex
• Conge n ital cho lesteato ma, ME-M • Congenita l cholesteato ma, PA
• Acquired cholestea to ma , ME-M • PA cepha loce le
• Cho lesterol gran ulo ma, ME-M • Trapped fluid , pA
•
•
Adeno ma, ME
Glom us tympan icum paragan glioma
•
•
Apical pet rositi s
Ch olesterol gran u loma, I'A 2
• Rhabdo myosa rcoma, ME
In t ratem poral facial nerve 3
Inn er ear • Herpet ic faclal n euritis (Bell pa lsy)
• Labyr int h ine dysplaslas • Facial n erve h eman gioma, T-bo ne
• Large endo lymp hat ic sac an om aly • Facial n erve schwa nno rna, T..bo ne
• I.abyrinthi tis &; labyr int b ine ossif tcans • Perin eural parotid malig nancy, T-bon e
• In tralabyrin th in e schwa nnoma
• Endo lym p ha tic sac tu mor Lesion s w it ho ut anato mic location
• Superior semici rcular canal deh iscence • Fibro us dys p lasia, T-bone
• Fenes tral & coc h lear otosclerosis • Paget d isease, 'l-bon e
• Osteopetrosis, 'l-bo ne
• Coch lea r duct: Contains perilym ph • Poster ior gen u

o Bony labyrinth • Mastoid segme n t: Leav es posterior genu to pa ss
• Bone co n fin ing coch lear, vestibule & semicircular in feriorl y to stylom astoid forame n; t st gives off
cana ls mot or n erv e to sta ped ius muscle, th en chorda
• Bony vestibular & coc h lea r aqueducts tympani nerve
o Coc h lea • Sty lo mas to id foramen: facial nerv e exits sku ll base
• Co n ta ins - 2 'h turns here
• Mod io lus = cen t ral bon y axis of coc h lea: Hou ses o CN? com po ne n ts
spiral gang lion (cell bod ies o f coc h lea r n erve) • Mot or roo t: Innervates mu scles o f facial
• 3 spiral ch am bers o f coc h lea: Scala tympani ex p ression, staped ius, platys ma &. po sterio r be lly
(pos te rior cha m be r), scala vest tbult (an te rio r of d igastric mu scles
cha m ber) & scala media (co n tains Organ o f Co rti • Nervus interm edius: Sensory (spec ial visceral
= hearin g apparatus) afferent fibers of an terio r 2/3 o f tongue ta ste) &.
o Sem icircu lar ca n als (SCC), su pe rio r (S), lateral (I.) &; parasympathetic (gene ral visce ral effe ren t
poster ior (1') secre tomoto r fibers to lacrim al, sub ma nd ibu lar &.
• SSCC: Projects ceph alad; bon y ridge ove r SSCC in sublingua l glands) root
roof of pet rou s pyramid called ARCUATE o CN? branch es-fun ctions
EMINENCE • Great er su perficia l pet ro sal n erve: Lacrimation
• LSe C: Projects into middle ea r with tym pani c • Stap edius n erve: Stap edius refle x == sound
CN? o n under side dampening
• pSCC: Projects posteriorl y alo ng petrou s ridge • Cho rda tympa ni nerve: Ante rior 2/3 o f tongue
• Cru s co m m un is: Co m mo n orig in of all 3 sces taste via lin gu al nerve
• Pet rou s ap ex (PA) • Mo to r bran ch es: Muscles of facial ex p ressio n
o Definition: Ant eromedi al to inner ear &. lat eral to • Muscles o f T-bone
petrooccipital fissur e o Ten sor tympani mu scle
o _ 33 1M) normall y pn eumati zed • Functi on: Dampens sou n d
o CN6 passes on media l sur face of PA • Dysfuncti on: Hyp eracusis
o CNS passes through porus tri geminus in to Meckel • Inn ervati on: V3 bran ch
cave o n cepha lad-media l surface of PA • Locati on: An tc rome d ial wall, mesot ympanum
• Pet rou s internal carotid artery (lCA) • Attachment: Tendon goes through coc h lea riform
o le A: Ver tica l &. horizontal segm ents in petrous process, turns lat erally to attach on man ub rium of
T-bone malleu s
• Verti cal segme n t: Rises to genu ben eat h coc h lea o Sta ped ius mu scle
• Hori zontal segmen t : Pro jects anteromedi ally to • Functi on: Dam pe ns so un d
turn ceph alad as cavernous segme n t • Dysfuncti on : Hyp eracusis
• Intra temporal facial n erve (CN ?) • Innervation: eN?
o CN? segme n ts • Locati on: Muscle belly in pyram ida l e mine nce
• lAC segme n t: Ante ros upe rior locat ion wit h in lAC • Attachment: Tendon attac hes o n head of sta pes
• Laby rin t h ine segme n t: lAC fundal ex it to • Bones
gen iculate gang lion o Ossicles of middle ea r
• Ge nic u late ganglion = an terior gen u • Mall eu s: An te rior ossicle; umbo, manubrium, h ead
• Tym pa n ic segme n t: Leaves gen icu late ga ng lion, • Incus: Poster ior oss icle; sh o rt pro cess, bod y, long
passes under latera l SCC p rocess, lenticular pro cess
2
4
Coronal graphic reveals cartilaginous & bony eaemet Coronal graphic illustrates 3 ossicle chain from
auditory canal, middle ear & inner ear. Notice antt'rolateral mallcus (arrow), lhrough incus to
Ixmy-cartilaginou5 j unction of El\C (arrow) , site of incurloStdpf0ial articulation (open arrow ). Stapes hub
drainage of some 1stbranchial cleftcysts. leads /0 2 crura and footplare (curved arrow) .
• Stapes: Med ial ossicl e; hub, crura , footplate • Perilymphat ic space & otic capsu le form s from
surrou n ding mesen chym e
IANATOMY-BASED IMAGING ISSUES I Practical Implicat ion s

• In EAe at resia, inner ear is spared since it fo rm s from
Key Co ncepts o r Question s m igratio n of oto cyst ind ep end ent from 1st & 2nd
• Assign lesion to o ne o f foll owing T-bo ne areas: EAC, bran ch ial groo ve-po uch-arch interact io n
ME-M, inner ea r, PA o r or int rate m pora l eN ? • Inner ear ano ma lies in most cases for m witho ut
o Co nstruct differential diagn osis based on specific EAe -m idd le ear anoma lies
an at om ic locati on
o Matc h imaging cha racte ristics to dif feren tial
diagn osis list ICUSTOM DIFFERENTIAL DIAGNOSIS I
o Always check en tire in t ratempo ral eN? co urse
Ret rotympanic "vascular" lesions
Normal Measuremen ts • Conge n ital
• Bony vestibu lar aq ued uct tra nsverse d iam eter .$ 1.5 o Aberran t int ern al carotid art ery
mm o Persistent stapedia l artery
• Coch lear turns: 2.5-2.75 o Deh iscen t jugul ar bulb
• Inflam mator y
o Hemorrh agic Infl am mation of ME-M
ICLIN ICAt IMPLICATIONS o Cho lestero l gran uloma, ME-M
• Ben ign tum or
Clinical Importa nce o Glom us tym pa ni cu m paragan glioma
• Facial n erve win ds through 'l-bone o Glom us jugul are paraganglioma
o Injury at an y poin t results in peripheral facial nerve o Men ing ioma, ME-M
paralysis
• EAC-tym panic membran e-ossicles-oval window
o Co n d uctive ch ain di sru pti on resu lts in con ductive ISELECTED REFERENCES
hearing loss
1. Silver RD et al: High -resolu tion magnetic reson ance
• Coch lea-mod iolus-coch lear n erve ima ging of human coch lea. Laryngoscop e. 112:1737-41,
o Lesion s p resen t as unilat eral senso rine u ral heari ng 2002
loss 2. Gu nlock MG et al: Ana tomy of the tempora l bone.
Neuro imaging Clin NA 8:195-209, 1998
3. Viraspongc C et al: Computed tomograp hy of temporal
IEMBRYOLOGY bon e pn euma tization : 2. Pctro squamosal suture and
septum. AJNR. 6:561-8, 1985
Emb ryologic Events 4. v rrasponge C ct al: Computed tom ograph y of tempo ral
• EAe fo rms fro m 1st bran ch ial gro ove bo ne pn eumat ization: 1. Normal pattern and morphology.
AJNR. 6:551-9, 1985
• Tym pa nic cavi ty forms from 1st bra nc hia l pouch 5. Swart z ]D : High -resolution computed tom ograp hy of the
• Ossicles form from 1st & 2nd br an chi al arch midd le ear and mastoid . Radiology. 148:449-54, 1983
• Endolym p ha tic system form s from otocyst
IIM AG E GALLERY
No rmal
(/.£1' ) I h i,)! T·!xmt' C T
IhrouRh cpnvmpsnum sho ws
m.ll1(·IJ_~ h('.ld (.m ow) r.
.m /(Jrior to incus Sh Of t
Pf(x:f's .~ (open ,J( fOW ) .
PrU SS,lk .~ p.1 C(' "" !<u£>ral
ep icymp.l num «('t / f\'eeI
,u f mv). I ymp -snic wgm"-'fJl
eN? ob viou s. (Rin1JO 1'''':;,)/
T-bone ( 1 through
me50ty mp J num show..
posterior \\'.111 sinus t )'mp.mi
(arro w) , p}',.lmid,l/l~m;n t'nn·
(open dffOW) ll\ mastoid
e N 7 (curved .lUOW, .
An[{'rior nS<iiclf' = m"l/l'uS.
Posterior ossi( It · = incu s.
No rma l
Coronet T-bone ( T
(/.('JI)
through ova/ window show..;
10l1g process or incus (J rr o w )
S. !< 'fJ/;cu /af p ro cess (opon
arro w). N otice ebsenc e of
IXHJ t' evide nt in normal oval
window niche (curved
,U((JW). ( Hig ht) Co ro nal
T-Iw flt , cr thro ugh anterior
middle ('J( shOlvs malleus
(.lfm w) . f.l h yrin th im> (0 fJf.'n
.,rrow) \~ tymp.m ic (ClUHV
." row ) f.l ci.11 nerve
SL'WJlf.-'nls. N o lin ' horizon tal
pdrouo; leA below coc hlea.
Normal
( 1.£/ 1) Sagitt,11graphic shows
CN7. i .Krimal n('rv('
(ar(Ow ). St.lpt·di al n('f\'('
(oocn olr (() w ) . Cho rd.1
tymp.lIli nL'f\'!-' {rur vv d
arrow). Yellow: Moto r.
O range : Anrt'rior i/J tongul"'
I.Hl t',· C fl 'f.'tl :
f'arasymp .Jthf,tin. (Hig" t)
No rmal venicsl &. horizonral
petrous leA within bone.
A rro ,,': Inft'rim tymp .m ic
arll'fy in c a/l.l1inJlu ~ . Op(-'n
arrow : Caroticotymp.mic
aft t"fy. Curve-d arrow : M id d!t,
m eningeal .If /pr y in foram£'fl
spinosum.
EAC ATRESIA
Coronal graphic of external ear _~hO\V5 deformed auricle Coronal t-bone CT shows severe CAC atresiJ with small
& lxm y EAe <If,( ~.~i.l (a rro w). Ossicutar fusion 8., rotation m iddle ('.lr c.wity. Note lateral anomalous facial nerve
with OV<ll window ;]i"2s;a (open arrow) are .1/$0 p( 'S('nt. coarse (arrow). lnner ear i.~ normal.
ITERM IN O LO GY CT Findin gs
• NECT
Abbrevi ati ons a nd Syno nyms o Ext ern al ear & EAC
• Abbrevia tio n : Extern al a ud itory ca na l atresia (EACA) • Sma ll, dysmo rp h ic pinna
• Syn on ym s: Co nge n ita l aura l dysp lasia, m icrot ia • Bony, so ft tissue, or mi xed ste nosis of en tire EAC,
Defin itions includi ng memhranou s Sr bon y port ion s
• EACA: Part ial or co m p lete atresia of EAC wit h o Midd le ear findin gs de pen d on severity of a tresia
associa ted a u ricle (externa l car) defo rm ity • Sm all m idd le ca r
• Fusion & rotation o f m alleus &. in cus
• Dysm orphlc m alleu s & in cus
IIM AG ING FINDINGS • Fu sio n of m alleo la r-in cudal a rt iculatio n
• Oval windo w at resia m ay be associated
Ge ne ral Features • Norma l m or pho log y « locat ion of stapes
• Best diagn ostic clue: Absen t EAC becau se of bon e o r important fo r su rgical reco n struction o f ossicular
soft tissue canal oblite ratio n fun cti o n
• Loca tio n • Congen ita l cholesteatom a beh ind at resia plate «
o EAC, m idd le ear & masto id co m p lex l OI}'cJ)
o Inner ea r usua lly spared o Facia l ner ve ca na l findin gs
• Size: Stenosis usually extend s from exte rn al opening of • Aberra n t cou rse o f tympan ic &: ma stoid por tions
can al to tym panic m em bran e of facial n erve com m on
• Mo rphology • Tym pa ni c segme n t may be delu scen t, ove rlying
o Dysp lasti c auricle (m icro tia) o va l o r roun d win dow s
o Mildest form ha s narrow ed EAC • Masto id segm en t usua lly a n te rio rly di splaced
o Mo re sev ere has no identi fiab le EAC • May exit skull base in to gleno id fossa, o r lateral to
o Hyp oplastic m iddl e ea r cavity & masto id co m p lex styloid process
o Dysm orphic ossicular cha in , especia lly m alleu s « o Inner ca r find in gs
in cus • In ner ea r &: lAC usuall y n ormal
DDx: Abnormal EAC

~~---
Exostosis Osteoma EAe Cho lesteatoma EAC Foreign Body

EAC ATRESIA
Key Facts
Term inology Top Differential Diagn o ses
• Abbrev iat io n : Ex ternal audit ory ca na l at resia (EAC A) • EAC osteo ma
• Syno nyms: Congenital aura l dysplasia, m icrotia • EAC cho lesteato ma
• EACA: Part ial o r co m p lete a tresia o f EAC wit h • Fo reign bod y
associated aur icle (ex tern al ear) d eform ity
Pathology 2
Imaging Find ings • EAC m ay be narrowed o r sten o sed , or co m p lete ly
• Small, dysmo rp hic pin na atret ic 7
• Bon y, soft tissue, o r mi xed ste nosis of en tire EAC, • Failure of canalizatio n lead s to EAC at resia
including mem brano us &. bon y port io ns • Inn er ear for ms earlier dur ing gestation, so ano malies
• Sma ll m iddle ea r of labyrin th & lAC rarely associated with EAC atr esia
Fusion &. ro ta tio n o f ma lleus &. in cu s
• Co n ge n ita l ch o les tea to m a be hi nd a tresia plat e « Diagn osti c Checklist
10%) • Seve rity o f au ricular d ysplasia parallels degree of
• Abe rra n t co urs e o f ty m pan ic & ma st oid por t ions of deformity o f m idd le ear &. osslclcs
facial nerve co m m o n
• Me mb ra no us atresia ch a rac te rized by ca rt ilag in ous

MR Findings plug in ca nal
• Un necessa ry o Emb ryolo gy-a natom y
• Of use o n ly if la rge co ngen ita l choles te a to m a • t st & 2 nd bra nc hia l arc hes & 1st pha ryngea l
Imaging Re commend ati ons pouc h d evelo p at sa m e tim e dur in g e m bryoge nests
• Associated m id d le ca r &. m a sto id a no ma lies a re
• High -resolut io n axia l &. coro na l pl a ne hone CT is best
co m mo n ly seen wit h au ricu lar d yspl asia & EAC
Im aging approach
a tre sia
• Bra nch ial groove & 1st phar yn gea l pouch giv<.' rise
to EAC
IDIFFERENTIA l DIAGNOSIS • In it ia lly, co re o f ep it he lia l ce lls a re so lid in futu re
Acq uire d EAC stenosis (surfe r's ea r) EAC location
• Usua lly bilate ral • In thi rd tr im ester , co re o f ce lls canali ze to form
• Exostosis o f EAC, o fte n pr esenti ng wit h h isto ry o f co ld EAC
wa te r swim m in g o r o t her lo ca l EAC tra uma • Failu re o f ca na liza t io n leads to EAC a tre sia
• Usua lly pr ese nt s later in life • 1st b ra n ch ial arch for ms ma lleus h ead , incus body
• Au ricle is norma l &. short p ro cess & te n sor ty m pa n i ten d on
• 2nd br an ch ial ar ch forms m an ub rium o f ma lle us ,
EAC os te o ma lo ng p ro cess of inc us , sta pes (excep t tootplate) &.
• Usua lly unila te ral stapedia l m uscle a nd te ndo n
• Ben ign bony growt h o blite ra ting EAC • In ner car fo rm s car tic r du ring ges ta tio n , so
anomalies o f labyr in th & l AC rarel y associa te d
EAC cho leste a to ma wit h EAC atresia
• Un ilateral wit h no rm al auric le • Gene tic s
• Soft ti ssue mass p rotrudes in EAC o 14 1}\"1 have positive p rio r fam ily hi sto ry
• Unde rly in g bony EAC sca llo ping o May be assoc iated w ith In h erit ed sy ndro mes
• May h av e bone frag men ts in so ft ti ssue m ass • Cro uzo n, Go lden har or Pierre Roh in synd ro mes
For eign body • Etiol o gy
o Etiol ogy o f m a lfo rmati on is p resumed to be in uter o
• Ma y sec su rgica l packi ng in ad ults
in sult
• Sm all to ys, bean s & beads in ch ildren
o Epit helial cells o f l st branch ial groove fail to split k
ca n alize , resulting in a tr esia
• Epide m iology
IPATHOLOGY o I in 10,000 birth s
Ge ne ra l Fe ature s o Bo n y > > m em b ran o us atresia
• Ge ne ral path co m me nts • Associa ted ab norma litie s
o No n -sy ndro m a t EA C a tres ia u suall y un ilat e ral o In n er ea r an o maly o ccu rs in 10%
o Bilat e ral atr esia co mmo n whe n EAC ma lfor matio n is o May be iso lated ma lfo rmat io n, or part of
sy nd ro ma l cra n ia l-fac ial syndro m e
o Atresia is mem b rano us. bon y or a m ix of two Gross Path ol ogic & Surgica l Features
• EAC m ay he na rro wed o r stenosed , or co mpletely • Pinn a is malformed & abnorma lly po sit io ned
atr et ic
EAC ATRESIA
• Atresia plate ca n be mem b ran ou s o r bony and o f • Seve rity of a uricu la r dyspl asia parallels degree o f
va riable thi ckn ess deformity o f middle ea r &. ossicles
• T-bo ne cr later in life to exclude assoc iated
Microscopic Feat ures cholestea to ma
• Co n gen ital cholesteato ma may occ ur in rudim entar y
m idd le ear cav ity Image Inte rpretat io n Pea rls
• Pre-o pera tive T-bo n e CT checklist esse n tial fo r surgi cal
Stag ing, Grading o r Classification Crite ria
2 • Mild a noma ly may have nor mal pin na, mini ma l
pla n n ing
o Type (bo ny o r m emb ran ou s) &. th ickn ess o f at resia
8 defo rmity o f malleus & incus, hypop lastic middl e ea r plat e
cavity o Size o f mastoid co m plex &. middle ea r cavity helps
• Mod erat e malfor mation ha s rudimentar y a uricle, m OTC det erm in e su rgical app roach
severe ossicu la r a nomalies &. abe rran t facial n erve o Status of ossicu lar cha in, includ in g presence,
course mor ph ol ogy, &. fusion to late ral m iddl e ear cavity
• Severe an o maly may have no pinna, rudim e nt ar y wa ll
midd le ea r cleft, a bsen t ossicles &. in ne r ear o Ca refu lly assess malleo in cuda l a nd in cudostap edi al
malfo rmati o n s arti culati ons
o Sta tus o f oval wi nd ow &. sta pes inspected for oval
window atresia
IC LIN ICA L ISSUES o Trace cou rse of facia l nerv e, as abe rra n t nerv e may
be at risk du ring surgery
Presentati on o Hypoplastic o r ap lastic lAC deficient coc h lea r nerve,
• Most co m mon signs/sym pto ms a surgica l contrai ndi cati o n
o Co nd uctive hea ring loss = most co mm o n sym ptom
o Phys ical exam
• Dys plasti c auricle
• EAC is absen t o r steno t ic
• Clin ical profile: For unk now n reasons, occurs more I. Blevi ns NH et al: Exter nal aud itory canal d uplication
commonly in righ t ea r an om alies associated with congenital aural atr esia. J
I..ary n go l 0 101. !I 7( 1)::Q·H, 200]
Demographics 2. Kllnge biel R et al: Multisllce co m puted to mog raph ic
imaging in tem po ral bo ne dy splasia. Ot o l Ncu ro tol .
• Age: Con gen ita l lesion 23(5):7 15-22,2002
• Gen de r: Occurs more co m mo n ly in ma les :I. Ben to n C et al: lm agtng of co nge n ita l ano ma lies of th e
temporal bo ne . Ncuroimaging Clin r\ Am. 1O( 1):3S-S:{ ,
Nat ural Histor y & Prognosis vii-viii, 2000
• Status at b irt h rem ain s u nc ha nged th rough life, unless 4. Calzclart F ct al: Clinica l an d rad iological evalua tio n in
t here is associated middle car cholesteato ma ch ildre n with microtia. Br J Audi ol . 33(S):303·12, 1999
• In u n ilate ral atresia, ot he r ea r has n or mal hea rin g 5. Dcclau F et 31: Diagnosis and man agement strategies In
• Bilateral atr esia may p resen t as bilateral co nductive congen ita l at resia of th e ex terna l aud itory ca nal. Study
hea ring loss Grou p o n Oto logica l Malformati ons and Hearing
o Afte r surgery, hea ring is adequate but gen erally n ot Im pai rmen t. Br J Audiol. 33(5):3 13-27. 1999
6. Selesn lck S et 01 1: Surgical trea tment of acqui red exte rna l
n o rmal
aud itory canal atresia. Am J Oto l. 19(2):123·30, 1998
• Au ricle reco nstru ctio n may requ ire 4· 5 staged su rgeries 7. Mayer TE et al: High-reso lut io n cr of the tem pora l bone in
Treatm ent dysplasia of the auricle an d externa l au d itory ca na l. AJNR
Am J Neurorad iol. 18(1):53-65, 199 7
• Un ilateral at resia n ot t reated if ot he r car is nor mal 8. Nish lzaki K et al: A co m puter-assisted o peration for
• Co smetic recon struction of au ricle d ysplasia usually congenital aural malfor matio ns. lnt J Pediatr
occ u rs in adolescen ce Ot or hino laryngo l. 36 (1):3 1-7, 1996
• Cou rse of facia l n erve, status o f oval window &. inner 9. Cha nd rasekha r S5 et al: Surg ery of conge nital au ral at resia.
ea r sh ould be estab lished by cr prio r to surge ry Am ) 01 01. 16(6):713-7, 199.\
• Bilateral at resia is treated a t 5-6 years o f age, wh en 10. j ah rsdoerter RA et al: Gradi ng system for th e selectio n of
head h as reached 90% of ad ult size patients with co ngeni ta l au ral atresia. Am J 0 101.
13(1):6-12,1992
o Reco nstructio n o f auricle preced es surgi cal t reat me n t 11. Andr e,..-s JC et al: Th ree -di men sion al cr scan
o f middle ea r &. ossicula r defo rmi ties reco nstructio n fo r the assessme nt of co ngen ital aural
o Su rgical reconstruct ion o n side wit h mildest EAC atres ia. Am ) O to l. 13(31:236-40, 199 2
atresia 12. Karh uketo TS et al: Vlsualizatlo n of the middle ear with
o Both auri cles a re repa ired fo r co sme tic reaso ns hi gh resoluti on co m puted tomography and supe rfine
fiberop tlc video m icrocn doscopy. F.ur Arch
Otor h ino lary ngol. 255(6) :277-80, 1998
IDIAGNOSTIC CHECKLIST 13. Cho YSet at: Narrow intern al aud itory ca na l synd rome :
pa rasaggita! reco nst ructio n. J Laryngo l Otol. 114(5) :392-4,
Co nsider 2000
• EAC at resia = clin ical d iagnosis
o cr provides preo perative roadmap
EAC ATRESIA
IIM AG E GALLERY
( / .ej / ) Axial NXJne ( 1 SIIU\VS

mild alreo;;.l with a talt'ral
Jxmy pl.1l(' (,m ow) , ."(
fUdiown/ary nJ(odidl/ X)( t ;OIl
2
of f Ae Midd le· (, J ( Cell/fly is
small but no /e normal 9
ma ~toid COIlJ/,/t·X. (Hin',t)
Axial 7~/x)fl(> C I (('v pals
lump y r(·.Jtu,('It's _~ pinna
(surow ), norm al mss taid
complex, sm.111 middle ('elf
cavity &. d ym lofphic m,llI('us
ht'Jd (opt'n arro w ). Inner Cd r
structures and lAC .If(!
no rma l.
(/ ..l'f/J Axial l-bon e C1

d emonstrates abcrrJrH
course of m astoi d p ortion of
(.lei,)! nerve. NerVI' ;s
,m lt'r;or!y disp',I(l'(/.I\ {');;b
into beck of glenoid fmsa
(,m ow ). (RighI) Ih i,11 1-bOflf'
r
C sho l\'s opa cified mirld ft·
('.If c.w ily, no ossicles .1{
sr dJloped ep it ymp.m ic walls.
N Oh ' anterior epitymp.lnic
is df'hisced (,Jrro w).
WoJ/I
Surger )' reve sted a.-.m ci.JtM
cbo testeetome.
Typical
(I.£j l) Clinical ph olograph
reveals .. mall, seven-tv
mal formp<1 euricto with no
iden tifiable no m ltll
component. Rem em her
se verity of s vtic uler
deform ity is directly related
to severity of EAC .ltrC'sid.
(Right) Axial I-bo ne C1
shows severe microtia
(arrow) & comple te bony
atresia (open arrow ), with
sma ll rudimt'nlary middle ear
cavity, no ide ntifiable
ossicles & norm.ll inner ('ar
struc tures.
NECROTIZING EXTERNAL OTITIS
2
10
Ih id!era 51JOWS .1 right intraparotic! absce5s (arrow) & Axial Ct.Cr ,,,vedIs infectious thickening of (he auricle ,I(;
/"lm/idiris [rom tlt-'CfotiLing L'xtf'fnal fJlilis af!f.'(Jing FA e so il (iSS-tIPS of the right ex({'rnal auditory canal (arruw).
E.. <w rid e (ol"J(.'tl arrow) "hove. The parotid sf-Met' is Tht' in!i'c rion has sprm d Jnleroinferior info subj.1H:.>nt
mo~t commonly .lffc·ctt.'fI. TMI (open arrow).
• Late: Dest ructi ve, osteo mye litic appea ran ce to

ITERM INO LO GY ho ny EAC especially affecting inferio r po rtio n
Abbrev iation s a nd Syno ny ms • CECr
• Nec rotizing extern al otit is (NEG)
o Early: Soft tissue of EAC & aur icle thi ckened ,
• Malign ant exte rna l otitis, sloug hi ng otitis cxte rna, ed emato us &. en ha nceme n t
mali gn ant nec rot izin g o t itis cx tcr na o Late: Cellu litis & a bscess pocket s ma y sp read in any
direct ion
Definitio ns • In ferio r: Parotid, masticat or &. paraphar yn geal
• NEO: Severe inf ecti ou s di sease invo lving bony ± spac es most co m mo n ly affected
car t tlaginous ex te rnal a ud ito ry canal (EAC) and • Posterior : In to masto id air ce lls
adj acent soft tissues • Med ial: In to m id d le ear cavlty / pe trous apex
• An teri o r: Int o tempo rom andibul ar joint
IIMAGING FIND INGS MR Find ing s

• T1 WI: Fluid to mu scle int en sit y th icken ed EAC, au ricle
Ge ne ral Feat ures & ad jace n t so ft tissues
• Best diagn ostic clue: Swo llen EAC soft tissues with • T2W I
hon y erosion & adj acen t deep space ce llulitis o r o Diffuse trans-spati al high signa l sugges ts cellulitis
a bscesses o Focal h igh signal areas suggests abscess
• Locatio n: EAC and su rrou nd ing sof t tissues • STIR: In creased signal in te nsity wit hi n infl am ed EAC,
• Size: Variable depending o n sprea d o f in fecti ou s aur icle and ad jacen t soft tissues
process int o adjace n t soft tissues • T I C+
• Morphology: Variable based on rou te o f sp read o With fat- sat u rat ion
• Tissues o f EAC & aur icle d iffusely en ha nce
CT Find ings • Adjacent so ft tissues show heterogen eous
• NECT en ha nce me nt with cellulitis
o 'l-bonc CT • If focal areas of rim-enhan cin g fluid see n in
• Early : Shows th icken ~d mucosa of EAC &. auricle ad jacen t soft tissues, a bscesses are p resent
DD x: EAC Le sion s
EAC Chole steatoma Med ial Can al Fibrosis Kera tos is O b turans EAC SCCa
Key Facts
Term inology • Post-inflammatory medial ca nal fibro sis
• NED: Severe infecti ous d isease involving bony ± • Kerat osis o btu rans
cartilagin ou s externa l aud itor y canal (EAe ) and • EAC exostoses
adjacent soft tissues • EAC squa mo us cell carcinoma
Imaging Findings Pathology

• Severe infectious changes involving EAe &: soft tissu e
2
• Best diagnost ic clue: Swollen EAC soft tissues with
bony erosion & adja cent deep space cellulitis or structures ad jace n t to EAC 11
absce sses Clinica l Issu es
• Tissues of U\C & aur icle diffu sely en hance • Clin ical profil e: Occur s in elde rly diabet ic patients
• Adjacent soft tissues show het erog en eous wit h severe o talgia
enhanceme nt with ce llu litis
• If focal areas of rim-enhancing fluid seen in adj acent Diagn osti c Checklist
soft tissues, ab scesses are present • Skull base o steo myelitis & NED may mim ic
malignancy requiring biopsy for pathologic
Top Differential Diagn oses differentiation
• EAC cholesteat oma
• MRV: Intracranial NED extension may lead to sigm o id • Ob stru ctive fibro us tissue wit hi n medial EAC without
sinus th rombo sis ho n y erosion
• Ch ro n ic otitis cxte rn a o r surgical proc ed ure causal
Nuclear Medicine Fi nd ings
• Bone Scan : Increased uptake in bony EAC &. ad jacent Kerat osis o bturans
skull base in more seve re cases with os teo mye litis • Rare co ndi tio n wit h abnormal acc u mu latio n &:
• PET obstruc tion of bon y EAC fro m desquamated keratin
o Impro ved ana to m ic localizatio n wit h PET o r single • Homogeneou s so ft tissue filling EAC wit h m ild
photon em issio n c r (SPEC !') en largemen t, withou t aggressive bon y ch anges
o SPECT indiu m- I l l white blood cell stud ies assist • Classically seen in pati en ts with chronic sin usitis &.
with d iagnosis of osteo myelitis b ronchi ect asis
• Gallium-6 7 citrate & tec h net iu m-99m (Tc-99m)
methyie ne d ip hosphonat e (MDP) scanning provide EAC exo stoses
infor ma tion in localiz ing focu s of infect ion • "Su rfer's ea r", co ld water ear
o Low specificity • Ben ign, broad-based overgrowt h of osseou s FA C wit h
o Gallium scan will reve rt to n ormal with qui escen ce norma l overlying soft tissues
of disease • Most com mon so lid tumor of EAC
o Tc-99m scintigraphy changes ma y lag heh ind for • No aggressive features
many months EAC sq ua mo us ce ll carci noma
Imaging Recom me nd at io ns • Bony invasi on & EAC ma ss may mimi c EAC
• Bone c r is preferred at initial diagnostic tool cholesteato ma
o \ViIl identify subtle co rtical erosi ons signaling early • Elderly pati ents with kn own EAC skin squa mo us cell
osteo myelitis ca rcinoma
• CT or Mil can be used to follow -up so ft tissue infectio n
o MR better for meningeal en h ance me n t, bone
mar row ed ema IPATHOLOG Y
• Nuclea r medicine helpful in ini tial NEG work-up
o Fails to provide anato m ic localizati on
Gen e ral Features
• Gene ral pa th co mme n ts
o Severe infectious changes invo lving EAC &. soft
IDIFFER ENTIAL DI AG NOSIS tissue structu res adj acent to EAC
o Necrosis and sloughing o f midd le ear and ad jacent
EAC choles teato ma tissues
• EAC mass com posed of exfoliated kerati n with o Embryo logy -anatomy
stratified squamous e pithelium • Disease typicall y exte nds in feriorl y from EAC via
• Often seco ndarily infla med or infe cted fissur es o f Santorini
• Un ilateral soft tissu e EAC mass with underlying bo ny • These vertica lly orien ted fissures in cartilagino us
destruction EAC allow flexibility, bu t also presen t rou te of
• Int ramural bo ny "flakes" (- 50%) infectious spread infe rior ly
• Etio logy
Post-inflamm atory medial cana l fib rosis o Pred isposin g facto rs
• EAC disease characteri zed by form ati on o f fihrou s
tissue in medi al aspect o f bo n y EAC
• Im munologic abn orm alities (i.e., neutropenia),
neoplasm, dermatitis, medicati ons, iatrogenic
pro cedures Con sider
• Epide miology : Diabetes m ellitus pr esent in 95% of • EAC SCCa can have a sim ilar appearanc e to NEO
adults with NEO • Skull base ost eomyeliti s & NEO m ay m im ic
Gross Pathologic & Surgica l Features malignan cy requiring biopsy fo r pat hologic
2 • EAC edema with gran ulati on tissue
• Gran ulation tissue may not be seen in
d ifferentiation
Image Interpretation Pearls
12 im munosup pr essed /AIDS patients • Small, early cortical erosions are best seen with CT at
Microscopic Features initial d iagnosis
• Severe infl ammati on with necrosis in subcutaneous • Careful com pariso n with norm al co nt ralateral ear ma y
tissu es in EAC be required to identify sub tle bo ne destructive ch ange
• Pseudomonas aeruginosa most com mo n age nt
o Gram negat ive aerobi c rod
• Classically described in m idd le-aged and elde rly ISELECTED REFERENCES
d iabetics 1. Urna pathy N: Necrotizing fasciitis: a rare occur rence in the
• Aspergillus fumigatu s and ot he r organisms ma y be extern al auditory meatus. J Laryngol Otol. 117(1):69· 70,
cause in immu no suppr essed/AIDS pati en ts 2003
2. Lancaster J et al: Non-pseudomo nal malignant otit is
exte rna and jugular foram en syndrom e secon dary to
cyclospo rin-ind uced hypertrich osis in a diab etic renal
IC LIN ICA L ISSUES transplant patient.) Laryngol Otol. 114(5):366-9, 2000
3. Soldati D et al: Necro tizing ot itis externa caused by
Presentation Sta phyloco ccus cpidermid is. Em Arch Oto rhino laryngo l.
• Most com mon signs/symptoms 256(9):439-41, 1999
o Most com mon presenting symptom: Severe 4. Kristiansen P: The diagn osis and managem ent of
persistent otalgia malign ant (necrotizing) otitis exte rna . J Am Acad Nurse
a Othe r signs/sympto ms Pract. 11(7):297-300, 1999
• Persistent ot orr hea 5. Tezcan I et al: Necrot izing otitis ex tem a, otitis media.
perip heral facial paralysis, and bra in abscess in a
• Facial nerve paresis (rare): Inferior spread of th alassemic child after a llogeneic BMT. Pcdiatr Hem at ol
infectio n Oncol. 15(5):459-62, 1998
• 9 ~ 12 cranial ne uropath y: Postero med ial exte ns ion 6. Kuma r BN et al: Beni gn necrotizin g oste itis of th e exte rnal
to jugular foram en or carotid space auditor y meatus. J Laryngol Otol. 111(3):269-70, 1997
• Persistent granu lation tissue seen at 7. Grandis JR et aI: Necrotizing (malignant) extern al otitis:
bo ny-cartila ginous EAC junction Prospective com parison of CT and MR imag ing in
• Clin ical p rofile: Occurs in elderly diabetic patien ts diagnosis and follow-up . Rad iolo gy 196(2):499-504, 1995
wit h severe otalgia 8. Weinro th SEet al: Maligna n t otitis exte rna in AIDS
pat ient s: case report an d review of th e literatu re. Ear Nose
Dem ographics Throat ). 73(10):772-4, 777-8, 1994
9. McElroy EAJr et al: Fatal necrotizing otitis externa in a
• Age: Midd le-aged or elderly
patient with AIDS. Rev Infect Dis. 13(6):1246-7, 1991
• Gen de r: M:F = 2:1 10_ Guy RLet a1: Com puted tomography in ma lignant external
Natural Histor y & Prognos is otitis. Clin Radio143(3):166-70, 1991
11. Barza M: Use of quino lones for treatment of ear and eye
• May progress to frank skull base os teomyelitis infection s. Eur J Clin Microbiol ln fect Dis. 10(4):296-303,
• NEO begin s as soft tissue in fection, spreading into 1991
surrounding osseous stru ctures with pro gression 12. Shupak A et al: Hyperb aric oxyge na tion for necrot izin g
• Poten tia lly lethal if u n t reated (malignan t) otitis extern a. Arch Otolaryngo l Head Neck
• Intracranial exten sion can lead to sigmoi d sinus Surg. 115(1 2):1470-5, 1989
thro mb osis, m en ingitis, int racran ial em pyem a 13. Wolff LJ: Necrotizing otitis exte rn a during in duction
• 209il recurrence rate th erapy for acute lym ph oblastic leukemia. Ped iat rics.
84(5):882-5, 1989
Treatm en t 14. Cun ningha m M et a1: Necroti zing otitis externa due to
Aspe rgillus in an im m unocom petent patien t. Arch
• Aggressive & meticulous debridemen t of gran ulations,
Otolaryngol Head Neck Surg. 114(5):554-6, 1988
top ical and syste m ic antibioti c th erapy 15. Matt ucci KF et al: Necrotizing otitis ex terna occurring
o Often combinatio n of an antipseudo monal conc urrently with epidermo id carcino ma. Laryngoscope .
bet a-lactam and an ami noglyco side 96(3):264-6, 1986
a Adjuvant th erapy wit h hyp erbaric oxygenation m ay 16. Green e SL et al: Pseudomonas aerugtnosa in fect ion s of the
be considered , if available skin. Am Fam Physician. 29(1):193-200, 1984
• Surgica l d rainage of an y deep facial abscess seen 17. Gold S et a1: Radiog raphic findings in progressive
necrotizin g "ma ligna n t" ext erna l otitis. Laryngoscop e
94(3):363-6, 1984
18. Pace-Balzan J: Necrot izing otitis exte rn a. J Laryn gol OtoI.
91(8):735-8, 1977
IIM AG E GALLERY
Typical
(u ft) (orond! t-bone C1
shows int7ammatory chdoges
in the region of the fight
external duditor y canal.
2
Destructive dehiscence
(arrow ) 0; inferior bony EAC 13
signals the beginning of
associdted osteomyelitis.
(Right) (orondl CEeT shows
inflammator y soft tissue
filling the left external
auditory csnet tenowt. with
;o;er;or ellension of cellulitis
into the cephil/ad aspect of
the msstics tor space (open
arrow ).
(/.£ft) Axial (fCT shows

infe ctio us soft tissues filling
the right externa l Juditory
canal (arrow) .Ii: mi ddle esr
with e xtensi on through a
bony dehiscen ce into the
right m iddle cranial {OSS.l
(op en arrow). (Righ"
Anterior view bone scan
5ho\\-'5 significant increased
up tak.eof the radioisotope
w ith in the It.'ti tempor,]! bone
(arrow ) ~~ sl..ull bsse (black
arrow ), consistent with
necrotizing e\terniJ l otitis .
Typical
(LIf t) Ax;.' CECT, how,
inferior extension of NED
into immedia tely inferior
pdrotid gliJnd. Diffuse
pdrotid swelting an d
enhancement signal acu te
psrotidltis (arrO'o 'o's). Facial
nen 'e paralysis was also
present. (Right) Axial CECT
reveals ceJlu litic changes
exten ding inferiorly into the
left mssucs ior spac e
("UfOW), anterior to the left
con dy lar need. Left
p arapharyngeal space fat is
in vol ved (open arrow) .
EAC CHOLESTEATOMA
2
14
Coronal graph ic shows .1 EAe choleslm loma .15 J Il Coronal T·bone CT r('vea/~ £/Ie cholestpatoma a~ an
crosiw-sc.1//oping submucosal mass in rhe inferior erosive lesion of in({yior hony EAe. Note bony CAe
external audiror y canal. Note bone erosion (arrow ) w ith erosion (arrCAv) with multiple bony tiecks within
bony fleck s wilhin lesion matrix. dlOicsleatoma matrix (Of'Jell arrow) ,
ITE RM INO LO GY MR Fi ndings

• T1WI: Soft tissue in ten sity ma ss in EAC
Abbreviatio ns a nd Syno nyms • 1'2WI: Signal can be in term edi ate com pared wit h
• Abbreviat ion : Externa l aud itory canal cho lestea tom a infl ammator y t issues
(EACC ) • T l C+: Low int en sity, rim-e n hancing EAC mass
• Synony m : Acquired cholesteatoma of EAC
Imagin g Reco mme ndations
Definitions • Axial I< co ronal thin-secti on bon e algor ith m C1' of
• EACC: EAC lesion com posed of exfo liated kerat in temporal bone is exa m ina tio n of cho ice
withi n st rati fied sq uamous epithe lium
jlM AGING FIN DI NGS
Ke ratosis obtura ns
Ge ne ra l Features • Bilateral keratin plugs filling EAC
• Rest dia gn ostic clue: Erosive EAe soft tissue mass ± • Mild d iffuse EAC en largement wit hout focal bony
bony necks in ma trix erosio ns
• Locati on : Co m mon ly posterior & inferi or EAC wa ll • You nger pati ents with sin usitis & bro nc h iectasis
• Size: 5 111m to 2 em
Post -in flammatory medial ca na l fib rosis
CT Find ings • Obstructive fibrous tissue within med ial EAC without
• NECT bon y erosio n
o Un ilateral, sca llo ping so ft tissue mass in EAC • Ch ron ic otitis exte rna or su rgical proced ure causal
o Bon e fragm ents com mo n ly found with in mass
(SO'Ji,) Ne cro tizing externa l otit is (N. E. otitis)
o May exte nd locally int o sub jace n t bony structures • Infecti ou s process of EAC & surrounding structures,
o Tympan ic mem bra ne in tac t in most cases dia gn osis con firmed with 1'c-99 m or gallium scan
• CECT: May dem onstrat e rim -enhan cem ent • Elderly diabet ic pati ents with pseudomonas aeruginosa
DD x: Externa l Aud itory Mass
Keratosis Obturans Media' Calla' Fibrosis N. E. O titis EACSCC"

EAC CHOLESTEATOMA
Key Facts
Termino logy • Axial & coro nal th in- section bone algorit h m c r of
• Synony m: Acqu ired cholesteatoma of EAC temporal bon e is examinatio n of choice
• EACC: EAC lesion co m pose d of exfoliated keratin Clinical Issu es
wit h in stratified squamo us epit he lium • Oto rrhea & ota lgia
Imaging Find ings Diagn osti c Checklist 2
• Best diagno st ic clue: Eros ive EAC soft tissue mass ± • Focal, unilat eral EAC ma ss + EAC bony scallop ing ±
bo ny flecks in matri x 15
bon y flecks = EACC
• Location: Co m mo n ly posterior & in ferior EAC wall
EAC sq ua mo us cell ca rcino ma Treatm ent

• Bo ny invasion & mass may mimic EAC cholesteato ma • Spo n ta neo us EACC adequat ely con t rolled wit h
• Elderly patients wit h know n EAC skin SCCa pe riod ic o ffice debridemen t N co nse rva tive med ical
man agemen t
• Su rgical exc ision fur larger lesions with bu ny invasio n
IPATHOLOGY
Ge ne ral Fea tures IDIAGNOSTIC CHECKLI ST
• Gene ral path co m me n ts: Most co m mo n ly in ferio r &.
poste rior EAC wall Co nside r
• Etiology • Imagin g ap pearance of EACe may exac tly mimic r..A C
o Co nge n ita l: EACe from ectodermal rest left with in sq uamo us cell ca rcinoma
EAC wall (rare) • Clin ical h isto ry guides thi s di ffere n tiati on
o Spontaneous: EACe seco ndary to ab no rma l
mi grati on o f EAC ectoderm Image Interpretation Pearls
o Acquired EACe: Pos to pera tive, post-t raum ati c or • Focal, un ilateral EAC ma ss + EAC bon y scallo ping ±
seco nda ry to EAC obstructio n bo ny flecks = EACC
o In all typ es, EACe mass co ntin uously en larges,
scallops ad jacent bo n es
• Ep ide miology: O.l l){) inci dence in new ENT pa tien ts ISELECTED REFERENCES
I. Heilbrun ME et al: External auditor y can al choles tea toma :
Gross Pathologic & Surgica l Features cli n ical and im agin g spectrum. AJNR Am J Neu ror ad iol .
• Different type o f cholesteato ma ; not "pea rly wh ite" 24 (4):75 1-6,2003
• Su bm uco sal soft, waxy material discol ored by 2_ Sapc i T et al: Gia n t ch o lesteato m a o f th e ex te rnal au ditory
infla m mato ry cha nge canal.l\n n 0 101 Rhlnol Lary ngol . 106(6):47 1-] , 199 7
• In tra m ural bo ny fragments possib le 3. Venk atra m an G ct al: Externa l aud ito ry cana l wall
cho lesteato ma : a com p licat io n of ea r surgery. Acta
Microscopi c Features Otolary ng o l. 117(2 ):293-7, 199 7
• Sim ilar to epi de rmo id in clusio n cyst
• Stratifi ed squa mo us epit he lium with p rogressive
ex foliatio n of keratin ou s ma teria l IIM AG E GALLERY
• Co n te n ts may be rich in cho lesterol crysta ls
ICLIN ICA L ISSUES

Presentati on
• Most com mo n signs/sy mpto ms
o Ot orr hea & otalgia
o Oth er signs /sy m pto ms
• Co nductive h ear in g loss
Demographi cs
• Age: Old e r po pulatio n, 40- 75 yo
(Left ) A xial lefl ear I-bo ne CT demonslr.l les su1Jl/e crcnivo change'S of
Natural History & Prognosis hony [AC (arrow) with hony (leek s (ope n arrow) within the EAC
• Relentless increase in size &. e rosion of EAC bon y wall cbokssuiuome. This M e cbolesteetom o is circumferential in medial
• Sma ll lesio ns usually a re cu red wit h co m plete excisio n EAe. (Rig111) Coronal T-bone CT shows so n tissue fillin g left vxtomst
• Recurr ences are mo re com mon wit h inc reas ing lesion auditor y canal, with erosive osseous changes (arrow) as well as small
size &. inv asio n o f su rround ing osseo us struct ures foci of hon y flecks within the' EAC cholesteatoma matrix (ope n
arrow).
EAC KERATOSIS OBTURANS
2
16
COfOllJ l /(>Ii f'.]r t-booe CT ~h()w!'o /KJfJlOgC'f)('OUS SOIt tW.JI ' -h OI if' LI of koIi ( ' J I s/)o.y; l>f'flit-:n ~r t~'o{JfO
filling the EAe (arrow ) without (b!Jf:'()U!J' cha nges.
liS51IP (J " c" ,,) fillin~ tbe &K with J fKXnJJI 05k'OU~ GInal in
The mklrl1eP.lf fj. UnJiieclff/ by lAC-KG (open arrow ). thi~ CJ S(' of EAe-KG. M iddle Cd T GJ\'iry and inner e.lf
[Ae i.~ diiilJ~ ffila rgrd. ~lfUCIUff'5 .:ITt' nclml<1/.
ITE RM INO LOGY MR Findi ngs

• T HVI: Hom ogeneous low-inte rmed iate signa l sof t
Abbreviations and Syno nyms tissue filling EAC
• EAC kerato sis obt urans (EAC-KO) • T2\VI: Iso- or low signa l in ten sity
• Cho lestca tos is. molluscum co n tagtosum • TI C+: No int ra m u ral e u h a nccmc n t: m ay rim -en ha nce
Defi nitio ns Imagin g Recommend ati ons
• EAC·KO: Ahn o rma l accumulatio n N o bstr uct io n of • No nco n trasted, th in-secti on bo ne algorith m 'I-bon e
bo ny ex ternal aud itor y canal (F./\ C) from desquama ted Cf exa m in atio n is study o f c hoice
kerati n with ou t e rosive bo ny ch an ges
IDIFFERENTIAL DIAGNOSI S
IIM AGING FIN DINGS
Benign EAC debris
General Featu re s • Partia l filli ng of FAC wit h so ft t issue densi ty m at er ial
• Best di agn ostic cl ue: EJ\ C·KO ap pear s as h om ogeneo us withou t bony erosion
so ft tissue fillin g EAC with mild EAC en la rgeme n t bu t
wit h ou t bo ny erosio n EAC cho les teatoma
• Locati on: Most co m m on ly bilatera l • T-bo ne CT: Un ilatera l EAC soft tissue wit h ho ny fla kes
• Mo rpho logy: Soft tissue co n forms to Et\ C in 50 l }1)
CT Findings Necrot izing ex terna l o titis (N. E. otitis)
• N l.C f • 'l-bone CT: EAC swe llin g ± bon e e rosio n ± a bscess
o 'I-bo ne c r
• Ben ign-appear ing soft tissue filling bo ny EAC, Sq ua mo us cell carci no ma of EAC
witho ut hon y ero sion • T-ho ne CT: Irregul a r mas s with und erlyin g ho ny
• Mild M e bo ny en largeme n t possible e rosio n
• CECT: No sign ifican t en ha nce men t o f t issue
DDx : EAC Mass
EAC Debris N , E, Olitis EAC SCCa

EAC KERATOSIS OBTURANS
Key Facts
Termin ology Path ology
• EAC-KO: Abn or m al accum ulatio n & obst ruc t io n of • Epide miology: Rare FAC lesion o n ly imaged wh en
bony ex terna l aud ito ry cana l (EAC) from seve re
desqu am ated keratin wit hout ero sive bo ny cha n ges
Clinica l Issues
Imaging Findings • Clinica l profil e: You ng m an ; bilateral o talg ia and 2
• Best d iagnosti c clue: EAC-KO a ppears as CHL
homogen eous soft tissue fillin g EAC with mild EAC • Excision of kerati n p lug; may requ ire a nest hesia 17
en largement bu t wit hou t bon y erosion
IPATHOLOGY Treatm en t
• Excision o f kerati n plug; ma y req uire a nes thes ia
General Featu res • Direc t treatmen t o f gran ulatio ns when present
• General pa th co mm en ts: Henign keratin plug filling o Excisio n, cauterization , to pica l steroids
bony EAC without focal bon y erosion • Per iodi c fo llow-up wit h EAC clean sin g routin e
• Etiology • Rem oval o f re-accum ulated debr is o ften required
o 2 commo n theo ries
• Abnorm a l epithe lial mi grati o n wit h kera tina ceo us
debri s bui ldup IDIAGNOSTIC CHECKLIST
• Sym pa thetic reflex sti mulatio n o f ceru mino us
glands in EA C ca use hyperem ia &. epiderma l Image Inter pretation Pearls
plugging, seco ndary to bronchi ecta sis • EAC-KO & EAC cho lesteato ma terms o fte n con fused
a Chro n ic radi ati on dermati tis can prod uce rad iati on o EA C-KO is lu min al in EAC wit hou t bo ny e rosive
keratosis ch anges
a Marked infl amm at ion in subepit helia l ttssuc with out o EAC ch olestea to ma sub mucosal with bony flecks &.
bony erosion eros ive cha nge s
• Epidem iolog y: Rare EAC lesion on ly imaged wh en o Bot h lesions cons ist of ex foliated keratin
severe
• Associated ab no rma lities: Ch ron ic sin usit is &.
bro nchiecta sis ISELE CTED REFER ENCES
Gross Path ologic & Surgica l Feat ures L Persa ud R ct al: Atyp ica l keratosis o btu ra ns. J l.aryn go l
• Tympan ic membrane (TM) usuall y thicken ed Otol. 117(9):72 5· 7, 2003
o TM is often norma l with EAC cholesteato ma 2. Sh ire JR et ul: Cho lesteato ma o f the exte rn al aud ito ry can al
an d keratosis obtura n s. Am J Ot o l. 7(5):36 1-4, 1986
• Mild circu m feren tia l widening of bon y F.AC co m mon :I. Naibe rg J et al: The pat holog ic features o f kerat osis
• Granulomas ma y form at jun ction o f hon y & obtu ran s and cho lesteato m a o f th e- external a ud itory canal.
cartilaginous cana l Arch Ot olar yng o l. 110 (10):690-3, 1984
Microscop ic Feat ures
• Desquamat ed keratin t issue
• Keratin tight ly organ ized in la mella r patt ern in
IIMAGE GALLERY
EAC-KO
o EAC cho lestea to ma sho ws ra nd om keratin
organ ization patt ern
ICLINICAL ISSUES
Presentation
• Most co m mon signs/sy m pto ms
o Acute severe o talgia. o ften bilateral (- 50% )
o Oth er signs/sym ptom s
• Co nd ucti ve h earing loss (CHL)
• Clin ical profil e: Young man; bilatera l otalgia and CHL
(I.ef l ) Axi.ll lefl esr l-bone C T shows tx'nign soft tissue (arrow) filling
Dem ographics the left EAC with an intact osseo us f AC canal in this CilS(' of
redtetion-iaduced k.eratosis obturens. (RigJrt) Axial t-booe CT shows
• Age: You nger pati en ts « 40 yea rs o ld)
keratosis obrurans findings of homogeneous soft tissue opacifying
Natural History & Progn osis right EAC (arrow) wi thoUl osseous destructive changes. Notice
averal/ size af EAC is slightly enlarged.
• Over time keratin pl ug en larges, wid eni n g EAC bony
can al
EAC MEDIAL CANAL FIBROSIS
2
18
Comn.JI graphic sho.vs medial canal fibr0si5 ac .1 thick' Coronal T·hone c r f('\,('ars a band oi soil tissue ;illing
fihro(l'-' CfP.'iCmC O\'t'fJ)'ing the lymp.1nk- ITIffilJ)fdJ)(', mt.'(/i,l/ CAe. abuning the lymp.mic mt.'1nbrane (J If(Jl,V}.
filling tJk'f.li.J1 Me (.1f m w). Ini1Jmm.ltory ch.J1ll,'('S oi !l.fiddlt· tOJ' is un,l ffpclpf! by mtx/ial c,ma/ l/brrx is. Op('fJ
nJt"Cli.J1LAC w,)lIs ,1/!>O depicted. ,maw: l}'m p.m k· ,lnnlllll~ .
• Morphology: Homogeneous soft tissue co n fo rming to

ITERM INO LOGY med ial FA C
Abbrev iatio ns and Syno nyms CT Find ings
• Abbreviat ion : Med ial ca na l fibrosis (MCF)
• NECf
• Syno nyms : j'ost inflarnmator y MCF, medial meatal o Un ilateral or bilateral me d ia l fibrous p lug in EAC
fib rosis, acq u ired rv1CF, acq uired at resia, chro nic o Early stage MCF
steno sing exte rna l otitis • Th ickened TM with ed ematous, m ild ly thi ckened
Defi niti ons EAC m uco sa
• MCF: Discret e clinicopathological disease o La te stage MCF
ch a racte rized by forma tio n o f fibrous tissue in medial • Ofte n look s like th ick "crescen t" of tissue o ver lying
as pect of bony exte rn al auditory ca na l (EAC) latera l sur face of TM
• T:vi ca n not be reso lved as sepa rate from ~{ C F
fibrous ma ss
IIM AGING FINDINGS • No underlying bon y cha nges presen t
• Middle ear-mastoid uninvolved
Gen era l Featur es • CECT: May sec sligh t en ha nceme n t o f edematous E,\C
• Best diagnostic clue: Fibro us crescent ove rlying lat eral thi ckened wa lls
su rface of ty m pan ic membrane (T!\t)
MR Findings
• Locat ion
• THV) : Ho mogeneous low signa l soft t issue in medi al
o Med ial EAC, ad jace n t to TM
ex terna l audito ry ca na l
o - 60 l M, hilatera l
• T2W I
• Size o Int erm ed iat e to low signa l soft tissue in medi al
o Variab le
externa l audito ry ca nal
• May have m ild th icken in g of 'I'M wit h ede ma tous
o Mo re fibrous tissue pre sen t, lower th e signal
EAC wa ll' ea rly
• T I C+: M ay sec sligh t enhancement of
• More advanced cases show ncar com plete
inflamed /ede matou s EAC thickened wall s in ea rly
opaci fica tio n o f EAC
stage
DD x: EAC l e sion
'
,
("""""'-
,...!"f .., J
4ft
rAC Debris KC'raIO.'ii.'i Obtumos rAC Cholesteatoma N. t. Otitis

Key Facts
Terminology • EAC cho lesteato ma
• Abbreviat ion : Med ial ca nal fibros is (MCF) • Necrotizing exte rnal otitis (N. E. o titis)
• Syno ny ms: Po stinflammatory MCF, media l meat al • Squamous cell carcinoma of EAC
fibrosis, acq uired MCF, acq uired atresia, ch ron ic Path ology
ste nos ing exte rna l otitis
• ~I CF: Discret e clin icopat holog ical di sease
• Gene ral path comments: Most co m mo n etiology is
ch ro nic exte rna l otitis
2
cha racte rized by forma tio n of fibrous ti ssue in med ial
Clinical Issu es 19
aspect of bony ex te rna l aud itory canal (EAC)
• Clin ical profile: 50 year old wo ma n with bilat eral
Imagin g Findings oto rrhea and CHL and hi story of ch ro n ic o titis
• Best diagnostic clu e: Fibro us crescent overlying lat eral • Su rgica l in te rven tio n alone co rrec ts con d uctive
sur face o f tympanic membrane (TM) hear ing loss
Top Differe nti al Diagn oses Diagnostic Checklist
• Benign EAC debri s • Long term follow-up is recommended to evalu at e risk
• Kerato sis obtu rans o f recurren ce
• EAC exostoses
• 'I-bo ne C l: EAC mu cosal swe lling ± underlying bone

Imaging Recommendati on s erosio n ± deep space abscess (exte ns io n of di sease
• T-bon e thin-sect io n (1 mm or less) nonenhanced , inferiorly)
bon e algorit h m cr o Diagnosis confirme d wit h nuclear med icine Tc-99m
• Do both ax ial and co ronal planes o r gallium scan
• Be sure to in clude en t ire EAC in mag n ified images
Sq ua mo us ce ll ca rcino ma of EAC
• Clin ical: Ulcerating lesion in EAC of elderly patient
ID IFF ERENTIA L DIAGNOSIS • CT: Irregu lar, ill-d efin ed mass with und erlying
aggressive bo ny ero sio n
Benign EAC de b ris o Can mi mic EAC ch o lesteatoma
• Clin ical: Usua lly obvio us on oto scopi c exam
• 'I-bo ne C f: Lu m ina l so ft tissue in EAC witho ut osseo us
erosion IPATHOLOGY
o Air oft en present in clefts & intersti ces o f EAC debris
Gen eral Features
Ke ratosis ob tu rans • General pa th comments: Most common etiology is
• Clinica l: You nge r pati ents with sin usitis &. ch ron ic exte rnal otitis
bro nc h iectasis • Etio logy
• T-bune Cf: Bilat eral keratin plugs fillin g EAC o Ch ro nic inflam matio n of medi al EAC heals via
o Mild di ffuse EAC en largemen t Seen wit hout focal gran u lation tissue formati on
bo n y erosio ns • Gran ulation tissue slow ly matures int o a mature
o Spa res m idd le ear cavity fib rous plug
EAC exostoses o MCF is fin al co mmo n path oph ysio logic pathway for
• Clinica l: Younger pati ents with repetitive exposure to multiple mechanism s of injury to EAC
cold wat er (sur fer's ea r) • Chro n ic otitis externa
• 'l-bo n e CT: Bilat eral osseous encroach me n t of EAC • Seconda ry to su rgical p rocedure or trauma
can al • Suppu rative otitis medi a
o Diffuse broad-based overgrowt h of osseous EAC with • Radi otherap y to EAC
n ormal mu cosal su rfaces • Epidemi ology: Very rare lesio n
o Usua lly beg ins at medi al osseous EAC • Associated ab normali ties: C h ron ic o titis ex te rna
EAC cho lesteato ma Gross Path ologic & Surgical Featu res
• Clinical: Otorrhea &. EAC ma ss in o lder pa tient • In flamed , ede mato us ma rgin s to fibrou s plug covering
populati on TM
• T-bone C'l: Un ilate ral EAC soft tissue wit h u nderlying Microscopi c Features
bon y destru ct ion • Early stag e
o Bony "flakes" seen wit h in mass in 50 l J{J o f cases o Granulati on tissue
Necrotizing exte rna l oti tis (N. E. oti tis) o May demonstrate lym p hocyte infilt ratio n
• Clin ical: Eld erly dia betics wit h pseud omonas • Late stag e
o Layered fibrous co n nec tive tissue
aerugi nosa EAC lnfcc tlon
o May demonstra te foca l areas o f calci fication
o Kerat osis o bt u rans: Look for co m plete opacificat io n
Staging, Grading o r Classificat ion Crite ria & subtle EAC bo ny wide n ing
• Early (wet) stage a EAC ch olesteato ma: Lo ok for foca l EAC so ft tissu e
o Ch ro n ic ot itis med ia wit h otorrhea &: co n d uctive ma ss wit h un d er lying bo ny erosio n ± in tram ur al
h earin g loss bone flecks
• Late (d ry) sta ge
o Med ial EAC fibrous plug wit h cond uct ive h eari ng Image Interpretation Pearls
2 loss • Cresce n tic so ft tissue plu g agai nst tym pa n ic
membrane h ighly suggestiv e of MCI'
20 • No role for MR ima ging in MCF di agnosis or imagin g
IC LIN ICA L ISSUES evaluatio n
• Lon g term follow-up is reco mme n ded to eval ua te risk
Presentation of recu rre nce
• Most common signs/sympto ms
o Cond uctive h ea ring loss
• Typi call y 20-40 decib el ISELECTED REFERENCES
o Ot her signs /sym pto ms
L Ho psu E et a l: Id io pat h ic infl am ma tor y m ed ia l meatal
• Ch ro n ic otitis exter n a fibro tizing o titis. Arch Otol aryngol Hea d Neck Su rg.
• Ch ro n ic d ermatit is (ecze ma o r psori asis) 128(11 ): 1]1 3-6, 2002
• Tinnitus 2. Sen C et .11: Jugular fo ramen : mi cro scopi c anato m ic
• Oto rrhea features ami implicat io ns for n eur al pre servati on wit h
o Ear ly sta ge reference to glo mus tu m ors Invol vin g till' tempora l bo ne.
• Ch ro n ic otitis me d ia with oto rrhe a &. co nd uctive Neu rosur gery. 48(4 ):838-47, 2001
hear in g lo ss (CII L) (wet) 3. Lavy J et al: Chron ic ste nosi ng ex tern al
o t itis/ post infla m m ato ry acq uired at resia: a review. Clin
o Late stage
O tola ry ngol. 25(6):435-9, 2000
• Mature fibrou s plug p resen t wit h CII L (d ry) 4. el -Saycd Y: Acqu ired me di al cana l flb ros ls. J Laryn gol O tol.
• Clin ical profile : SO year old woman wit h bilateral 112(2):145-9 , 1998
o to rrhea an d C HI. and h isto ry o f chron ic otitis 5. Slatter y WII Srd ct al: Post infl an un ato ry med ia l ca na l
fibrosis. Am J O tol. 18(3):294 - 7, 199 7
Demographi cs 6. Birma n CS et al: Media l ca na l stcnosts-c h ro ntc stenos ing
• Age ex te rn al otiti s. Am J O to l. 17(1):2-6, 1996
o Mean age = 50 yea rs o ld 7. Gor en flo M et al: Morphometri c tec h niq ues in th e
o Range: 5-S0 years o ld evalua tio n o f pulmo nary vasc u lar changes d ue to
• Usually rare in pediatric popu lation co n gc nt ta! heart dis euse. Path o l Res l'rart. 192(2): 107- 16,
1996
• Gen der: M:I' = 1:2 Magliu lo (j ct al: Medi al meata l fibrosis: cur ren t ap p roac h.
8.
Natural History & Progn osis J Laryn go l 0'01. 110 (5):4 17-20, 19 96
9 . Birma n CS et al: Media l cana l srcuosts -c h rontc stenosing
• Surg ica l co m plica tio n
ex te rna l ot itis. AmJ Ot ol. 17( 1):2-6, 1996
o Recurren ce o f EAC ste nos is « 5%) 10. Keohane JD et al: Medi al meata l fibrosis: th e Un tversuy of
o He-sten o sis m ay occ ur years aft er treatment Western Onta rio ex perience. Am J O to l. 14(2 ):172-5, 19 93
11. Ka tz kc D et a l: Po sti n fla m mat ory med ial mea tal fih rosis. 1\
Treatm ent neglected en t ity? Arch O to laryngol. 108(12):779· 80, 1982
• Surg ical interve ntion a lo ne co rrects co n d uctive 12. Paparella MM ('t a1 : Surg ical t reatmen t for ch ro nic
hearing lo ss stenosing ex te rn al o titi s. (In cl ud in g fin di ng o f u nusual
• Early phase ca nal tu mor ). Laryngoscope. 76(2):2 32-45, 19 66
o Topica l an tibiotics & steroi ds
• Late phase
o Surgical in te rven tio n requ ired to co rrect CHL
o Excisio n o f all fib rou s tissue &. in vol ved skin
• Wide can alo plasty
• Meat oplast y fo llowed .by reco ns tructio n by split
skin graft
• Squa mo us ep it heliu m may be need ed to repopulat e
EAC and lateral TM
o Skin graf ts ma y be need ed fro m po sterior pin na
Consider
• Differentia te MCI' fro m keratosis ob tu rans & EAC
ch o lesteato ma
o Medi al ca nal fibrosis: Look for med ial EAC tissu e
plug with no EAC bo ne cha nge s
IIM AG E GALLERY
Typical
(/.£11) A xi,ll ri(;hr ear I-bone
C T shows medial canal
ii/)(Q.';;s JS ticmogenec us »ctt
!issue in Ih(' medi al ElK
2
(arrow). Notice undN/ying
bom' i~ flo l eroded .ltl d the 21
mkklle (''' ' ;S spaw d. (High"
Corond l /c{r car T-b o ne C r
shows an example of li n'
earl y p ha$f:' o f mt'dial canal
fibrosis. Note thick tympanic
mom brane (arrow) with
cc/t'm illOus cha ngt'5 0; the
mt'(Jial [A e wall.~ (op l'n
arrows ).
Typical
(/ .ef' ) Coronel toit PJr T-bone
C T reveals class ic CT
findings of med ial canal
tibrosis. N ouco so ft tissue
Cft'Kt'n t ahulting tympanic
membrane (arrow) wi/ho u!
lIndl'rlyifJp, IX)fJ('
f'f( J~io n/mid( lfp I-'df
involve..' m l' ll l. ( HiK" t) Axi,)1
l -bc no CT of Ihe IPI; C'el r
demcnst rates <1 band arm!/
tissue iitling lh(~ lett media.l
EAC (arro w) and sparing th('
m iddle ear cavity in this case
of media! ctillal fibrosis .
(Lef t) COfOfl.lf rstct th fo ugh

the te mporal bo ncs shows
50 ft tLmlf." density material in
the m edia l EACs bilaterally
(arro w s), without exp ansi on,
and relatiV(' sp aring of thf'
middle ear cavities in M CF
case. (Hight) Coronal Nf er
5how.~ thick m ing of the walls
0; the right EAC (arrow) with
prominen t soit tissue
adjacent to tht' tympanic
membrnne, con sistent with
edemetous ch angl:'s to the
EAC w alls with 1\ 1Cf.
EAC EXOSTOSES
2
22
Corondl graphic shows 1Jt-./Jign dpFx'aring bony or

Axial N XXIf:' CT right Cd r sholVs broacl-bd'iecl ()'is('Ou.~
o\,prg/O\\th of tile right [ Ae (a u (JlYs) in J esse of [A e F.AC t'flCf{hlChml'fll on oolh ant erior 8. IXJ,>Il.'riol' 1V.l1I~
f'\o.~t()~l'5. tnscn Sh O l 'o'5 oto.~c0l'ic view of (a mnV'iJ in /his Cc15(, of f AC (>X05 /09::'5 . N otin' EAC
("irn Jln k' relllial subnwrn\.l l EAC rumxving. lunwn i~ 'i('\'{',l'Iy narrOIVf::'(I.
ITE RM INO LO GY M R Findings

• T I \ VI: Low signa l, t hic k, nar rowed bon y Ei\C
Abb reviat ions and Syno nyms • T2\VI: Low sign a l o f osseous ove rgrowt h in to CAC
• Syno ny ms: Surfer's ear ; co ld wa ter ea r
Imagin g Re com m en dations
Definit ions • Noncon trasted. h igh-reso lut ion CT with bone
• Exte rna l aud ito ry ca n al (EAC) ex os tose s: Ben ign algo rit hm of tempor a l bo nes
ove rgro wt h of bon y exte rna l a udito ry can al • ~1 R o f no h elp wit h t hi s d iagno sis
IIM AGING FINDINGS ID IFFERENTIAL DI AGNOSIS

Genera l Featu re s EAC cho les teato ma
• Best d iagn osti c clue: Ben ign , broad -based overg row t h • Un ila teral EAC ma ss wit h bon y dest ruct ion
o f osseo us EAC wit h norma l overlying soft tissues, • May see hon y "flakes" in m ass (S{Nil)
wit hou t agg ress ive fea tures
• Location Media l ca na l fibrosis
o Begin at m edia l OSSL'OLIS EAC • Fib ro us p lug aga ins t no rm al tym pa n ic mem b rane
o Bilatera lit y is ru le • Usua lly fo llows o titis extem a o r surg ical proced u re
• Size : Varia ble, n a rro win g o f EAC
• Mor pho logy Necrotizing e xte rna l o tit is (N. E. otitis)
o Va riable, osseous ove rgro wt h encroach ing on EAC • Severe in fect io us process of EAC
o Mu lti lo b ul ar, clr cu mfer entlal • G ran ulatio n tissue wit h possible bo ny erosio n a t
infer io r bo n y-ca rti lagi no us jun ct ion
CT Fi nd ings
• NEeT: T..bo ne CT shows broad -based bon y ex pa nsio n EAC ost eo ma
o f osseou s EAC wit h out agg ress ive fea tu res • Sing le focal, peduncu lat ed , ba lly overgro wt h , wit ho u t
• C ECT: No rma l soft tissues ove rlying ho ny ex pa ns ion, agg ressive featu res
with ou t abn ormal en ha n ce men t
DD x: EAC Le sions
. l"" ~_,r~
, ',."
-», . .)"
. (\
EAC Cho les teatoma Medial Canal Fibrosis N . E. O titis EACOs/coma
EAC EXOSTOSES
Key Facts
Termin ology Clinica l Issu es
• Synony ms : Surfer's ea r; co ld water ea r • Co n d uctive hear in g lo ss (CHL)
• Extern al audi tory canal (EAC) exos toses: Ben ign • Clin ica l profile: C HL in you ng ad ult m a le w it h
overgrow th of bony ex ter nal auditory canal ch ro n ic h ist or y of p rolo nged co ld water ex posu re
(swim me rs, surfers, div ers)
Pathology • Co m plete occlusion of EAC is rare 2
• Lesions usually locat ed m edi al to ist hm us of EAC • Typ icall y exostoses are ben ign, req uiring no
t reat m en t 23
• May req uire su rgical excisio n

!PATHO LO GY • Co m plica tio n s of su pe rio r CAe d rilli ng
Gene ral Features o Tympa n ic me m b rane (TM) pe rfora tion
• Gene ral pa th comments o Tempnro ma ndibu la r jo int (TM» dehi scen ce
o Com m o n ly a rise n ear tym panic annulus, at • Drilling exc ision a long post erior, infe rior &. a n terio r
tympanomas to id & tympanosquam ou s su tures wa lls pe rformed with less risk of co m p lica tio ns
o Lesions usuall y located medi al to isthmus of EAC o Allow s preser vation o f cana l skin , likely leading to
• EAC osteoma usually locat ed lat eral to ist hmus perma ne n t cure
• Etiology
o Direct co rre latio n wit h a mou n t o f co ld wate r
expos ure & prese nce of exostoses shown in su rfers IDIAGNOSTIC CHECKLIST
o Occu rs excl usively in hum an s Co nside r
• Epidemio logy: 7391J pre valence in surfers
• Ost eoma if sing le, foca l osseo us p rotuberance p rese n t
Gross Path ologic & Su rgical Features Image Interpretati on Pearls
• Benign, bony ove rgro wt h of osseous EAC
• Bilat eral circ umferen tial, m u ltilobu la r bony n ar rowing
Microscopic Features of EAe ch aracteristic
• Path ologica lly sim ila r to os teo m as
• Parallel, co ncen tric layers of subperioste al bone
1. Vasam a JP: Surgery for externa l auditory canal cxos toscs. .a
ICLIN ICA L ISSUES repo rt of 182 o peration s. O Rl. J Otorh inolaryngol Rela t
Spec. 65(4):189-92, 200 3
Presentat ion 2. Sme lt GJ: Exostoses o f the Inte rnal a uditor y ca nal. J
• Most co m mon signs/sy m pto ms La ryngol Otol. 98(4 ):34 7-50, 1984
o Cu nd uctive hearing loss (CHL)
a Ot he r signs/sym p to ms
• O ti tis ex ter n a, tin nit us, ota lgia IIM AG E GALLERY
o Alt hough bilatera l, smw ) prese n t with unil at eral
sym pto ms
• Clinica l profile: CHL in yo u ng ad u lt male wit h chron ic
h isto ry of p rolonged co ld wa ter ex pos u re (swim m ers,
surfers, d ivers)
Demograph ics
• Age: Typ icall y seen in you nger age gro up
• Gender: Ma le predominance
• Eth n icity : Lesio n n ot fo und in African-America ns,
reaso n is unkn own
• Co m plcte oc clus io n of CAC is rare (Lef t) A xial left ear T-bo ne CT revoe!s severe narrowing of bony EAC
• No rma l hear in g & n ormal e pit helial m igra tion (arrows) in lhis ca...e of [ AC exostos('s (surfer's ear). N ote thin lin e of
pa tt erns seen po stopera ti ve ly ear wax "seals" [ AC shut ft',\ ult ing in conductive hearing /(h 5. (I{j~hl)
• SIMI su rgica l co mp lica tion rate Coronal lefl car T-bone CT . .hows benig n app earing bon )' EAC
o Ca n al stenos is, TMJ prol ap se, sen sorineur a l loss &. oVt'rgw w !h (arrows) in Ihis ra se of EAC exostoses. There is sign ifican t
pe rsiste n t tympan ic m emb rane perforati o n na rrowi ng of EAC medially, w ith normal lateral cartilagino us [A e.
Treatm ent
• Typ ically exostoses a re benign, requ irin g no treatm ent
EAC OSTEOMA
2
24
A xial /p(r t>.lf rlXJfJC CT clem on s/fal es a small, focal Coronal left {m t-boc e a shows a focal 05 SftXJS
benign <lppeilring 055f'(JWi peduncu/.lted mass within density ma5~ (arrow) within the lalel'a! left Me c1<l55ic
lhe anlc'rio( !t'lf EAe (arrow), com istent with <Ill (or a lx-nign ~tl'Oma .
incidental fAe osteoma.
ITERM INO LO GY Imaging Re commendati on s

• Ro ne CT in ax ial & co ro nal plane fully characterizes
Abb rev iat ions a nd Syno nyms this lesio n
• Ext ernal aud ito ry cana l (EAC) osteo ma • MR not useful in th is sett ing
De finitions
• EAC osteoma: Rare. benig n , focal, ped un cu lat ed, bo n y
overg row th o f osseo us EAe with nor m al ove rlying so ft ID IFFERENTIA L DIAGNOSIS
tissues Beni gn EAC d ebris
• Soft tissue den sit y in Ei\ C witho ut bon y changes
IIM AGING FINDINGS EAC exostoses (surfe r's ear)
• Most com mon soli d tumor of EAC
General Features • Broad -based , benign-appearing, bil at eral bon y
• lIest diagnostic clue: Soli tar y, pedunculated, bony overgrowt hs o f EAC
overgrowt h wit h n o rm al ove rlying soft ti ssue, wit hout • Circu mferen tia l, multi lobu lar
agg ressive featu res
• Locat ion: Un ilateral EAC, single lesio n typical Medi al ca na l fibros is
• Size: Va riable, usually small « 1 cen time te r) • Fib rous mass in medi al EAC wit ho ut ho n y ero sion
• Mor p ho logy: Variable, usua lly oval • Fo llows otitis externa o r surgical proced ure
CT Findings EAC cho lesteato ma
• NECT: 1I0 0 e c r sho ws ben lgn -ap pcartng, fo cal, • Unilateral Ei\C d est ructive mass
pedu ncu lated , bo n y overgrowt h of osseous EAC • In t ramural bony "fla kes" (50% )
• CECT: Nor mal soft tissues overlying bo ny expans io n,
without ahno rmal en ha nce m en t Necrotizing ex te rna l o t itis
• Seve re EAC in fectious pro cess
• Gran ulatio n tissue wit h po ssible bon y erosio n at
lnferior bon y-cart ilagin ous juncti on
DDx: EAC lesion
Debris EAe Exostoses Medial Canal Fibrosis EAC Cholesteatoma

EAC OSTEOMA
Key Facts
Terminology Clinical Issues
• EAC osteoma: Rare, ben ign , focal, ped un culated , • Asympto m atic, usuall y an inciden ta l finding
bony ove rgrow th of osseo us EAC wit h n ormal
ove rlying soft tissues Diagnostic Checklist
• Differentiate fro m EAC exostos es
Pathology • EAC exostosis : Broad-base d , circu mfere n tial, 2
• EAC osteo ma is m uch less co m mon t h an exostose s m u ltil ob u lar, me d ial EAC, b ila teral
• EAC osteoma: Narrow-based, single lesio n , la te ral 25
EAC u n ilate ral
o Persistent tympani c membrane perfora tion

IPATHOLOGY
Treatm en t
Ge ne ral Features • Usua lly med ical th erap y is adeq ua te with out surgic al
• Gene ral path co m me nts excisio n for sym pto ma t ic lesions
o Osteo ma found in o the r 'l-bo ne sites • Surgica l rem oval may be performed th rou gh EAC
o Em bryo logy-a nato my und er local a nes t hes ia
• May be att ac he d to tympanosquarnou s o r
tympan om asto id suture line
• Most commo n ly seen ncar osseo us-cartilaginous IDIAGNOSTIC CHECKLIST
ju ncti o n of EAC
• Etiology: Likely spon taneous bony grow t h Image Int erpretati on Pearls
• Epidem iology • Differentiate from EAC exos to ses
o Usua lly unilat eral & solita ry o EAC exostosis: Broad-based , circumfere ntial,
o EAC osteo ma is m uch less co m mon tha n exosto ses mu ltilobular, medial EAC, bilateral
o 20 l } [) preva lenc e in su rfers (po ssib le ea rly exostoses) o EAC osteoma : Narrow-ba sed, sing le lesion , lateral
• Associated ab no rmalit ies EAC unilateral
o Osteomas associated with EAC & m idd le car
cholestea to mas
o Possible association of osteom as with prior EAC ISELECTED REFERENCES
surgical proce d ures
1. Rami rez-Ca macho R et al: Fibre-osseo us lesions o f the
Gross Pathologic & Surgical Features external aud itory ca na l. Laryn goscope.. 109(:i):488-91, 1999
• Osteoma usually connect ed to u nderl yin g EAC bone 2. Orita Y et al: Osteoma with cholesteatoma in th e ex te rna l
auditory can al. In t J Ped iat r Otorhi n o la ry ngo l.
• May not be connected to underl yin g EAC bo n e 41(1):289-91, 199M
however 3. Keminkl l. et al: Osteo mas and exostoses o f th e ex te rn al
au d ito ry cana l - m edic al and surgical man agem en t. J
Microscopic Features Otolaryngol. 11(2 ): 101-6 , 19HZ
• Pathologicall y sim ilar to exos toses
• Irregularly or iented lam ellated bone su rro un di ng
abunda n t, di screte, fibro vascular cha n ne ls IIM AG E GALLERY
IC LIN ICA L ISSU ES

Presentatio n
• Most co m mo n signs/sym pto ms
o Asymptomatic, u sually an incidental finding
o Ot he r signs /sym p to ms
• If associa ted wit h cholesn..-atom a, sero us o titis
media
• If la rge, co nd uct ive h ea ring loss
• Clin ical profile: No ne, usua lly incidental finding
Demographics
• Age: Broad age ran ge (L eft) Axial left ear r..hone CT reveals a small, focal ossooos
pedun culated lesion (arrow) within the anterior and lateral If'ft CAC
Natural History & Prognosis diagnostic of a n external auditory canal osteo ma. (Right) Axial right
• Permanent cur e with ad equat e surgical exci sion car I-bono Ct domcnsiretes a smaf/ osseo us lesion (arrow) within the
right anterolateral EAC withou t surrounding aggressive features,
• Possib le su rgical com plicatio ns
consistent with an EAC osteoma .
o EAe sten os is
o Tem poro man d ibu lar join t prolapse
EAC SQUAMOUS CELL CARCINOMA
2
26
Anll right ear ' ·boot" (1 ( "''l'afs EAC SCC.a inv,1C!ing A xi.l l T1C+ M R .11 tht· h 'l'l of tilt· ('};,ICffl..ll .lUditory
in{('fiorly fmm FA e. cdusing osseocs ifJVil~ion of cite canal 511()\ ~ invam-c. mh..1flcing EAe ~W1TlOU5 n41
,mc('ro!aleraJltJiI.\loid (arr ow ) g fJ05lC'rior condyl.1f{05XJ csrciooru (am x v) ~lerKx .md IJtPf<Jl to right
(open arrOlv) . rond)4ar he.1d (O()f.'fl Jrra.v}.
• SCCa lend' to spread to all parts of a uricl e I< EAC.

ITERM INO LOGY sparin g middle ear struct ures
Abbreviations and Syno nyms • CEC r
• External auditory ca nal (EAC) ca rcino ma a Heterogen eou sly en ha nci ng EAC lesion
a Pre- & post-a uricu lar, par ot id ma lign a n t n od es
Def initi ons possib le
• Squa mo us ( ell ca rcinoma (Se ea) cen te red wit hi n walls
o f EAC
MR Find ings
• T l \-VI: Low to in ter m edia te signa l
• T2 \VI: Heterogeneo us high sign al in vasive lesio n o f
EAC
IIM AGING FINDINGS • T I C+: Ho mogeneo us o r h eterogeneo us en ha nce me n t
Ge ne ral Feat u res Nuclea r Med icin e Findings
• Best di agn ost ic clue : FAC mass with aggressive
underl yin g bon y c ha nges • PET
o I-D G-PET is accurate in d etecting head & ne ck
• Locati on pr ima ries
o EAC
o Nodal staging also possibl e with PET
o Middl e ea r us ually spared
• Paro tid, pre- & post -au ricu lar nodes most likely
• Size: May infiltrate ce n time ters of periauricular tissue
affected
• Morphology: Lobu lar, in filt rating
CT Find ings Imaging Re commendati ons
• Bon e algo rit h m tem po ral hon e cr best predi cts tum o r
• NECT ex te n t (osseo us dest ruct ion)
o Temporal bo ne CT
• Larger SCCa lesio ns: Use ho th unenhanccd T-hone c r
• Ea rly seea p resent wi th EAC soft tissue mass
& en ha nced MR imaging
without bo ny destru ctive c han ges
• Lar ge r seea shows under lying bon y destru ctive
ch anges
DD x: EAC Lesion
EAe Debris KC'r.1 loS;S Obturens N. E. Otitis EACCholesteatonu:

Key Facts
Imaging Findin gs Patho logy
• Best dia gn ostic clue : EAC mass with agg ressive • SCCa clin ically & pat ho logica lly sim ila r to
underlyin g bo ny cha nges pseudoeptthelio ma tou s h yperplasia
• Bone algorit h m t em poral bo ne CT best pred icts • Ma ligna nt tu m o rs o f EAC are relati vely rare
tumo r extent (osseo us destru ction )
• La rger SCCa lesion s: Usc both un cn ha n ced 'l-bo nc cr Clinica l Issues 2
& en ha nced MR im aging • Ulcerat ing EAC mu cosal lesion
• Wit h co m plete resection wit h free ma rgin s, 5 year 27
Top Differential Diagn ose s su rvival = l 00 l MI
• Benign EAC de bris • In com plete resection 5 year su rviva l = 66911
• Med ial can al fib rosis
• Kerat osis ob turans
Diagnost ic Checklist
• Necrotizing externa l otitis (N. E. oti t is) • Secondary EAC inv olvement from region al pri mary
• EAC cho lesteato m a SCCa is much mo re co m mon tha n p rim a ry EAC
SCCa
o Increased incide n ce rep or ted in pat ients wit h

IDIFFERENTIA L DIAG N O SIS o to logica l d iseases
Benign EAC debris • Possibly seco n dary to risk factor of chronic
• Soft tissue m ater ial in EAC wit ho ut bon y erosio n inf lammati on
• Ep ide mi ology
Med ial ca na l fibrosis o Ma lign a nt tum o rs of EAC are relati ve ly rare
• Thick, fibrous m ass wit h in medi al EAC wit ho ut bon y • SCCa > > basa l cell carci no m a > > aden oid cys t ic
erosion ca rci no ma
• Follows otitis ext e rn a o r otit is m edia or su rgica l o Incid en ce o f can cer of EAC: 1: I,OOO,OOO/yea r
procedure o Carcinom as o f EAC < 19i1of a ll h ead & neck
• Classica lly bila tera l ma lign ant neo plasm s
o H51X) of a ll EAC malign a n t tu mo rs a rc SeCa
Ke ratosis ob tura ns • Asso cia ted ah norm a lities : Cl in ica l hi sto ry o f EA C
EAC obstru ct ion with d esq uam at ed kerat in ch ro n ic in fla m ma to ry processes
• Hom ogeneous soft t issue fillin g EAC
• Nor mal EAC osseo us cana l Gross Pa th ol ogic & Surgica l Feature s
• Presents as lll-defined, u lcerati ve ± in d urated lesio n
Necrotizing exte rn al otitis (N. E. otitis)
• Severe EAC infectio us p rocess sp reads to sub jace n t Microscopic Feature s
struct ures • Seea is defined as having predom inant ly sq uamous
• Older di ab eti c pa tients; seco nda ry to pseudo monas d ifferent ia tion
• Gran ulatio n tissue with possibl e bon y erosion at o In t racell ular bridges o r kera ti n izat ion (± kera tin
inferior bo n y-ca rtil agin ous ju nctio n pear ls) present
• Diagn osis co n firmed with Tc-99 m o r gallium sca n
Staging, Grad ing o r Classification Crite ria
EAC cho les teato ma • '1' 1: Tu m o r lim ited to EAC w ithou t bon y ero sio n or
• Un ilatera l so ft tissue wit h in EAC associa ted und erlyi ng soft tissue invo lvem en t
bon y destructi on • '1'2: Tumor wit h lim it ed EAC osseo us eros ion o r soft
• Often Sl!C bony "flakes" in m ass t issue invo lvem en t
• May exactly mim ic EAC SCCa • T3: Tum o r erod ing osseous EAC with lim ited sof t
tissue/middl e ca r/ m astoid in vol vem ent
• '1'4: Tu m or erod ing deep er inner ea r
IPATH OLO GY st ruc tu res/ te m po rom a nd ibu lar join t (TMJ)/cx te n sive
soft tissue extensio n, or facia l nerve paresis
Gene ra l Fe atu res
• Gene ral pa t h co m men ts
o SCCa clinica lly & pathol ogically sim ila r to ICLIN ICA L ISSUES
pseudocptthcliomat ous h yperp lasia
o Se Ca is mo st co m m on ma ligna ncy o f F.AC Pre sen tati o n
o Relevan t a na to my • Most co m m o n signs/symptom s
• EAC, au ricle & adj acent scalp nodal drainage is to o Ulcerating EAC m ucosal lesio n
pre- & post-a u ricu la r an d parotid nodes • Presentati on may m imi c oti t is ex tern a o r EAC
• Et iology cholesteat o ma
o O the r signs /s ym pto ms
• Other ea rly sym ptoms: Ot orr hea , ota lgia & o Paro tid gla nd (d irect in vas ion O f nodes)
co nd uct ive h earing loss o Tem poroma ndi bu la r joint
• Othe r lat e sy m pto ms (poo r pro gnosis): Facial o Ho rizontal pc tro us in ter na l ca rot id ar te ry ca na l
nerve paresis. ex te ns ive bulky tumor primary &
paro ud /pertpa rot ld/ccrv ical noda l di sease
Image Interp retation Pearls
o Early-small lesio n s mim ic benign processes • Look for osseous destr uctive ch anges!
• Both clintcal &. rad iological mimics • EAC seca lesions cru cial as bon y in vasion predict s
2 o Late-large lesions cl in icall y less co nfusi ng t reatm ent o ut come
• CECT or MR shou ld inclu de parotid gland , lookin g fo r
• Any EAC lesion wit h bo ny erosion should he
211 co nsi dered malig nan t 1st o rder nodes
• Early diagn oses grea tly affects ou tco m e o Parot id nod es are l st order dr ain age n od al gro up
• Clin ical pro file: Elder ly wo man with clin ical o t itis
ex te rn a N. ulcerati ve EAC lesion
Demographics I. l'u tcc JI.c t al: Sq ua mous c('11 ca rcinoma of th e e xte rn al
• Age a uditory ca nal. Ear Nose Throat J. 8] ( 1):9 ,2004
o Disease of eld erly 2, C h oi J Y c t ill: Mode o f parotid involvem ent in exte rna l
o Med ian age - 6S yea rs o ld a udit o ry ca na l ca rci no ma. J l...aryn go l Oto l. 117( 12 ):95 1~ 4 ,
• Gender: More com mon in women 21X}]
:l _ Kucg t PI' c t al: Sq ua m o us ca rcin o m a o f th e ex te rn al
Natural Histo ry & Prognosis a ud ito ry ca n al: a differen t a pp roa ch . Clin Otola ryngol .
• EAC seen destroys osseous EA C, t hen in vades 2 7(3 ): 183-7, 21X)2
su rro undi ng anatom ic landscape 4. Nyrop ~ 1 ct al: Ca ncer o f the e xt erna l aud itory ca nal. Arc h
Otola ryngol Head Nt"l"k Surg. 12K(7):K34· 7, 2002
o Mo st co mmon ly extending inferiorl y int o so ft
5. Wol fe SCi ct a l: Bilat eral 5oquamo llS cel l ca rci noma of th e
tissu es belo w tem po ra l bo ne ± ma stoid li p ex tern a l au d itory ca n als. I..a ry ngcscope- 112 (6): I(Xn·s.
o Medial ex ten sion into mi ddle ear ca vity is rare 2002
o Sup e rior ext e nsio n in to mid dle crania l fossa also h. Lim l.H c t ,II: M alignan cy o f til l' te m poral hone an d
rare exte rnal a ud itory can al. Oto lary ngol Head Ned Surg.
• Wit h co m plete resectio n with free margin s, S year 122(6):882-6, 2IXlO
su rvival = 10<)l}'i. 7, Moody S1\ et al: Squa mous l"<'11 ca rcin oma o f t ill' ex ter na l
• Incomplete resect ion 5 year surviva l = 66r.Yt. a ud itory canal: an evaluat ion o f J staging system . Am J
0 '01. 2 1(4 ):582-8, 21X)0
o Recurren ce rare stage I & II: 2Sr.Ytl
8. Ilash i N et al: The role o f rad io th erap y in treating
o Recurrence rate stage III & IV: l Q(Yl'il ~q u a m o u s cel l carc inoma o f th e ext ern al a ud itory can al.
• Middle ea r cav ity involve men t I 5 yea r surv iva l by cvpcctally in ear ly ~ Ia~ l'~ of d isease . Radiothcr 0 111..'0 1.
SO'X, 56(2):22 1-5, 2000
• Exte n sio n into sku ll base or TMJ ind ica tes poor 9, Kami ya M c t ul: Pigm e n ted sq ua mous ce ll ca rcinom a wit h
progn osis dendriti c melan ocy te colonization in th e ex te rna l auditor y
• Lymph node meta stases al so a poo r prognostic ca na l. Pathol In t. 49 (10) :909 -12. 1999
ind icat o r 10. Uc h id a N et 'II: Sq ua m o us ce ll ca rcinoma o f th e exte rnal
a ud itory can al: t WO GI \<" ~ tr eated wit h hi gh dose ra te 19ZIr
o Any noda l disease should be co nsidered advanced
rem o te afte rloa di ng sys te m (RA1.S). Radlat Med .
d isease {stage IV} 17(6):H ] · 6, 1999
• Dista nt met astati c disease is a very poo r prognost ic 11. Okada E c t a l: Hbc rsco pc-asslvtcd e valuatio n o f
ind icato r prereconst ructcd an d postr econ struct ed exte rn al a uditory
o Any d ista nt metastat ic d isease sho uld he co n side red canal. J Cranio fac Su rg. 9 (3):22 8-32, 199M
stage IV 12. Test a J R ct al: Prognostic factors in ca rcinom a o f the
exte rn al aud itory ca n al. Arch Otolar yngo l Head Neck Su rg.
Treatment 12317):720- 4, 1997
• Con t roversia l U. Pra sad S ct ill: Efficacy o f surg ical tr ea tm ent s for sq ua m o us
• Surgery nea rly a lways performed cel l carc inoma of th e te mporal 1>011<.': <l lite rature review,
o With loca lized tumors, en bloc resecti on o fte n Otolar yng cl t k ad :'>Jed . Surg. 11O(3):2 70-MO. 1994
14. Aust in J R c t al: Squamous cel l ca rd noma of the e xte rna l
curative a uditory (a l1,1 1. Th e rap eutic pro gn m i'i based o n a p roposed
• Hest o u tco me s repo rte d wit h co m bined surge ry &. stagin g system . Arch Otola ryngol Hca d Nec k Surg.
posto pe rative radiati on therap y 120(11):1228-:l2, 1994
• Topica l S ~ FU &. preoperative rad iot herapy also I S. Kin ney SE: Sq ua m o us c('11 ca rcino ma o f th e ext e rnal
advocate d a ud itory ca n al. Am J Oto !. 10 (2): III +6, 19M9
16. Hahn SS ct al : Ca rcino ma o f the m idd le ear a nd ex ternal
a udit or y ca na l. In t J Radiat On co l Bio i Ph ys. 9( 7): 1{)OJ-7.
IDIAGNOSTIC CHECKLIST 17.
19M]
Olsen KD c t al: Rad iogra p h ic assessment of squam o us re t!
Consider ca rcinoma o f the tem poral bo ne. Laryn goscope.
93 (9):1 162-7, 19M3
• Seconda ry EAC invo lveme nt fro m regiona l prima ry IX. Wang CC: Radia tion th e rap y i ll th e management o f
SCCa is much m or e co m mo n tha n prima ry EAC SCCa carcinoma of th e ex terna l aud itory canal, middle ear , o r
• Radi ologicall y int errogate sur rou nd ing structu res for mastoid . Rad iology. 1 1(l C~) : 7 IJ~ S . 19 75
possible in vo lve men t
IIM AG E GALLERY
Typical
(IL/ I) Coronal right ear
T-bone CT demonstrates _~ oit
tissue densit y fillin/{ the right
fA C (arrow) with in{{'fior
2
osseous destruction tope«
arrows) without bony neck s. 29
cons istent with CAe seed.
(RighI ) AX;,ll ( Eel shows
large hl·ll·(ogt'Of!Ously
enh.mcing mass Misillg from,
snd filling the laler.ll right
lAC. displacing tht> pion,]
and 50f' tissues of lhe ear
latt'fJlly (arrow) . Biop sy
confirmft/ Seed.
Typical
(/ LI t) Axial Ct ct shows a
mildly ('nhane;0f.: soit tissue
mJSS involving pmtC'rio r wa ll
of/prl cAe (,m o w ), with
encroachm ent of {,lIl'ra/ EA C
canal. Hon e' C t confirnlL'cJ
bony oew ucuon w ith /his
ire seea. (RiKIII ) A xial
Ci Cl shows .1
heterogenoously enha ncing
mass ems ruuing from
posterio r mllJ o f right EAC
wilh extension into the mft
tissues of the p in na of th e e.u
(arr o w). fAC remains palent
in this r ase.
Typical
(/~JI) Axial right ear I-bone
CT revests 5ubllf>EAC Seed
cetu ered in the inferior left
EAe with sssocis ted osseous
(~ truction of tbe 170m of tbe
EAe (arrow) end posrerior
1.\1/ (open .mow). (High"
Coronal /('Il t'<lr t-booe CT
shows d c ircumietvntie t leit
EAC SCCd with infeJior
ossccc s dp stru ct;"/P changes
(a rro w). Notice .~pa ring of all
middff! ear structU((·S.
CONGENITAL CHOLESTEATOMA, MIDDLE EAR
2
30
uxoiMI i:f<lphiC sha.vs uX"b'f..YlitiJI dkM.·slealOfTliJ AWl T-bone CT ('('\'Pills J lobu1.lr but smooth,
involving the middk.~ esr: Notice till' lesion hJs (!).lended w(,/k ircumscribed middle (>.lr congenital
nl<'di.ll to (,..~kks (clrrVIV) as it mgtJli5 the entire ossicle c~ /(',l/oma medi.11/o ossKuLJr ctuin (arrow ). Nolke
ctuin . I M;\ in /dCl. rht> os!>k.Je chain k ¥Jared (rom f>frn;Otls.
o En tire m idd le ear (ME) ca vity no t invo lved at

!TE RM INO LO GY p resentati on
Abbrev iations a nd Syno nyms • Mo rp hology: Lob u lar, di screte ME ma ss
• Abbreviation : Co ngen ita l cholesteato ma, middle ca r CT Find ings
(Cel,-ME) • NEeI'
• Syno ny ms : Primary cho lesteato ma, ep iderm o id, "skin o T-ho ne cr ap peara nce de pen ds o n size of lesion
in th e w rong place" o Sma ll CC h-ME: Detected early, a ppears as
Definition s we ll-circu m scribed XIE lesion
• Cell-ME: Middle ca r aberra n t epit he lial rest of o Large CC h· ME: La rger mass may erode oss icles,
exfoliated keratin within str atified sq uamo us m idd le ear wall, lat eral semic ircu lar ca na l o r tegmen
epithe liu m tympani
• Bo ne erosio n less co mmo n t ha n in acq u ired
cholestea to ma
IIM AGING FINDINGS • Occ u rs lat e in d isease
• Ossic u lar erosio n unusu al wit h anterio r
General Features mesot ympan um in vo lve me n t
• Best diag nosti c clu e: T-bo nc cr shows smooth , • Long p rocess o f in cu s &. sta pes su pe rstr ucture
well -circumscribed midd le ear mass ± ossic u lar mo st co m mo n ly d estroyed ossicles
erosio ns • La byrin th ine exte nsio n may occu r b ut o n ly late in
• Locati on d isease process
o Mult iple m idd le car locati ons • If ad itu s ad ant rum occlude d, mastoi d air cells
• Ant erosu pcrior tym pa nic cavity near eustac hian o pacify wit h retained secretions
tu be o Co mmo n locat ion s o f CCh-ME
• Posteri or ep tty m pa n um a t tym pa n ic isth mi (a rea • Ant erosu perio r middl e car, ad jacen t to eustach ian
bet ween m idd le ear cavity &. atti c) tube &. ant erior tym pa n ic ring, media l to oss icles
• Size • Ncar stapes
o Usua lly small beca use identified o n otoscopic exam • Posterior epitym pa n u m, at tym pan ic ist hmi
DDx: Middle Ear l esion s
I\cq . Cho /('Slealom a (flO/esterol Granulom a Glom us Tvmpen icum F.trial Sch wannom a
Key Facts
Termin o logy Path ology
• Syn on ym s: Primary cho lestea to ma, epide rmoi d, "skin • Co nge n ita l ectoderma l rest is left be hi nd in m idd le
in t he wrong p lace car cavity
• Mechanism : Abnorma l m igrati on of ex terna l ca na l
Imaging Fi nd ings ectode rm beyond tympanic rin g
• Best diagnostic clue: T-bone CT sh ows sm oo th, • Epidemi ology: Account for 5% of all temporal bon e 2
well-circu mscribed midd le ear ma ss ± ossicula r cholesteato ma s
erosions 31
• Early or "closed" Ceh: Sma ll, enca psulated focal
Top Differe ntia l Diagn oses a nterior tym panic cavity ma ss
• Late or "open" Ceil: Large ME ma ss, exte nd s
• Pa rs flaccid a ch olesteato ma, acqu ired
th rou gh out cavity & mas toid co mp lex
• Pa rs te nsa cholesteato ma, acq ui red
• Cho lestero l gran uloma. m idd le ea r Clinical Issu es
• Glo m us ty mpa nicum pa raganglio ma • Most co m mon signs /sy m pto ms : Avasc ular ME mass
• Facial nerve sch wa n no ma , midd le ea r be hi nd in tact 'I'M
• Un ilatera l co nd uct ive hearing loss (CHL)
MR Find ings Cho lesterol gra nulo ma, middle ea r

• T l WI: 150- to hypointcn se ~t E mass • Clin ical: Otoscopy reveals blue Tl'.l
• T2WI o Usua lly ha s history o f prior surger y o r recu rrent ME
o Interm ediat e in ten sity ME mass inf ection
o With larger lesio ns, aditus ad antrum obst ruct ion • c r findings
seen as h igh signal retai ned secre tions in ma sto id air o Midd le ea r ma ss with ossicu lar erosio n common
cells • Mil find ings
• '1'1 C+ o T l C· MR shows h igh signa l in ME
o Perlphcra lly-e n hancing ME ma ss
• CCh· ME is non enhancing material surrounded by Glom us tympani cu m par agan glio ma
thi s subtle rim -enhancement • Clin ical: Otoscopy shows pulsat ile, vascu lar mass
o If lesion is lon g-standing. associa ted sca r may be behind 'I'M
seen as thi ckened a rea of en ha nce men t adjacen t to o Un usua l in pedia tric o r ado lescen t patien t
CCi l-ME • cr findings
o Sessile mass o n coc h lea r pro mon to ry
Imaging Re commendations o No bo ny eros ions present
• Bone o nly f- bon c cr is exa m ina tio n of choice • MR findi ngs
• 1'1 C+ MR is co m plime ntary exa m in certai n o focal enhanci ng mass on T I C+ MR
circu ms tances
o Recom me nded if recurren t or large CCh· ME Facial nerve schwanno ma, middl e ea r
o Also recom mended if diagn osis uncertain wit h • Clinica l: Otoscopy shows avasc u lar mass beh in d in tact
glom us tym pa n icu m or facial nerve schwa n no ma 'I'M
possible co ns ide ratio ns o Appeara nce ca n closely mimic CCh·ME
• c r find ings
o Tubular mass e ma na ting from tym panic facial n erve
IDI FFERENTIAL DIAGNOSIS canal
o En larged bony facial nerve ca nal
Par s f1accida cho lesteato ma, acq uire d o En larged genl cu late fossa
• Clin ical: Ot oscopy reveals retracti on poc ket o r par s • ?vlR find ings
flacc ida TM perforation o Tubu lar e n ha nci ng mass on 1'1 C+ MR
• cr find ings o Exte n ds fro m geniculate ga ng lion along tym pan ic
o Scutum erosion with lesion in Prussa k space of segme n t of facial nerve
lat eral cpttym panu rn
o Ossicu lar chain & lateral se mici rcu lar ca na l more
likely eroded IPATHOLOGY
o Chronic infla m matory cha nges presen t
Ge neral Features
Par s ten sa cho leste a to ma, ac q uired • Ge nera l pat h co m me n ts
• Clin ical: Otosc o py reveals pa rs ten sa Th1 perfo rati on o Co nge n ital cho lesteato ma 'l-bone locations
• c r findings • Pctrous apex , mastoid , m iddle ea r, middle
o Lesion en larges medial to ossic les ea r-mastoi d & external audit ory ca nal
o Ossicu lar eros io n comm o n • Etiology
o Con ge n it al ecto d erm al rest is left behind in midd le o If e ustac h ia n tube obstr uct ed , ME effusio ns & o titi s
ear cav ity occu r
o Mechanism : Abno rma l m igrati on of exte rnal canal a Large r lesio n s w it h infection m ay be diffi cult to
ectoderm beyond tympan ic ring di fferentiat e fro m acq ui red choleste a to m a
o Becomes a m ass-like middle ea r accumulation of • Large lesio n s o r po steri o r ep itym pa n ic CCh have
st ratified epit helial sq uamo us cells recurren ce rat es as h igh as 201M)
• Epid erm oid formation is poin t of t ran sfo rm atio n o Sta ged su rgica l resectio n o fte n used for large lesio n s
2 bet ween tympan ic cavity & eustac hia n tube • 't-bo ne c r recommended to assess for recurren ce
• Rest o f st ratified sq uam ous epidermal cells where
32 man y cell ari se Treat ment
• Epidemi ology: Account for SIX) of all tem pora l bo ne • Co m p let e surgi ca l ext trpatlo n e t rea tm en t o f ch oice
cho lesteatomas o My ri ngo to my for sm all, we ll-e n ca psu lated lesions
• Associat ed abno rm alities o Ma stoi dect o m y for la rge r lesio n s
o EAC at resia can present wit h associat ed Ce ll • Ossicle ch ain reconstructi on m ay be n ecessar y
o Rarely asso ciated with lst b ran chi al cleft remnant
Gross Path ologic & Surgical Features IDIAGNOSTIC CHECKLIST
• Circu msc ribed, pearl y-w hit e ma ss with capsular sheen
• When detected early, no associated infl ammat ory Co nsider
ch an ges • Ce h -ME m ass is seen behind in ta ct TM
• No h isto ry o f rep ea t or c h ro n ic ME in fect ions present
Micro scopic Features • ME is o pacified w it h wa ll ero sio n in pati e nt wit h
• Identica l to ep idermo id incl usio n cyst ex terna l a ud ito ry ca na l a tresia
• Stra ti fied sq ua mou s ep it he liu m , with prog ressive
exf o liat ion o f keratin ous m a te rial
• Co n ten ts rich in cho leste ro l cr ysta ls ISELECTED REFERENCES
Staging, Grading or Classificatio n Crite ria 1. El-Bita r MA et al: Co n ge nital middle ca r cholesteatoma :
• Early o r "close d" CCh: Small, encapsu lated focal need for ea rly rccognition--rolc of comp uted tomography
scan. Int J I'cd iat r Otorhinolaryn gol. 67(3):231-5, 2003
an te rior tympan ic cavity m ass
2. Po tsic WI' et al: Congenital cholestea to ma: 20 yea rs'
• Late o r "o pen" Ce h : Large ME m a ss, exte nds ex pe rience.' at Till' Children's Hospital of Philadelphia.
th roughout ca vity & m ast oid co m p lex Otolaryngol Head Neck Surg 126:409-14, 2002
3. Darro uzet V et al: Congenital middle ear cholest eato mas in
children: our experience in 34 cases. Otolaryngol Head
IC Ll N ICA L ISSUES Neck Su rg . 126 (l ):34-40, 2002
4. Koltai I'J ct <II: The natural history of congenital
Presen tation cholesteatoma . Arch Otolaryngol Head Neck Su rg.
• Mo st co m m o n signs /s ym ptom s: Avascu lar ME m ass 12H(7):H04-9 , 2002
5. Nelson M et al: Congenital cholesteatoma: classificatio n,
behi nd int act 'I'M managem ent, and outcome. Arch Ot ola ryngol Head Neck
• Ot her sym pto ms Su rg. 12H(7):81lH, 200 2
o Un ilateral cond uctive heari ng loss (CHL) 6. t'o tstc WI' ct al: A stagtn g system for co ngeni tal
o Large lesions ca n o bst ru ct eus tach ia n tube with ch o leste ato m a . Arch O tola ryngo l Head Neck Surg.
resultant ME e ffusio n & in fecti on 1 2 ~ (9) : J(X)9-12, 200 2
o May he di sco vered su rgica lly aft er ch ro n ic ME 7. EI -Bitar MA ct al: Bilateral occurrence of congenital midd le
effusio n u nresp onsive to tyrnpano sto my tubes ear cholesteatoma. Otolaryngol Head Neck Surg.
12 7(S):4HO-2, 2IX)2
Demograp hics 8. Shoh ct JA et al: The m an agem en t of pe dia tric
cho lesteatoma. Otolaryngol Clin North Am . :~ S ( 4 ) : 8 4 1 - S I,
• Age
o Average age o f presen ta tion or d et ecti on 2002
9. Yl'O SW ct al: The clinical evaluations of pathop hysiology
• Ant eri o r or ante rosupe rior: 4 yea rs for congenital middle car cholesteatoma. Am J
• Po sterosu perio r & m esotympanurn: 12 years O tolaryngol . 22( 3 ):184-9 , 200 1
• Att ic & masto id a n t ru m in vo lvement: 20 yea rs to . Yammine FG ct al: Anterior and posterior middle ear
• Ge n de r . congenital cholesteatomas in child ren. J Otolaryngol.
o M :I' = 3 : 1 3D! I ):29-33, 200 1
o No expla na tio n fo r m a le pr ed omina n ce I I. Melero GA ct al: Facial paralysis: An unusual present ation
of congenital cho lesteato ma. Otolaryngol Ilead Neck Surg.
Natu ral Histo ry & Progn osis 122(4):61 5-6, 2000
• Sm a ller, a n ter ior lesio n s h ave better o u tco me, w it h 12. UUJI let al: Congenital cholesteatoma of the middle ear.
co m ple te su rgica l resection Clln I'ediat r (Phil a ). 39(9):5"'9 -5 1, 2000
13. Tos M: A new path ogenesis of mcsotympnnic (co ngenital)
o Sm a ller les io ns m a y be encapsulated, o r cys tic, &
cholesteatoma. Laryngoscope. 110(11):1K90-7, 2000
easi ly rem o ved 1.... De la Cruz A et al: Detection an d managemen t of
• If u nt reat ed , keratin d eb ris accumu lat es o ve r tim e, child hood cholesteatoma. Pcdlat r Ann. 28(6):370·3, 1999
w it h resultan t la rger lesion 1.5 . Frledberg j : Congenital cho lesteatoma. Laryngoscope
o En la rging, cyst -like CCh m ay ruptu re, ex te n d ing !tH: 1-24 , 1994
throug h out ME
IIM AG E GALLERY
Typical
(I.e/I) Axial Nr CT "~b OIl L' CT
of le(l ear sholVs cl
we ll-de fined middle paf
abu ttin g the 1 1 ldlfeu~ (arrow)
ma ss 2
...':: disp/.lcin8 Ih (' o ssicular
chain laterally. No ossicuier 33
erosion is .w e n despilP lesion
p ro xim it y. (Rig/II) Corolla I
t-bor e CT sh ow.~
circ um scr ibed cOflg f;'nit al
cbolesteetoma at ti ll'
cochlear promom orv
(arro w). Yo ung age .11
p rese nta tion S. avascular
appmrance _~ lJgg( 'S I5 this i_~
not J glomus tympanicum.
Typical
(/.t>JtJ Co ro nal T-bo nc C1 of
feft t'ar shows late ral
tym panic: cavity congenital
c h()/('.~tf'.1 toma, displecmg
m5ic/(' ~ fIlt>< lially &
scalloping l-uerel
f'pil ymp.1nic wall. 1M \Vd S
touci during otoscop ic
exsmtnation. (Ril: h l) Coronal
rJ C+ MR rovosts
IlOf}('I lIJ""cing ;so;nlellw
co nf!,cll ital cholestt' ..Homa
(arrow ). No te t>nh,mcing
SC' lf (op en .l rro\\!.~ ) latt'r al to
cb otesteatorna . CT cl/on t'
can not dif(C'renti.lte scar
(rom c "o ft'~(('a toma .
Va riant
(/.('[1) lI xial [ -bo/le CT sho ws
smal/ middle ear congt'nital
cllO/es,patomJ, colltig t lOU ~
with the tympanic
membrane (arro w).
Otoscop ic exam ind tion
sho veed int ac t TM wil h ~mJII
avascula r retrotvmpsn ic
ma ss. (RiKIl I) A lo;iall- bonp
c r rcwa/s [ IIC bony atresis.
J\<lid d/(' ear cavity is smstt S
{iI/pel wit h c ongpnila/
chotes te-uom a sc."a //opin g
t>pirymp anic walls (arrow) .
Anterior waif is dohiscent
(open arru w ).
OVAL WINDOW ATRESIA
2
34
Coronal graphic illustrates oval winclv.v atresia (p.1ltJre s A~ i.11 T-lXJnl' CTof right ear $!Jow's absence of normal
including dysp/Jsi<l of slapes cnsre and roo/plate (arrow) oval window (a/row) . D istal incus & sIJfX'S are
and lymp.mic sC'gmenl oi facial nerve abnormal localion deformed. Sofl tissue overlying oval window <lrea is
toocn arrow) . anom,]/ou.~ facia/ nerve topori arrow).
o lnfero rnedl all y positi oned facial nerve (tym pa nic

!TE RM INO LO GY segme n t) overlying expec ted location o f OW
Abbreviations and Syno nyms • Co m mon associate d find ing (> 60(M))
• Ov al window at resia (OWA) • Critical su rgical im portance
• Co ngen it al oval w i n dow (O W) n on -development; • Appreciated o n axial & corona l images
conge n ita l absen ce of OW o Norma l exte rna l auditory ca nal (EAC)
• Most co ngen ital m idd le ear (ME) ano malies are
Definitions associ at ed with EAC atresia
• OWA: Absen t cleavage plan e (OW = stapes • CECT: Co n t rast mat erial no t need ed o r recommend ed
foot plat e-annol ar ligam en t) between lateral
semic ircular canal abov e &. coc h lear p ro monto ry
MR Findings
belo w associat ed wit h an omalo us stapes &. • MR no t useful o r reco mme nded
rnalposit ioned e N ? Imaging Recommendations
• Th in-sectio n (1 nu n ) axial & co ro nal T-bo nc c r
images target ed for m axim al bony detai l
IIM AGING FINDINGS o 0.5 mm ima ges o r overlap th rou gh OW adds
General Features add itio nal in for mati o n in most cases
• Best di agn o stic clue: OW covered by thi n bo ny plate
on co ron al bo ne CT
• Locati on: Oval window &. n iche
ID IFFERENTIAL DIAGNOSIS
CT Findings Tympanosclerosis
• Clinical: Histo ry co ns iste n t wit h ch ro nic
• N EeI'
o No rma l OW repl aced by ossific web o to ma stoid ilis
• Best see n in co ro nal pla ne • Imaging
o Malfo rmed stapes su perst ruc tu re (absenc e of normal o In vol vemen t o f OW resu lts in thick, irregular area o f
pa ired cru ra) &. d ista l incu s den se hone
• Best see n in axi al plane
DDx: l esions Ca using Co nd uctive Hearing l oss
Malleus Fixation Absenl Distil l Incus Tympanosc/erosis Fen . Otosclerosis

OVAL WINDOW ATRESIA
Key Facts
Terminol ogy • Malfor med sta pes supe rstruct ure (absence of normal
• OWA : Absen t cleavage plan e (OW = sta pes paired crura) & distal incu s
footplate-annu lar ligam ent) betw een la teral • In feromedially posit ion ed facia l nerve (tympa n ic
semicircular canal above &. co ch lear promonto ry segmen t) ove rlying ex pec ted location of OW
below associated wit h a no malous sta pes &
rnalpostttoned eN ?
Pathol ogy
• Best h ypothesis: Primitive sta pes fails to fuse with
2
Imaging Findings pri m itiv e vestibule d uring 7th wee k of gestation
• Normal OW rep laced by oss ific web • If stapes forms but a nnul a r ligamen t does n ot,
conge n ita l stapes fixati on results (ins tead o f OWA)
o Middl e ea r debris & sclero t ic mastoi d ind icating

chron ic oto ma sto id itis Natu ral Hist ory & Prognosis
a Stapes & facial nerve are no rmal • Surge ry less successfu l tha n p rost het ic stapedecto my
for ot oscle rosis
Fenestra l otosclerosis
• Clin ical: Rare in ch ild ho od Treatm ent
• Imagin g • Vestibu loto m y wit h stapes prosth esis o r tot al oss icular
o Typically results in abno rmal ho ny den sit y localized rep lacem en t p rosth esis (TO Rr)
to a nterior O\V • e N7 ecto pia into OW n ich e is surgical relative
o Obliterati ve variety « 1OIJ{») results in sim ila r co n t ralnd ic at to n
appea rance to OWA hut sta pes &. facial nerve are Surg tca t repa ir more d ifficult du e to locatio n of eN? &.
intrinsically nor mal diffi cu lty ide n tifying appropriate a r:..'a fo r fe nestr ation
IPATHOLOGY IDIAGNOSTIC CHECKLIST

Gene ral Features Image Inte rpretati on Pearl s
• General pat h co m men ts • Ca refu lly inspect O W in c h ild ren with co nge n ital
o O \V no n-develo pmen t results in osseo us oh lite ratio n co ndu ctive deficit
• Th ick hon y plate or by co n cen tr ic narr owing wit h
"d im ple-like" de pression
• Etiology ISELECTED REFERENCES
o Best hypot hesis: Primitive stapes fails to fuse wit h I. Boot h TN ct al: Imaging a nti clinica l ev alua tio n or Isolated
primitive vestibu le during 7th wee k o f gestatio n at resia o f the ov al window. I\JNl t 2 1: 1 7 1 ~-I , 2000
o If stap es for ms but annu lar ligam ent do cs not , 2. Zcifcr B ct al: Congeni tal absen ce o f the o val win dow:
conge n ita l stapes fixa tio n results (in stead of O\VA) Rad io lo gic dia gn o'iio; and as\oria tl'd ano mali es. i\Jl'\ lt
2 1:322 · 7,2()(XI
• Cautio n: Th is may result in conge nita l cond uctive
3. La mbert Pit: Congenital ab sence o f t he ova l windo w.
hea rin g loss in absence of imagi ng find ings! Lar y ngosco pe. 101 ::i 7--10, 1990
• Epld crmology: OWA bilateral in - 40 1.I'C 1
Gro ss Path ologic & Surgica l Features
• At surgery ma y see t he fo l lowt ng IIM AG E GALLERY
o Tym pa nic segm ent of CN ? ahno rmal in all cases
• Usually infero med ially position ed
o Abno rma l incus len ticular process associat ed
• Expecte d sin ce di stal in cus & stapes su perstr uct ure
a rc bo th fo rmed fro m 2nd bran ch ial arch

Prese nta tio n
• Most co m mo n signs/sym ptoms
o Profound co nd uctive hea ring de ficit in ch ild
o Lack of histo ry o f oto masto idi tis; n o rmal EAC
(I~JI) CmOfl .11 T-hon e CT 0; righ t ear shows .lbst'n c(' o( normal oval
Demo graphi cs window w hich i~ (ppl,lced h y an .1(P.1 of msifi c.ltio/J (a / ro w ) . Soft
• Age: Usua lly d iscovered in ch ild ren tissue stftlclurp (open arrow ) represents IHJlpositivf) ('d facial n er ve.
• Gende r: M > F (Hi1:'''' Coronal T-IxJlJe CT of left ear, d varian t example or oval
windo w .l/rt·Si<l. A bifid tympanic s('gmc'nt of fad,11 norvo (, I rro\\'~)
Cd lJ h e seen overlyillg the OVJ I windolY ,l tf e5i,1 plate (o / JP/l dfroW) .
LATERALIZED INTERNAL CAROTID ARTERY
2
36
M ial ft>It mf l -baw cr sha."15 (Ii.~al cervical 10\ with AAAJI lrli ear T-bone a (('\'l'afs ,1 Jateralized lCA
no bony aA~.,ing along its IJll" .l l margin (.m aw). (arrow ). NOll:' tf'I.ltionship of \'[",,'ieI to basal turn of
Nolin.' tftt:o protrusion of tilt> arrcvy into the .mlefoin;ffiof n xhlt,.) (open .JfW.V). The dnll.,inr loc.Jtion of llk-~
mkklk' (>Jf cdvi/y. wl-ICA exclude'S dbt"fanl /CA.
o Excellen t mod alit y to co n firm Lat-ICA

ITERM INO LOGY o Bo ne window shows lateral wall de h isce nce
Abbreviati on s and Synonym s o Inte rmed iate wind ow/level sett ing shows co urse «
• La teralizcd in tern al ca rotid a rter y (Lat- le A) co n tour of Lat-ICA pro jecting in to midd le ca r
Definitions MR Findings
° tat-ICA: Deht scent lateral bon y wall o f petrous ICA as • T l WI: Lat-ICA invi sib le because of surroun di ng low
it borders a n te rior middle ea r cavity signa l ma stoid air &. bo ne
• T2W I: Non-v isua lized
o MRA
IIM AGING FINDINGS o Sou rce image s from MR angi ograp hy may show
lat eral position of genu o f vertical &. h orizo ntal
Ge nera l Feature s po rtio ns of petrou s ICA
• Best d iagn ostic clue: Petro us le A ca na l has dc h iscen t o Basilar repro jecti on reveals bu lbous, po stero latcrall y
latera l wall placed pct rous lCA
° Loca tio n: Late ral aspec t o f bo ny ICA ca na l wa ll at Imagin g Recommendati ons
pet rous segme n t
° Bone o nly te m poral bo ne cr
• Size: Dehiscen ce usually small, with no aneurysmal
• MRA or CfA co n firms d iagn osis
d ilatati on o f leA
CT Findin gs
° NECT IDIFF ERENTIAL DIAGNOS IS
o Bon e c r shows dch lscent late ral wall o f pet rou s leA
wit h protru sion o f le A in to midd le ca r
Aberrant ICA
J • Deh iscence usually n ear ba sal turn of coch lea • Enlarged infer ior tympan ic ca na licu lus present
o Coro nal T-bone CT sho ws laterally d isp laced lCA at • Aberrant ICA mor e postero lat eral as it rises in to
level of coch lear promo nto ry midd le ea r
° CEC r: En ha nci ng lCA p rot rud ing into midd le ea r
° C rA
DD x: Vascul ar Mid dle Ear Masses
Aberrant IC/\ Glomus Tympanicum Glom us lugulare ICA Ane urysm

LATERALIZED INTERNAL CAROTID ARTERY
Key Facts
Term inology • Glo mus tympan icum paraganglioma
• Late ralized intern al ca rot id ar tery (Lat-K'A) • Glo mus jugulare paraganglio ma
• l e A ane urysm, petrous apex
Imaging Find ings
• Bone CT shows deh iscen t lateral wall of pet rous l eA
Diagno st ic Checklist
wit h pro t ru sio n of l e A i nto midd le ear • Alwa ys check course of ICA o n T-bone CT or e r A
• Always check integrit y o f latera l wall of pet rous l e A
2
Top Differ ential Diagn oses 37
• Aberr an t lC A
Glo mus tym pan icu m par agan glio ma IDIAGNOSTIC CH ECKLIST
• Focal mass o n coc h lear prom ontory
• No tubu lar shape
Co nsid e r
• Be sure to differen tiate from aberran t lCA
Glomus jugul ar e paragan glioma o Lat·ICA lacks of en larged inf erior tympani c
• Arises from m argin of jugular for amen &. proj ect s cana licu lus
supero laterally into midd le ear cav ity o I'ctrou s ICA docs n ot cou rse from poster ior to
• Perm eati ve-destr uctive bony cha nge s on CT an terio r across coch lear promon to ry
• High -velocit y flow vo ids o n T l C- MR • Im portan t n ormal vascular varian t to recogn ize &
rep ort to avo id surgical in jury to le A
ICA ane urysm, petrou s ape x
• Focal o r fusiform expansion o f petro us l e A canal Image Int e rp retati on Pearls
• MRA &. crt\ arc d iag n o stic • Alwa ys check course o f ICA o n T-bone CT or CIA
• Always chec k int egrit y of lateral wall of pct rou s l e A
IPATHOLOGY
Ge ne ra l Features
ISELECTED REFEREN C ES
I. Pak MW ct al: La terall zcd carot id artery : An unu sual caus e
• Ge nera l pa t h co mme nts o f pulsat ile tinn itu s. ENT J. 80:14K· 9, 200 1
o Embr yology-an atom y 2. Saada AA et al: Ecto pic in tern al carotid arte ry (ICA) with in
• Lat·l CA isolated finding th e pctro us temporal bone. Arch O to laryn gol Head Neck
• En larged infer ior tympanic artery & persistent Surg. 122:792-4, t 996
staped ial arte ry are not present 3. Sin nrei ch AI et at: Arte rial ma lformations o f th e middle
• Gen u of vertica l & hor izont al segme n ts of petr ou s car. Otola ry ngo l Head Neck Surg. 92:194·20 6, 19R-t
ICA are anatomically no rmal, just lateral to
normal location
• Etiology: Failure of formation or ossificatio n of lateral IIM AG E GALLERY
wall of petrou s ICA
• Epidem io logy : Very rare vascul ar 'l-bo ne lesion
IClI N ICA L ISSUES

Presentati on
• Most co mmo n signs/sym pto ms
o Asymptom atic
o Lesion in ciden tally detected at otoscop y or c r
• Ot he r p resenting sym pto ms & signs
o Objective or subjective pu lsatile tin n itus
o When prominent, vascu lar retrot ympan ic mass
(u fl) Axial MRA shows fighf tetereiired int emal carotid al tclY
Natural Hist ory & Progn osis (arrow ) is norm ,l! in sire & conto ur, but is latefull y !ocatt ·c1, wh t'n
• No lo ng term seq uelae reported wit h Lat -ICA compa red with contra lateral normal side. (Right) Coro nal left ear
l-bo tw CT c!cmomtrates a Ii.l tt.'ralizl' d internal cc1lOtid artery (arrow) .
Treatment The vessel dtsmeter is normal while the superio r bon y waJJ is intact
• None n ecessary
• Important radi o logic observation
• Inadvertent surgica l injury can result in sign ificant
ne u rologic deficit s
ABERRANT INTERNAL CAROTID ARTERY
2
38
Ax;,'ll graphic iII/J<;trare.s classic Ah lCA NOlice Ab/CA Anwroposlerior intPfflcll csnskl angiogram show s a
ri~ing along ,.,()~tl'riornx'h/Pdf promufl/or y, crossing {aleral course of A/)JCA (.m ow). N()f/ll.lJ arteria{ co urse
.110 ng /llff!i,11 middle (',u \Vall 10 rejoin horizontal is sh()\\In with d.1 ..bed li/lP".
pt:'trous {CA (am ll v).
• Lacks statis tica l sign ifica n ce , du e to sm a ll n u m ber

!TE RM INO LO GY o f repo rted cases
Abb reviatio ns and Syno nyms • Rar el y bilatera l
• Abbrevi at ion : Aberran t int ernal caro t id a rtery (AhICA) • Size: Sma ller t h a n co n tra la teral l eA
• Synon ym s: Late ra l inte rn a l caro tid a rte ry (l e A); • Morp ho logy : 'Tu bu lar m o rph o logy key obse rva tio n
a berra nt ca rotid a rte ry CT Findings
Definitions • NEeI'
o Appearance o f Abl CA o n thin -section ( 1 m m)
• AbI CA: Co ngenita l vascu lar anoma ly result i ng i ll
disp laced le A runnin g th rough mi ddle ear te mpo ral bon e c r is di agn ostic
o Axial b on e CT
• Abl CA ap pears as tubu lar lesion crossin g m idd le
ea r posteri o r to a n te rior
IIM AGING FINDING S • ENLARGED inferior tym pan iCca na licu lus
Ge ne ral Features im po rta n t obse rva tio n
• Best di agno sti c clu e: Tu bu la r st ruct u re runni n g • Ca rot id fora men & vert ical segmen t of pe trous
ho rizo ntall y th ro ugh m idd le ca r cav ity from pos terio r ie A are abse n t
to a n terior o Corona l bone CT
• Locati on • Abl CA a p pears as ro un d, soft tissue lesion o n
o AbI CA en te rs po ster ior m iddle ea r t h rou gh en larged coch lea r pro m ont or y
inferior ty mpani c ca na licu lus • On sin gle slice look s d ist ur hin gly like glo m us
• Ab lCA is po ster ior & late ral to ex pec te d site of tympan icum paraga nglioma
caro tid foram en • Viewi ng m u lti p le slices revea ls tubular n ature
o Co urses a n te rio rly across coc hle ar p rom onto ry to o Th in n in g a n d re m od elin g of bo n e su rro u nd ing
join hori zonta l ca rotid ca na l t hrough a deh isce nce m idd le car, in cluding coch lea, ma y oc cu r
in carot id plate o If persisten t sta pe di a l a rter y associa ted
o Right m ore co m m o n th a n left AblCA • Enlarged a n te rio r ty m pa nic seg me n t o f facial
nerve ca n al
• Abse n t fora m e n sp lnosu m
DDx: "Vasc ular-Appea ring" Retrotympanic Masses
D ehiscen t lug. BulLJ oc: Granuloma ME G lo mus Ivrnpsnicum Glom us lugulet«

Key Facts
Te rmin o logy Top Diffe re nt ial Diagnoses
• Abbrevi ation: Abe rra n t in terna l ca ro tid a rtery • Deh isccnt jugu lar bulb
(AbICA) • Ch olestero l gran uloma, middle ea r
• AbICA: Co ngen ital vascu lar anomal y resu lting in • Para gan gli oma, tympani curn o r [ug u lare
di sp laced ICA running th ro ugh middle ear • Aneurysm , pet ro us internal ca ro t id arter y
2
Imaging Find ings Clinical Issu e s
• AbICA enters posterior middl e ea r th roug h en la rged • Mo st co m mo n sym pto m: Pu lsatile tinnitus (PT) 39
infe rio r tympa nic ca na liculus • Oto sco pic appea rance m im ics paragangli oma (glo m us
• Courses ant eriorl y across coc h lear promontor y to join ty m pan icum , glo m us [ugu lare)
hori zontal carot id ca na l thro ug h a d eh iscen ce in • G reates t risk is mi sd iagn osis lead ing to biopsy
carotid plat e
• Appea ra nce of AblCA o n th in -secti on (J m m ) Diagnostic Checklist
tem po ral bone cr is d iagn ost ic • Radio lo gist m ust remain firm o n im ag ing d iagn osis
• Carotid foramen & ve rtica l segm en t o f petro us l e A d esp ite cli nic al imp ression of pa ragan gli oma
are absen t • Biopsy or att emp ted resectio n o f m isd iag nosed AblCA
ca n be di sastrous
• CEeI" o "Bud" fro m su pe rol ate ra l jugu la r b u lb en te rs m id d le

o En ha nceme nt eq u iva len t to o t her arte ries car as "mass"
• Glo m us tympa n lcurn par agan glioma a lso
enha nces Cho les tero l granuloma , m idd le ea r
• Use m o rpho lo gy to di ffer en tiat e (tu b ular Ab lCA • O tos co py: va scu lar -ap pea rtn g retrotym pa n ic mass
versus ovoid paraga nglio ma) • Im agi ng
• CTA: Dem o nst ra tes AhICA, bu t is usua lly no t o CT ap pea ra nce o fte n id e n ti ca l to acq ui red
necessa ry becau se bo ne ch anges a rc ev ide n t o n bone cho lestea to ma
cr o T I unenha nced A'IR hi g h sig na l is sugges tiv e
MR Findings Par aganglio ma, tympani cum or jugu lare

• Conven t io nal M R d ocs not reli ably iden ti fy Abl CA • G lo m us tympa ni cum
o l.ow signal o f ho ne is d ifficu lt to distinguish from o Otos co p y: C he rry- red, p ulsatil e retro tympan ic mass
low signal of arterial flow void o Im ag ing: f o cal o vo id ma ss o n co chlea r prom o ntory
• MRA so u rce & refo rma tt ed im ages show abe rran t • Glo m us jugu lare
natu re o f vessel o Oto sco p y: C he rry-red, pu lsat ile ret ro tympan ic mass
o AblCA en ters skull base posterio r & late ra l, o Im agi n g: Permeat ive-destru ctive m ass exten di ng
co mpared to no rma l contralateral side fro m jugula r fo ra men th rough flo o r o f m idd le ear
o Fron tal reformat: Pet rous segme n t o f l e A exte n ds Aneurysm, petrous inte rnal ca rot id artery
later all y ins tead o f medi a lly
• Otosco py : Negat ive u nl ess la rge
• In le ft ear, Abl CA lo o ks like "7"
• Im agin g
• In righ t ear , AbICA loo ks like "reverse 7"
o Bone CT sho ws foc a l smoot h ex pans io n o f pet rou s
Angiograph ic Findi ng s ICA can a l
• Fro n tal view: Pet ro us seg me n t o f ICA ex te nd s la terall y o ICA has nor ma l course, b ut is ab normal in size &.
instead o f med iall y sha pe
• Con ven tion al angiography no lo nger n ecessary to o MRA is dia gnosti c o f n on -thrombosed an eu rysm
co n firm th is im ag in g di agnosis
o Th ln -sect ion c r o r ~ 1Ri\ is su fficie n t
IPATHOLOGY
Imag ing Re co m me ndatio ns
• Tem po ral hon e c r is most usefu l; con trast no t Ge ne ra l Fe atu res
necessa ry • Gen era l path co m men ts
• If MR is used, ~ IR A SO llfCC &. rcpro jection images ar e o Ana to my o f AblCA
cr it ica l • In ferio r t ym pan ic arte ry. b ran ch o f asc en d ing
ph a ry ngeal a rte ry, su p plies pet rous le A via m iddle
ea r
ID IFFERENTIA l DIAGNOSIS • In ferio r tympan ic cana licu lus en larges to
accom modate en larged inferior ty mpan ic artery,
De hiscent jugula r b ulb post erior &: lat eral to ex pe cte d lo ca tio n o f ca rot id
• O tos co p y: Ret ro tympa nl c vasc u la r m ass in fo rame n
post ero in ferio r q uadra nt • If associ ated wit h persiste n t stape di al a rtery,
• Im ag ing passes bet ween stapes cru ra
o Fo ca l abs en ce o f sigm o id plat e
• Bony margin o f posterolatera l horizontal petrou s • If tinnitus is loud, Ab lCA can he deb ilitating
lAC ca nal dehiscent to allow AblCA to rejoi n ICA
• Etiol ogy
Treatment
o Etio logy o f AblCA is n ot kn own • NO TREATMENT is best t reat men t
o Rest h yp othesis: Abn ormal regression of cervical lCA • Grea test risk is m isdia gn osis leading to biopsy
duri ng emb ryog enesis tri ggers en large ment of • Most pati ents h ave min or sym pto ms th at d o n ot
co llate ral art eri es require tr eatment
2 • Cervica l lC A &: 1st portion o f petr ous ICA are • Ra re tr eatm ent: Place syn t he tic mat er ial betw een
art er y a nd ossicles
absen t
40 • Inferior tympanic a rte ry ana stomoses with • Persist ent staped ial a rtery ha s bee n treat ed with
ca rotico tym pa n ic a rte ry (b ra nc h o f pet rou s ICA ligation o r ca ute rizatio n
flowing retrograd e)
• Dehiscence o f h orizon tal petrous l eA may lead to
arterial d isplacem ent I DIAGNOSTIC CHECKLIST
• Trac tio n from persist ent e m bryo n ic vesse ls ma y
pu ll ICA into midd le ear
Image Interpretation Pearl s
• Ep ide m io logy: Ver y rar e di sorder (45 case s io English • Radiologis t must rema in firm on imaging diagn osis
litera ture) d espit e clinica l impression of paraganglioma
o Biopsy o r att em pted resecti on o f misdi agn osed
• Asso cia ted abnorma lities
o 30% o f AblCA have persi stent sta ped ial a rte ry AhlCA ca n be di sastrous
• Hem orrhage, st ro ke o r deat h ma y result from
• En la rged anterior tympan ic segme nt of
intraternporal facial n e rve ca n al vessel injury
• Absent ipsilate ral foram en sp in osum • Retro pharyn geal ce rvical ICA is so me ti mes referr ed to
as AblCA in Briti sh literatu re
Gro ss Pathologic & Surgical Features o This should n o t be confused wit h Ab lCA of
• ICA e n te rs sku ll base po ste rior &: lateral to o ormal temporal bone
location
• Pulsatile ab err ant a rter y is found in mi ddl e ea r cav ity
Micro scopi c Features
I. Roll JD et al: Bila tera l abe rra nt ln tcma l ru rotld a rteries wit h
• Hist o logicall y n ormal a rte ry bila teral persistent stapedia l arteries and bi lat eral
d uplicated in terna l ca rotid arte ries. AJNR Am)
Neurora<! iol. 24(4):762·5, 200:1
ICLINICAL ISSUES 2, Rojas R ct al: Aberrant in te rna l ca rotid a rtery as a cause of
pu lsatile tin nitu s a nd an lntratym panic mass. Ea r Nose
Presentation Throat J, 8 2(:1): 17:1·4, 200:1
• Most common signs/s ym ptom s :I, Ko jima H et aI: Abe rran t carotid arte ry in t h e m idd le ea r:
o Mo st co m mo n sym pto m : Pulsat ile tinnitus (I'T) m ul tlxllce CT imag ing aids in di agnosis. Am] O tol ary ngol.
2~(2 ): 92·6, 200:1
• Ma y be sub jecti ve or ob jecti ve 4, Biller JA et al: Aber ran t carotid a rte ry. Otol Neuro tol.
• Obj ective JYI' when stenosis is pr esent at juncti on 2:1 (:1 ):407 ·8,2002
o f AblCA &: n ormal h ori zontal petrous ICA 5. Jain R et al: Manage ment o f ab erra nt in terna l carotid arte ry
• Pul sati ons may transmit direct ly in to m id dle ca r inj ur y: a real eme rge ncy . Oto laryngol Head Nec k Surg.
o r through co ch lea r p rom o n tory to ba sal turn o f \ 27(5): 470·:1, 2002
co ch lea 6. Bo tm a M ct al: Aberra n t in te rna l caro ti d art ery in till'
• Less co m mon sym pto ms m iddle-ca r space. J Laryngo l Ot o l l l -t:7H4-7. zooo
a Con d uc tive hea ring lo ss 7. Silberglei t Ret al: The persistent staped ia l arte ry. AJ NR Am
o O tos co p ic exa m: Midd le ca r mass
J Ne uro rad iol . 21(3 ): 57 2-7. 2000
8. Davis WI. et al: MR an giograph y o f a n abe rran t inte rna l
• Vascula r in fer ior ret rot ympa ni c ma ss behind ca rotid art ery, A) N R 12:1225, 1991
intact tympanic m embran e (T M ) 9. Lo W \\' et a l: Aberran t caro tid arter y: Rad io logic d iagn osis
• Ot oscopic a ppea ran ce mi mics paraganglioma wit h em phasis in hi gh -resolut ion co m pu ted to mography.
(g lo m us tympanicum , glo mus [ugu lar e) Radiofl ra phics S:9 K5-93, 1985
o Vertigo
o Bruit n ear ea r
o Otalgia
o Often asy m pto ma tic o bserva tio n durin g ea r
exa m in a tio n
Demographics
' . Ge nd e r: M < 1=, b ut lacks statistical sign ifica nce, due to
small n umber o f reported cases
Natur al History & Prognosis
• No long term sequelae rep orted wi th Ab lCA
• Poo r progn o sis results o n ly if mi sdi agn osis lead s to
biopsy
IIMAGEGALLERY
Typical
11.1.11) ,,\ \i,,1 I-bone C1 of 1141
C,lf sl ,o w!> ,m AblCA <15 ,UI
.1Jmorf1lJllubU/,lf Sl ruc ture
tunmng .110118 flJl'f.li,J/ .Hl)f'Ct
2
of middlL' ( ', If (,If(mv,. On
J.xi.ll ;m.1Sl'S till' tuhu/,l( 41
n ,HUff> of lhi!> lesion is
unm M .JJ".l blt>. (Ri1:I !ti A"i,11
I-lxnw C T of It'It c-u fl' Vl ' J / !>
stC'nosis of .1Il ,l ht'" .ln l
internal c.lf o lid .Jtlt·f~' .15 it
(pja ios hOri Lofll.IJ wgm t"1I1 of
petro us lCA ( ,1f fOW5' . This
can ceuw ohjl"C'in· tinniru5.
Typical
(1.1.10 COfOrM I l -bone CT of
Iplt par sno ws Ab lCA ,H afJ
ovoid m d SS on cocbteer
promon tor y (arrow). In .1
singft· coro ll al CT slice,
Allle A c.l n pXilCtly mimic
J.: f()nHJ ~ l ympclflic um
/ ),If,'g,mglio /l),l. (J(i!: " ,)
COIOll,ll ten o.v T-bone C l
('VI',,!S ill ferior tymp anic
c.IIl.l /ic fl/u5 (.lflm n' is
l'nl,lfgp(! to .II/O\\' P.J..... I/iW oi
..Jberr.lnt intt'''MI csroud
.1ftt'ry.
Typical
(lA10 ,h i.,1 t-lv vw C I of
f ight t '<if ." how!) enlarged
interior tymp.lI1ic c.Jf1J/icu/w;
(<1((o w ), jus' ,m tl 'm l,l tl 'f,l l to
j uglilar bu ll}. Tll i' Ilx '.ltion i~
pos terior ,m el IJt"f.11 to
wht'if>nOfm .11c,lfotid
toranwn would be. (Ril:lI tJ
A nt c ropostt'riol l e{()f m at o f
tim C'-o{·"'igllt ,\ IRA ~hf1\ \'S
Chd /J c tef'5,ic ~ 7 ., ign -
tonowst. ..IS ..Jht'ff,m t in tt'fIlJI
c.l ro tid artt" y C'.\tends
''' '(,(dlly in to middfl' "<f l .
Con vpsre with co ntr,I /JEt'roll
norm.J/ ICA.
PERSISTENT STAPEDIAL ARTERY
2
·t2
l.llt'I,,1Wdphic _~ h()I\'.~ 1'.",>\ ,ui"ing li"om \'(·rl;c,l/.\f'g m r=n t 1.I/I'r,I1 oW, l/l hie ,hows P).-\ arising linm A1>ICA (,m o w),
petlou... leA, Ikh., in~ through ... /,1/)(.... ,I; fr;l\ 'f'ling a/ollfj p.Jssin!-lI!lmugh <;/,l/JPS, to '-0 1101\' dn/eriO/ tpn/wlII CN i
tvm p.uuc ...egmf'1J/ of (",\ /7 ( ,IIU I\ V) to lJt'<vml' middle (u fJ( '1l d l fO \V) . Intracranially it IX '("Of1li'_' middle
fHeo ingf'al artf-'f y (Op t' ll , lfflJ l \'! . Im 'llin/-:I',11 , ll ll'Iy (cu rved arm\ \' ).
o " SA a rising fr om gen ll o f vertica l ~ ho rizon ta l

ITERM INO LOGY pct ruu s ICA
Abbreviations and Synonyms Angiograph ic Findings
• Persistent sta ped ia l art er y (PSA) • Exte rn a l carotid a rtery arteri ogram
Definitions o Sh o ws absen ce of n or ma) middl e m en ingea l artery
• PSI\ : Raft.' co ngcn fta l va scular nuo maly in w h ich • In ter nal ca ro ti d a rte ry a rter iogra m
e m b ryo log ica l stap ed ia l a rtery IK' fSis IS a Sho ws PSA ari sin g from ln frncochl ea r pct ro us le A or
from a n Abl e ,\
Imaging Recomm endations
IIM AGING FIND INGS • Th in -secti o n, co ro n a l N ax ial te m pora l h on e CT
Ge ne ral Fea tures
• Bl'S! d iag nost ic elm': l.n largcm cn t o f an terior tympan !c
s('g llle ll! eN ? can al + ab se ll t for a m en Sp iIlO." UIIl
ID IFF ERENTIAL DIAGNOS IS
• Locat io n : PSi\ pasws th rou g h sta pes foot plat c Herpet ic facia l neuritis (Be ll palsy)
• Size : Do ubl es size of an te rio r FN tym pa n ic seg men t • Bo n e CT n o rm al
CT Findings • TI C+ t\lR sh o ws e n ti re int ra tem po ra l F;\Il'llhan ces
• N ECT Facial nerve he ma ngio ma
o Absen t ip'iila tl' Ta l [ora m cn spl nos u m • BOlle CT co m mo n ly sho ws in tratu mora l oss ifica tio n
o Enl argem en t o f FN ca na l o r se par a te par a II cl can al • '1' 1 C+ M R sho ws e n ha nc ing ma« in gc n lcu latc fossa
o C u rvll lnear str uc tu re cro ssing m ed ia l wa ll o f mid d le
ear cav it y over co chlea r pro m o n tor y Facia l nerve sc hwan noma
o Sma ll ca n ali cul us leaving caro t id ca n al • HOll e CT SllOWS tubular or fo cal en largem ent o f FN
o PSA Sl 'P Il w it h or wit hou t abe rra nt int ern al ca ro tid ca n a l
arte ry (,\ hleA ) • '1' 1 C+ M R sho ws m ass en ha nci ng
• e TA
o Shows absen ce o f n o rma l m iddle n u-n in geal ar tery
DDx: Lesions Invo lving Horizo nta l Segme nt Petro us Facia l Ne rve
Belll',,/, y TN Ilemangioma FN Sch wannom,J I'prill(-'ur,,1 CN? Ca

PERSISTENT STAPEDIAL ARTERY
Key Facts
Termin olo gy Path ology
• PSA: Rare conge nital vasc ular anomal y in wh ich • Associa ted abn or ma lit ies: Abe rra n t in ter na l ca rotid
embryological sta pedia l a rte ry persists artery
Imaging Find ings Clinical Issu es
• Best d iagnost ic clu e: En largemen t of ant erior • Most co mmo nly asymptomatic find ing o n 'I-bo n e CT 2
tymp a nic segm en t eN? ca nal + ab sent foramen o r dur ing surgery
spinos um 43
Perine ural CN? carcinoma (Ca) Trea tm ent

• Bon e CT shows enla rged FN ca nal • NO treat me nt is best treat me nt
• T l C+ shows en hanc i ng tumor com ing li p fro m • If presu med C<lUSt' of pulsati le t innit us, surgica l
parotid ligati on o r endovasc u la r occl usion m ay he co ns tdc red
o O n ly do ne if pu lsati le tinni tu s is in tract abl e
• PSA su rgica l imp licati ons
IPATHOLOGY o Ca n co m p licate sta pedecto my o r cho lestea to ma
resec t io n
Ge ne ral Features o May preven t coc h lea r im pla ntatio n
• Genera l path co m me n ts
o Conge n ital a no ma ly
o Em bryo logy-ana to my
• Prim iti ve 2nd ao rtic arc h gives rise to h yoi d a rtery
IDIAGNOSTI C CHECKLI ST
• Hyoid art ery gives rise to staped ial a rter y Co nsider
• Staped ial arte ry d ivides into dor sal (m idd le • If Ab lC A di sco vered, loo k for assoc iated PSA
men ingeal artery) & ventral division s (to maxilla
& mand ibl e) Image Interpretat ion Pearl s
• Gene tics • Largv anter io r tympani c eN? + absent foram en
o Othe r avsoctat tous repor ted in clu de spl nos um = pst\
• Trisomy 13, IS, &. 2 1, Paget d isease, o tosclerosis,
anencepha ly, ncu roftbromat osts
Etiology : Stape dlu l artery fails to Involu te in Srd fetal ISELECTED REFERENCES
mont h l. Yilm az T et al: Persisten t sta ped ial artery: MR a ugiogruphlc
Epidem io log y: Very ra re lesio n and CT find in gs. AlNR'\m J Ncuro radlol. 2-1(6 ):11:n ·s,
• Associat ed abnor ma lit ies: Aberra n t in ternal ca ro tid 21XJ:l
arte ry 2. Stlbcrg lclt It ct al: Th e persis ten t sta pedia l artery, AJI'\R Am
J Ne uro radlol. 2 1(] ):S72·7, 2000
Gross Path ol ogic & Surgica l Feat ures :I. Thiers FA ct .11: Persistent stapedia l artery: CT findings.
• Otoendoscopy dem o nst rates PSA pa ssing th ro ugh I\It'R Am J Ncur o radlol. 2 1lH):155 1--I, 2000
stapes
IIM AG E GALLERY
ICLIN ICA L ISSUES
Presen tati on
• Most co mmo n signs /sy m pto ms
o Most co m mon ly asympto ma tic findi ng 0 11 'l -bo ne
C f or durin g surgery
• Rarely prese nt s wit h tin n itus ± pulsatile
rctrotym pant c red mass
Cltntcal profile
o Otosco pic exa m usu ally no rm al
• Ra rely pul satil e, red, ret ro tym pan ic ma ss seen
Dem ograph ics
• Age: Co nge n ital, m ay be d isco ver ed at a ny age (/£JI) enroll,ll rigll t ('d r l -borw c r sho ws emerior d 5fJ IT( of " I)('rra/J(
caro tid ,Ulery (arro w) .m d ,H!>oc; aled oetsistent ,Q,1pcd ia/ .lf l pf Y as all
Natura l History & Pro gn osis (·nl.1fgp<! i,!Cial npf\'l' t pnp.lfIic sC'gm t' /lt (ope n <lrrow ) . (NiJ.:hO Axial
• Excellent : just needs left a lo ne riMht ear (-honE' C I df:'mon.~ t,ates ttw dbsent for amen sp ino!>um
(.UfOl\). po...ll' ru l,lI(·/& 1o 111(· norm,ll fo ramen U\lah· (op en arro w) ill
th is esse of persisunn . .t apcd i.l / .1flef r .
AOM WITH COALESCENT OTOMASTOIDITIS
2
44
Axial '-bon<' right ear CT shows (!i/iuw op.lCific.ltion of A,;al right PJr a
,.lJont:> ff'\'Pal.~ m.btoid r1t'bri_~ with
middlt· eelr li. mastoid in febtilt> p<Jcit>tJt with (J{d/nia . (lt~trucliOfJ 01 *fXdtions COm islt'fl l with (wlcscent
Clearly ut!findblc thinning & erosion of sigmoid sinus mastoiditis. E.\temJI (arrow) and in/l'm,)1 (opm arrow)
pl.ltc 5t:'en (arra. v). (ort('.~ di'>flIlJtion present.
o In co m plicaled cases " COM may involve lab yrin th,

ITE RM INO l O GY men inges, sigmoid sinus &: remainder o f in tra c rania l
Abbreviatio ns an d Syn onym s cavity
• Acu te coalescen t o to mas toidi tis (ACOM) CT Findin gs
• Acute oto rnasto id itis o r acu te o titis medi a (AOM)
• NECT
• Syno ny m s: Erosive o to mas to id itis, coa lesce n t o Opaci ficat io n of mi dd le ca r & masto id air ce lls
o to masto id it ts o Destru ctiv e c ha nges of ma sto id septat lo ns ± co rtex
De finitions identified on thin-secti on ho n e CT
• Definitions • CECT
o AO M : Active infection in m iddle ear & masto id air o En hancement of in fla m ma tory d ebri s within midd le
cells witho ut de struction of masto id scpta tlons or ca r &: mastoid
cortex o Enhancing debris beyond ma rgin s o f Tvbone in
o ACOM : Acut e inf ecti on of midd le ear-mastoid ai r co mpli cated cases
ce lls wit h progressive reso rpti on &: d emi n eraliza lio n MR Findi ngs
o f mastoid septae due to ed ema -loca l acid osis with • T1W I: Isointen se d ebri s withi n an d ad jace n t to midd le
devel opment o f intrarna stoid empyema ea r-masto id
• T2W I
o Hyperintense d ebri s within &: adj acen t to middle
IIMAGING FI NDI NGS ear-masto id
Ge ne ra l Features • Associated cholestea toma is relatively hypointen se
o Trabecular lo ss &: co rt ica l d ehiscen ce at times visibl e
• Best diagn ostic clue: Erosion of ma stoid septa tions is on T2WI
most sensitive &. specific cr finding d istingui shing
• FLAIR: No ns pecific T2 hyp erintensity
coa lescen t from n on-coalescent AOM
• TI C+
• Locatio n o Diffuse enhancement of infl amm at ory debris
o Middl e ea r, mastoid, petrous apex
DD x: Midd le Ear-Mastoid Lesions
CongoCholesteatoma Acq. Cholesteatoma Rhabdomyosarcoma

Key Facts
Termin olo gy • Acu te u ncom plicated ot omastoiditis
• Acute coalesce nt o to masto idit is (ACO M) • Acq uired cho lesteatoma
• AOM: Active infection in mi ddle ear & ma sto id air • Lan gerhao s cell hi sti ocytosis
cells witho ut destruc tio n of ma stoid scptations or • Rhabdomyosarcom a
cor tex
• ACOM: Acut e infect ion of midd le ear-masto id air
Path olo gy
• Prolo nged in fection crea tes hyperem ia, veno us stasis
2
cells with progressive resorption &- dem ineralizati on &. in creased osteoclastic act ivity 45
of mastoid sep tae due to ede ma-loca l acidosis wit h • Su ppurat ion under pressure causes local acidosis,
development of intramasto id em pyema decalcifica tio n, ischem ia &. osteoclastic act ivity
Imaging Findin gs Clinical Issu es
• Best diagnostic clue: Erosio n o f masto id septations is • Wit h failure to co nt rol AOM wit h a n tihiot ics,
most sensitive &- speci fic CT find in g distingu ish ing mu coperiosteal disease invades bon e (ACOtvn
coalescen t fro m no n-co alescent AOM • Masto idectom y is becom ing more freq uentl y n eeded
Top Differen tial Diagn oses • Surgery may be requi red to esta blish good
• Congenital cho lestea to ma, m idd le ea r-mas to id co m munica tio n between m iddle ea r and mastoid
o Enhan cement o f ad jacen t men inges in po sterio r &. • When large may cause masto id trab ecula &. cor tical
middle cra nial fossa is om inous fo r im pend ing destru cti on
intracranial co mplicat ions • Cho lestea to ma docs n ot en ha nce on '1' 1 C+
o Excellen t for iden tification of assoc iated abscess
Lan gerhan s ce ll histi ocytosis
• MRA
o Petrous carot id in vasion or pseu doaneurysm rare • Clinical: Usua lly pa rt of system ic process; can mim ic
o Invo lvemen t of leA may be du e to spread fro m co m plicate d ACOM
adjacen t nodes near int ern al jugular vein (UV) • Bon e CT: Sign ifica nt bo ne destructio n usually present
• MRV: Importan t mod alit y as IJV t h rombo ph lebitis is a • Tl C+ MR: En ha ncing mass associat ed wit h bo ne
life threatening com plication destruction
Imaging Re commendati on s Rhabd omyosarcom a

• High-resolution th in -section (0.5- LO m m ) bon e CT • Clinica l: Present ation may he sim ilar to co mp licated
makes th is diag nosis ACOM
o Must be targeted for maxim al bo ny detail • Bon e CT: Sign ificant bon e destru ction usually presen t
o Comparison to op posite side is crucial • Tl C+ MR sho ws en hanc ing mass associated with bon e
• MR best if any regional co mplica tio ns suspected dest ruct io n
o Inclu de T1 C+ in all three planes
o Always include ?\.IRA an d MRV
I PATHOLOGY
Gen er al Features
IDIFFERENTIAL DIAGNOSIS • Etio logy
Conge nita l choles teato ma, middl e o Prolon ged inf ection creates hypere mia, venous stasis
& in creased osteo clast ic activity
ea r-mas to id o Sup puration und er pressure ca uses local acidosis,
• Clin ical: Wh ite mass beh ind In tact tym panic decalcification , ischemi a & osteocl astic activity
membrane; no in fectious sym pto ms o Bacteria in volved in clude
• When larger, may cause trabecula r ± cor tical eros ion • Streptococcus pn eumon tae (some resistan t)
Acute un complicated oto mas to idit is • Haemophilis influ en zae
• Clinical: Painfu l ea r associated with infect iou s • St reptococc us pyogencs
sym pto ms o Fungal disease: Invasive Aspergillosis mu st he
• Opacified m iddl e ear & mastoid co ns idered in immune co m prom ised ind ivid uals
• Bony margin s a ppea r in tact wit hou t destruct ive (espec ia lly HIV)
changes • Epidemiology: Rare co m plicatio n o f oto masto idi tis
• Pa rticula rly wor risome if air cell no rmal awm metr v Gross Path ologic & Surgica l Fe atures
suggests ACOM . .
• Soft osteo myelit ic bon e with pus filling co n fluen t
Acquired cholesteatoma mastoid air cells
• Clinical: Tympanic membran e perfora tion wit h Microscop ic Features
middle ear ma ss • Offend ing o rga n ism ofte n not cu ltured
• Originates in m idd le car, usually attic o Patients are alread y on multip le an ti biotics
• Middle ea r ossicles destroyed
IC LIN ICA L ISSUES IDIAG NOSTIC CHECKLIST
Presentati on Co nside r
• Most com mo n signs /sy m pto ms • ACO M may be diffi cu lt to separate clin ically or hy
o Ota lgia imaging fro m rhabdo myosarcoma o r Langerhans cell
o Other signs/sym pto ms h ist iocytosis
2 • fe ver (o ften spiki ng)
• Post-a u ricula r swe lling if ruptures throu gh la te ra l
o Usually t hese ca use mo re destructio n &. have mo re
associa ted mass t han wi th ACO M
4 (, masto id cor tex
Im age Interp retati o n Pearl s
Demograp hi cs • Carefu lly examine mastoid septat lons o n hon e CT an d
• Age: Most aggrl'ssive cases in ch ild ren compare 10 o ppo site side
• Beware o f overcalling o to masto id itis w it h asym me tric
Natural H isto ry & Prognosis masto id air ce ll sizc as ACO l\,.1
• \Vith fa ilure to co n tro l r\O~1 with antt blot ics, o ACO M d iagno sis o ften lead s to su rgica l int er vention
m ucop crtosteal d isease in vad es bone " \COM) whi le oto masto id itis co nsidered uo n- surg lcal d isease
• A CO ~ I typically has exce llen t prognos is unl ess
regional co m plicatio ns ens ue
• A CO ~ ( regio nal co m plicatio ns ISELECT ED REFERENCES
o Subpe riostea l abs cess
I. ~ li ~ iro\' L ct .11: Co m p uted to mogra phic \'{'r, u\ surgica l
• Erosio n of extern al masto id cor tex wit h abscess
find in gs in co m plica ted ac u te o to masto kn us. Ann 0101
formation i n post-auricular locati on Rhino! l.aryn gol. 112 tHj:6 75· 7, 2003
o Men ingitis &. brain abscess 2. Agarwal A et .11: Natura l hi sto ry o f stg motd sfn us
• Occurs from di rect co n tig uity to coal escen t t h ro mbosis, Ann 0 101 Rh inol Laryn gol , 112(2 ): 191 --1 , 200 J
disease, erosio n o f int ernal masto id cortex J. Vazquez E ('I .11: Im ilging o f co m plica ti ons o f ac u te
• Rarely seco ndary to ret rograd e th romhoph leb iti s m astoidi ti s in chtlcl n-n. Radiog ra p hies. 23 (2 ): :~5 9· 72. ZOOJ
o Sigmo id sin us th rom bosts/ rhrombophlcbl tis (SST) ~. Taru n t fno V ct <I I: Acute ma vtoidi tis: a 10 y l'iH rc trovpccti vc
• Usually 'il'COI HIJry to e pid ural abscess, rarely from 'lo t lilly. In t J l'cdi at r O to rhi nola ryng o l. II ;66(2 ):l -1 :{·H, ZOOZ
S. Mats ubara K ('I ill: Acu te coa lescen t rnastonnu s and
erosio n o f sigmo id sin us plate
aco ustic seq uelae in I n in fa nt wit h seve re con ge ni tal
• Results from pressure nccro slv on sin us wall, ncu t £ope n ia. In t J l 'cdia t r ( lt orh in o la ryn go l. 11;62( 1):I'l.{ . 7,
platelet ad heren ce &. m ural th ro mbu s wh ich 2(K)2
propa gat es to form occl usio n c,. Zapalac JS et .11: Su pp ura tive co m p lica tio ns o f acu te o tit is
o Bezo ld ab scess m edia in th e vra of a n tibiot ic resistan ce. Arch O tola ryngol
• Result') from erosion o f mas to id tip wit h tracking Il eall !\'l'(-l\ Surg. 12H:h(;O-3 , 2002
of debr is med ial 10 inser tio n of 7. Ma ro ld ! It e t al: Comput ed tomograph y and magnetic
stc ruoctci do mavtold m uscle reson an ce imagi n g of pa t hologic co nditions ()f the middle
ca r. Em J Radi o l. -IO(2 ):7H-93, 200 1
• Ant eriorl y lim ited by po ster io r cervica l and
H. Go C, ct al: lntracr a nl al co m plications of acu te m astoid it is.
phnryngobastla r fa...cia 1nt J I'ediatr Ot or hino laryn go l. 15;52( 2): I -I :~·H , ZIXXt
o Serous o r supp u rative labyrinth itis 9. Dobben (il) ct .11: O tog en ic int rac ra n ial in flanu n at lo ns:
• Pat hogens u s uall y en ter round win dow result ing ro ll' o f magn enc rcv ma ncc im agi n g. Top ~ I ;l g n Rcson
in ver tigo N. vcnso rinc ur a l hear ing lo... s Im aging. 11(21:76·H6 , 2000
o Pet rou s a pic itis: Extl'nsio n to petrou s a pex may 10, An tonelli 1') ct a l: Co m p ut ed to m ogra phy lind till'
occ u r in ] Ol}fl of ind ividuals who have di agn o sis of co alesce n t mastoi d it is. Ot ola ryn go l I lea d Neck
developmentall y pn eumatized pctru u... apex Su rg. 1 20 : _~ 5 0- -I . IlJ99
I I. Kaplan D M c t al: O togen ic lateral sinus th rombovls in
• Apical pctro ...itis co u ld be called "co nfluen t ap ical
chil d ren . In t J I'cdiat r Ot or hi n olar yn gol . 20;4lJ(3 J: 1 7 7 · K~ ,
pet rositi s" 1999
o Otic h yd rocephalu s 12 , Cavtillo :-' I l't al: Im agin g o f Bezol d's ab sce ss. M R.
• Co m m un icatin g (ex t rave n tricu lar} h ydrocep halus 17 1: 1491 -5, 199 8
due to o bstruc tio n o f ara ch noid gran ulatio ns 13. IlhoOgl' 1.I ct al: Value o f co m pu ted tom og ra ph y o f the
te mpora l bo ne in acu te o to m astoi di t ls. Rev La ryngo l Otol
Treatm ent Rhi nol . 119(2 ):9 1·4 , 199M
• Acut e o titis media 1-1. Spit'gt:l Jlll,t .a l: Co n te m pora ry p resen tation a nd
o Usually treat ed success fully with antibiotics with no m an agem ent of a spect ru m of m astoid a bsces ses .
ima ging req u ired Laryngoscope. IOHI6 ):822-H, J99H
o Antibiotics, myringot omy 15. 7.agardo ~ rr: Prominent arachnoid granulations. AJNR.
I HIH): t S 9 ~ -S. t 99 7
o Incisio n and d rainage
• Acute co n fluen t oto mas to iditis
o Masto idect om y is beco ming more freq uently need ed
o Su rgery may be requi red to establish good
co mrn u n lca rlo n bet ween middle ear and mastoid
o Reststa n t o rgan isms mo re co m mo n ly enco u n te red
IIM AG E GALLERY
(I.e/I) Axial riMht P elf t-booe

CT rt>vt'<1/s fluid throughout
mkkite ear S ma stoid with
intact o.m des. Co,lfpsCf:'nI
dist.·<I.~f..·in m,lstoid m,lfIifl'sl
2
by erosion of sigmoid sinus 47
p late' (arrow). (Rig1l0 A't; al
righf [ -bone ( 7 sho lVs fluid
in middIL· ear-mastoid air
Cf:'f1.~ with dreas of
oS/t'omyeliti... ,m c!n m t1upnn "
sigfl all'{ f by ira/x ·c ul.1f
b rf:'.lkdmvn (arro w s) 1\
sigmoid pl.1le dehisccnce
(op en arrow).
Typi cal
(I.e/I ) AxiJI bon e C T shows
middlp ear-mastoid antral
cbotesteetcms (arro w)
<l .~ s od.l ted w ith cortical
dehiscen ce (ope n Jrrows).
Confluent mastoiditis spills
lale'rally int o p ostauric ulaf
abKPss (CLIf\'C'd arrow).
(Rig ht ) Axial right ear t -bo ne
CT sho ws de bris in ma stoid
with focal area of con fl uen t
ma.~ loiditis (arro w}. No tice
the inkx tion has broken
through the la/eral mastoid
cor/ex (open arrow}.
(/40 Axial l~ bone Ct of It-fl

ear shows mastoid
op acification w ith a larg<'
art'i! of trsbecu ter 10 .\5
(arro ws) siglJJ/ing the
tran sitio n from otomastoiditis
to co nfl uent ma!>toidi ti s.
(Hix hl ) AX;,ll t 1 C+ AIR
rf' vf'a/.~ (Iiffuw enhanCf'mf'nt
of right ma stoid ,lir n :'!ls wi,h
in lt 'ctio n spreading through
m edial cortex (arrow) to
invol ve of p m /erior fossa
meningc s. Focal pus (open
arrow).
AOM WITH ABSCESS
2
48
Axi.ll ceo sho.vs classic prnhwricul1f ab5cess (arrow) Atial T-I>one a sho.vs opJciflCiUion of mkkJ/e edr in
as a sessile>. fluid density mass O'tfflying an opaciflt'Cl pdtif.'fll with acute confluent ITIDtokJiris. ConfltJCfJIT of
m ,lStoid (diifiw lt to see). Note single oa of ai, within m.l5l.oid .1ir cells (affOlv) prt!Sf!Ol \'trith focal COOic.l 1
the a~.. C.WilY. bredkthrough (0fJffi .]ffOoV) .
o Abscess locati on de pends o n d irectio n o f midd le

ITE RM INO LO GY car-masto id co rtica l deh iscenc e
Abb re viatio ns and Synonyms • Post-auricul ar (co rtica l bone here t hi n n er)
• Acu te coalesce n t oto masto id itis (ACOM) • Pre-au ricu lar
• Acu te o to mas toi d itis o r acute otitis med ia (AOM) • Tempor al lobe (tegmen tympani o r teg men
• Eros ive oto mas to iditi s O ( coalescen t o to ma stoid itis masto tdeum d eh isces)
wi t h abscess o Post-au ricul ar > > pre-au ricu lar & m idd le cranial
fossa
Definiti on s • Size: Variable; usuall y p resen ts with > 1 em flu id
• AO M: Active in fect ion in m iddl e car-mastoid wit h out pocket
destructio n of m asto id septa tion s or corte x • Morpho logy: C rescen tic, len tiform or sp heri cal
• ACO M: Acu te middl e ear-masto id infecti on with
prog ressive resorption & dem ineralizatio n due to
CT Findi ngs
in t rama sto id em pyema • NECT
• ACO M + ab scess: Coalescen t mastoiditi s with resultant o Masto id ai r cell opacifi catio n wit h variable
po st-auri cul ar, m iddl e cranial fossa o r ex tracran ial so ft trabecul ar & co rtica l erosio ns
tissue abscess • Air-fluid levels in masto id air ce lls &. mi ddl e ea r
(AOM may h ave reso lved)
o Subtle to gross foci of dehiscen t cortex just d eep to
IIM AGING FINDING S area of a bscess
• CECr
Ge ne ral Features o Pre- o r post-au ricul ar fl uid co llectio n = subpe riosteal
• Best d iagn ost ic clue: Rim-en hancing fluid collection abscess
ad jacen t to eroded ma sto id co rtex + mastoid ai r cell • Th ick, en h anc ing lateral wall represents infl am ed
o pacificatio n periosteum
• Locatio n o Bezold abscess : Walled -o ff pu s in & arou nd
o Ad jacen t to masto id air ce lls ste rnocleidoma sto id m uscles
o Middle cranial fo ssa abscess: Focal flu id (p us) in
tem por al lobe just above T-bone tegm en
DDx: Dest ructive T-bon e Lesion in a Child
ff:"
.'
-~
-'-'. ' . .•
, I
I'".i " ' ..

I
\
I
2td.1~,~ . ;.:
p.--- ., " ~ J1
Acq . Cholesteatoma Chondroblestoma t.C Histiocytosis Rhabdo myosa rcoma
AOM WITH ABSCESS
Key Facts
Termino logy • MR as needed for be tter definition of in t rac rania l
• Acut e coalesce n t o to mas toi d itis (ACO M) co m plications
• Acu te o to masto td tt ts ur ac ute o tit is media (ADM) Top Differen tial Diagn oses
Imaging Findin gs • Acquired (Acq .) cho lestea to ma
• Best diagnostic clu e: Rim-enhancing fluid co llectio n •
•
Apical petrositi s
Lang crhans cell (LC) hi stiocyt osis
2
ad jacent to eroded ma stoid co rtex + masto id air cell
opacification • Rhabdomyosarcoma 49
• Masto id air ce ll opacifica tion wit h varia ble trab ecu la r Pathology
&. cortica l erosio ns
• 46% ch ildre n have > 2 ep isod es AOM by age 3
• Subtle to gross foci of dchiscent cortex just deep to • O.24 I X, patien ts with ADM develop ACOM
area of abscess
• c r needed in pati ents with mu copurulent ea r Clinica l Issu es
discharge, mastoid tend erness o r neurologic signs • Young ch ild with 1 da y to 1 week hi story otalgia,
• cr accuracy 95% for AOM com plicatio ns pos t-au ricu lar swelling. fever &. o to rrhea
• crv • Usually n o associate d intracranial abscess

o May sho w d ural sinus thrombosi s
Chondrob lasto ma
MR Fi nd ings • Clin ica l: < 20 yea r old with peri au ricular mass lesio n
• T I\VI: Low signa l flu id in sub periosteal co llection &. • lmagln g: Het erogeneously enhanci ng, ero sive mass
mastoid air ce lls wit h vari ab le calc ifica tion
• T2\ VI: High signal flu id in subperiostea l collec t io n &. • Benign tu mor of sku ll base, T-bo ne affec te d rarely
mastoid a ir ce lls
• UWI: Restricted d iffusio n in abscess Lan gerh an s ce ll (LCl histi o cytosis
• Clin ical: Ch ild w it h dr ai nin g ea r &. periau ricul ar m ass;
• T1 C+
o Pre- o r pos t-au ricu lar abscess : Rim -en ha n cing pus in cra n ial neu ropathy un usual
p re- or post-auricular soft tissues • Imaging: Extensive. o fte n bi lat eral . mastoi d
o Bezo ld abscess: Rim-enha ncing pu s in &. around de st ru cti o n with e n ha nci ng soft tis sue
sternocleido m asto id muscle Rhabdomyo sar coma
o Temporal lo be a bscess: focal fluid (pus) with thin
• Clin ica l: Ne urolog ic deficits co m mon, including CN?
en ha nci ng rim & meningea l thickening
pal sy
• MRV: May sh ow dural sinus thrombosis
• Imaging: c r shows exte ns ive bone destruction;
Imaging Re com me ndati ons in trac ra n ial exte ns io n
• cr needed in patients with mucopurulent ea r 0 '1' 1 C+ MR shows enhancing so ft ti ssue mass
disch arg e, ma stoid tenderness o r neurologi c signs em ana ting from mi dd le car
o High-reso lu tion 'I-bo ne shows subtle bone di sease
o CEC r skull base/head ide n tifies ex tracra n ial ab scess
&. intracrani al co mp lica tions IPATHOLOGY
o CT accu racy 95 1Xl fo r AOM co mp lica t io ns
• MR as n eed ed for better definition of intracran ial
Gen eral Features
co mp lica tio n s • Ge ne ral path co m men ts
o Macewen trian gle
• Surgica l access point to mastoid antru m at
IDIFFERENTIAL DIAGNOSIS posterosu perio r EAC
• Weakes t bone-loo se pcrtovtcu m a llo w b reako ut of
Acquire d (Acq .) cholestea to ma infecti o n in post-auricu lar location
• Clinica l: Ret racti on o r ruptu re of tympanic m embrane; o Friedrich Bezold ( 182 4· 190l!) descrtbed S pattern s of
may he su pertnfect ed abscess formati on as seq ue lae o f ACOM
• Imagin g: cr shows erosive mass with poor • Subpe rioste al m asto id ab scess
en ha nce me n t • Bezold ab scess
• Wh en associa ted wit h ACOM may a lso have • Subperi osteal zygoma t ic a bscess
extracra n ial o r intracranial ab scess • Other mastoid ab scesses with or without epo nym
(n ot described by Bezo ld )
Apica l pe trositi s • Etio log y
• Clin ica l: C N6 pal sy, ret roauricular pain , o to ma stoid itis o Infl ammati o n , g ranulation tissue or ch oleste ato m a
• Imagin g: cr sho ws coal escen t ch an ges in petrous a pex b lock ad itus ad a n tru m &. preven t ma sto id ai r cell
o T l C+ MR sh ows e n ha nci ng meninges & focal dra inage
wa lled o rr fluid in pctro us a pex
AOM WITH ABSCESS
o Local hypercmia-actdosts crea tes enzymatic o If co m plica t ion o f acq uired choleste ato ma, o fte n
resorptio n o f tr ab ecul ae (ACOM ) o lde r age gro up affected
o Co rtical subtle or gross deh iscen ce conveys infection • Gen d er: M > F
in to ad jacen t tissues • Socio-eco no mic
• An te ro lateral: Pre-a u ricular abscess o Lower in com e, malno ur ish ed c h ild ren have I risk of
• Post erol ateral: Post-a uricu lar abscess ab scess after AOM
• Off masto id ti p: Ste rn ocle ido masto id abscess
2 (Bezold abscess) Natural History & Prognosis
• Isol ated ex t rac ran ial subp er io steal abscess
• Su pe rio r th rough tegmen: Tem por al lobe abscess
50 o Less co m mon pa t h op hysio logy: Mastoi d cortex o Excellen t p rogn osis wit h prompt th erapy
remain s in tact wit h septic thro mboph leb itis of o Worse if pr io r in com plete a nti bio tic t herapy,
em issary vein s seed in g peri ost eum viru len t organism or im m u noco mpro m ised h o st
• Ep ide m iology • Int racranial absc ess (te m po ra l lobe m o st co m mon)
o 46 % chi ld ren have > 2 episod es AOM by age 3 o Worse prog n os is
o 0. 24% pat ients with AOM develo p ACO M o If co ncomi tan t co m p lica t io ns, even worse p rogn o sis
• Em bryo logy-anatomy • Ven o us sin us th ro mbos is
o Mastoid a n trum present at bi rt h; periph era l • Epidu ral abscess or subd ura l em pye m a
p n eumati zat io n o f masto id d u ring ch ild hood Treatm e nt
o Mastoid tip aerat es lat e
• Intraven ou s a n t ib iotics ± tym pan ocentesis with
• Classic stern ocleidom asto id abscess (Bezold)
m yri n goto m y t ub e placeme n t
un comm on in chil dren
• Su rgical treat men t
o Masto id ai r cells d rain via m u ltiple small em issa ry
o In cision & dr ain age (1 & D) of ex trac ran lal
vei ns
sub pe rios teal ab scess wit h cortica l mast o idect omy
• Exte rna lly in to periosteal ven ou s plexus
• Surgical th erapy m u st be pe rforme d wit h h ear in g
• Intern ally in to sigmo id sinus prese rvat ion in mind
Gross Path ologic & Surgica l Features o Rad ical masto id ecto my if cho lestea to ma p resent
• Pus in mastoid , adjacent abscess cavit y
• Gran u lat io n ti ssue or ch o lestea to ma occ asiona lly
ide n tified in midd le ea r-mastoi d IDIAGNOSTIC CHECKLIST
o Mo re com mo n in subac ute-ch ro n ic di sease
o Requi res m ore ex te ns ive surgery
Co nside r
• Seek o t her co m plica tio ns o f ACOM
Microscopic Features o Tempor al bo ne findings (Tl C+ MR)
• Polymicrobial aerobes &. a na e rob es • Facial ne rve pa ra lysis sh ows as en ha n cing eN ?
• Strep tococcus spec ies co m mon • Labyrinth itis shows as en ha nceme n t with in
mem branous lab yrinth
• Petrositi s (m ucosal en ha ncemen t in pet rou s air
IC LIN ICA L ISSUES cells o n T1 C+ MR)
o Int racranial find in gs (Tl C+ MR)
Presentati on • Ep id u ral abscess, subd ural em pye m a, men in git is ±
• Mo st co m mon sig n s/sy m p to ms d ural sin us th rombosis
o Otalgia (ea r pai n )
o Oth er sig ns /sym ptoms Image Interpretati on Pearls
• Feve r • ACOM u sually mani fests after AOM reso lves
• Otorrhea (ea r dr ain age)
• Post-auricul ar pain &. swelling
• Late ralized au ricle (ea r push ed ou tward by abscess) ISELECTED REFERENCES
• Co nd uctive > sensorine ura l h ea rin g loss I. Taylor MF et al: Indications for mastoidectomy in acu te
• 1 WBC, 1 ES R mastoidi tis in child ren: Ann Otol Rhinal Laryngo l.
• Clin ical profIle 113(1):69-72, 2004
o Yo u ng ch ild wit h I d ay to I week h istory ota lgia, 2. Khan I et al: Mastoiditis in ch ildren: J La ryngo l Oto l.
pos t-au ricu la r swellin g, fever &. otorrh ea 117(3):177-8 1,2003
3. Migirov L: Com pu ted tomograph ic versus surgical findin gs
o 35-?O'){j pati ents already received an tib io tics for in co mp lica ted acute otomastoid itis: An n Oto l Rh inol
AOM l.aryngol 11 2(8):675-7, 2003
o Post-au ricu lar ede ma (Griesinge r sign) co m mon in 4. Bauer PW et al: Mastoi d subperiostea l abscess man agem en t
un complicat ed acu te m astoid itis (85%) in ch ildre n . Int J Ped tatr Otorhinolaryngo l. 63: 185-8, 2002
• En ha nc ing fluid co llec tio n n eeded to co n firm 5. Spiege l JH ct at: Con tem por ary present at ion and
subpe rio stea l abscess managem en t of a spec trum of mastoid abscesses.
Laryngoscope. 108:822-8. 1998
Demograph ics
• Age
o In fants & yo u ng ch ild ren
AOM WITH ABSCESS
jlMAGEGALLE RY
Va riant
(u f t) A xial CfCT sho ws
opacification o f mastoid
(arrow) associated with both
a post-auricular abscess
2
(op en arrow) and an
epidural abscess (curved 51
arrow ). U nusual examp le of
complicated otomastoiditis.
(RighI) Axial T1 C+ M R
reveals p atient after
masto idec tomy for ACOM
now with apical peuosuis
(arro w), m eningit is (op en
arrow) and thrombosis of
sigmoid venous sinus
(c urved arro w ).
Varia nt
(/.efl) Coron al T1 C+ M R
reveals a rim-enhancing
abscess (arrow ) abo ve
tegm en tympani of the
middle ear. Notice empyema
o f mastoid (op en arrow)
extends up through
de biscem tcgm en
masto ide um (cur ved arrow).
(RighI) Coronal T1 C+ MR
shows tempo ral /abe abscess
[errow} associated w ith
meningitis (ope n arrows).
External ear swelling is
present. Otomas toiditis
visible as enhancing mastoid
air cells (curved arrow).
Va riant
(u JI) Axial CECT shows
con fluent mastoiditis (arrow )
0; tett mastoid air cells with
loss of cortical margins a l
mastoid tip (open arrow).
Bezold abscess arises after
mastoid tip erosion. (RighI)
Axial CECT reveals
enlargement o f
sternomastoid muscle with
foeal abscess (arrow)
em bedded in perimuscular
ph legmon . Bezold abscess
extends down from infected
maslOid w ith eroded mastoid
tip.
COM WITH OSSICULAR EROSIONS
2
52
Coronal graphic shmvs IXJ!'t-int7ammatory Q<i<;;cu/ar Coronal bone CT .~ hmvs no f'vidmcf:' of inflamma tory
('ro.~ ion of long pron~5 of inCIJ.~ (arrow )
& hub of .~ la1X·s (K·hris. A portiotl of incus is visualized (arrow) .
(opt O
n arrolV). Note ch,mb'<-'s of tympJn5cfpwsis of 11(MIE'\!l'r the long process & kmticul.1f prOC('5_~ of incus
tympanic membrafJ(.' & ossc les. ~lnd stapf!S are absC'nt (open arrow).
• Axial CT plan e: Epity m pan u m shows "ice crea m

!TERM IN O LO GY co ne" (an terio r icc cream = malleu s head; posterio r
Abbreviatio ns and Syno nyms co ne = in cu s body/sh or t pr ocess)
• Abbr eviati ons: Ch ron ic o titi s m edi a (COM); oss icu lar • Axial CT plan e: Meso tym pan um shows 2 "parallel
ch ain (OC) lin es" (a n te rio r lin e = ten sor tympani tendo n
• Syno nyms: Non-cho lesteatomat ous ossicular erosio n; leading to mall eu s neck; posteri or line = in cu s
post-infl ammator y oss icu lar eros ion lenticular process, incudo stap ed ial joint & stapes
head )
Definitio ns • Coro nal CT plane: Mesotym panum shows
• Definition: Erosive changes in vol vin g OC in absence ossicular "righ t ang le" (vertically o rien te d in cu s
of cho lesteato ma in patien t with long hi sto ry o f COM long pr ocess; hori zontally o rien ted inc us
len ticul ar p rocess)
o Absenc e of segment of ossicular chain
IIM AGING FINDINGS • Co m mon c r findi ng ; usuall y ove rloo ked
• Often with out ad jacen t in flam ma to ry de br is
General Features o Axial bone cr ima ges
• Best d iagnostic clue • Absen ce o f portion o f posterio r lin e of normal 2
o Absenc e o f segm en t of ossicular cha in "pa rallel lin es"
• Most com mon ly found in di stal in cus • ln cudo stap ed ial jo in t (IS) may he repl aced by
• An y o ssicula r seg men t may be invo lved fib rou s tissue
• Ofte n wit hou t sur ro u nd ing infl ammat or y debri s • IS) th erefor e may ap pear widened o n ax ial bone
• l.o cat ion: Middl e ea r o ssicular chain CT
• Size: FOGIl o r diffuse • Erosio n o f ice crea m co ne, especia lly co ne = in cu s
bod y/sh ort p rocess also occu rs
CT Findings o Co ro nal bone c r im ages
• NECT • Long p rocess o f inc us most co m m on ly absen t
o Imaging d iagnosis req uires st ro ng ana tomi c • Vertica l segmen t o f "righ t ang le" mi ssing
foundati on • Tympan ic membrane retracti on o fte n p resent
o Ana to my o f o ssicular chain
DDx: Norm al Ossicu lar An atom y
2 Parallel Lines Incus Lon g Pro cess Ice Cream COile Malleus H eed / Neck
Key Facts
Terminology • MR im agin g h as no ro le
• Abbreviati ons: Chron ic otitis medi a (CO M); ossicular Path ology
cha in (OC) • Lon gstandin g hi story of COM in itiates lesion
• Defin itio n : Erosive cha nges in vol ving OC in absenc e • Subsequent osteoclasia & decalcification crea tes bo n e
of cholestea to ma in patient with long h istory of
COM
loss
• Acid ph o sph atase is stro ng ly implicat ed = well known
2
Imaging Findings marker for lysoso mal activity 53
Most com mo n ly found in distal in cus • Incu s is most vu lne ra ble portion o f oss icula r chai n
• Imagin g di agn o sis requires strong anatom ic du e to te n uo us blood supp ly
fou nd ation Clinical Issu es
• Absence o f portion of poste rio r lin e of norma l 2 • I'o st -in fla m m ato ry co nd uctive hearing lo ss
"pa rallel lin es" • Usua lly there is lon g h isto ry of chron ic otitis me dia
• Sclero tic m astoid com mo n • Suc h hi stor y not always present!
• Axial &. coro na l thln -sectl on hone c r im ages thro ugh
middle ea r
o Sclero tic masto id co m mo n o In itial ph ase in clu des periostiti s &. osteiti s
• Eustac h ian tube dysfunction o Subseq uent osteoclasia &. dccalcification crea tes
• COM during ma sto id formation bon e loss
• CECr • Ostco clasia defin ed as a bso rptio n & destru cti on of
o Some en ha nce me nt o f deb ris possible bon y tissue
o Co n t rast not needed or reco mmended • Multin ucleat e osteoclast & mononuclea r
h istiocyte implicated
Imaging Recommendation s o Seco nda ry path o ph ysiol ogic hypo th esis
• Axial & coro na l th in -secti on bo ne CT im ages th rough • Ossicu lar erosio n is fro m enzy ma tic activ ity
midd le ear associated with m idd le ear inflam mati o n
o 0.5-1.0 m m th ickness • Acid phosp ha ta se is st rong ly impli cat ed = we ll
o Overlap th rough ova l window desirab le in axial kn own marker for lysoso mal activity
plan e o Incus is mo st vul ne rable portio n of oss icular chain
o Be fami liar wit h com mo n site s of erosio n d ue to te nu ou s blood su pp ly
• MR imag ing has no ro le • Isch em ic n ecrosis th erefo re likely play s a
o Unless co m plicated COM or AOM is presen t sign ifican t role
• Wate rshe d region in vicin ity of di stal inc us
o Bon e resorptio n furt h er facilitated by capillary
IDIFFERENTIAL DIAGNOSIS prolife rat io n &: h igh oxyge n te nsio n
• En ha nce in flam matory sti m ulus &: foreign bo dy
Congenita l oss icular deformity reactio n
• Very rare, especi ally as a n isol at ed fin di ng • Ep idem io logy
• Co nd uctive hea ring lo ss in ch ild ren o Very com mo n clin ical & cr en tity
• Usually associat ed wit h EAC atresia o Lack of recognitio n by rad io logists make specific
• Typic ally limited to a specific bra nchial arc h in ciden ce figu res u nava ilab le
o Lat eral OC (1st a rch) : Malleu s I< proxima l incu s • Associated abnor ma lities: \ Vit h or wit hou t
o Distal OC (2n d a rch) : Distal in cus I< stapes in flamm ato ry de b ris
Cho lestea to ma with ossicular erosion Gross Patho logic & Surgica l Features
• Non-de pe nden t so ft tissue mass assoc iated • Exp lo rator y tym pa no to m y reveals eroded ossicu lar
• Perfo rat ed o r retracted tym pan ic me mb ran e cha in
o Clin icia n is usually awa re of cholestea to ma presen ce • Gran u latio n tissue o fte n asso cia ted
Post- ope rati ve oss icular loss • No cho lestea to ma
• Evide nce for masto idecto my o fte n present Microsco pic Features
• Stapedecto my for fene st ral o tosclerosis most co m mo n • Mu ltinucleat e osteocl ast & mon on uclea r h istiocytes
cause ofte n presen t
• Foreign bod y reacti o n
IPATHOLOGY
Ge ne ral Features
• Etio logy
o Lo ngstand in g h isto ry of CO M in itia te s lesion
o If ab sent, distal long process & len ticular process o f
IC LIN ICA L ISSUES incus has been eroded
Presentati on
• Most co mmo n signs /sy m pto ms
o Post-inflammato ry co nd uctive hearing loss ISELECTED REFERENCES
• Usua lly th ere is long history of chro nic o titis L Dawes P]: Myrtngo stap ed iopcxy: su rgical expec tatio n. J
2 media
• Such hi stor y not always present! 2.
I.ary ngo l 0 101. 1 17(3 ):182-5, 20m
Ishim o to S et al: Use o f car tilage plate as tym pan ic
mem brane in to tal m idd le car reconstructive sur gery for
54 • Clinica l profile
in fected radi ca lized ear. O tol Neu ro tol . 24(1):2·5 , 200 3
o Adult wit h co nd uctive hearing loss 3. jung JY ct al: Bone resorpt ion in ch ro n ic o titis med ia : th e
o Otosco py sho ws no soft tissue mass ro le o f t he osteoclast. ORLJ Oto rh inolaryngol Relat Spec.
o Tympanic membrane may have healed leading to 64 (2):95-10 7,2002
con fusio n Lesinski SG: Causes o f conductive hearin g loss after
• Clin ician may suspect otosclerosis if TM healed staped ecto my or stupcd o to my: a p rospective st udy of 279
co nsecutive su rgica l revision s. Oto l Ncu rotol. 23( :~):28 1·8,
De mo gra phics 2002
• Age: All ages 5_ Srin ivasan V ct al: Pars tcnsa ret racti o n pockets in chil d ren :
t reatmen t by exc isio n and ven tila tio n tu be in sert ion , Clin
Na tural History & Progno sis Ololaryngol. 25 (4):253 -6, 2(K)0
• Surgical repair resul ts var iahle 6_ Dor nhoffcr JI. ct al: Evid ence o f residu al di sease in osstcles
• Relat es to severity of ossicular loss &. associated o f pati ents undergoi ng chole steato ma remo val , Acta
O tolaryngol . 119 (1):89 -92, 1999
tym panic membra ne status 7_ I.i Y et al: Prospect ive study o f tym panic membrane
Treatm en t ret ractio n , hearing 10\5, and m ult ifreq uc ncy
tym panom ct ry, Otol ar yngol Head Neck Sur g.
• Explo ratory tym pan otomy with oss icu lar 12 1(5):514-22, 1999
reco nstruction as needed 8_ Yu ng MW: Type IV tympa noplasty rcvlsttcd . Am J O tol .
o Depends upon amount of bo n e resorp tion 19 (6):700-3, 1998
o Decision made at tim e o f su rgery 9_ Alhu S et ;11: Prognostic factors in tymp ano plasty. Am J
• Su rgical lin k is created 0101. 19 (2 ): 136-40 , 1998
o Bet ween malleu s hand le &: head of sta pes 10. Swa rtz j l) ct al: Imag ing o f t he Tem po ral Bone, Th ieme,
o uetw cen tympa nic membran e &. head of sta pes Inc., Ch . :~ . 199K
o PORP = partia l ossicular rep laceme n t prost hesis 11. Lemmcr ling M~ f : Nor ma l and o pacified midd le ca rs: C I'
;lppcaran ce of th e stapes and lncudostapedl al join t.
o TORr = to ta l ossicular replacem ent prost hesis
Radiology 203 :25 1-6, 199 7
o Syn the tic prosth eses 12. Mills RP: l'h yslologica l reco n structio n of def ect s of th e
• Go lde n be rg in cu s lon g process. Clin Oto laryngol. 2 1(6) :499·5 0] , 1996
• Appleba u m L3 . Qua ranta A ct ill: Cho lesteato ma in child ren:
• Richa rds cen tere d h isto pa th o logic find ing s in m iddle ear osst cles. OIH.J
• Black o va l-to p Ot orhinolaryngol Re lat Spec. 57(5 ):296-8 , 1995
o Ho mograft, autog raft l -l. l'alv a T et al: Epity m pa nic co mpartme n t su rgical
• Incus int erp ositi o n co nside ratio ns : reeva luat ion , Am J Oto l. 16 (4):505- 13. 1995
IS. Uno Y et al : Bone reso rp tion in h u m an cho lesteatoma :
• Alternatively, "sleeve" techniq ue is used
mor ph ologica l stud y wit h scan n ing elec tro n m icro sco py.
o Co rtica l graft co n nec tion is placed over malleu s Ann Oto l Rhino! Laryngol. 104(6):463·8, 1995
stu mp &. co n nec ted to stapes head 16. Vartiainen F. et al: Hearin g resu lts o f sur gery for acq uired
cho lesteato ma. Ear Nose Throat J. 74(3):160·2, 164 , 1995
17. Vartiai ncn E et al: Hear in g resu lts o f sur gery for chro nic
IDIAGNOSTIC CHECKLIST o titis media without cho lesteato ma. Ear Nose Th roat J.
74(3) : 165-6, 169 , 1995
Co nside r 18. Wright CG ct al: Patho logy o f ot itis m ed ia . Ann Oto l
• Fenestral otosclerosis in indivi d ua ls with co nd uctive Rh in o l Laryn go l Su pp!. May; 163:24-6. 199 4
19. Va rt iainen E ct al: Success and pitfa lls in m yringopl asty:
hear ing loss if ma stoid is well pn eumat ized
follow -up stud y of 404 cases. AmJ O to l. 14(3 ):301-5, 1993
o Ca reful exa m ina tion of ova l window area on bone 20 . La nn igan FJ et al: The cellular m ec hani sm o f ossicu la r
CT ero sion in ch ronic sup p urative o tit is me dia, J Lar yngol
• Conge nita l ossicu lar defo rm ity 01 0 1. 107(1): 12-6, 1993
o Very rare en tity un less EAC atresia is present
Image Interpretati on Pearls
• Make su re that bot h an te rior &. posterior of "two
para llel lines" are in tact as seen o n axial bo ne CT
images th rough meso tym panum
o Posterior lin e of "two par allel lines" is most
co m mo n ly absen t in COM ossicular erosio n
o Represen ts erosion o f long process of incu s & stapes
• Be certa in that "righ t angle" is visualized o n co rona l
bo ne c r ima ges at level of vestibule
IIMAGE GALLERY
Typical
( oron.l! bOTJf-' (7
( I .tlt)
rotmction of
(t'VE',I/S
tym p,mic memh,.lIlt> ( ,Jrr Ol V ) 2
wilh dt'fIJine(a!i/dlicm of IOllg
pruc('!iS of incus (u /f w d
arro w ), 5lrJ f}( lin~ Sofl li m JP 55
in m iddfe t~J r is dssoci<1lt'c/
inl1,lIJl1lJa lory ctobris. (Hig ht)
i\ xi.1 /IJom' CT _~ how.'i norm,, 1
tensor tympani tenck m 0<
mal/pHS neck anterio rly
(arro w). Vis/al incu s &
.~ « l')(,,~
,lh"elJl (op ell arrow)
pos/e riorly. Long his/Dry of
CO,\-I. N o to .1bSC'flCC' of
Inflammdtor}' debris.
Typical
(l .I!fl ) A'l:i.ll bono C1
ctetnonstrstes normal stapf:'s
crura (arrow). Distal
incus-lenticular proces.' S
."tapes huh are ahsenl (open
arrow). N o te flamMI mal/em
fuxk (c ur ved arrow ) in ih l'
antf'(()la/(',.l l mk kito e.n.
( R;~ " t) Coronet bunt' C1
((oVC'.l/,'; (ot.ll (/t>f('ct in IOllg
pf()( P,'iS of (/1.,,(,11 incus
(arro w) Js; ociJted with
<Ji((uo;.f' pat ch y in l1.1mm,lIory
dehfis w ithin aWc dnd
hvpct vtnpatwm . , hefe was
-10 df'cil)( ·' conduc tive-
ch'(icit.
Typical
(LeJI) Coronal bone ( I
shows broad tympanic
nwmbmno Ihickening
(.l rrows) wilh assuciated
retmction. The en tire
().,<;iCIJJ,lf chain 1J.1.~ bf'f'n
f(..'50r/}C'd resulting in maximal
co nd uctive hC'. lfing de ficit .
(Ilil:/it ) A'(ial bOn!- CT f('VN/S
the "ice cream cone" (short
process 0; incus ) h as been
destro y<'{/ by COM (arrow ).
N o te absence 0;
cbolestestometous mass.
"Ice cream " (/)ead 0;
m alleus) is present .
COM WITH TYMPANOSCLEROSIS
2
56
Coronal graphic .~ /JOWS severe tympanoscJerosis in Axial right ear T-1>one CT shows tym panm d cfm i.5 as
sell ing of chronir. otom.lstoi(Jitis. Post-inflamm ator y I XJst.infl.JlJlln l tory calcifications within (·pitymp.llw m
c.alcification can be seen in tympanic membrane (arfD'l'Vs) in p dricn/ with long history of COM & severe
(arrow) g throughout micld/(' car. conductive h<-'ilring h~.~ .
o High density foci surro u nd ing oss icular cha in

ITERM IN O LO GY associat ed with par tial o r co m plete middle
Abbreviat io ns and Syno nyms ea r-masto id o paci fica tio n
• Abbreviatio n: C h ro nic o titis medi a wit h • Ca lcific deb ris (tr ue tympan osclerosis) is ca use o f
ty mpa noscJe rosis (CO M-T) high d en sit y on c r
• Syno ny ms : CO M with foca l calcificatio n o r • Variably sized ca lcificatio ns
ossi fica tion; post-in fl ammator y ossicular fi xati on • Calcificatio n no t necessar y fo r di agn o sis (fib ro us
tissue fixat ion)
Defini tions o Oval window invo lvemen t occurs
• COM-ToCalcific, bony or fib rou s midd le ea r foci form • Whe n iso lated, referr ed to as peri -stap ed ial "ten t"
seconda ry to chronic otitis me d ia (CO M) • Differentiated from fenestral o toscle rosis by lack
of in vo lvem ent o f o tic capsule
o Co m mon locati ons o f tympan osclerosis
IIM AGING FINDINGS • Tympa n ic memb ran e
• Epity m pa n um (attic) : Ossicles &. ligamen ts
Ge ne ra l Feat ures • Mesot ympanum: Ossicles
• Best diagnostic clue : Bone CT shows high d en sit y • Less co m mon in posterio r tympa num &.
(calcific or ossific) foci in middle ea r-mas toi d h yp ot ympanum
associated with spo radic inflammator y debris o CT findings associa ted with COM
• I.ocati on • Het erogen eou s soft tissue (inflam mato ry tissu e)
o Epitympan um (attic) with in mi dd le ca r &. ma stoid
o Meso ty mpa n u m (m idd le ea r cav ity p rop er) • Unde rdevelo ped , poorl y-aerat ed sclerotic mastoid
o Tympan ic memb ran e (long histo ry o f ch ro nic o titis)
• Size: Varies from m illim et er foci to thi ck areas of bone • CEC r
d epo sitio n o Co n t rast plays no role in diagn o sis
• M orphol ogy: Lin ear o r ovo id foci o Co n t rast is not reco m mended
CT Findin gs MR Findings
• NECT • No ne, in typ ical case
DDx: Middle Ea r-Mas to id Radio -Opaqu e Lesions

- ---.,
Stapes Pro sth e$is Tympanostom y Tube Incu s Ilo m og ra(t

Key Facts
Termin ology Patho logy
• Abbreviation: Ch ron ic otitis media with • Diffuse h yalinizati on &. d eposit ion o f calciu m «
tympanosclerosis (COM-T) phosphat e crysta ls in multiple foci within middle
• COM-ToCalcific, bony o r fibrous middle ea r foci form car- masto id
seconda ry to chron ic otitis med ia (CO M) • Th ree varieties of post-infl ammat o ry ossicu lar
fi xation 2
Imaging Find ings • Fibro us tissue fixat ion
• Oval window invo lveme n t occu rs 57
• Classic tympan o sclerosis
• Axial &. coro na l un enhan ced thin-sectio n T-bo ne cr • Ossific tym pan osclero sis
best exa m
Clinica l Issues
Top Differen tial Diagn oses • Clinical profile: l.ong hi stor y o f COM
• Conge nital cho lesteato ma , middle ear • Otoscopy : Thi ckened TM ± tympanoscleroti c plaq ues
• Granulatio n tiss ue, middle ear -ma stoid • Su rgical intervention less success ful th an with COM
• Acqu ired cho lestea to ma withou t ty m panosclerosis
• Fenestral o tosclerosis (FO)
• Ossicular prost hesis
• MR not ind icated unl ess suspected regional

comp lications of COM O ssicu lar prosthesis
• Clin ical histo ry crucial
Imaging Recommen dation s • If hi stor y u n known, observer may co nfuse wit h
• Axial &. coro na l u nenh anced thin-section T-bon c CT tym pan osclerosis
best exa m • Incus in ter pos ition graft may be especia lly co nfusing
o Overlap ping ax ial images through ova l wind ow may
be part icu larly useful
• MR has no role in average case IPATHOLOGY
o Useful for co mplicated CO M
Gen e ral Features
• General path com me n ts
IDIFFERENTIAL DIAGN O SIS o COM-T is healin g respo nse to repeated
infl ammatory event s in midd le ear-masto id
Congenital cho leste ato ma, midd le ea r o Often develo ps duri ng q uiescent periods
• Clinical: Variab le co nd uctive hea ring loss (CHl.) o Formed by fused co llagen o us fibers
• Most com mon in mesot ympanu m • Fibers hardened by dep ositi on o f calciu m &:
• Mastoid is well pn eumatized pho sp hate cryst als
• Calcificatio n very un usual o TM inv olvement common
• Localized mass most com mo n • May oc cur as an isolated even t
• Etiology
Granulatio n tissu e, middle ea r-mas to id o Diffuse hyaliniza tion & d eposition of calciu m &
• Clinica l: Co nd uctive hear ing loss va riable; not phosphate crysta ls in multiple foci withi n middle
hallmark o f process; COM history ear -ma stoid
• Patch y, non-destructive middle ea r d ebri s o Chalky calcific de position th en occurs
• Debris not calcific • Epidemiology
Acquired cho lesteato ma o l Ql.Yi) of pati ents with chronic su p pu rative otitis
medi a develop tympan osclerosis
• Soft tissue mass with associated ossicle loss ±
o 21MI have co inciden t midd le car cho lestea to ma
underlyin g bone erosio n
• Chronic o to masto id itis findings present o n cr Gross Path ologic & Surgica l Featu res
• Calcific foci (tyrn pancsclerosis) rarel y associated • Inflammato ry debris binds ossicles, tympanic
• Usua lly localized to att ic membran e (TM) & ligaments to m idd le ear wa lls
Fe nest ral otosclerosis (Fa) • At otoscopy, opaq ue TM o r ex tra "bo n y" foci seen
through ruptured TM
• Clin ical: More co m mo n in fema les; no histo ry of COM
(well pneu matized mastoi d ) Microscopic Features
• FO usually loca lized to an terior ova l window • Calcificatio n of previously h yalin ized mu cop eriosteum
• Diffuse calcificatio n of ova l window more likely • "Onion skin-like" lamellar arrangem en t
tympan osclerosis
• "Obliterative d isease" may appear id en tical Staging, Grad ing or Classificat ion Criteria
• May be associa ted wit h cochlear disease • Th ree va rieties of post-in flammatory ossic ula r fixatio n
o Fibrous tissue fixation
• No calcificatio n
• Midd le ea r d eb ris with dispro por tio nate profound
CHL
IDIAGN O STI C CHECKLIST
• Severe CHL help d ifferen tiate from gran ulatio n Consider
t issue' o r c ho lestea to ma • Fen estral otoscle ros is first in patien t wit h CH I. wit h out
• May occ u r anyw here in meso tym pan u m (m idd le COM hi stor y
car cav ity p rop er) or epi ty mpa n um (attic)
o Classic tympan osclerosls Image Interpretation Pearls
2 • Mu ltiple sm a ll ca lcificat io ns • Loo k for in flam m at ory debris in mi ddl e ear-ma sto id in
• Usua lly associa ted with non-calcific deb ris co m bi nati o n wit h m ultip le ca lcific depo sits
5A (gran ulatio n tissue o r even cho lesteato ma) o May loo k like "ex tra ossicles"
• May be locali zed to TM • CI IL d isp ro po rt ion at e to a mou n t o f m id d le ea r deb ris
o Ossific tym panosclcrosis • Ca lcifica tio n o r oss ifica tio n n o t n ecessa ry for d iagno sis
• New bon e fo rma tion
• Usua lly occu rs with in e p ity m pa n u m (att ic)
• End stage process ISELECTED REF ERENCES
• May a ppea r to "coa t" ep ity m pa n u m I. Karlldng T ('1 .11: Comparison of fret" radicals an d
• All 3 va rieties co m m o n ly refer red to as antiox idan t enzy mes in ch ro n ic otitis media wit h a nd
"tyrn pa no scle rosis" wit h ou t tympanosclerosi s. Laryngoscope. 1 14 (1):85-9,
2004
2. Wl'llin g DB e t al : l'rcdi ct lve facto rs in pcdl a trlc
IC LIN ICA L ISSUES sta pedec tomy. Laryngosc ope. 1 U(9) :IS 15-9, 2003
.1. Batt aglia A et .11: Reco nstructio n of th e ent ire oss icu lar
Presentati on con ducti on mechani sm . La ryngoscope . 11:\(4):654-8, Z003
• Most co m mo n sign s/sym pto ms 4. l'ulcc J l.l't .11: Lo n g-term ven tila ting tube wit h
tympa no sclero sis. Ear Nos e Throat J. 8Z(1 ):8, Z00 3
o Severe Cl IL
• Usually o ut of p roportio n to exte n t o f
s. Vincen t R et .11: Stnpcdotcm y fur tym pa no sdero tic sta pes
fixation : is it safe and efflcl en t? A review of 68 cases. O to l
in flam ma to ry deb ris Neur otul. 23(6):866-72, 2002
• Clinical profile; Long histor y of COM 6. Fo rsen i Flodin 1\1ct a l: Po ssib le inflammatory m ed iator s in
• O t h er p re sent ing signs o r sym pto ms tym pa n osclcro siv d eve lopm ent . IIlI J Pcdi at r
o Ot osco py: T h ic ke ned 'I'M ± tym panosclero tic Ot o rh inola ryngol. 25; 63(Z ): 149-S4, ZOOZ
plaqu es 7. Tcufert KB ct .11:Tympano sclcr o s!s: lo ng-term h earing
result s a fter o ssicu la r reco nst ructio n . Ol o lar yn go l Head
• No v isible c h o le stea to m a
Ned: Surg. 1 2 6 CH : 2 64 ~ 7 2 . 2002
De mograp hics 8. Albu Set al: Surg ical treat me nt of ty m panoscleros is. Am J
Otol. 21 :63 1-5 , 21X)0
• Age 9. Palacios E et .11: Tym pa nosc lcrosis. Ear ~OSl' Th roat J.
o Ave rage age at d iagn os is e 3S yea rs 79( 1):17-23,20 00
• Age docs no t h el p to diffe ren ti a te fro m fen estral 10. Alza m il KS ct .11: Tympa n o scleros ls. Am J Otol. 2lJ(SI:6K6-7,
o tos clerosis (sign ifica n t age ove rlap) 1999
o All ag es bu t m ore co m m o n in ad ult 11. Asiri S CI al: Tym pa uo sclc ro sis: revie w of lite rat ur e and
• Disea se ta kes yea rs to develop in ciden ce ,II Bong pat ients with midd le-ca r in fect io n . J
Laryn go l O to l. 113(12):1076·80, 1999
Natural History & Prog nosis 12 . Ka ma l SA: Su rger y o f ty m pa no sclc rosis. J La ryn go l 0 101.
• Depends on severity o f COM 111(10 ):9 17-23 , 199 7
• Su rgic a l int ervention less successfu l t han wit h CO M 13. Lcm merltn g M~ l et al: Nor mal and o pacified mid d le ca rs:
wit ho ut tympanosclerosis cr a pp ear an ce of sta pes a n d incud o stapcdl a l joint.
Radiology. 20:\:251-6, 199 7
• Su rgica l in tervention less successfu l th a n with H. Fo rscn i M ct a l: Development of tym pano sclero sls: can
fen estral o to scle rosis predict in g facto rs he identified"! Am J O to l. IXO ):29 X-3(H ,
199 7
Treatm ent 15. Malec MF ct .11: Ch ro n ic o tom astoldi tls: a concept ua l
• Atticoto m y with m obilizat ion of ossicles o r understanding of CT find in gs. Rad io lo gy. 60( I): 193-200,
cp ity m pa n lc bypa ss p ro cedu re 19X6
• In sertion o f p rost h esis o r h omogra ft d evice 16. Swartz JD ct al: l'ostlnflamm atory o vslcula r fixation: CT
o Dependen t u po n exten t o f d isea se anal yviv with surgical correlation. Radiology.
o Prost heti c stapedecto my ty pica lly less effect ive when 154 1:l):69 7-7()O, t 9 85
fcucstr a l (o va l window) tym pan osc lcrosts is p resen t 17. Kin n ey SF.. Po st in flam ma tor y ossicula r fixa tio n in
tympan oplast y. Laryn go scope. X X(S) : K2 1- :~ 8, 1978
o TORP = tota l oss icu la r rep lacemen t prost h esis
o PORP = pa rtial ossicu la r re placem en t prosthesis
• Ma n y au t ho rs recommend rem ova l o f Ko rner sept u m
(pe t rosq ua mous la min a)
IIMAGE GALLERY
Typical
(Lef t) Coronal t-bo tw C T
reveals large den se bone
dcposit with in medial
ep itympanum (arr o w) in
2
patient with long histor y o f
COM . This type of 59
lympanosclewsis o ftell
referred to as Iib ro -osseou s
sclerosis. (R iglll) Coronal
' -hone CT sholVs sublle
l ympanosclf'(()sjs as Io cus of
calcification within oval
Iv/nc/ow (arro w). Note
.1!Js(,rll distal incu s whi ch
1U'lp.5 dir(('((' nlia/f> from
fl:>flt'slfa { otosclerosis.
(I.l'jH !\ X;il / ' f' ft fI,1f f~J)(JIJ(,

CT S!JO W.5 lym,J.lIlmdt'w"is
calcific foci sUfro tl/ lC li nR
,l'i
msic/es (arro w ) rp SLI/ling in
1'0 m clt"i'niliolJ of "icc Cft.'<1IU
COIlt ''' , Ctv nn ic in l1,lmm,lI(}(y
dl'/Jri.s fill.. {'pitymp,lIlum .
( /{ i£htJ CO/Ofl.!1 ft'{/ l'a l
U JOfJ(' C T (ew,lls
tymp.Jnow·/erm is a s msi(i("
dt'1)tL~ "coa ting " /1l('(Ji,I/ IV,11I
of {'pilymp,lIl11fll (,,,ro ws ).
Tll i" i" ,m f' xaml ,h' of "ocw
hOfJ(' fo rm,l lion ".
Typical
(1.£11) Axia l T-bOlw CT ill
p.1ti plJl with COM S
l ympd nos dpro ~ is ~"O\1/S
fuzzy. "enlarRed" ossicle,
(arrow) due 10 calci fic
d ep ositi on . Diffuse
inflam nMt o r }' debris pr esent.
OpPfJ arro w : Os siii c foci in
f1 "'-'iloid. ( N ight) Corona l
T-ho ne C T .~ 1JOIV5 ca lcific
de hrio; o;urrounrh'ng ttw
f1Jal/f'u.'i (arro w s) res'ul ti ng in
f1ldlle us fixation and
conductive hearing {0';5.
Calcifica tio ns suggcst
tympanosclerosts no l
c·lw/t's/e a to ma.
ACQUIRED CHOLESTEATOMA, PARS FLACCIDA
2
60
("orOIl. )1graphic 4 10\\'\ small ,ho1('.\ /mlOIIM ori~ inalitJ~ G JlIJflJI ri/-:/II ( '<If T-bOIl l' CT ~ho\\'~ pars flaccid,l
,it Pd'~ t7JC<'ic/,1 portion of /he t ympanic (lK'mlJl',lIJe, c!Jok'st('.l tofJIa filling PtllHa k ',~ Sp..1C(' (arrow). with
fjlfing "russak's sp ace (arrow ), wil h slight media! biunting of tin" K ucum (open arrvl v ) l\ erosion of
dispfa c(>fll('n l of o~ 'id('s . m<'dially di5pfac'{'c/ mall('m .
• Morpho logy
ITE RM INO LO GY o w cl t-ctr curn scribed mi ddl e ea r ma ss
Abb reviatio ns a nd Syno nyms o t.arge lesion s o fte n associat ed wit h sca r &. effusio n,
• Par s flaccida c holeste ato m a (PFC ) m ay be less well defined
• Syno ny m s: "Attic" or "Prussa k space" ch o les teato m a CT Find ings
• Previously ca lled middle ea r kerato ma
• N ECT
Definitio ns o Soft tissue ma ss in Pru ssa k's space (me d ial to attic
• l'l-C: Exfo liated keratin with in stratified sq ua m ous wa ll, lateral to head of ma lleus & bo dy o f inc us,
e p it heliu m; begins in Pru ssa k's space above la teral rna llcar ligament) ,
• May exten d poste rolat er a l to ad it us a d a n t ru m ,
into m ast oid a n t ru m
IIM AGING FINDINGS • Inf erior exte n sio n to posteri or m iddl e ear recesses
may occur in ch ild re n
Gene ra l Fea tures o Scutu m erosio n co mmon
• Best d iagn ostic clu e: Mass in Prussa k's space with o May widen ad it us ad a n tru m or re m odel lat eral a tt ic
scu tu m, os sicle o r lateral e pity m pa n um wa ll ero sion wall
• Lo cat ion o Ossicu lar eros io n in 70 1M,
o Pars Ilaccid a (PF) portion o f tympan ic m emb ran e • Long process of in cu s m ost co m mon lv e rode d
(TM), a lso known as Sh rapn ell's m e mbrane, is sm a ll • In cus bod y &. ma lleu s h ead ma y also i>c eroded
sup erior portion o f TM • Ossicles d ispla ced m edi a lly
o PF retract io n pocket O [ per foration predi spo se to • CECT: No en h a n cem e n t of ch o lestea t o ma, but
c ho lestea to ma formation in Prussa k's space su rro u nd ing gra n u lat io n tissue m ay en ha nce
• Size MR Findin gs
o Ea rly Pru ssa k's space cho lesteato ma m ay be o n ly
millimeter s in widt h • TlWI
o Sma ll lesions poo rly see n in pn eu mati zed mi dd le ear
o Neg lected PFe may gro w to cen ti me ters in size,
& m astoid co m p lex
fill ing e n ti re middl e ea r ~ beyond
o Hypo in tc nse midd le ear m ass
DD x: Middl e Ear Masses
IlT Cholesteatoma CongoCho lesteotome Cholesterol Granulom a Glom us Ivmpenicum

Key Facts
Terminology • Glo mus tympanicum pa raganglioma
• Syno ny ms: "Attic" o r "Prussak space" cholestea to ma Path ology
• PFC: Exfo liated keratin within stratifi ed sq uamous • 'I'M perforation o r retr acti on results in m iddl e ear
epitheliu m; begin s in Prussak's space acc u mulatio n o f st ratified epithe lial sq uamo us cells
Imaging Find ings • M ost com m on middle ear-mastoid lesion 2
• Best dia gn ostic clue : Mass in Prussak 's space with Clinical Issues
scutu m , ossicle o r lat eral epitym pan um wall eros ion 61
• Clin ical profile: Pati ent wit h histo ry o f ch ro n ic
• Soft tissue mass in Prussak's space (me d ial to atti c middle ea r infl ammat or y di sease &. TM ret ractio n o r
wall, lat eral to head of malleus & bod y of in cu s, perforation
above lat era l rnallear ligament)
• Ossicu lar erosio n in 701M) Diagnosti c Checklist
• Wh en ME & mastoid com plete ly opacified, difficull
Top Differential Diagn oses to d ifferent iat e effusio n frn m cholesteatoma
• Pars ten sa (PT) cholestea to ma • Ossicular erosio n su pports dia gn o sis of cho lestea to ma
• Congen ital cho leste ato ma, midd le ear in com plete ly o pacified ea r
• Cho leste rol gran uloma, midd le ear
• T2 WI: Usually homogen eo usly hyperin tense

IPATHOLOGY
• T1 C+
o Lo w signal in te nsity mass, nonenhancing except for Gen eral Features
periphera l rim o f gran ulation tissue • General path co mmen ts
o If tegm en tympani erosion pr esent, du ral o Mo ist ure & d esq uam atio n o f epit helial cells result in
en hance me n t at bon y d efect inv ag inat ed po uch filling wit h epide rmal deb ris
Imag ing Recommendation s o Exfo liated sq uamous epit he lium grow s into
• lti gh -rcsolution tem pora l bone CT, with out co n t rast, cho lestea to ma
is test o f cho ice o Seco nd th eo ry: TM perforati on lead s to sq uamo us
• Prussak's space mas s, att ic, scut u m arc best see n 01 1 epit he liu m fro m 1'M su rface exten d ing in to middle
co ro nal CT ear, resulti ng in cho leste ato ma
• Co ronal 1'1 C+ MR used whe n ce p haloce le, m iddl e o TM perforati on o r retracti on results in mid d le ca r
cran ial fo ssa infectio n o r intracra nial cho lesteato ma accum ulatio n of stratified epit he lial sq uamous cells
ex tension suspected • Etiology
o Deve lo pmen t of cho leste ato ma hegi ns wit h negat ive
m id d le ear pressur e leading to TM retr act ion pocket
ID IFFERENTI A L DIAGNOSIS o 'I'M perfo ration o r retractio n results in mi ddl e ear
accu mulat ion o f stratified epi the lial sq ua mo us cells
Pars tensa (PT) cholesteato ma o Sq uamo us epit he lial cells produce kerat in, wh ich
• Otoscopy: 'I'M rupture o r retraction in posterosu perior beco mes mass-like
par s ten sa area • Epide m iology
• Less co m mo n t han pa rs flaccida type o Most co m mo n midd le ear-masto id lesion
• Imaging: Sin us tympani & facial recess in vo lvemen t o Mo st co m mo n type of cholesteato ma (80% o f all
arc classic acq uired cho leste ato mas )
o Ossicles push ed laterally • Associa ted abnor malitie s: Cleft palate is risk factor for
cho lesteato ma fo rma tio n
Co nge nita l cholesteatoma, middle ea r
• Oto scopy: Tan-whit e mass beh ind in ta ct 'I'M Gross Path olo gic & Surgical Features
• On ly 21M} of cho lesteato mas are congen ital • "Pearly tumor, co m posed o f so ft, waxy, whit e material
M
• Imaging: Ossicular erosion may occur • Often has ch ro n ic infl am mator y co mpo ne n t which
may ca use osseo us &. ossicular erosio ns
Cho leste ro l granuloma, middl e ear
• Otosco py: Rctr ot ympan ic "blue" mass Microscopic Features
• Imaging: Ossicul ar & bon y ero sio ns may he sim ilar to • Identical to epidermo id cyst
cho lesteato ma • Stratifi ed sq uamo us epit he lium, with pro gressive
o Hyperin tense o n 1'1 C- im ages ex foliatio n of keratinou s mat erial
o Co n te n ts rich in cholestero l crys ta ls
Glo mus tympa nicum paragan glioma • Chron ic infl ammat or y changes also usuall y pr esen t
• Otoscopy: Het rotympanic red , pu lsatil e mass
• Ima gi ng: Focal ma ss o n coc h lea r p rom onto ry witho ut Staging, Grading o r Class ificat io n Crite ria
oss icular o r hone erosio ns • Pars Ilacc ida ret ract io n pocket o r perfor atio n is earliest
stage & may be t reat ed effectively wit h tymp an o storn y
tu be
• Small c holestea to ma ma y be iso lated witho ut oss icular
erosion or loca l exte ns io n Image Interpretation Pea rls
• Large lesions stage d based o n local ex te n t, ma sto id • \ Vh e n ME & mastoid completely opacified. diffi cu lt to
involvemen t N. ossicu la r erosio n d iffer entiat e effus io n from c ho lestea toma
• Ossicu lar e rosio n su pports d iagn osis of cho lestea toma
in co m p lete ly o pacified ea r
IC LIN ICA L ISSUES • Detecti o n of sin us tym pani ex te ns io n of
2 Pre sent ation
cholestea to ma associated wit h h igh post-surgica l
recu rrence rat e
62 • Most commo n signs /sy m pto ms
o Fo ul-sm elli ng aur al d isch arge
o Co nd uct ive hearing 10 5 \ ISELECTED REFER ENCES
o Other sign s/ sym pto ms I. El-Mewl aty K et a l: En d osco pe affect s d ecisio n m akin g in
• Ve rt igo if la teral sem icirc u lar ca nal is dch tsced cholesteato ma ~ urg"ry, O to laryngoJ Head Neck Surg .
• Ota lgia may occur if cho lesteato ma is large. or 129 (51:490·6, 200.1
in tracra n ial exte ns ion 2. Shohct JA et at: The manageme nt o f pedia tric
• Clin ica l profil e: Patie n t with hi st ory o f c h ro n ic mi dd le ch o leste atoma . O to laryngoJ C1in North Am . 35( -1): 8-1 1·5 1,
ca r infl amma tory d isease & 'I'M ret raction O f 21Kl2
perfor ation
3. waus .s et al: 1\ systema tic ap proach to in terpretation of
com p u ted tomography sca ns p rior to mi dd le ca r
• Oto logic exa m ina tio n cholesteato ma. J Laryn go l Oto!. 1 1-I:2·a~·53 . 2lX)()
o Retraction poc ket , perfora tion or obv ious pearl y 4. Presco tt CA: Ch olesteato ma in ch ildr en- th e expe rien ce at
white tum or at pa rs f1a ccid a Till' Red eros.. War Mem o rial Child ren's Ho spita l in South
o PFC locat ed a t antc rosuperlor quadrant of T ~ I Afrlca 19&8· 1tNfl . lilt ] l'cdi at r Otorhl no laryngol.
49 l l) : 15·9,1999
Demograph ics 5. fino Y e t .11: Risk factor s for recurren t <in ti resid ual
• Age cholestea to ma in ch ildr en d vtcr mlncd by ~ en)IJ tI ~ t a gl'
o May occ u r in ch ild ren o r ad u lts opera t io n. ln t ] I'edin tr O to rhi n oln ryngo l. -I6 ( 1·2):5 7-65,
o Un us ual in ch ild ren < 4 yea rs of age 199M
h. Miyanaga S ci al: l' tu ssak's space: ch rou olog tcal
o Ch o lestea to ma in ch ild re n more aggressive
develo p m en t a nd rout es o f aerat io n , Auri s Nasus Laryn x.
• Extensive dis ease &. recur rence co m mo n 2-1( 3 1:2 55-ll-l, 1997
• Et h n icit y: Rare in Ame rica n Ind ian &. Alaskan Eskimo 7. Gyo K et ill: I{ l'~ l d u l' of mi ddle ear ch olesteato ma af ter
po pu lati ons. despite high occu rrence of o to masto id itts intact ca na l wall tym pa nopla..ty : surg ical findi ngs alone
yea r. An n 0101 Rhinol La ryn gol . 105(X):6 15-9, 1996
Natural History & Progn osis 8. Kobayavh l T et .11: Pat ho gen e..i.. of att ic retracti on IKX )..: N
• Progressive I in size of cholestea to ma, wit h and ch oles tea to ma as studivd by comp u ted tomography,
d estruction o f su rro u nd ing st ructu res, incl udi ng Am ] O to l. 15(5 1:65X-62, 1\)9-1
oss icular cha in, ot ic ca psu le & semici rcular canals, 9. Kikuchi S ct al : An analysis of hOJ1l' d estruction in
tegm en tym pan i & transver se sin us invas io n c h olestea to mas by hig h reso lution co m p u ted to mo grap hy,
Auriv NiI\U SLarynx . 20( I 1: 11·7, 199 3
• e N? in vo lvemen t, ve nous sin us th ro m bosis. &
10. Vartiai nen E vt ill: Long-ter m rc..u lts o f ..urg ical treat m ent
in tra cran ial exte ns ion a rc late co m plica t io ns in diffe ren t c h olestea to m a ty pe s. Am] O tol. 1-1:507·1 1,
• Sma ll c holesteatoma : Exce llen t for total e rad lca tlon & 1993
no rma l h ca riug I I. Schuri ng AG et al: Slilgin g for cholesteato ma in t he ch ild ,
• La rge cho lestea to ma : Residual co nd uct ive hea rm g loss adolesce n t, and ad u lt. An n O to l Rhin ol Laryn gol . 99( 4 fit
is po ssible 1):256. hO, 1990
12. You ngs R ct .11: The spatial o rgan isation o f kcra tlnocytcs in
Treatment acq ui red mid dl e ear ch olesteatom a rese mbles th at of
• Early trea t me n t o f retracti on pocket wit h ex ter nal au ditory cana l skill and pJ r ~ Iluccida. Acta
tympa n ostom y tube may prevent c ho lestea to ma Ot ola ryngol. I I O( 1-2 ): 115-9 , 1990
formati on 13. C h o le Rt\ et .11: Compamttve histology o f t ill' tym panic
mem bran e and its relat io nsh ip to cholestea toma. An n 0101
• Su rgica l gO.I) is co m plete e rad ica tio n o f cho lesteato ma
Rhinol l..a ryng ol. 9X( IO):76 1-ll, 19M9
N inf ecti on , out recurre nce rat e is 6-H% 1-1 . Michaels L: Biology o f ch olesteato m a. O tu laryngo l Ch n
• Hea ring preser va tion or restorat ion are seco nda ry go als North Am. 22 (5) :Xll9·St , 19M9
• Su rge ry includes mastoid ect om y &. fo rma tio n of a 15. Meyer h off W L et al: Cholesteato m a ..1;lgin g. La ryn go sco pe .
co m mon cavity be twee n mastoid antru m &. exte rn al 9 6(91't 1):9 :1 5·9 . 19X6
a ud itory ca nal + TM &. os sicle reconst ructi on 16. Suzuki ~f et al: l.u ng term follow-up of secr eto ry utiti..
media in children: th e effects of adcnotonsrllcct o my with
in ..crtion o f a venti lati on t ube . Au rb Na..us Larvnx . 12
IDIAGN OSTIC C HECKLIST 17,
Su pp l I:S2:l 7· 8, 198 5
Swar tz ] D: Cholestea tomas o f t he middle ear, Diagnovi s.
.
et iology, and compl lcatlo ns. Radi o! Cltn No rth AI1I .

Co nside r 22(1) : 15·35, 198 4
• C ho lestea to ma wh en NEeT sh ows ME o r masto id
mass &. o ssicu lar eros io n
• Assess for tegmen tympani &. la teral sem icircu la r can al
erosio n
IIM AG E GALLERY
Typical
(/.~Jt) Axial right cs r T-bonf!'
CT (pve.lls lateral tym panic
cavity PFe remodeling Icllf.·f al
.l~p('ct of mal/e(15 heJ d ~~
2
incus body. M.H .~ f>Xlends
lIuounh aditu s (ilrf mv), bur 63
f1ltls{oid cav ity is clear:
(Hig ht) COrDn.1! t-bono CT
(/('m om /ral es Pru55ak '~
~pcJ ("e .1\ atti c cho/t'~ leafOnJ.l.
The walls of tympanic c.w ity
appf.>ar scalloped. but the
tegmen tympani remains
int s ct (.1rrrn v).
Typical
(loR/ I) A xial T-Ixme CT show)
complete opacification of
right tymp anic c.w i'y f..
ma ~t()id comph.'x . Ero sion of
entire ossicular civsin lid S
occurred with .1.\ ~ ()d.l ll..'(J
d e!liKC'/l("(' or
1,lIpr. l l
w m icircu lar Ci.lfM I (arr o w) .
(Righi) Coro nel I-bon o
({'Com/fUrl io n shows
m.rrJ..t'd lhinnin/: of the
legmen tym p.mi (d m J1,\,)
with loss of the l M icular
cha in. Note chotestes toms
dehisces u x )f uf fJciJI nerve
postC'rior genu topen ar(()w ).
Variant
Coron.ll grap hic shows
( I .../ I )
l.u1:<' pars flaccida
cho fe~ r ea (of1 ld.
Complications inclur/c'
('ro.~i()11 of osstctos. ('rmiorJ of
la/('r,,1semicircular cdrlal
(." ro w) So thinning of h ogIHf'rJ
rymp.lni (open .U fOW ) .
(Rig" ' ) CoronJ I reveetv 1.1II:t·
par s f!,Jcdda choles'c'.ltomJ.
with hlun ting of scultJm .\
erosi on 0; malleus l'to incus .
/'Fe c/ehiscl's lateral
w mkirculer canal (.l rr m v) .'.:
foca lly thins tegm en ' ympJni
(open arrow).
ACQUIRED CHOLESTEATOMA, PARS TENSA
2
64
Coroml le(t ear T-bone C1" shows small inferior Axial left Cdr T-bone a shows middlf.' car PI C, nu.>dia!
tymp,m ic GlViry pars lefl5<l cho/estl'Jtoma, nK"<.liaf to to os_~ icles . No (e subtle scalloping of mff!i.ll body of
TM perforation no t Sf>f>Tl on C1-
(etr<1ctp() 7M ,,\- ossid es. incu <; (.1,,0\ \,). Latt·r.l/ semicircu!ar cenat wall is imea.
hut was visihle during otoscopy:
o La rge PTC may reach 2-3 em , filling middle ear

!TE RM INO l O GY ca vity
Abb reviat io ns a nd Syno nyms • Mor p ho logy
• Par s ten sa cholestea to ma (P'I'C ) o Lobu la r, well-circumscribed ma ss
• "Sin us" cho lesteato ma, du e to involvem en t of sin us o No ne n ha n cing tympanic cavity mass, wit h ossicu lar
ty m pa ni o r bo n e e ros ion
• Seco n da ry acq u ired typ e; "ke ra to ma" CT Findings
Definitions • NEcr
• lyre : Foca l accu mu lat io n of exfo liated keratin within o Sma ll P'I'C
stra t ified sq ua mo us epit he liu m at site o f perforatio n or • Early rrc shows soft tissue m ass th at involves
retra cti on pocket in in ferior par s tensa po rt ion of sin us tympa n i & facia l n erve recess of post eri or
ty mpan ic me mbr an e (TM) mesot yrn pan u m
• Mass projects MEDIAL to os sicula r ch ain wit h
sub t le lateral d ispl aceme nt of oss icles
IIM AGING FINDING S • Ea rly oss icle erosion pr esen t
o Large PTC
General Features • f ills m idd le ea r cav ity
• Best di agn o stic clu e: Eros ive mass in poster ior • In vad es masto id th rou gh wid en ed ad itu s ad
tympanu m th at may involve sin us tym pan i, facial a n t ru m
recess, ad it us ad a n tru m and /or ma stoid • Destroys ossicl es fro m medi al to lateral
• Location • Ossicu lar eros ion co m mon, especially alon g
o Middle car (tympan ic) cav ity med ial in cu s long process, sta pes supe rst ruc ture &
o Po sterior cavi ty, o fte n medi al to ossicles man ub rium of ma lleu s
o Sp reads poste romed iaily to a posit ion medial to • Poster ior tegme n tympa ni & an te rior tegmen
o ssiclcs ± th rou gh ad itus ad an t rum in to mastoid mast o idcum ma y be deh iscent
• Size • CECr: Midd le ca r mass does n ot e n h a nce
o Variab le, fro m severa l m illimet ers to large
DDx: Middl e Ear Mass
Congo Cho lestea toma PF Cholesteatom a Cholesterol Granuloma Glomus Tympanicum

Key Facts
Terminology Top Differential Diagnose s
• "Sin us" cho lestea to ma, du e to in vo lve me n t of sin us • Co nge nita l cho lesteato ma of m idd le ea r
tym pani • Pars f1 accida (PF) acq ui red cholestea to ma
• Cho lestero l granuloma
Imaging Findings
• Early ('TC sho ws soft tissue mass th at in volves sin us
• Glom us tympanicum paragan gli om a
2
tympan i &: facial nerve recess of posterio r Pathology
mesotym panum • 1O-2Cr ll> of all middle ear cho lestea to mas are l' fC 65
• Ossicu lar eros ion co m mo n, espec ially along medi al • Signi ficantly less co m mon th an par s flacci da
incus long process, sta pes su perst ructu re & cho lestea to ma
manubrium of ma lleu s
• T l C+ MR used to answer specific issues raised by Diagn ostic Ch e cklist
ho neCf • Coronal cr necessary to assess lat eral semici rcu lar
• Cepha loce le, midd le crani al fo ssa infection o r ca na l integrit y &. intact tegmen
int racranial cho lestea to ma bett er deli neat ed by MH • If m iddle ea r co mplete ly o paci fied, bony wa ll o r
ossicular eros ion helps di fferentiate eff usio n from
cholestea to m a
MR Find ings IPATHOLOGY

• T IWI: Mid d le ear mass , isoin tense to CSF
• T2W I: lfigl] slgnal l' fC less than signal of trapped
Ge ne ral Featu res
secretions of mastoid • Ge ne ra l pat h comme nt s
o Co llectio n of kera ti nize d stra tifie d sq ua mo us
• T1 C.
o PTC itself does not en hance epit heliu m called cho lestea toma hy pa tho logist
• Gra n ulatio n tissue & o the r sca r ma y e n ha nce wh ereve r fo un d
• If tegmen erosio n p resen t, coronal '1' 1 C+ shows o Histol ogy is sam e, whe ther cho lestea to ma is
du ral en hance me n t at bony defect acq uired pa rs f1 accida or par s ten sa typ e, co nge n ita l
m idd le ear, pet rou s apex o r cc rchcllopo n tlne angle
Imaging Recommendations cho lesteatoma
• Axial &. co ro nal thin-section f -bo ne c r is stud y o f o Embryo logy-ana to my
choice • Pars tcn sa portion of tympani c m embrane: "Ten se"
• 1'1 C+ MH used to a n swer specific issues raised by bo ne lower 2/3 of TM
cr • Eti ology
o Ce pha locele. m iddl e cra ni al fossa inf ecti on or o Pa rs ten sa TM perforation ± retracti o n crea tes par s
int racran ia l cholestea to ma bett er delineated by MR tensa cholesteato m a
o Triggers pos teri or midd le ear deposit of st ratified
sq ua mous epithelium
ID IFFE RENTIA L DIAGNOSIS o Resul ting m ass en larges, erod ing osstcles & tym panic
cavity walls
Co ngenita l cho les teato ma of middle ea r • Enzy ma tic bo ne resorption mad e more severe by
• Otoscopy: \Vhite mass behind intact Thf assoc iat ed infecti on
• Often loca ted po steri orl y like par s tensa cholestea to ma • Resu lts in furt he r bon y dest ructio n
• T I C+ MR: Rim-enhancing middle ea r ma ss • Ep ide mio logy
o 10·20% of all m iddle ca r cholestea tomas a re JYfC
Pars flaccida (PF) ac q uire d cho les tea to ma o Significa nt ly less co m mon tha n par s flacc ida
• Otoscopy: Pa rs flaccid a per forat ion o r retracti on cholesteato ma
poc ket • Associated ab no rma lities : Pars ten sa c ho lestea to ma
• Pru ssak space mass with scut um erosion; oss lcles co m mo n co m plication o f cle ft palate
pu sh ed mediall y
• T I C+ MR: Rim-enhan cin g m idd le ea r mass Gros s Pathologic & Surgica l Fe atures
• "Pea rly tum or " see n at su rgery
Cho leste ro l gran ulo ma • \Vell-circumscrihed , so ft, wa xy, whi te m ater ial
• Oto scopy: Blue m ass heh in d intact TM
• Ossicu lar & hon y erosions m ay mim ic cholesteato m a Microscopi c Features
• T I C- MR: Hype rin ten se ma ss in m idd le ea r • Stratifi ed sq ua mous e pit he liu m wit h progressive
exfo liation of ke rati n ized m ate rial rich in cho lestero l
Glomus tympanicum paraganglioma crystals
• Oto sco py: Che rry red pul satile mass behind inta ct TM • Squam ou s e pit heliu m may result in cyst-like co llect io n
• Mass o n coc h lea r p rom onto ry wit ho ut ossicu lar o r • May he associated ch ron ic ln fcct lo n
bon y e rosio ns
• T l C+ M it In te nse e n ha nceme n t of ma ss
• If middle ear com pletely o pacified , bon y wall or
Stag ing, Gra ding or Classificat ion Criteri a ossic ular erosion helps differentiat e effusio n from
• Retraction pocket or TM atelectasis &: perforation are cholesteatoma
initial even ts
• Forma tio n of ch olesteatoma then occu rs
Will iam s Mf ct a l: Detecti on of postop erative residua l
2 IC LIN ICA L ISSUES 1.
choleste atoma with delayed co n trast-en han ced MR
imaging : init ial find ings. Eur Radiol . 13 (1):169 -74,2003
6& Presentati on 2. Aikele P et al : Diffusion-weigh ted MR imaging of
• Most co mmo n signs /sympto ms cholesteato ma in pedia tr lc and ad ult pati ent s who ha ve
o Histo ry of o titis med ia o r ch ro n ic middle ear undergone midd le ea r surgery, AJR Am ) Roen tgc nol.
in fecti on is typ ical 18 1(1 ):261 -5 , 20m
o Middle ea r effusio ns wit h 'I'M ret ractio n 3. Goc me n H et al: Surgical trea tment o f ch olestea toma in
o Co nductive hearing loss (Cll l.) ch ildre n , lilt) Ped la tr Ot orh inolaryn go l. 67(8 ):86 7-72,
o Oth er signs/sym ptoms 20 m
4_ Min or LB: Lab yrint hine fistu lae: pa th obio logy an d
• Foul-smelling ch roni c, intermitten t oto rrhea
man agemen t. Curr O pin O to laryngo l Head Nec k Surg.
• Clin ical profile: Cll L in pati ent with recurren t m idd le II (5):340-6 , 2003
ear infection 5. Shoh et j A et ,11: The managemen t o f pediat ric
• Other presen tin g sym pto ms &: com plications cholestea to ma. Otolaryn gol Clin No rth Am. :-1 5(-1 ):8-1 1-5 1,
o Vertigo (lateral sem icircular cana l de h iscen ce) 2002
o Facial nerve pa lsy 6. Yates PO ct al: L' sca n ning o f m idd le ea r cho lestea to ma :
• Otoscopi c physical exam what does th e surgeon wa nt to kn ow'! Br J Radiol.
o Retractio n po cket, per fur at ion o r visible 75(89 81:8 47-52 , 2002
7. Waddell A et al: Ch olesteatoma causing facial nerve
cho lesteatoma at pars ten sa
t ra nsecti on .) I.a ryngol Ot ol. 115(3 ):2 14· 5, 200 I
o Exte nt of lesion incom plete ly assessed with oto scope 8. Soldat l D et <11: Know ledg e ab out ch oles teatoma , fro m tile
• Axial T..bone CT best shows enti re lesion first description to the mod ern h istopathology. Oto l
Nc u ro tol . 22( 6):723-30, z oo t
Demographi cs 9. Watt s S et al: A syste ma tic appr oach to in terpretati o n of
• Age: Occurs in ch ildren &: ad ults co m puted to mograp hy sca ns pr ior to surgery of m idd le ear
• Gender: Iv! = f c holesteato ma. ) Laryngol O tol . 1 1-I ( -I ) : 2-1 X - .s :~ , 2000
• Ethn icit y 10. Sadc ] : Surgical pla n nin g of th e treat men t of choles tea toma
o No eth nic predi spo sitio n an d posto pera tive foll ow-up . An n Otol Rhino l Laryng o l.
o Has been repor ted as rare lesion in Eskimo, 109 (4):3 72-6 , 20 (JO
American Ind ian & Australian ch ildre n , despit e 11. Watt s S et al: A system a tic ap proach 10 in te rp retati on o f
com puted to mography sca ns prio r to midd le car
mid dl e ear in fect ion s cholcsteatoma.] Laryu gol Oto l 11-1 :2-18-:) 3, 2000
Nat ural Histor y & Progn osis 12. Stangcr up SE et a l: Cholestea toma in ch ild ren, pred ictor s
a nd ca lculatio n of recur ren ce rates. lilt ) l'edia t r
• Progressive en largemen t wit h growi ng symptom Otor hino laryngo l. -It) Supp l I:S69-7:i, 1999
com plex du e to local extension 13. Fagce h l':A ct al: Surgica l t reat men t o f ch o lestea tom a in
• Post-surgical recurr en ce rate - 10<*1 chil d ren. ) Oto laryngo l. 2X(6)::m 9· 12, 1999
• Sma ll lesio n : Exce lle n t for tota l eradica tion &: no rmal 14. Alexa nder AE) r et a l: Clin ica l an d su rgical a pplication of
hea ring reformat ted liigh-rcso lution CT of th e tem pora l bon e.
• Large lesion: Residual conductive hearing loss Ncuroima gi ng Clin N Am . X C~ ) :631 · 50 , 1998
com mon I S. varuo tncn E ct a l: Long-ter m result s of su rgic a l treat men t
in d ifferent ch ole stea toma types. Am ) OtoI 14:507 - 11,
Treatm en t 1993
• Surgical remova l of cholestea to ma req uires t 6. Swartz ) D: Chole stea tomas of the mid dle ca r: Diagnos is,
etio logy a nd compltcat km s. Radi o ! Clin Nor th Am
mastoidectom y with formation of com mon cavity 22 :15-:15, 19 84
betwee n mastoid an trum &. external auditory can al
• 'I'M & oss icular reco nst ruction req ui red for hearin g
resto ration if ossicular chain invo lved
I DIAGNOSTIC CHECKLIST
Co nside r
• PTC if middle ear mass is ce ntere d posteriorly, extends
medial to ossiclcs & displaces ossicies lat erally
• If medi al surface of ossicles shows signs of eros ion
first, con sider PTC
• Coro nal C I' necessary to assess lateral semicircular
ca nal integ rity &: int act tegm en
IIM AGE GALLERY
Typical
(1.(1' ) Axial/pit mf ' -born'
C1 reveals small m i(/llle 1:".1'
P tc, nwdid/ro w!>ide!>,
ab utting eroded incus Ixxi y
2
(armw ), P.atit>nt p resented
with con (/uc ti Vl' hearing loss. 67
diler chronic l'Jr infC"Ctions .
(Highl' Coron.ll /("'t es r
T-ho ne C T dt'monWiJ lt;'}
p sr s {('n sa cho/l·~tt'.l rom.l
surrounding tht' ossicies.
No te K utum w nJains intac t
(arrow) whi ch w ould not be
the C.H e if this were pdrs
/( '05el cholesteato ma.
Typical
(/ £/ 0 Coronal t-oone CT
!> holVs a large pars tldccida
porioretion (arro w) It._'ading
to hollolVed out pars tellsa
c!Jult'.\ ((',rlo m.l . Th l! PTe
.l/mh /ht· nwdi.ll w,lll o f tbo
micJdlt' Pclf (",w ily topt' n
,m ow) . (High" Coronal
l -Jx >Il1' ( 1 5110 1\'5 mJ.lII
middle l 'df m.l'>!>. mt'<1iJI to
os sicles. /x'n eath f.ldal
norve, filling oval wine/ow
nichf' (.m ow). Unlike pars
Odcdc/.I cho/l'stl·dlomJ.
K utum & PrUSSJk space are
nonrul.
l-boo o C T shows
(/,.('/t) A'(iJI
1,lrl:W posterior rymp.mic
(',1Vit y PTe. Incu s body &
sbon process ,l r(' dC'5rro}l:>d,
maf/po/J' 1weld di~p/dced
.lntt'm l,u{>'J lly Idrrol'l). No te
chronic mastoid
int7ammatory changes.
(Righ' ) All;JI left ear I-bone
CT revcets a large PTC filling
middle ('Jr c.lvity & mastoid
air cells. Oss;culJr chain has
been destroYf.>d. No te fistula
of latt'ral semicircular canal
(., ((m v) .
ACQUIRED CHOLESTEATOMA, MURAL
2
68
CC)f()(),J/ T-IJ()f)P CT shaovs large m.lsloid cavity with A'(i.ll T-bone CT rovesl... mural cholesteatoma as ~UiJI
ff"lkJU.ll mUT.II cho/esteclton1cl rind (,m e)',v) in pafient rinds of ti,,5UC-' in d hoIlout!(j out tl1iJstokloo..\" (d frO'oVS).
wi th no hMOfy of rn.J5toid 5urgt'fy. Open JITOW: wlera/ The bion h..1S btoken into the pos terior bony {''(f{.,nal
!>efnicirruf.lf canal ckhkence. auditory canal.
• Scutu m seve rely trun cated

ITE RM INO l O GY • Labyrin thine fistula o fte n p resen t: lateral
Abb revia tio ns a nd Syno nyms sem icircu lar canal mo st co mm on
• Auto rnast oidcctorn y, at ypical ch o lestea to ma sh ell • Large lesion can fistulize any area of inn er ea r
Definition s MR Findings
• Resid ua l cholestea to ma rin d left behind after mi dd le • TlWI
ear-masto id acq uir ed cho lesteato ma ext rudes ce n tral o Mastoid cav ity a ppea rs ide n tica l to su rgical de fect
mat rix either th rough tympan ic memb ran e • May be com plicated by cepha locele
perfo ration or EAC bo n y wa ll • T1 C+
o Peripheral enhancement with in cavity if granulation
present
IIM AGING FINDI NGS • More if co m plicated by meningitis or brain
abscess
Ge ne ral Features • More easily seen th an with cr
• Best diagnos tic clu e: "Masto id ecto my" cavity with o If inner car fistu la: In ne r ea r ma y enhance
resid ual soft tissue along cavity wall wit hou t histo ry of o If tegmen tympan i d ehi scen ce: Dural enhancem ent
mas toidect o my ma y be present
• l ocation: Middl e ca r & masto id Imaging Reco mm e ndation s
• Size: Cholesteato ma rin d o f va riab le t h ickness
• T-bo ne CT in axia l &. coro na l planes
CT Find ings • T1 C+ MR reser ved for co m plicated cases
• Nl.C]'
o 'l -bon c cr
• "Ho llo wed out " m iddl e ea r-mastoid with residual IDIFFERENTIAL DIAGNOSIS
cho lestea toma rind seen alo ng walls of cavity
• Variably sized ma sto id cavity
Coa lescent masto iditis
• Common cavity co nn ects m idd le ea r &. antrum • Middle car cavity is no t en larged
• Ossiclcs usually destroyed
DDx: Mastoid Defects
Confluent Ma.Hoiditis Simple Mastoide ctom y Canal Wall Up Modiiied Radical

ACQUIRED CHOLESTEATOMA, MURAL
Key Facts
Terminology Path ology
• Residua l cho lestea to ma rin d left beh ind after m idd le • In te rn al cholesteatoma mat rix dr a in s t hrou gh TM
ea r-masto id acqu ired cho lesteatom a ex t rudes cen tra l perfor ati on o r directl y into EAC
m atri x eit her th rough tym panic membrane • Erosive membrane persists afte r d rai nage
perfo rat io n or EAC hony wa ll • Co nti n ue d growth of cavity d ue to enzym at ic act ivity 2
Top Differential Diagnoses Clinica l Issu es
• Coa lesce n t ma stoiditis • Clin ica l profil e: Long h istory o f CO M wit h out su rge ry 69
• Masto idecto my
• Ma stoid a ir cells become co n flue n t in sett ing o f acute • Ge nde r: M = I:

o tit is med ia
• Middle ear &. ma sto id co m p lete ly opaci fied Natural History & Progn osis
• Restor at ion o f hearin g diffi cu lt becau se of co m plete
Mastoide ct om y oss icle loss &: bone eros ion
• Posterolat eral wall o f mastoid is a bse n t
• Surg ica l hi stor y is kn own Treatm en t
• Su rgery dep ends o n lesion size and ex tent
o Excis ion o f tissue lining cavity imperat ive
IPATHOLOGY o Ossic ula r reconstructi on
Ge nera l Features
• Gene ra l path co m me n t s: O n ly "lin in g" o f IDIAGNOSTIC CH ECKLIST
ch olesteato ma is ava ilab le fo r eva lua tion
• Etiology
Co ns ider
o Acqui red cho lesteatom a fo rm s in midd le • Imaging findings rem in isce n t o f m astoidect omy
ea r-m astoi d wit hout hi stor y o f su rgery = "auto mas to tdcctomy"
o Internal cholesteato m a matrix drain s th rou gh 'I'M • Pati ent may have had act ua l m astoidectomy and is
perf ora tion o r directl y into EAC poor hi stori an
o Eros ive membrane persists aft e r d rain age Image Interpretati on Pearls
o Co nt tnued gro wt h o f cav ity d ue to enzy ma tic
• Pat ient h istor y of ma stoidectom y crucia l to dia gn osis
activity
• Epide m iology
o Mura l cholesteatom a is rare var iant form o f acq uired
cho lesteato m a
o Pars flaccid a > > par s ten sa > mura l acq utrcd 1. Swa rtz jD et al: Imaging of 111(' Te mporal HOll e , Thieme,
cho lesteato m a 11lC., Chapter 3, 1998
2. Aberg B ct a l: C lin ical cha racteristics o f cholesteatoma. Am
Gross Pathologic & Surg ica l Features J Oto laryn gol 12 :254- 8, ] 991
:I. Nard i PF er al: Un usual ch o lestea to m a shell: CT fin dings.
• Hind of tissue found along wall of cav ity
JCAT 12 :1084·8 7,1 988
• Aggressive keratini zin g st rat ified sq uam ous epithelium
IIM AG E GALLERY
ICLIN ICA L ISSUES
Presentati on
• Most co m mo n sign s/ sym pto ms
o Long h istor y of ch ro n ic oti tis
o Other sign s/sy m pto m s
• Draining ea r
• Con ductive h earing loss
• Intermi tt en t vertigo if fistula presen t
• Clin ical profile: l.ong h istory o f CO M wit hou t su rgery
• Past med ical hi stor y
o May rep ort mat eria l "falling out o f ea r"
o No hi story of m asto idecto my (LRftJ Axial T·lx m e CT shows a thin-wetted rind of mural
cholesteatoma (arrows) in I/)(' mas/Did cavity in this otherwise poorly
Demographics poe umstired mastoid. O, \ic1('s ti sve been deslro)'ed. (Rig ht) Axial
• Age: Usua lly in o lde r pati ent reveal" a thick -walled mu ral d JO/('stfl.llo ma (arrow) along l!Ie back
w all 0; tilt> f' ro(/pel m astoid c.1Vil y. Thf-' sigmoid pl ate' ha s !Jet' 1J
Ihirlfl ed and partially c!l ,hlsfWI (01'(' /1 ,m ow ). o.\'!jidl'S destroyed.
CHOLESTEROL GRANULOMA, MIDDLE EAR
2
70
Coronal graphic depicts large middle ear cholesterol !lxi,11T 1WI MR shows high !)ignal cholesterol granuloma
granuloma. entire middle ear fillt-''C/ wilh dark brown (arro w) filling middle ear, expanding tympanic
(" cm X"()/ate-) fluid wilh 05.~id('.\ l;olle . Ot oscopy shows membr.11J(' lawrally. J Ugh signal is from bkxxl products
" h/ue f:'ardrum~ . in cholesterol granuloma.
ITE RM INO l O GY CT Find ings

Abb rev iat ions a nd Syno nyms • NEcr
o Early CG-ME bo ne CT find in gs
• Ch olestero l gra n u lo ma, m idd le ea r (CG-ME) • Sma ll midd le ea r mass
• Cholestero l cyst, chocolate cyst, chocolate car, • No ossicu lar loss or ho ne remodeling
blu e-do me cyst • Difficul t to make spec ific diag nos is
Definit ions o Lat e CG-ME bo ne c r findings
• CG-l\1E: Recu rrent hemorrhage in to m idd le ea r cavity • Opacified mi ddl e ear &: masto id
ca uses In flam mator y m ass o f gran ulation tissue • Expa ns ile bo n y changes wit h scallo pin g of
surro u n d ing bo ne
• Ossic u lar d isplacement ± dest ruction
IIM AGING FINDING S • CECT: May be usefu l to d istingu ish small CG-M E from
glo mus tympani cum paragan glioma, whi ch en h a nces
Ge neral Features br iskly
• Best diagn o stic clue MR Findings
o Bone Cf: Smoot h ly cx pa nsile mass of midd le ea r ±
• T 1WI: t Signa l fro m param agn eti c effect o f
mastoid cells
methem oglobin
o MR: HIGH T1 & '1'2 sig nal in midd le ea r
• T2WI
• Locati on o Cen t ral I signal fro m gra n u lation tissu e
o CG occ urs thro ugh ou t body, includin g pet rou s a pex
o Peripheral I signa l from hem o sider in deposition
& or bit
• STIR: Follow s '1'2 signal
o Mo re com mon ly arises in mi ddl e ear
• Size: Dep ends o n chro nic ity ; m illim eters to • Tl C+
o Inherent h igh T l signal may be co n fused with
cen ti me ter s
cn ha nce rnen t
• Morphology: Expa ns ile natu re critical to diagn osis,
• Co m pare to un enhan ced T1WI
di st ingu ish es CG-ME from hem orrhagic o titis med ia
wi th out gran u latio n tissu e • MRA
DD x: Vascular Retrotympan ic Mass
Dehiscem lug. Bulb Aberranl lOl Glom us Tympanicum Clomus II/gulare

Key Facts
Termin ology Top Differential Diagn oses
• CG-ME: Recurrent hem orrhage into middl e ea r cavity • Deh iscen t jugula r bu lb
causes in flam matory mass of gran u latio n ti ssue • Aberrant int ern al carotid a rtery
• Ch ron ic otitis med ia with hem o rrhage
Imaging Findings • Paraganglio ma
• Bone CT: Smoo th ly expansile ma ss of mid dle ear ± • Encep h alocele 2
masto id cells • Hem ot ym panu m
• MR: HIGH Tl & T2 signal in m iddle ear 71
• Mo rph o logy: Expan silc na ture cr itical to dia gn osis, Clinical Issu es
distingu ishes C G~ M E from hem orrhagic otitis med ia • Slow ly progressive co n duct ive h ear ing loss
without gran ulatio n tissue • Otos cop y: No n-pulsa ting bluis h di sco lora t ion of
• CT and MR are co m plime n ta ry; both may be neede d tympan ic mem brane = "blue eard ru m"
if lesion la rge
• Exp ansion on bo ne CT, alo ng wit h h igh signa l o n Diagn ostic Chec klist
both T l and T2, establishes d iagn osis • CG-ME m uch m ore co m m on tha n CG· PA
• Do not mi st ake hi gh T1 signal for enhan ce ment;
co mpare wit h un enhan ced T1
o May be useful to d ist inguish CG-ME from vascula r • Like CG-ME, associa ted wit h recurren t pr ior in fect ion s
anom alies (e.g., aberra n t in ternal carotid a rtery) ± effusio ns
• cr is preferred to elim in a te vascular • Microscopic: Cho lesteatom a lined by sq uam ous
cons iderat ion s epithe lium; CG-ME lin ed with fibrous co n necti ve
tissue
Imaging Re commen d ations
• CT an d MR are com plime n ta ry; hoth may be n eed ed if Paragan glioma
lesion large • Oto sco py: Red mass in midd le ea r
• Expan sion o n bon e CT, alo ng wit h hi gh signa l on • Bone c r
bot h T1 a nd T2, establish es di agn osis o Glom u s tympa n icum: Co n fin ed to coc h lea r
promon tor y
o Jugu lare: Perm eati ve bone cha nges fro m jugular
IDIFFERENTIAL DIAGNOSIS foram en up into m idd le ear cavity
Deh iscen t jugul ar bulb En cephalo cele
• Oto scopy: Blue ma ss in m iddl e ear • Surgical view: Ca n m imi c CG-ME stron gly
• Bon e C I': Absen ce o f thin bon e bet ween jugu lar bu lb • Bone CT: Show s dehi scen t tegmen tym pan i with b rain
& hypo tympanu m herniati on in to ME or m astoid cavit y
o Divert iculu m of jugu la r vein exten ds in to m idd le ca r • MR: Coron al T2 ma y defin e co n te n ts
• Th in -sect ion c r n eed ed fo r d iagn osis; both axial & • Usua lly post-trauma tic o r posr-su rgtca l
corona l plan es useful
Hem o tympanum
Abe rrant int ernal ca ro tid arter y • Oto scopy: Blood in m idd le car fro m recen t trauma
• Oto sco py: Red mass in midd le ea r • Bo ne Cf: Associa ted T-bo ne fractur es
• Bone CT: Tubula r mass crosse s middle ear cavity to • MR: High T l m et hem oglob in do es n ot fill o r ex pan d
rejoin ho rizon tal pet rou s l eA middl e ea r (not obstruc ted, like CG-ME)
o Large infe rior tympa ni c cana licu lus
• En larged co llate ra l vessel traverses m iddl e ea r when
in tern al ca rotid ar tery (ICA) rails to deve lop IPATHOLOGY
Chro nic o titis medi a with hemorrhage Ge ne ral Fe atures
• Oto sco py: In flam matory tissue & b lood in midd le ea r • Gen era l pa th co m m en ts: CG-ME Ist report ed by
± rupt ured tym pa ni c mem b rane Manasse in 1894
• Bone Cf: Infl am m atory tissue & b lood fill middle ear • Etiology
wit h ou t expans tle bony ch ange s o Ob st ructi o n-vacuu m h yp othesis
• MR: Variable T1 & T2 sign al • Ch ron ic o tit is medi a, cholesteatom a o r previous
surge ry obst ructs air cells of ME ± m astoid ai r cells
Acquired cho lesteato ma • Resorptio n o f gas in obstruc ted ai r cells creates
• Ot oscop y: Tympanic m em bran e ret ractio n- rupt ure ± relat ive vac uu m
visible cholesteato ma • Decrease in p ressu re ~ mucosal engorge me n t ~
• Bon e c r: Erosive m idd le car-ma stoid ma ss wit h ossicle blood vessel rup ture
loss
• MR: Low T l & hi gh T2; rim en han ces o n Tl C+
sequence
• Anaerobic red blood cell d egradation to o Middle-age d patient with "b lue eard ru m " &
choleste rol crys tals incites multinucleat ed foreign co nd uctive h eari n g loss
gian t cell response > infl ammation wit h sma ll o Easily co nf used clin ically wit h vasc ular
vessel proliferati on => vessel rupture malformation o r vascular tum or
• Gra n u latio n tissue forms from rep eated o Histo ry of recurr ent middl e ear infections helpfu l for
hemo rrhage, expand ing m idd le ear ± masto id d iagn osis
o Expose d marrow h ypothesis
2 • In yo ung adu lth ood , en larging mu cosa crea tes Demo graphi cs
• Age: Middl e age H th -7t h decad es)
hon y defect s into hematopoietic marr ow of
72 T..bone Natural History & Progn osis
• Recurrent mi crohem orr hage => accumulation o f
• Great va riabi lity in gro wth rat e o f CG-ME
red cell degradat ion produ cts o Depe nd s o n frequen cy & seve rity of
• Anaerobic red blood cell degrad ation to mi cro-h em orrhages wit h in lesion
cho lestero l crysta ls inci tes mu ltinucleat ed fo reign
• Most CG-ME grow ove r decad es
gian t cell response
o Sym pto ms a rise year s afte r ini tial ep isodes o f otitis
• Obstruc t ion seco nda ry to infl ammati on, rathe r media
th an obst ructio n as primary cause
• Recurren ce rat es for CG-ME mu ch low er th an for
• Ep ide mio logy: CG-ME sign ifica n tly mo re com mo n CG-PA
than CG of pctrou s apex (CG-PA)
o Easier surg ical ex pos ure
• Associat ed abno rma lities
o Recu rrent ot it is m ed ia o r ef fusion Treatm ent
o Ch o lesteato ma • Ini tial surge ry: Resectio n of wall k con te n ts
o Be ni gn gra n u lat io n tissue • Intractab le d isease: Masto idect om y with ven tilation
Gross Path ologic & Surgica l Features tube
• Po st-op e rative MR useful to d ocument reso luti o n o f
• Cystic mass with fib rou s ca psu le, filled wit h browni sh
high T1 signal
liquid co n ta in ing o ld blood N cho lestero l crysta ls
• Fluid described as "cran kcase oil " or "chocolate cyst"
Microscopi c Featu res IDIAGNOSTIC CHECKLIST
• Lined hy fibrou s co n nective tissue Co nside r
• Red blood cells
• Multinucleat ed gian t cells su rrou nd ing cholesterol • Possrblc extensio n into eustac h ian tu be ± ma stoid air
crys ta ls e m bedd ed in co n nective tissue ce lls
• l Icm osldcrin-lad en macrophages, c hro nic • Mat ch oto sco pic impression of "blue tympani c
inflammat or y cells & blood vessels membran e" wit h Cf-MH findings
Stag ing, Gradin g o r Classification Crite ria Image Interpretati on Pearls

• Diffe rences between CG-PA & ee,-ME • CG-ME mu ch more co m mo n th a n CG-PA
o CG-PA presents with pain; CG-ME presents with • Do not mistake high T l sign al for en ha nce me n t:
h ear in g loss compare wit h uncn ha nccd '1'1
o CG-PA associat ed with neuro pat hies of e NS, 6, 7;
CG-ME h as n o cra n ia l neu rop athi es
o CG-PA h as no inf ect ion hi story; CG-ME has ISELECTED REFERENCES
recurrent infecti ons h isto ry I. Maeta M ct al: Surg ical in terven tion in middle-ear
o Large CG-PA ma y ha ve exte n sive hone erosion; choles ter o l gran ulo ma. J Laryngo l Otol. 11 7 ( S ) : :~44· 8 , zor)]
CG-ME has hon e erosio n late 2. j nckler RK tot al: A nev..' th eor y to explain t he genesis o f
pc tro us apex cho leste ro l gran ulo ma . Ot o l Ncu ro tol .
o CG-PA occ u rs in highl y pn eumat ized T-bone;
24( 1):96- 106; d iscussio n 106, 200 3
CG-ME occ urs in poorl y pn eumati zed 'l-bo ne (resu lt 3. Kos ling S ct al: c r an d ~I R imag ing a fte r m iddl e ea r
of prior infections) surge ry. Eur J Rad io l. 40(2):I 13-H, 200 1
4. Ca m pos A ct al: Cho leste ro l granu loma of t he m idd le ear:
Report o f 5 cases. Acta O tor h inolaryngo l Belg. 50: 125-9,
ICLINICAL ISSUES 1996
5. Mart in N et al: Cho lestero l granuloma of the m idd le car
Presentati on cavitie s: MR imagi ng . Rad io logy. 172: 52 1-5, 1989
• Most com mo n signs/sym pto ms 6. Pa iva T et al: Large cho leste ro l gra nu loma cysts in t he
o Slow ly progressive con d uctive hearing loss ma sto id . Arch Otola ryngol. 1 11:786·9 1, 1985
o Ot he r signs/sym pto ms
• Pulsati le tin nitus
• "Pressu re o n th e ea r"
• Ot oscop y: No n -pulsating b lu ish dis colorati on of
t ympanic membrane = "blue ear d ru m"
• C lin ical profile
IIM AG E GALLERY
Typical
(I.('!I) Axial bone CT shows
opa cification of m iddle ear
by cho/esterol granuloma 2
which bulges tympanic
membrane lat erally (arrow) .
Ma:.roid opacification d ue to 73
obstruction of aditus ad
an /rum. (Righi' Coro nal
hone CT re veets
opacification of middle ear
lJy cholesterol granuloma
with associated outward
bulging of tvmosnic
membrane (a,rmv) . Long
procl-·!> .~ of incus shows
sub tto ero sion (open arrow).
Va riant
(u f l) Axial T IWI MR shows
high signal cholesterol
granufoma extending from
m id dle esr (arrow) into
extemal car (op ('o arrow)
and bon y e w,t.1c hian tuhe
(c ur ved arrow). (Rigltt ) Ax;.11
T2WJ M R shows exp<1nsile
higll .~ jg n a l chofeSf{'rol
w anu/oma primarily IOGHed
in thC" ma stoid air <.: d /5
(arrowsJ. Usu.llly c hole.~ t('rol
grdnuloma extt'nds irom
m iddle ear into mastoid area.
Va ria nt
(/£jl) A xial T1W I MR shows
T-ho ne wi th multiple lesiom .
Medial low signal lesion
(arrmv) (('(u(' s{-nts
cho!es{('<)foma while l.lwral
h igh signet lesion (o pen
arrow) is cholestf'rol
granuloma. (Righ' ) Axia l
h igh -resolution T2WI M R
sho ws h igh signal throughout
midd le ear. Both medial
cholesteatom a (arrO\v) &
lateral cholesterol gran uloma
(open arrow) are p resent
but indi sting uishab le o n
T2WI M R.
MIDDLE EAR ADENOMA
2
74
Axial h-ft T-bon(' CT sha.vs \\'PII fJl,}rgin.lted mkkllf' m , A v.)1 T1 C+ MR of /Pit mr show'S mh.lncing middle t'.lf
,IC!f '1l0 H J.I (arrow ) that encases the os5k k.~. NOlt' acknoma (arm .,,). TlJis t:'limin.l f('5 cho/(osledloma as a
t1 lJ.~t(JicJ i.~ well pn eumatized. The tympanic mem bra"''''> d i.lgnoslK: posSibility. (\"CJtmculatt'{/ (acial / X'f W
WJ 5 olo.~coflically intact. schw .l nnoma stiff {X)5sible mimic
• Ind istin guish ahl e fro m co ngen ita l cholestea to ma

ITERM INO LO GY • Co nge nita l cho lesteato ma I11l1 ch m or e com mo n
Abbreviation s and Syno nyms • Well pneu ma tized masto id (n o histo ry of ch ro n ic
• M idd le ear aden o ma (MEA)
o titis medi a)
• Aden om atou s tumo r of middle ear • CEC1, Will en ha nce but d lfflcu lt to a pprecia te
Def init ion s MR Findings

• Beni gn, indo len t epit he lia l tum or s o f middle ear • T l WI: In termed iate signal lesion
o Not ceru m m o m a • T2WI: Hyperinten se
• Middle car (ME) ha s no apocrine glan d s • '1' 1 C+: En ha nc ing soft tissue mass in midd le ea r
• Ce rurn ino ma =EAC lesion Imaging Recommendations
o No t choristo m a • Axial & coronal bo ne CT im ages with out co n t ras t
• Salivary cho risto ma = norma l saliva ry tissue in • If large adenoma presen t, Tl C+ MR may be helpful in
abno rm al locati on, not adenoma defi ni ng lesio n exten t
IIM AGING FINDINGS IDIFFERENTIAL DIAGNOSIS

General Features Co ngenita l cho lestea to ma
• Best diagnostic clue: Soft tissu e mass + well • Most co m mo n mass be h ind int act TM
pneumati zed masto id (no ch ro n ic o titis media • Ch ild or yo u ng adult; no his tory of chron ic o titis
fmdtngsj m edia
• Lo cati on: Midd le ear cavity proper (mesoty m pan u m) • Wh en large. typically ero sive o n cr
• Size: Ea rly sym pto m s so sma ll « 10 rnm ) at diagn osis • T1 C+ MR sh ows no en ha n ceme n t
• Morphology : Oft en irregul arly margina ted
Acqui red cholestea to ma
CT Findings • TM retracti on po cket or perfo ratio n
• NECT • All ages affected: history of chroni c otitis media
o Mass with in ME beh in d in tact TM
DDx: Middle Ear Mass
CongoCholestcetotne Acq. Cbotesieetome Glomus Tympanicum Pedunculated FNS

MIDDLE EAR ADENOMA
Key Facts
Termin ology Top Differential Diagn oses
• Benign, indo lent epithelial tumors of middle ear • Conge nital cholesteato ma
• Acquired cholestea to ma
Imaging Findings • Glom us tympanicum par aganglioma
• Mass within ME behind in tact TM • Pedunculated facial nerve schwa n noma (FNS)
• Indi stingui sh abl e from co nge n ital cho lestea to ma 2
• Congen ita l cho lesteatoma mu ch more common Path ology
• Etiology: MEA arises from modifi ed respirat ory 75
mucosa
• CT shows erosive middle ear mass o Conductive hearing loss

• Tl C+ MR shows no enha ncement
Demographics
Glomus tympanicum paragan glioma • Age: Mean age at presen tati on is 4S years
• 2nd most commo n caus e of mass behind in tact TM
• Vascular, pulsat ile mas s behind an intact TM Natural History & Prognosis
• T l C+ MR sho ws en hancing mass • Slow-growing benign tumor
• No history of chro nic otitis media • If aggressive type, facial nerve in jury possible
• 18% recu rrence rate
Pedunculated facia l nerv e schwa nnom a
Treatment
(FNS) • Co mplete su rgical excision is treatmen t of cho ice
• 3rd most com mo n cause o f ma ss beh ind inta ct TM
• Avascular mass mim ics co ngenita l cho lesteatoma
• Tl C+ ~:IR sho ws en ha ncing mass con nected to facial
nerve
Co nsider
• If 1'1 C+ MR shows en ha nc ing m idd le ea r mass,
IPATHOLOGY consider MEA, glom us tympanicum para gan gliom a Sr
ped un cu lated facial nerve schwa n no ma
Gen er al Features
• Gene ral path com men ts
o Diagnostically cha llenging lesion for pathologist ISELECT ED REFERENCES
o Benign , ind olen t epithe lial tumors of middle ear
th at rarely invade bone 1. Devaney KO et al: Epit helial tumors of the midd le ear-care
midd le car carc inolds really d istinct fro m m iddle ear
• Etiology: MEA arises from mod ified respiratory muco sa adeno mas? Act a Otol aryn gol. 123(6): 678-82, 2003
• Epidem iology: MEA is very rare midd le ear tum or 2. Malnt z 0 et al: MRI ami CT of adenomatous tum ou rs of
Gro ss Path ologic & Surgica l Features the m idd le ear. Neurorad iology, 43(1):58-61, Jan , 200 1
3. Smith MM et al: Chortstomas of the seven th and eigh th
• Pinkish , shimme ring, soft tissue mass cran ial nerves, AJNR. 18:327-330, 1997
• Gene ral comme n ts
o Mode rately cellular &; un encap sulated IIMAGE GALLERY
o Arran ged in glandular, trabecular &; solid pa tt ern s
• Mucosal ad en om a
o Cubo ida l &; low colu mna r cells formi ng empty acini
• Papillary ade no ma
o "Papillary" patt ern = exte nsive local invasion
o More aggressive than mucosal aden oma
• Inverting papilloma .
o Iden tical to those tha t arise in nasal cavity
• Carcinoid tumor
o Arises from argen taffin cells
o Histologically iden tical to int estinal variety
(!Rf l) Axial right T-bone CT reveals middle ear adenoma in posterior

ICLIN ICA L ISSU ES tympanum with ossicle encasement (arrow). Lesion conforms to
shape o f posterior middle ear but there is no evidence of bone
Presentation destruction. (Right) Axial right T-bon e CT snows invasive middle ear
• Most commo n signs/symptoms adenoma associated with welt pneumatized mastoid, There is some
o Pin k soft tissue mass behind in tact TM expansion anteriorly (arrow) and destructive change posteriorly
(open arrow).
MIDDLE EAR SCHWANNOMA
2
76
A..;al T-Ixm{~ cr shows facial f)P(W K h w a lJ/lfJ m a AI::;.ll " c+ MR rP\'(',1/5 homog(Ifl('OtJ.~ (~"h. lIln-·m('nt of
protruding from Ix)~ tf'ri()r gt'fJU into pm /crior f..Jdcll scbws nnoma (arrow) prolrudilJ{; from
!lPfV('
me~oIYnlp..lf)Um (il (f() W). Note tumor obuts shorl pmrerio f b't'nu into postt'fior mesotvmpenurn. P,ll;pnl
pr ocess of incu_~ (open arrow). pfe<>en/('(/ with nmduclivf' hearing lo.S5.
o Facial nerve schwa n no ma

!TE RM INO LO GY • Well-ma rgina ted mass ema nati ng fro m facia l
Abb rev iat ions and Syno nyms ca nal
o Midd le ea r schwa n n om a (no t from 7t h n erve)
• Jacobso n ner ve sch wa n no ma , cho rda tympa ni
sch wan no rna • We ll-m argin ated ma ss fillin g middl e ea r without
invo lving facial ca nal
De finitions • Bo ny rem od eling wh en la rge
• Prima ry sch wa n noma: Tum or a rising with in mid dle o Trans laby rin th in e o r int racoch lear schwannorna
ear cavity wit h m id dle ca r p rotru sion
o Facial nerve > > j acobson nerve (9th bran ch ), chorda • La byri nth eros io ns wit h mass prot rudin g into
tympani nerve (7th bra nch ), Arnold n erve (10 th mid dl e ea r via rou nd o r oval wi nd ow
bran ch ) • CECT
• Seconda ry sch wan no ma: Arises outside midd le ear o Lesio n e n h an ces wit h co n trast
o Large 9-11 jugul ar fo ram en schwa n no ma erod ing • MR preferable to C ECT
into middle ea r
o Tran slabyrint h ine St h cran ia l ne rve sch wa n n o ma
MR Findin gs
• T IWI : Isoin tcn se to h yp ointen se (n o n specific)
• T2W!; Hype rinten se (non specific)
• T l C+: En ha n cem en t differentiat es from
IIM AGING FINDING S cho lestea to ma
Gene ra l Feat ures Ang iog ra phic Find ings
• (lest di agno st ic clu e: T l c+ MR shows en ha nci ng m ass
• Blush is rare
in m idd le ea r • Blush suggest ive o f glo m us tympant cu m
• Location: Midd le ca r cavity paragan gliom a o r perh ap s m idd le ca r ade noma
• Size: Vari a ble, usually < 15 m m
• Mo rp ho logy: We ll-m a rgin at ed , lobul a r mass Imagin g Re commend at ions
CT Find ings c r in ax ial & coro n al plan es
• Thin-sectio n
• NECT
DDx: Middl e Ear Ma sses
CongoCholes teatom a Glomus Tympanicum ME Adenom<l

MIDDLE EAR SCHWANNOMA
Key Facts
Terminology Top Differential Diagn oses
• Prim ar y sch wa n no ma : Tum o r arising with in middl e • Co nge nital cholesteato ma
ear cav ity • Glomus tympani cum pa ragan glio ma
• Secondary sch wan nom a: Arises outside midd le ear • Middle ear ade no ma
Imaging Findings Path ology 2
• Best diagn ostic clue: '1'1 C+ MR sh ows en ha nci ng • Epide miology: 3rd most com mo n middle ear mass
ma ss in midd le ea r (afte r conge n ita l cholestea to ma &. par agan glioma) 77
• Th in- secti o n ax ial & coro nal T I C+ MR d ifferen tia tes

from cho lestea toma Treatm ent
• Surgica l removal
ID I FFERENTIA L DIAGNOSIS
Co nge nita l choleste ato ma
• Most co m mo n ca use of mass behind int act 'I'M Co nside r
• Ch ild o r yo ung ad u lt • Co ngenital cho lesteato ma
• '1'1 C+ MR show s no en ha ncemen t o Most common m ass behin d intact 'I'M
• G lo mu s tym pa n icum pa raga n glio ma
Glo mus tympani cum para gan glioma o Mos t com m o n e n ha nci ng m idd le ea r ma ss
• 2nd most co m mo n ca use of mass behi nd intact TM
• Adu lt patient gro u p
Image Interpret ati on Pearl s
• Vascul ar, pul satil e mass • Angiographica lly negati ve co n tras t-en ha nci ng pr imary
• T I C+ MR sh ows en ha nci ng ma ss m iddle ear m ass
Middl e ear ade no ma

• Very rare mi ddl e ear tum or ISELECTED REFERENCES
• "1'1 C+ MR reveals enhancing middle ear mass I. Swa rtz]D. Imaging diagnosis of middl e car tcsron s. Cu rt
l'robl Diag n Radio!. 3 1(1 ):4·2 1, 20 02
2. Magliu lo G et al: C hor da tympa ni neu ro ma: d iagnos is an d
IPATHOLOGY ma nagement. Am J Otolaryngol . 2 1(1);65-8, 2000
3. Ayd in Kct al: j aco bse n's nerv e schwan no ma presenting as
General Features m idd le ear mass. t\jl': R Am J Neur o radiol . 2 1(7 ): l :n l ·3,
• Genera l pat h co m me n ts 2 (XlO
o Slow-gro wi ng, encapsu lated ben ign lesio n
o Neuroectoder ma l or igin
• Epidem io logy: 3r d m o st common middle ear m ass IIM AG E GALLERY
(after co nge n ita l cholestea to ma & paragan glioma)
Gross Pathologic & Surgi cal Features
• Encap su la ted ta n or gray neoplasm
• Antoni A areas co nsi st o f palisadi ng spind le cells
• An toni R areas con sist of pleom or ph ic cell s i n myxoid
grou nd substa nce
ICLIN ICA L ISSUES

Presentation
(Lef t) Coronal /t'ft T-bone CT shows pedunculated fad al nerve-
• Most common slgns /syrnptoms sdw a nnome (arro w) extending intc middle par cavily from
o Co nductive hearing loss mid -tympenic segment. I.( ·~ ion nearl y fills oval w indo w niche (op t-'n
o Ot o scop y: Flesh y-wh ite ma ss beh ind in tact TM arrow). (Right) A xial Tf C+ MR. reveals seconda ry middle ear
sctwa rmome as enhancing mass (arrow) protruding (rom round
Demographics window of cochlea. NOlin' the lesion emerges fro m prox imal basal
• Age: Adult tum of cochlea (open arrow).
Natural History & Prognosis
• Total excision is cu rative
MIDDLE EAR MENINGIOMA
:(~;-~~~,:_<.~2~:·~~-~};7 _ , \ ,~\
• :"~. ~,1,'\» J
-,
'.
.,
J', .TH
:.' i
r': ' , - '. ,', ,"\I' V " r' r:
. :---'-:: ......... ~ ,,~
2 ~ ., .
~ ..."
78
~
",' · f · · ,
.. ~
e" Ii .f
~
Corona! T1 c+ MR shows enhancing meningioma in Coronal t-booe CT shows opacification of middle ear
middle ear (arrow ) surrounding ossides. Dural (Mf M). Tegmen hyperostotic bone (arrow) irnlicdle5
thickening & enhilnCemen! (ofX'n arrows) indicate site of origin. No te abnormal bOlle ex/ending inferiorly
tegmen dural origin. from tegmen (open arrows) .
o Jugul a r foramen o r tegm en tympani com ponen ts

ITE RM INO l O GY may be large
Abbreviations a nd Syno ny ms • Midd le ear co m po nen t may rep resen t "ti p of
• Middl e ear meningioma (MEM) iceberg" for larger intracra nial co m pone n t
• Temp or al bone meningiom a, intrat ympanic • Morphology: Globular o r en plaque
meningiom a • Vector of spread
o Tegm en tympani meningioma spreads inferi orl y
De finiti ons int o midd le ea r
• MEM: Beni gn , un en cap su lated tumor arisin g from o Jugu lar foramen men ingiom a sp reads cen trifugally
arach no id cap ce lls, extend ing in to m iddl e ea r or alo ng dural su rfaces
arising with in middle ear • If spread su perolatcral, en te r m idd le ear th rou gh
floor, m imi ckin g glom us jugu lare par agan gliom a
IIM AGING FINDINGS CT Findings

• NECT
Gen eral Features o Bone c r shows perm eati ve-scleroti c cha nges in
• Best dia gn ostic clue: Avid ly-en ha ncing mass (T1 c+ affected bon e
MR) wit h in middle car cavity with • Tegmen ty m pa n i men in giom a: Tegm en tympan i
permeati ve-sclerotic bony ch anges (T-bo ne CT) in & mastoid bones affected
surround ing hones • Ju gu lar foram en meningioma: Sigmoid plat e,
• Location middle ear floor affected
o I.ocati on within middle ear depends on site of origin • CECr
• Extend ing down from dura overlying tegm en o 90 1M. strong, uniform en han cem ent
tympani o IO{),{) het erogen eou s en ha n cemen t
• Extending up fro m jugular foram en MR Find ings
• Arisin g within middle car
• T1WI: Iso intense to gray matter
• Size
o Midd le ca r co m po ne n t gen erali y smali « 15 mm) • T2WI
DDx : Vascular Middle Ear Mass
Dehisceru luguler Bulb Aberranl /CA Glomus Tym panicum Glom us Jugulare
Key Facts
Termin ology Top Different ial Diagn o ses
• MEM: Ben ig n , unencap su lat ed tu m o r ari si ng from • Deh iscent jugu lar bu lb
arach no id ca p ce lls, exte nd ing in to middle ea r o r • Aberrant in ter n al ca ro tid artery
arising wit h in m idd le ear • Cho leste rol gra nu lom a, midd le ea r
Imaging Findin gs
•
•
Glo m us tympan icum para gang lio m a
Glomus [ugul a re paragangli o ma 2
• Best diagn ostic clue: Avidly-en ha ncing mass (TI C+ • Middle ea r ade no ma
MR) wit hi n midd le ear cavity with 79
permea tiv e-sclerot ic bony changes (f -bone CT) in Clinical Issu es
surro un di ng hon es • Clin ical profile: Midd le-aged femal e wi t h co nd ucti ve
• Midd le ea r co m po nen t ge nera lly sm all « IS m rn) hea rin g loss
• Jugular foramen o r teg men tympani co mpo nen ts • Relativel y h igh recurrence rate , d ue to diffi cult y of
may be la rge co mplet e excisio n
• Bone CT shows permea tive-sclerotic changes in
affected bone Diagn o sti c Checklist
• Associat ed du ral "ta il" ma y allow pr ecise d iagn osis • Precise di agn osis of M EM di fficult unl ess la rge
intracran ia l co m po ne n t
o Iso in tense o r sligh t ly h ig her signa l th an br ain gray • T-bo ne CT: Tubu la r mass crosse s middl e ea r cav ity to
matt er re jo in h ori zontal pet rous ICA
o Oc casio na lly, scattered low in t en sity foci from a Enl a rged in fer io r tym pa n ic cana licu lu s
calc ifica tion • MRA: So urce ima ges & repro jection sh ows asym me t ric
• T1 C+ abe rra n t vesse l
a 90 91(, e n ha n ce stro ngly • En la rged colla te ra l vesse l t rav er ses m idd le ea r w he n
• Associat ed d u ral "ta il" m ay a llow p reci se d iagn osis in tern a l carotid a rte ry (ICA) fai ls 10 devel op
o Teg men tympani m enin gio m a
• En plaque thi ckened en ha ncin g dural lesio n
Choles tero l granu lo ma, mid dle e a r
• En ha nc ing ti ssu e p ro ject s down int o m idd le ear • Clin ica l: Blue-bl ack rct rot yrn pa nk m ass
cavit y • T..bo ne C f: Appea ra nce mimi cs acq uired
o Jug u lar foram e n m en ingioma ch olesteato ma
• En ha nci ng m ass fills jugu lar fo rame n • '1'1 C.. M it High signa l from m et h emoglobin h igh ly
• Enha ncing lesio n exte nds th rough sigm oid plate sugges ti ve
& m iddle ear floo r • In fla m mato ry mass with he mo rrha gic com po nents
• MRA: En larged fee dings vessels so meti mes visib le Glomus tympani cum pa ragan gliom a
Angio graphi c Findin gs • Clin ical: Red..v ascu la r retrotym pani c m ass beh ind
• Vascu lar tu mor w it h prom pt b lush a nt eroinferio r q uad rant of TM
• "Sun bu rst" pattern o f e n larged d ural feed ers co m mo n • T-bone C f : Focal mass o n co ch lea r p rom ontor y;
• Prolonged vascu la r "sta in " in to ve no us phase midd le car floor intact
• Midd le ea r co mpo ne n t m ay he o bsc u red by • '1'1 C+ MR : Foca l en ha n cin g m ass, coc h lea r
subtract io n a rt ifact p rom o nt or y
• Il ighl y vascu la r beni gn tum or a rising in m iddl e ca r
Imaging Re commendati ons
• lIegin w it h s ku ll ba se focused Mit wit h T1 C+ Glomus jugul ar e par agan gliom a
fat-satura ted MR • Clin ica l: Red ..vasc u lar retrotym pa n ic mass he h ind
• Fo llo w wit h 'f-bo ne CT an tc roinfer io r q uad ra nt of TM
• T-bo ne Cf: Perm eative-destr uct ive bo ne eros io n a lo ng
su pcro la te ra l m argin of jugu lar bulb
IDIFFE RENTIA L DIAGNOSIS • '1'1 C- MR: Ju gul a r fo rame n m ass with flo w voids
(vpeppcr") ex te n ds in to midd le ear
Dehi scent jug ular bulb • Vecto r of sp read : Su pc ro late ra l fro m jugu lar bulb to
• Clinica l: Blue-vascu lar postcroinferio r retrot ympan ic m idd le ear h ypotympanu m
ma ss • High ly va scula r benig n tu mor ero d ing in to m idd le ear
• 'l-bonc CT: Absen ce o f bon y plat e bet ween jugular from jugul a r for am en
bulb &. m iddl e ca r allows va scu lar diverticul um in to
m idd le ea r
Middle ea r ad e no ma
o "Bud" (d ive rticu lu m ) o ff supero late ral jug ula r bu lb • Clin ica l: Pink-tan rctr ot y mpan lc mass
en te rs m idd le ea r • 'l-bo n e CT: Rounded midd le ea r mass wit h mini m al
eros io n
Aberran t internal ca ro tid arte ry • '1' 1 C+ MR: En ha ncing focal mass in m iddl e car
• Clin ica l: Red -vascu lar ret rot ympan ic mass crosses • Ben ign. no naggressive neo plasm a rising within m idd le
coch lea r pro mo ntor y fro m poste rio r to ant erio r ea r
IPATHOLOGY • Rela tively hi gh recu rren ce rate, d ue to d ifficulty o f
co m p lete excisio n
Gen eral Feature s • Progn osis relat es to su rgical o utco me &. com p licati ons
o Po ssible facia l nerve In jur y
• Ge ne ral path co m me n ts
o Embryology-ana tomy o Possib le per manen t h ea rin g lo ss
o 5 year su rv iva l: 85 1M,
• Rout es o f sp read from mid d le/post er io r cranial
2 fossa t hroug h 'l-bone
• Ge ne tics
Treatm ent
• Com p lete su rgical excis io n
o Long arm de letions of ch ro mosome 22 co m mon • Aggressive surgery advoca ted beca use bon e in vasio n
80
o N F2 gene ina ctivat ed in 609"6 of spo rad ic cases h ar d to see a t su rge ry
• Etio logy
o Arises fro m arac h noid "cap" cells, no t dura
o Arach noid ce ll rests ca n be fou n d wit hin midd le ea r
• Embr yonic mig ration ano maly
• Epid emiology: ]C)6 of int racranial men ing iomas Co nside r
origin ate from an te rio r or poste rio r su rface of pe t rous • Iden tify site of or igin (tegm en , jugular fora men o r
bo ne posterior wa ll 'f-bo ne)
• Con v ey to su rgeo n exte n t and vecto r o f sp read o f
Gross Pathologic & Surgical Features t um or
• Sharply circumsc ribed, unen capsul at ed
• Ad jacent d ura l th icken ing (collar o r "tail") is usually Image Interp retati on Pearls
reactive, not n eoplastic • Precise d iagn osis of MEM difficu lt unl ess lar ge
• Usua lly globular (most co m mon ) or en plaqu e type in tracra nial co m pone n t
Microsco pic Features • Rad io logist provides key info rmati on o n exten t o f
di sea se
• Wid e ran ge of hi sto log y with litt le beari n g on
• Clin ical &. imagin g appeara n ces mim ic paraga ng lioma
outco me
• Rare ly a rises with in m iddle ca r
o Meni ngoth elial, fib rous, t ran siti o nal,
o More co mmon ly exte nds fro m in tracran ial
psammomatou s, a ng io mato us, mi scella n eou s ot he r
me ning ioma
(mic rocys tic, cho rdo id, clea r cell, secreto ry)
• C lues to d iagn osis
• Nests &. wh o rls of "me n ing iom ato us cells" o Pe rmeative-sclerot ic unde rlyin g hone
• Psamm oma bodies o Con nectio n to intracra nia l menin gioma
• Im m un oh istochem ist ry: Po sitive EMA; va riab le 5 100 o Dura l "ta ils"
IC LIN ICA L ISSUES ISELECTED REFERENCES

Presentati on I. Thom pson LD ct al: Prima ry ea r and te m poral bon e
• Most co m mo n signs/sym pto ms men ingio ma s: a cllnl co pa tbo logic study of 36 cases with a
o Co n d uc tive h ear in g loss review of the literature. Mod I'at hol. 16(3):236-45, 200 3
2. Lawand A ct al: Path ology qui z ca se. Middle ca r
o Ot he r signs/sy m p to ms
men in gioma. Arch Oto lary ngol Head Neck Surg.
• Sensorineu ral hearing loss 128(8):9 75· 7, 2002
• Ota lgia 3. Sch ick B c t al : Magn et ic reso na nce im agin g in pat ients
• Ch ron ic otitis med ia wit h sudden h earing loss, tinnit us and vertigo. Otol
• Fac ial n eu ro path y Neu rotol . 22t6):808· t2 , 2lXII
• Vertigo 4. Prayso n RA: Middl e cur mc ntngtom as. An n Diagn Path ol.
o Symptoms from la rge r in tracra n ial com po ne n t 4(3):149-53, 2000
• Sku ll ba se: Co m plex cra n ial n eur opath y ma y 5. O' Reilly RC ct al: Prima ry ex t racran ial mcnin gioma of th e
tem por al bo ne. Oto laryngo l Head Neck Surg . ] 18(5):690-4,
involve S, 7 & 8
199M
• Ju gular foram en : 9- 12 cra n ial ne uropa t hy possib le 6. Langfo rd LA: Patho logy o f men ingiomas. J Ncuroo nco l.
o Otoscopic examinatio n: Vascu la r (b lue) 29( 3):217-2 t , 1996
retrotympa n ic mass 7. Civantos F et al: Tem pora l men in giomas p resen tin g as
• C lin ica l p rofile: Midd le-aged fema le with conductive chron ic otitis med ia. Am] Otol. 14(4):40 3-6, 199 3
he a ring loss 8. Hooper R et a1 : Tem por al bo ne meningiomas. Aust N Z J
5urg . 60( 10):779-86, 1990
Demographi cs 9. Salama N et a l: Men in giomas presen ting in th e m iddl e ear.
• Age: Avera ge age at presentation = 45 yea rs Laryngosco pe. 92( 1):92-7, 1982
• Gender 10. Par isicr SC et al: Th e evaluat ion of middle ca r
o M:F = I :3 meningiom as using co m pute rized axia l tomog rap hy.
Laryn goscope. 88 :] l70· 7, 19 78
o Sim ilar to int racrani al meningioma 11. Guzowski ] ct al: Men in gio ma s of th e tem po ra l bone.
Natural Histor y & Prognosis La ryngoscope. 86(8): 1141·6 , 19 76
• Slo w grow ing benign tu m or
IIM AG E GALLERY
(I£/I) Axial T1 C+ MR
reveels enhancing MEM
filling epitympanum (a" mv),
surrounding ossicles. Tumur
2
causes aditu_~ ad an/rum
block (open arrmv) with Hl
obstruc ted 17uidin /ll,u(oirl
air cells (cu rved arrow ).
(RighI ) Co ronal T2WI AIR
d emonstrates midriff' e-sr
nJcningionJ.l (arrow) ,
;so;nlenst> to gr.ly ma ll('(.
Bright secretions fill
remainder of middle (l.U.
Notice the lC1:men is norm .".
Typical
(/ Llt) Coronal t -borw cr
shows hyperosfosis of
(egmcil «lr m w), with
.1h no rmal hom" t'xtt>lldinf:
do wn to involve cssictcs
(op on .lrfmv) S tymp.lIl ic
!o{'gmt ' /JI of eN7 (n Hv(·d
.1"0\\' ). MR show5 legmen
tympa ni mC'ning;om,l . (RighI)
Coronsl t I C+ MR fPWJ/s
I.lfge tegown tympani
mt'ningioma (arrow )
sp rtw fing along 11001 of
m;(l</le 0 ,10;.11 {ossa. Lesion
t"xlt>nd> int o middll' ear
(orH'n JfroW ' ..t pfl'St'nt5
with retrotvmpsnic va>cul,Jf
mass.
(/.K/tJ Coronal 7·bolll' CT

~ h()\vs middfe eJr
meningioma (arro w) bl41;/I(/
intact tympan ic IJw m IJ{,lIl t ,.
Lesion ex rt'nd~ up (rom
j ugu/tlf toremon. 601lt'> of
skull base src itwolvod on
MR bu t hafd to .'>t>l. on thi~
C T image. (Rigl", CumrM/
T 1 C+ AIR sh ow s jugu/.lf
foramen meningiom.l
s!J'mding into millllll' ('ar
(d f ro w) S lAC (opt'n arrow ).
Middle ear compo nent is
- rip of iceberg " with mmt of
lumor in jugular iorJmen
(curved arrows ).
GLOMUS TYMPANICUM PARAGANGLIOMA
2
112
Coron.,! gr,ljlhic slu.J\vs <J higlJly vascular glomus (orooal T-lxme CT .~h ()ws glomus tympenicum in
tympemi cum p"lfi.llj dnglioma perlunculating o ff ccchtosr meso- S hypo tympanum bulging against tymp"m ;c
!,romontory into /0 \\'('( middle ear cavily. The bon y membra ne (arrow). Ope n arrow: Floor of middle ear
floo r of midd le m r cavieyis inta.t. intact. Cut vod arrow: Caro tid canal wall intact
• Mo rphology
ITE RM INO LO GY o Round mass wit h flat base most com mon
Abbreviation s a nd Syno nyms o La rger lesion resem ble "New Jersey" o n co ro nal
• Abb revia tio n: Glom us tympan icu m paraganglioma image whe n they fill midd le ear cav ity
(GTI') CT Findings
• Syno ny ms : Glom us tym panic um ; chemodecto ma • NEC I'
Definiti on s o Focal mass with flat base o n coch lear pro montor y is
• GTP d efinition : Benign tum or arising from glom us characte ristic
bod ies situated o n coc h lear promo n to ry o Small GTI'
• May be subtle soft tissue bu mp o n coch lear
promontor y
IIM AGING FINDINGS • Project off coc h lear promontor y int o lower
meso tympan urn
Ge ne ral Features • May reach as far lateral as lower tympanic
• Best d iag no stic clu e membrane (TM)
o c r: Mass wit h flat base on coch lear promo n tor y o Large GTI'
o M R: Enhancing mass wit h flat base O il coc h lea r • Fills midd le ear cavity, creating at tic block
pro mon tor y resulti ng in fluid co llectio n in m asto id
• Locati on • Tum or margins may not be di scern ible o n hone
o Prim ar y location : Co ch lea r promon to ry C I'
o Varian t locations: An te rior to pro mon tory, benea th • Floor o f m idd le ea r cavity is intact (if deh iscen t or
coc h learifo rm process; inferior to pro mo n to ry, in permea tive, glo mus jugulotympanicu m)
recess ben eat h basal tu rn o f coc h lea • Larger lesions may show "aggressive" bo ne
• Size changes with eros ion of medi al wall of mid d le ear
o Millim ete rs to 2 em cav ity ± ossicles
o May be so sma ll tha t rad io logist mi sses lesion • Rare invol vem en t o f air cells along infe rior
alt ogether cochlea r pro mo n tor y ma y be mi staken fo r
in vasion
DD x: Retrotympan ic Mass
.
~.
I
). .
.. "\ _-
i
ic.· 1',....--s:«, /
Ab lCA Dehiscenl Jugular Hulh CongCf} Cholesteatoma Glo m us Jugu /arp
Key Facts
Terminology • G lo m us jugu loty mpanicum pa ragan glio m a
• Abbreviatio n : Gloom s tympan icum pa ragang lioma • Facial n er ve sch wa n n o m a, tym pa n ic seg men t
(GTP) Pathology
• GTP defin it io n : Benign tu mor arisin g fro m glo m us • GTP is most co m mon t umo r of m iddle ea r
bodies situated on coc h lear promontory • GTP rarely associa ted with mu lticentric
pa raga nglio ma s
2
Imaging Findin gs
• CT: Mass with flat base o n coc hlear pro montor y Clinical Issu es 83
• MR: En ha nci ng m ass w ith fla t base o n coc h lea r • Clin ical profile: 50 yea r o ld fem ale wit h vascular
prom ontory retro tympanl c mass & pulsatile tin n itu s
• CT: Bo ne-on ly CT wit ho ut co n t rast best if GTP
sus pec ted clin ically Diagnosti c Chec klist
• GTP may be clinically ind isti nguishable from glom us
Top Differential Diagn oses jugulotympan icu m paraga nglio ma o r AblCA
Aberr an t internal carotid ar te ry • Pre-operati ve im agin g must d ifferen tiate th ese
• Dehi sce nt [ugul a r bu lb d iagnoses
• Congenit al cho lesteato ma of mid dl e car
• CEC I': Difficult to identify e n han ci ng m ass in m iddle • Clin ical: Asym ptom atic, incide n ta l otosco pic
ea r wh en GTP sm a ll observation
MR Find ings Co nge nita l cho lesteato ma of middl e ea r
• TlWI • Imaging: Tl C+ M R shows no en ha nce me n t
o Tissue intensit y m ass o n coch lea r prom onto ry • Clin ical: "White" ma ss beh ind in tact TM
o GTP too small to sec high velocity flow vo ids in
mass Glo m us jug ulo tym pa nic um paragan glioma
• T2W I: GTP di spl ays lower signa l co mpare d to • Imagin g: CT sho ws permeative cha nge in bon y floor
obstructed flu ids o f midd le car
• TI C+ • Clin ical: Otosco pic exa m id enti cal to GT P
a Focal en hanc ing mass o n coc h lear promont o ry Facial nerve schwa nno ma, ty m pa nic
a With larger, obst ructi ng lesion s co n t rast helps
d ifferentiat e tumor from obstructed secre tions segment
• MRA: Does not show en larged vessels • Imagin g: Peduncu lat ed mass off tympanic segme n t,
facial nerve
Angiog rap hic Find ings • Clin ical: Tan-white mass behind supe rior 'I'M
• GTi' a rteria l su pp ly
a Ascend ing ph aryn geal artery & its inferior tym pan ic
branch , via inferior tympanic cana liculus IPATHOLOGY
Imaging Re commen dati ons Ge ne ra l Features
• CT: Bone-only cr witho ut co n trast best if GTP • Gene ral path co mme n ts
suspected clinically o All paragan glio mas have same histopath ology
• M It On ly used if glom us jugu lotym pa nic u m suspected o Named by location
from bone CT findings • GTI': M idd le ea r o n ly
o Small GT P ma y he m issed if slice thickn ess > 3 mm • Glo m us jugu lot ympan icu m : Jugular
• Angiograph y: Un necess ary if GTP d iagnosis clea rly fo ramen-m iddle ear
established hy CT • Glom us vaga le: Nodose gang lion o f
nasopharyngeal caro tid space
• Ca rotid bod y paragan gliom a: In no tch o f carotid
ID I FFERENTIA L DIAGNOSIS bifu rcati o n
• Etio log y
Abe rra nt int ernal ca ro tid arte ry a Arise from glom us ( L. "a hall ") bodies (paraganglia)
• Imaging: Tubular mass crosses mid dle car cavity to fou nd alo ng in fer ior tympani c nerve (lacobson
rejoin hori zontal petrous lCA; large in ferior tympani c nerve) o n coc h lear promo nt o ry
ca nalicu lus • Che mo recepto r cells derived from prim itive neu ral
• Clin ical: Vascu lar ma ss behin d T~1 ± pu lsatile tinn itus crest
Dehiscent jugular bulb a No nc h romaffln (no nsecret o ry) in t his locati o n
• Imaging: CT sho ws d chi sccn t sigmo id plate: venous • Epidemiol ogy
prot rusion into mid d le ear cavity from su pe rolate ral a GTP is most com mo n tu mo r o f middle ear
jug u lar bu lb a GTP rarely associated with multicen tric
paraga nglioma s
Gross Pathologic & Surgical Featu res IDIAGNOSTIC CHECKLIST
• Glisten ing, red , pol ypoid ma ss a ll coch lea r
prom on tor y
Co nsider
• Fibro us pseu d oca psule • GTP may be clin ically indi stinguishahl e from glo m us
[ugu lot ympa ni cu m paraganglioma or AblCA
Micro scopic Features • Pre-operati ve im aging mu st d ifferen tiate th ese
2 • Bip ha sic cell pattern co m po sed of ch ief cells &
suste n tacular cells su rro u nde d by fib rom uscular
dia gno ses
stroma Image Interpretation Pearls

84 • Ask referring clinician co lo r &. locati on o f
• Chief cells arranged in characteristic compact cell
nests or "ba lls" o f cells ret roty m pa n ic mass
o Referred to as "zellballen" o Red an tcro inferio r mass: GTP
• Immu nohistoch em istr y: Ch ief cells sho w a d iffuse o Blue posteroi nf crior mass: Dehi scent jugul ar b ulb
reactio n to ch romagranin o Red mass cross in g TM fro m pos terior to an te rior:
• Elcctronmicroscopy: Sho ws neuro secretor y gran ules AhlCA
o Whi te mass: Co ngen ital choleste ato ma (infer ior ) or
Stag ing, Grad ing or Classificatio n Crite ria facial ner ve schwan no ma (supe rio r)
• Glasscoc k-j ac kso n cla ssificatio n of GTP
o Type 1: Sma ll mass lim ited to coc h lear prom ontor y
o Typ e II: Tu mo r co m plete ly filling mi dd le ear space ISELECTED REFERENCES
o Type III: Tum or fillin g m idd le ear & ex te nd ing in to t. Rohi t ct al: Glomus tym pan icum tumo ur: an alt e rn ative
mastoid air cells sur gical tech niq ue. J Laryn gol O to l. 117(6):462-6, 2003
o Typ e IV: Tu mor filling m id d le ea r, extending in to 2. Forest jA 3rd et al: Lo ng-term co nt ro l of surgicall y treated
mastoid o r through tym panic memhr ane to fil l glo mu s tym pan tcu m tu m or s. Ot o l Ncuroto l. 22 (2):232 ·6,
ex te rnal aud ito ry canal; may ex te nd ant erio r to 200 t
ca rotid art ery 3. Ja ckson CG : Glo m us tyr npanicu m a nd glo m us jugulare
t umors. O to lary ngo l Clln Nor t h Am . 34(5):94 1-70, vii,
200 t
4. Noujai m SE et al: Paraga ng liom a of t he tem po ral bo ne : role
IC LIN ICA L ISSUES of m agnetic reso nan ce im aging versus co m puted
to m ograp hy. To p Magn Reso rt Im aging. 11(2):108· 22, 2000
Prese nta tion 5. Ma fee MF et al : Glo m us tac tale, glo m us [ug ula re, glo m us
• Most com mo n signs/sym pto ms tym panicum, glo m us vagalc . caro t id bod y tu m or s, and
o Vascular, pulsatile ret ro tympan ic mass sim ulating lesio n s. Role of M R im aging. Radi o ! C lin North
• An teroi nf erio r q uad rant o f tympanic mem b ran e Am . 38 (5): 1059 -76, 2000
o Other signs/sym ptom s 6. Rao AB ct al: From the a rch ive s of th e AH P.
• Pulsatil e tinnitus (90'J'h), co nd uctive hea rin g lo ss Paragangliom as of th e head and ne ck:
rad tologlc-pa th ologjc co rrela tio n . Armed Fo rces Ins titute of
(50%), facial nerve paralysis (5%) Patho logy. Rad iogr aph ies. 19(6):1605-32, 1999
• Clin ical profile: 50 year o ld fem ale with vascu lar 7. Maier W et al: Pa ragan glio m a as a syste m ic syndrome;
retr o tym pan ic mass &. pulsatile tin nitus pitfa lls a nd stra tegies . J Laryn go! Oto l. 113(1 1):9 78-82,
1999
Demographi cs 8. Weissman JL c t al: Beyo nd t he prom o n tory: the m u ltifocal
• Age: 66911are between 40-60 years o f age at diagn o sis or igin o f glo m us tym pa n icum tum o rs. AJNR Am J
• Gend er: M:F ~ 1:3 Ncu ro radiol. 19(1):119-22, 199M
9 . Llsca k R et al: Le ksell ga m ma kn ife radi osur gery of t he
Natural Histor y & Progn osis tu mor glo m us [ugu lare an d tym pa nic um . Stcrco tac t Funct
• Slow-g row ing , noninvasive tu mo r Neuros urg, 70 Su ppl I:152-60 , 1998
• Average ti me fro m o nse t o f sym pto ms to su rgical 10. j acobs IN et a l: Glo m us tym pan icum in in fancy. Arch
treatment is 3 yea rs Ot ol ary ngo l Head Neck Su rg. 120 (2):20 3·5, 1994
11. Al vhalkh ly A ct <II: Glom us tym pani cum chem odectoma:
• Co mplete resecti o n yields a perm an ent su rgical cu re
unu sual radi ol ogical find ing s. J Laryng o l Otol.
Treatme nt 108(7):60 7-9, 1994
12. Baguley OM ct al: Audi o logical findings in glo mu s
• Tympa no tom y fo r sma ller lesions; mastoidectomy for tu mo urs. Br .l Audi oJ. 28 (6):29 1-7, 1994
larger lesion s 13. Balatso uras DG ct al: Mult ip le glo m us tum ours. J l.aryng o l
• Incom plete su rgery o f glomus jugul ot ym pani cum 0101. 106(6):S38-43, 1992
paraganglioma o r bio psy o f an AblCA may result in 14. O'Leary M] et al: Glo m us tym pa ni cu m tumo rs: a clin ica l
serious pa tien t co m plicatio ns perspect ive. Laryngoscope. 101 (10 ):1038-43, 199 1
• Critical for rad iologist to co rrec tly int erp ret 15. Larso n T'C et al: Glo mus ty m pa n icum ch em od ect o mas:
pretreatment images Rad iograp hic an d clin ica l cha rac teristics . Radiolo gy.
163(3):801-6, 198 7
IIMAGE GALLERY
(u ft) Ax.ial t-bone CT
revce ts a sess ile ma ss on the
cccblenr promontor y 2
(arrow). Thi!> parag angliom a
is wen on otoscopv as J
cherr y ( prJ ma ss behind th p 85
ant erio r in ferior tymp"nic
membr ane. (Rig llt) Coronal
T-bone CT domonstretes tllis
smaller glomus tymp smcum
p araganglioma as an ovoid
m.l!; _~ abutting tilt' ("(x"hlear
promontor y (arrow ). No /in'
the 170m of 111(' middle e.l'
cav ity is inta ct (oIX' fI ,Jf(OW ).
Variant
(LeJt) Coronal I-bone CT
sholVs sholVs complete
opacificafion a; middle ear
and m ,Htoid .l i, ce lls. This
farw' glonJtJS tymp-m icum
p.lfag.mglioma f>\te/ll call no l
be ascertained by CT alon e.
Arrow: Tu mo r lobulation .
(Righi ) Coronal T1 C+ 1\4R
rcvvals large glom us
tympanicum p.lfagangliom a
in tnf:'50- dnd
hypo tymp.lnurn (arro w) .
Lesion extends into
epitvmpenum. o /ls/rueting
mastoi d air cells (open
arrow). CUf\'~/ arrow:
COCM.'il.
Other
(Lef t) Axial graphic shows
glomus bodies (arrow ) along
CO tHS(' 0; lnicrior tvmponk;
norve on ccctvoer
pro montory. Glom us
tym panic-urn !Umors iJriw
{mm I h i~ norma l cellular
cottvctkm. Open ,m ow:
cc chie s . ( Righi) Surgic.ll
phu /ugraph shows ~/omw.
Iympdn;cum par.lgalJ#lioma
as .l glistl'nillg. cbotrv (cd
m<1SS (arro w) . l he.' t ympanic
mem brane has been Wed.
Open arrow: Surgical probt'.
MIDDLE EAR RHABDOMYOSARCOMA
2
Be,
Axial /e(1 ear T-bon e CT 511rn1l5 dc~'rrtlctjve m iddle Axial CECT revm ls enhancing, destructive rh.1/xlo with
t'.lNmstoid rhalxlo (arrows) that has ex/t.·ncll'cl lil lerally eXlftlS;Uf1 imo ('xlraCfan i.11 soft tissues (arrow ). No /c
into $upertid.11 soft rissw.'S. Ossides and facial ne rve in rracrani.l/ l''''tc /lsion (ope n arrow) w ith sm ooth m argin
(emain intact. _~u~es lil1g dura intact.
• Inferior via internal carotid or jugular foram en

ITE RM INO LO GY into nasopharyngeal carotid space
Abbreviatio ns a nd Syno nyms • Ant ero inferior into TMJ, m asticator o r paroti d
• Middl e ear (ME) rhahdo myosa rcoma (rha hdo ) spaces
• "l'aram eni ngea l type" rhabd o myosarcoma • Size
o La rge at presen tati on : > ] cm
Definit ions • Fills midd le ear-mastoid co mplex, wit h
• ME rhabdo: Rare aggressive, destru ctive middle ea r intracrani al ex tens ion
t u m o r o f ske leta l mu scle o rig in , in PEDIATRIC • Mo rp ho lo gy : Poorl y defin ed , locally destr uctive mass
population
Cf Find ings
• NECT
IIM AGING FINDINGS o T-bo ne CT
• CT shows middle ear-mas to id destructi ve mass
Genera l Features • Often wit h associa ted EAC extensi o n
• Best di agn ostic clu e • Lytic, destructive bone &. ossicle changes
o 'l-bo nc Cf: Destruct ive m iddle ear m ass • Sku ll base & cra nial nerve foraminal in volvem en t
o '1' 1 C+ MR: Irreg u la r, in vasi ve en h a nci ng mi dd le ea r co m mo n
ma ss in ch ild • CECT
• Lo cat ion o Mass usually homogen eou sly en ha nces
o Middle ear &. masto id sof t tissue mass o Mass may be hem o rrh agic & necrot ic
o Possible areas o f extens io n o 30 tJtb have nod al met astases at presen tation
• Lat era l exte nsio n int o ex te rn al audi to ry canal MR Findings
(EAC) co m mo n
• T I WI: lso- to hypoint ense mass
• Medial ex te ns ion into intern al aud ito ry canal
• T2W I: Hyp erinten se. usually homogeneou s un less
• Ce ph alad in to m idd le cran ial fossa necro tic o r hem o rrhagic
• Posterior in to posterior crania l fossa
• TI C+
o T l C+ MR revea ls tumo r en hance s hom ogeneou sly
DD x: Destructi ve Middl e Ear-Mas to id Mass

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. -, '<O ~
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.' ;': ~, 'lLl1
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I- ". I
I
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Acq . Cholesteatoma Cholesterol C renulome LC Jlisliocytosis

Key Facts
Terminology Top Differential Diagnoses
• "Para rn en ingea l type" rh abdo m yo sa rcoma • Acquired cho lesteato ma
• ME rhabdo : Rare aggressive, destructive midd le ea r • C ho lestero l gra n u loma. middle car
tumor o f skeletal muscle o rigin, in PEDIATRIC • l.an gerhans cell (LC) histi ocyt osis, 'I-bo ne
po pulatio n • Endolym phat ic sac tumor (EL5T)
2
Imagin g Findings Path ol ogy
• c r show s middle ea r-masto id destructive mass • Rhabdo is most co m mon soft t issue sa rco ma in 87
• Lytic, destructive bo ne & ossicle cha nges c h ild ren
• Tl C+ MR reveals tumor en ha nces homogeneou sly • 50% of rha bd o in ch ild ren occ urs in H&N
• Coro nal images best for det ecting intracranial • Orbit > na soph arynx & associated spaces > midd le
exte nsio n through tegmen, mastoid roof ± skull ba se ca r-mastoid > sinonasal
for am in a
• Both CT & MR recomme nded to stage sku ll base Clinical Issues
destru ction, middle ear disease &" intracra nial • Clin ical profil e: C h ild under 5 yea rs with ch ron ic
extension oti tis media, o to rr hea & ca r pain
o Co rona l images best for det ecting intracra nia l

exte nsio n th ro ugh teg me n , mast oid roof ± skull base Cho leste ro l granulo ma, middle ear
fora mina • Clin ical: Otosco py shows "vasc ula r" hu e behi nd
o Irregu lar in terface between tum or &" d ura suggests ty m pa ni c me m bra ne
in t ra-d u ral ex tension o Past hi sto ry of m ultiple p rior ear inf ections
• Imaging: MR shows hig h T l sign al, h igh T2 signal
Nucl ear M edicin e Find ings m ass i n m idd le ca r ± ma sto id
• Bone Sca n : Midd le ca r &. ad jacen t sku ll base Increased
upt ake Langerh ans cell (LC) histio cytosis, T-bone
• Clin ica l: Pedi atri c pat ient wit h po st-a u ricu lar swe lling
• PET
o Im po rta n t tool fo r stag ing ME rha bdo • Imagin g: T-bo ne CT shows unil at eral o r bila teral
o Primary, nod al &. d istan t rn ct s FOG-avid dest ructive m asto id -cente red m ass
o Ofte n bila teral o r o the r assoc iated osseous lesions
Imagin g Recomm endation s • May st rongly m im ic m idd le ea r rh a hdo
• Bes t imag ing too l • Biopsy needed to m ake final diagnosis
o Bot h C f I'< MR recom m ended 10 stage skull ba se
dest ruction, m iddle ca r di sease Ex in tr acran ial Endol ymphati c sac tumo r (ELST)
exte n sion • Clin ical: Adu lt with unilat eral sensorine ura l hearin g
o Co rona l T l C+ to map intracranial exte ns ion loss
• Protoco l advi ce • Imaging: Bone cr sho ws d estructive mas s cen tered in
o Co m plex skull base mass , with po te n tia l for posteri or pct rous bone a t e ndolym pha tic sac-d uct
intracrania l ex te ns io n, d istant meta stas es & cervica l o '1' 1 MR: Mul ti ple hi gh signa l foci in tum or matri x
ade nopat hy req u ires ca reful multi-modality wor k-up ty p ical
o Thin-secti on bo ne cr in axia l plane, with
hi gh-resolution coron al reformat ions o r dir ect
coron al im agin g recommen ded IPATHOLOGY
o Multi-planar MR pre- & po st-co n trast, through sku ll
General Features
base
o Ce rvica l ade nopa thy ca n be staged with eit he r • Ge nera l path co m me n ts
o \Vlt O cla ssifica tion h as 6 hist ologi cal su bty pes
CECI; MR o r PET
o In te rgroup classificat ion has -I groups
o Th ese 6 subtypes &. -I grou ps h ave no ima ging
IDIFFERENTIAL D IAGNOSIS equivalen t
• Ge ne tic s
Acquired cho lestea to ma o Increased inci den ce in ch ild ren with pS3 tu mor
• Clin ica l: Tym pa n ic m embra ne pe rfo ratio n ± retraction su ppresso r ge ne m ut a tion
with visibl e ch o lestea to m a o May be increased in cidence in c h ild ren wit h
• Imagin g: Ho ne C I' shows hon e & ossicle destructio n hered ita ry retinobl asto ma
usuall y less ext ensive t ha n rh ahdo • Etio logy : Malign ant tumor o f skeletal muscle
o Pars f1a ccida type: Cho lestea to m a en la rges from • Epid em iology
Prussa k space o Rhabdo is mo st co m mo n soft tissue sa rco ma in
ch ild re n
o SOI}h of rh ahd o in ch ild ren occurs in l iNN
o Head &: neck sites o f o rigin
• O rbit > nasop haryn x & associated spaces > middle • Co mbined ad juvan t rad iatio n & che mot herapy
ear-masto id > sino nasal
Gross Patho logic & Surgical Featu res IDIAGNOSTIC CHECKLIST
• Smooth , lobul at ed necrot ic or hemo rrh agic tu mo r
• Sites o the r tha n ME may have grape-like polyp oid Co nsider
gross appearance (sarco ma botryo ides) • Clinical: Co ns ide r ME rh abdo if aura l po lyp or facia l
2 Microscopic Featu res nerve palsy found in ch ild with "ch ro nic o titis "
• Imaging: Consid er ME rhabd o if u nil ateral de structive
88 • Three gene ral histologic sub types: Pleom o rphi c, petr ou s ho ne mass in child
alveo lar &: emb ryo na l • Lange rha ns cell hi stiocytosis of T-oone at tim es very
o EMBRYONAL rhabdo is most co mmon in head &: d ifficult to di sti ngu ish fro m ME rhabd o
neck
o Prim itive rou nd skeleta l mu scle cells with Image Inte rp retatio n Pearls
h yperch romatic n uclei &. mitosis • Both CT &: MR impo rtan t for staging primary site,
• Immun oh istoch emi stry positive for dcsmi n, vi mentin, local d isease & nod al ± dista nt metastases
an tibo d ies to muscle-specific act in • Co ro na' plane ne ed ed to assess in tegr ity o f skull base
• In all subtypes, rh abd o rnyo blasts (cell of o rigin) & det ect intracrani al extens io n
presen t
Stagi ng, Grad ing or Classification Crite ria
• ~l Erhabdo co nsidered "param cningeal" &. ca rries
wo rse progn osis t ha n o rbit al o r lesio ns arising in othe r t. Mc na JC c t al: Case 3: Te m por a l bone rha bdo m yosarco m a.
sites Aj lt Am J Roentgenol. 17t (3):873, 877-9, t 998
2. Cas ti llo M e t al: Rhabdo myosarcoma of t he m idd le ea r:
• Intern ati onal Rhabd om yosarcom a St ud y G rou p ima gin g featu res in two c hild ren . AJ ;'\I [{ Am J Neuro radiol .
grad ing system used clin ica lly 14(3):7JO-3, 1993
o Gro up 1: Co m plete resection o f localized disease 3. Zam pa V ct 31: Rhabdo myosa rco m a of t he pet ro us ridge.
o Grou p II: Co m pletely resected regio na l d isease or CT a nd MR imagin g i n a n atyp ica l case with mult ip le
mi crosco pic residu al cran ial ner ve pa lsy. Acta Radlol . 33( 1):76-8, 1992
o Gro u p III: Gross residu al d isease 4. w ta truk IlJ e t 31: Rhabdo m yosarcom a of th e ea r and
o Grou p IV: Distan t metastases, ca rries wo rst tempor al bo ne. Laryngosco pe. 99(11):1188-92. 1989
p rogn osis 5. Sai d H e t <I I: Rhabd om yosarco m a of the middl e ca r and
m astoid in ch tld rc n. ] Lar yn go l Ot o l. 102 (7):6 14 -9, 1988
6. De SK e t 31:Tu m ours o f the mast oid tempor a l bo ne: with
in ter esti ng cases in th e paed iatr ic age group. J Lary ngol
ICLINICAL ISSUES Otol. 102 (7):582-7, 1988
7. Ccm ilog lu R et 31: Rhabdo myosarco ma of the tem pora l
Presentat ion bo ne: cl inica l rep o rt. Arch Otorhinolar yngol. 2 4-4 (4) : 195-7,
• Most co m mon signs/sym pto ms 1987
o Presen ts like ch ro n ic o titis medi a, with oto rrh ea, 8. Lesser RW (It a l: Malig na nt tu mor s of t he mid dl e car and
so me times blood y &. car pa in exte rn al audi tory ca na l: a zn.ycar review. Otola ryngo l
Head Neck Surg. 96 (1):43-7, t 987
o Other signs/ sym pto m s
9. Latack ] 'F ct al: Imagin g of rhabdom yosarcomas of the head
• Aura l pol yp an d neck. AJNR Am ) Nc u ro rndio l. 8 (2 ):353-9, 198 7
• CN7 palsy 10. Misra SK ct al: juvenile rha hdo m yosa rco ma of m id dl e car
• Clin ica l profil e: Ch ild under 5 yea rs with ch ron ic o titis and mastoid . Ea r Nose Th roa t }. 64(8 ):39 7, 19 85
m edi a. o to rrh ea & ea r pain 11. Chas in w u: Rhabdo m yosarco m a o f th e te mpo ra l bo n e.
Ann Otol Rhin ol Laryngol Suppl. 112:71-3, 1984
De mographi cs 12 . Wells SC: Embryo na l rhabdo m yosa rco ma of the ear : a
• Age review of th e litera ture and case hi stor y. } La ryngol Oto l.
o Bimod al , occurr ing in ch ild ren 2-5 years, and lat e 98(1 2): 126 1-6, t 984
teens 15 -1 9 years 13. Ca na lis RF c t al: Temporal bone findings in
o Rarely ca n occu r in ad ults rhabdo m yosarco ma wit h predom in antly petro us
involvem ent. Arch Olo la ryngol. 10 6(5 ):290 -3, 19 HO
• Gender: Sligh t male pred ominan ce
14 . Schw a rtz RII et al: Rhabdo m yosarco ma o f t he m idd le ear: OJ
Natural History & Prog nosis wolf in shee p's clo th ing. Pediat rics. 65(6): 1 13 1-3, 1980
15 . Goe pfert II et a l: Rhabdom yosarcoma of th e tempo ra l
• Delay to d iagn osis co m mo n , as ch ild is in itia lly t reat ed bo ne. Is surgical resectio n n ecessary? Arch Otola ryn go l.
for acute or ch ron ic o titis med ia lO S(6):3 10-3, t 9 79
• ME rhabdo is param entngeal type, ex tremely poor 16. Mahl ndra S et a l: Embr yo nal rhabdosa rcomn o f th e middle
progn osis d ue to in tracran ial spread &. distan t ear a n d m asto td] La ryngo l O to l. 92(3 ):253-8, 19 78
metastases 17. Gross CW tot a l: Rha bd o m yosa rcoma o f th e ca r; a cause for
• If local invasion into skull base, ex tracran ial sites & su ppurative ea r diseases in child re n. Arch Otolary ngol.
intracran ial co mpartme n t has occ u rred, resectio n fo r 90(5):609 - 16, 1969
cure im po ssible
Trea tment
• Prim ary t reatment is su rgical
IIMAGE GALLE RY
( I..ef l) A ' i al T-"(Jllt' C1 of

righ t car show s cotnl'lt'tf:'
middle ear-m asto id
op ociiicotto n. No le sulJl /e 2
lytic chan ge in tempof,)1
bone (arrows), indic ating 81)
underlying rhabdo is p resent .
(Rig"' ) Axial T1 C+ MR
rc vee ls J difiu.~ ely en hancin g
rhabdo myo sarcom a of
m iddle ear-mastoid. CT
sh o w ('(/ .HJb t/(· .1(('.15 o f b o ne
1().~ 5 . Olom(1)( oid ithi with M E
effusio n or eve n
choles /ealom J wo uld no /
enhance.
Typi ca l
(/. (·f l) A xial l-bone CT _~ h O\ V5
middle fw-masfoid ,~ f AC
opdcific,ll ioll. SulJlh.. lxm y
onwicm of cor ti cal walls of
[1\ ( (arrows ) 51Jf.:l-:l>StS .10
ilgWl·... .i w
PfO<.'f::'5 5. Wop sy of
CAe poly!, (l'W' ,lh' d
,h.1h doT! lym, trn ml,l. (Hig ht)
Ax/oJ/ ctct <ll'moll strates
m,lfkpd. homogeneou s
Pllhanc enll'nl in muktto
ear-CAC rhabdomyosarcoma
(arr ow s) w ith intr .1Cr.lni.l/,
eXIra-d ural e xtension ifJttl
middle Cfdflidl fm _...' (0 l't'n
arrow) .
Typi ca l
(LeJI) A xial T2WI M R sh()w~
middle esr ,habdo (.1rro w)
obstructing ddilUS ad an lfll lJl
(open arrow). Tumor is
slightly lowe' in Signal the n
o1J.wuctt'd St'Cft'tiOIlS in
I1Mstoi(/ air n'1/s (c u r w (/
..1frow ). (RiKhI) Coro nal T1
C+ MR roveeis di ffuse
t' Oh,l fl Ct.·m l.'Tll of middle
('.Jr-m ,ls (oid
,h.lbdomyosarcoma. Coron al
ima ge shOl vs tegmen
cJt,/* n >JKt ' (arr o w ) w it h
sutnte la/pral m '-' Il in~wal
in volvem en t (op en ar ro w ).
POST-OPERATIVE CEPHALOCELE, MIDDLE EAR
2
90
Ax';,11 ' -bone CT 5110\.\15 focal ceneceoce of anterior Coronal T-lx )I}(' a r('wals J pose-maseuidectomy ear
tf'gmm tympdni (arrow ) with proftusion of imfaCfclni.l l with focal ckohi'iCt.>nc(' of leg m m tympani (,1rfOLV).
L-Ofl/r.'n15 lhrough dehiscence. Surgery shOlvl.'(f small No tice the pt.'f..hmcul,lled ceph alocele hanging through
tt·mpof.lIIO/X· {·na ·ph.l/oce/e. the delliscencf:' (open Jrro w ).
ITE RM INO LO GY MR Find ings

• T l W I: Co ro nal ma y sh ow tem por al lobe her n iati o n
Abb rev iations a nd Syno nyms
• T2W l
• l'ostoperative men ingoencephalocele or encepha locele o Th in-secti o n coro nal best reveals cran ial co n ten ts
• Acq ui red ce p h aloce le ± CSF leak, T-ho n e h erni ation
• Misno me r: Neu rog lial h et erot opias • If men in ges &. brain : Men ingoen cephalocele
Def initions • If tem por al lob e o n ly: En ce ph alocele
• Prot ru sion o f cra n ial co n te n ts follow ing 'l-bo ne • If men inges o n ly: Men in gocele
su rgery o r tr aum a o If associat ed dural leak, high signal CSF in midd le
ear-m asto id
• T l C+: Po ssible min im al rim -en ha nce ment (sim ilar to
IIM AG IN G FINDING S int racranial h ypo ten sion )
Ge neral Features Nuclea r Medicine Findi ngs

• · l c-9 9 m ~ l)T PA cistern ogr a p hy ca n assist wit h possib le
• Best d iagnosti c clue
CSF leak
o Cf: Tegmen tympan i o r masto ld eum d ehi scen ce
o 1,,1R: Tem po ral lo be hern iati o n int o mi ddl e ea r Imaging Recommendations
• Locat ion : Tegm en tympani o r masto id eum • Bo ne CT I st to look for fo cal bo ne defect
• Size: f ew millimeter gap to cen timeter O f more • Pocu scd th in-sectio n MH used if CT po siti ve
• Morpho logy: "Ho urglass" sh a pe wit h ist h mus whe re a Co ronal T2 Mil defin es ce ph alocele co n te n ts
brain passes th ro ugh d eh iscen ce o T I C+ MR sh ows in t racr ani al co m plicatio ns if
CT Fi ndin gs presen t
• Men ingiti s, b rain ab scess ± subdura l em pyema
• NECT: Cor o na l bo ne C l: Focal bone d efect, usu ally o f
tegmen tympan i
• C ECT
a No en ha n cem en t, unless in fected (e.g., mening itis)
o CT cistc mog rap hy usefu l for detect io n of CSF lea k
DD x: Post -Ope rative Ce pha loce le Mimics
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Congen Cephalocele Acq Cholesteatoma Cholesterol C renulom a
POST-OPERATIVE CEPHALOCELE, MIDDLE EAR
Key Facts
Terminology Top Differ ential Diagnoses
• Protrusion o f cra n ial co n te n ts fo llowing T-bon e • Con gen ita l cepha locele, T-bone
surge ry o r traum a • Acq uired cholestea to ma with tegm en deh iscen ce
• C ho lestero l gran ulo ma of m iddl e ea r
Imaging Findings
• Cf: Tegmen tympani or mastoideum dehiscence Clinical Issues 2
• MR: Tem po ral lobe herniation in to middl e ea r • Pat ient with history of T-bo ne su rgery wit h m idd le
ea r mass ± CSF rhin orrhea 91
o Histo ry o f cholesteat o ma rem ova l, ch ron ic otitis

ID IFF ERENTIA L DIAGNOSIS med ia o r t rau ma
Co nge nita l ce pha locele, T-bon e De mogra ph ics
• Clin ical: No surgery • Age: Age at presen tatio n: SO years
• Imagi ng: Foca l bone defect ± brain her niati o n
Natu ral Histor y & Prognosis
Acquired cho lesteato ma with tegm en • Su rgical or trauma tic inju ry may be un noti ced
dehi scen ce • Ceph alocele may present im med iat ely o r years later
• Clin ical: Tym pan ic mem brane perfo ratio n wit h
cholestea to ma evide n t Treatm ent
• Imagin g: Oss icle lo ss, tegmen de h iscen ce, • Immed iate clos u re of defect is ma ndat o ry du e to risk
n on -de penden t soft tissue mass of life-threat en ing me n ingitis
• Transmasto id with mi dd le fossa :{ layer clos ure
Cho leste ro l granulo ma of middl e ear
• Clin ical: No n-pulsating blu ish mass + ret ract ed TM =
"blue eard ru m" !D IAG N O STI C CHECKLIST
o Ca n mimic po stop erati ve cepha locele at surgery
• Imagin g: High '1' 1 & T2 signal is ch aracte ristic Image Interpretation Pearls
• Define bone gap o n co ron al hone CT
• Clar ify ce phaloce le type wit h co ronal T2 MR
IPATHOLOGY
Ge ne ral Features ISELECTED REFERENCES
• Gene ral pat h co m me nts I. ja ckson CG et a l: Brain h ern iatio n in to t he mi d d le ea r a nd
o Encephalocele: Brain tissue only ma sto id: Co n cepts in d iagn osis a nd surgical mana geme n t.
• If arachnoid n ot intact, an en cepha locele resu lts Am) OtoI 18(2): 198-205, 1997
o Meni n goen ceph alocele: Men inge s &. brain 2. Lundy LB et a l: Temporal bo ne encep halocele and
• If arac h noid rem ains intact, a ce reb rospi n al fluid leak s. Am ] Oto I 17(3): -1 61·9,1 99 6
men ingoen cephalocele results 3. l'\ecly]G et al : Diagn osis and tr eat ment o f iat rogen ic
• Etio logy: Iatrogen ic po st-surgical lesio n cer ebrospi nal flu id leak and b rain he rniation d u rin g or
follo win g mastoidectomy, Laryngoscope 95 (11):1299·1 300,
• Epidemiology: Rare po st-su rgical co m plicatio n
1985
• Associated abn ormalities: Tear in meninges + CSF leak
Gross Patho logic & Surgica l Features
• Her n iated brain usually non-function al IIM AG E GALLERY
• No rma l o r necroti c CNS co n te n ts
!C Ll N ICA L ISSUES
Presen tati on
• Most co m mo n signs/sym ptoms
o Ceph alocele: Midd le ea r mass
o CSf leak: CSf rh inorrhea
o Oth er signs /sy m pto ms : Recu rrent m eningitis
• Clin ical pro file
o l'atlcn t wit h history o f T-ho ne su rgery wit h m idd le (/.('/1) Coro nal l -bonc..' CT shows bro,lClll'gmef) dl'hiscc'f)n' (aTrOlvs)
ea r ma ss ± CSF rhin o rrh ea with mil tissue in epilym pa num (oP(>/J arrow). (Rig hI) Coronal n c+
MR n-vcels meningouAe (arrow) h( >rnic1fin8 thro ugh U'gmen
dohiwvnco.
OSSICULAR PROSTHESIS
2
92
A xial /eft ear T-lxHle CT sllows incus intef/xJsilion gralt Ih ial T-bone CT reveil/~ nM~toidt'(' romy dt'{e("t with
(arrow) surrounu('( / by middle cor debris resulting in IJom ogr'lt i pm.~th(,s;5 (arrow) in din'Ct ap/x,",;t;O/l to
sound d lm,x'ning &. (('c urfm l conductive hl'ilring f()(. ~ . f}Orma/ ~ lape.~ (open arrow). Ap/X'.l(.l f)Cf" .~ imilar to
Opl-'n .mow: Mastoidectomy. traumatically di.sloc<lle(/ illcu.s.
• Lo cat io n
ITERM IN O LO GY o Mesot ympa num (m idd le ear cav ity prop er)
Abb rev iat ion s a nd Syno nyms • Stap es prosth esis: Localized to o val wind ow n iche
• Ossicular repl acem en t prosthesis • Size: Variab le, so me sta pes prosth esis are 3S gauge wire
• Stapes prosthesis • Morphology
o Incus inter posit io n
• Incus in terpos itio n
• Aut ogr aft (patient's ow n) o r homograft (cadaveric)
Definitions • Incus remo ved and resculpted to in terpose
• Su rgically im planted devices used to co rrec t between ma lleus &. stapes head
co nd uctive hear in g loss (CHL) • Usua lly perform ed for lo ng sta nding ch ro nic o titis
o Tota l ossicu lar replacem ent prosthe sis (TORP) med ia (COM)
o Partial ossic ular replacem ent pro sthesis (PORP) o TORI' & PORI' (Black, Richards, Go lde nbe rg)
o Incu s interpo sition: Incu s is ro ta te d betwe en malleus • Synthe tic pro stheses
Or sta pes • Util ized for mo re adva nced d isease
o Stapes pro st hesis: Prosthe tic replace me nt for sta pes • Co nnects tympan ic m embrane ('I'M) to stapes
head = PO RI'
• Co n nec ts 'I'M to stapes footplate = TORP
IIM AG ING FIN D IN GS • Head of pro sth esis is hyd ro xyapatite
• Shaft of prosth esis is pol yethylen e spo nge
General Features (Plastlpore, Sm ith & Nephew)
• Best dia gnostic clue o Sta pes prosth esis
o Prost hetic failure: Non-ana to m ic o rie nta tio n of • Synthe tic pro sthesis
prosthesis o n 'l- bone c r • Often wire or Teflon
• Req uires prec ise knowledge of normal ossicular • Olde r prosthesis we re larger a nd metallic
anatomy • Utilized most co m mo nly in co nj unc tio n wit h
• Disto rted appea ran ce of prosth esis o r abno rmal sta pedec to my for pati ents with fen estral
soft tissue lead s to expectation of prolapse o r o toscl eros is
othe r prosthesis malfu nction o Appleba um prosthesis
DDx: Range of Normally Po sitioned Prostheses
Wire Stape s Prosthesis Incus Interposition Goldenberg I'rosth. Applebaum Ptosth .

Key Facts
Termino logy • Axial an d co ro na l noncontrast 'l-bone c r best
• Total ossicular rep lacemen t prosthesis (TORP) imagi ng approach in eva lua ting oss icular pros theses
• Par tial oss icular replacemen t p rosthesis (pOR r ) • Become fam iliar with no rma l appea rances o f local
• Incus interpositi o n : Incu s is rotat ed bet ween malleu s neuro-oto logist's common ly used ossicular prostheses
• Review init ial c r wit h your surgeo n co rrelat ing
& stapes
• Stapes pro st hesis: Prosthet ic repl acem ent for sta pes otoscopic exam with cr findings 2
Imaging Find ings To p Differen tia l Diagnoses 93
• Incus in terpositio n: "Int erposed" between malleu s &. • Chron ic o tit is medi a with tymp an osclerosis
stapes head • Po st-t raum ati c inc us dislocati o n
• Appl ebaum p rost hesis: "L" shaped • For eign bod y in middl e ea r
• Black (ova l top) p rost hesis: Egg-shap ed Diagn o st ic Ch ecklist
• Go lde n berg p rost hesis: Flat hea d, o ff-cen te r sha ft • Co nside r prost hesis subluxation if hi stor y of
• Richa rd s p rosthesis: Flat head ce n tered on sh aft recu rren t CHI... o r vertigo
• Stapes pros t hesis: Artic u lated directly to oval wind ow
mem br ane
• Synt he tic br idge fro m resid ua l lon g process to • May become encased by debri s (gran ulatio n o r
sta pes head recu rrent cho lesteatoma) resulting in d iminish ed
• For patients with ab norma l in cud o stapedi a l joint fun ction
o TORI' or I'O RI'
Radi ogra phi c Findin gs • Suhluxa tio n: Most ofte n at ova l window nich e
• Rad iograp h y • Enca semen t (gran ulatio n o r cho lestea to ma):
o On ly me ta llic devi ces can be appreciat ed Abno rma l area o f soft tissue no t p resent
• Insufficien t for d iagn osis previously su rrou nd ing pro sthesis
CT Find ings • Ext rusion : Incid en ce increases in pati ents wit h
eusta chia n tube dysfun ction
• NECT o Stapes pr osthesis
o Incus int erposition: "In te rposed" betw een malleu s &
stapes head • Graft lat erali zati on: l'rosrhcsis may co mplete ly
di slocat e or may appear o nly slig h tly laterall y
• Normal incu s is "m issin g"
• Look s like "d islocated" in cu s if histo ry o f su rgery is po sitioned relative to oval window
not kno wn! • Post-stapedecto my gra nu loma-fibro sis: So ft tissue
o Appleba u m prost hesis: "I." shaped d evelops in oval wind ow niche 4·6 wks afte r
su rgery
• Articulates directly to stapes
o Black (oval top) prosthesis: Egg-shap ed • Regrow t h of fene stra l oto sclerosis: Usua lly obvio us
o n follow-up T-bone cr
• Cha rac teristic hor sesh oe appea rance
• Perilymp hatic fist ula: Rare; p neu molab yrint h o r
• TORI' o r r ORr
o Go lde n be rg p rost hesis: Flat head, o ff-cen ter shaft u ne xplain ed mi d d le ear effusio n may suggest its
p resenc e
• TORI' o r I'ORI'
• Design gives su rgeo n clear view and increases • Disloca ted in cu s (malleo inc uda! subluxatio n) may
occ u r du e to tor sional seq ue la resulting from
accuracy o f placem ent
prost hesis which is ma lpositionecl o r too lon g
• In creased ext rusio n rat es
o Richard s prosthesis: Flat head cen te red o n shaft MR Findings
• "Mod ified" Richa rds: o ff-cen te r head with a groove • MR no t needed o r usefu l for p rosthesis evaluatio n
fo r malleu s
• TORI' o r rO Rr Imagin g Re commendation s
o Stapes prosthe sis: Articu lated directl y to oval • Axial an d co ronal noncontrast 'I -bon e cr best
windo w memb ran e ima ging approach in evalua ti ng oss icu lar prost heses
• w trc, teflo n or meta llic • MR has 11 0 ro le in th is rad io logic evaluatio n
• \Vith in oval window nich e • Become famili ar with normal ap pea rances of local
• Mo st o fte n best see n o n axial im ages neuro-ot ologist's co m mo n ly used ossicular prostheses
• CECr: Co n trast not need ed o r reco m me nded for o Review init ial C f with yo u r surge o n co rrelati ng
prosthesis evalua tio n o tosc o pic exam wit h cr fin d ings
• Prosthesis Co m plications o n T-bone CT
o In cus interposit ion
• Subluxation: Incu s beco mes d isor ient ed relative to
TM & stapes
• No rm al postoperati ve baseline is usefu l
IDIFFERENTIAL DIAGNOSIS IDIAGNOSTIC CHECKLIST
Chron ic otitis medi a wit h tympan oscler osis Co nsider
• Th is er rant di agn osis co uld he made if clinica l h isto ry • Co ns ide r prosthesis sub lu xatio n if h isto ry o f recu rren t
is n ot soug h t C IIL o r ve rtigo
• Im agin g specia list m ust be fa m iliar wit h n or ma l
2 prosthesis a p pea ranc es
• Must have clini cal &: su rgical h istory
Post-trau matic inc us dislocation • Mu st have kn owl edge o f fo llowing pr ior to e valua tio n
'14 • May appear ident ical to in cu s interpositi on graft o No rma l o ssicu lar an at o m y
• Incu s m ay be found a nywhe re in middle ea r o r EAC o No rma l p rost hesis ap peara n ce
o Det ailed su rgica l hi sto ry
Foreign body in middle ear
• Clin ical hi sto ry is crucia l
IPATHOLOGY I. Kelly DJ et a l: The effec t of prost hesis d (,' ~i g ll o n vlbratl o n
o f th e rec o n str ucted oss icu lar cha in : a co m parative fin ite
Gen e ral Features cle me n t a nalysis of four prosth eses. 0 10] Neuro tol.
H( I): 11-9.2003
• Et iology 2_ Scn nurog lu Let a l: Antero po sterior approach wit h split ear
o Prostheti c dysfun cti on ca nal for coc hlea r implan tation in wvcrc malformat io ns.
• Recurrent cholestea to ma & ot itis med ia Otol Neurotol . 23( 1):39-42, 2002
• Trauma 3_ Keslin g S ct al : CT and ~Il{ imaging after m iddle ear
• Su rgica l erro r sur gery. Em J Radio!. 40 (2 ): 1 1;{· K, 200 I
• Fo rm ati on of gra n u latio n &. ad hes io ns 4_ Sf.:hdd SC et al: l'u cumolabyrtnt h : a late co m plicat ion of
sta pes sur gcry. Ear Nose Throat J. HO( 10):750- 3, 2001
• Mecha n ical probl em s including su bluxation ,
ex t rusio n & fract u re
s. Rang heard AS et .11: Posto pe rative co m plica tio ns in
o to spongtos ls: usefulness of MR imagin g. AjNR.
22 (6): 1171 -H, 200 1
Stone JA et a]: c r evalua t ion of prosthetic ossicular
ICLINICAL ISSUES reco nst ruction procedures : \VIMt t he o to log ist needs to
know. Radiot i rap h ics 20 :59J-h0 5, 2(XKJ
Presentatio n 7. l'ickuth D et al: Vertigo afte r stapes .!<lurgt.·ry: th e roll' of
• Mo st co m mon signs /sy m pto ms h igh resolutio n CT. Rr J Rad ial. n(R73): 102 1-:{, 2000
o No rma lly fun ction in g device H_ Niyazov D ct al: rc nest ranon surg l'ry for otosclt..rests: C I'
• lIi sto ry o f m iddle ea r su rgery fo r CHL findi ngs of an o ld surgical proced ure. AjNR. 2 1(9): 1670-2,
2000
• Precise kn owl edge o f os sicu lar a na to my is cruc ial
'1- Schwetschcnau El. et al: Osslculoplasty in yo ung children
o Prost het ic malfuncti on with th e Apple baum incudostaped la l joint prosthesis.
• Recurren t C HI. o r vertigo weeks to mon th s afte r Lary n go scope . 109( I 0 ): 162 1-5, 1999
successfu l surgery 10. Co llett i V et al: Malleu s-tu -foo tpln tc prosth eti c
ln tcrposi tlo n : experience wit h 265 pat ients. Ot olu ryngo l
Dem ographi cs Head Neck Su rg. 120 (3 ):4:U -44 , 1999
• Age: All ages I I. Albu S et al: I'ro gn o vttc factors in tympanoplast y. Am J
o ror. 19 (2 ):136-40 , 1998
Natural Histo ry & Prognosis 12. Zhe ng C et al: Ossiculoplasty by in te rposition o f a mi nor
• Depends o n skill a n d experie nce of su rgeo n col umella between th e ty m pan ic membra ne and 'ita pt.'S
• Stapes p rosthesis head . Am J Ot nl. 17(2 ):200..2, 1996
o Relat ed to longev lty I'< severi ty of fen est ral l3. Kovling S et al : Value of co m p u ted to mogra phy in pat ient,
o to sclerosis with pe rsist ent ver tigo aft er sta pes surgery, Invest Radlcl.
'. 30( 12):7 12-5 , 199 5
• Other pro sth etic d evices 14. Muk her]i SK et al: C1' of the tempora l bone: Fin d ings after
o Recu rren ce-prog ression o f chronic o to mastoi d itis is m astoidect om y, o ssicular recon st ruct io n, a nti coc hlear
a n impo rta nt risk facto r implantation. t\jR 16] :1467-7 1, 19 94
15. Cho le RA: Osstcu fo plasty wit h co mposite prosth eses: PORI'
Treatm e nt .:Jn L! TO IU'. Ot olaryng o l Cli n No rth Am 27:727- 45, 19 94
• Chron ic oto masto id itis ± acquir ed cho lesteato ma 16 . Hirsch Br. et al: Imagi ng o f oss icul ar pro stheses.
o Tym pa noto my \v·it h ossicu lar reco nst ruction Otolaryngolllead Neck Surg. 111(4 ):494 -6 , 199 -4
o Mast o idect omy with ossicu lar prost hes is pla cem ent 17. Wein s RE: Incu s interposition and ossiculoplasty \...ith
• Fenes t ral o tosc lerosis hy droxyapatite prost heses . Otolaryngol Chn Nort h Am.
o Sta pedecto my wit h stapes p rosthesis insertion 2 7(4):677-8H. 1994
IH. Shcll ock FG et .11: Metallic ot o logi c implant s: in vitro
• Prosth esis malfu n cti on
assessm ent of ferromagn etism at I. S T. t\jN R. 12(2 ):279·H1 ,
o Usually req u ires re-o pe ratl on 1991
IIM AG E GALLERY
Typical
( 1..1'/ 1) Ih id/ left eer t-txxw
CT shows normally
pmirioned Sl.Jpes pro:.. thesis
(arr o lV) with irs medial tip in
2
direct dpposirion to ova l
window membran e (open 95
arrow). ( RighJ) Coronal left
m f I-bon e CT ~ llows radical
m.lsloicJ('ctom y dok -ct with
LJI<lck. "ovel-top " IO Rr wil h
d('me h('iJd (arrow) ,f,. lo wer
.1It"'fJua tion Sh.l /; (op. ·n
_mow ). Shaft a 'ti("UJfllt~ with
ova/window membcuw.
(lA'l t ) A xial/eft ear I -booe

("1 (eveals 5u bflp wait
"lleraliza tion. No tice 'hal
medial tip o f stapes
prm t!U'.~i5 (.1((Ow) i.~ flO
IOllgt" ill direct ,lppmilioll to
ova l window m('mhraot'
(OPf'fl arrow ). (RighI) A~ ;J I
'('ft e,lf T-bone CT (' veals
di s/oc .lt (yf mt·tJlIic piston
S lap('~ p ro.-.rhpsis (arr ow)
wh ich hd5 migrated
in fero laterally 10 abut
tympanic m('mbr,lIw.
(1"'11) Coro nat !('(t edr T-bont'

Ct (ll'monstral f' S f".lo: trucftxl
~ t .l Pt:1S prosthesi.;which has
miW<1 /cd into Y(~s tilJU/('
«lf ro w) u;>sulting in
v('rtiginnu.'; complaints in
addition to rec urrent
co nductive /waring loss.
(RighI) Axial t-bone C I
shrnvs anterio rly positioned
stepcs prosthesis (arfO\Y)
lying in direct apposition to
oval window membrane.
Regrowth of otosclerosis
(open enow) h.l5 re.~u/loo in
rt'Cllffent O IL.
LABYRINTHINE APLASIA
2
96
Axial graphic depicts labyrinthine apl<1.~ia . Note the A xi al T-bonc C T shows comp lete ab5('(l(l' of all inner
complete absf.'nce all inner ear ~tructu(es with the ear .~ tructures diagnostic of lahyrinthine aplasia. No te
exceptio/l of small lAC with only eN7. Latera! wall of the l1at latN<11 wall of innL'r eer (arrow ).
inner ear j_~ flattened (arrow ) .
• Petrou s apex h yp opl astic

ITERM INO LO GY • Middle ear no rm al
Abbreviat ions a nd Syno nyms o Mo re severe form o f labyrinth ine aplasia
• Co m plete lab yrin thin e apl asia • Absent lAC
• O ld syno ny m: Mic he l d eform ity • Petro us ap ex bo ne abse n t
• Midd le ea r abnorma l wit h o ssicle ab sen ce or
Definiti ons fusion
• Co m plete abse nce of co ch lea, vestib ule & sem icircular o Lateral wa ll o f inner ea r is FLAT
ca nals a Facial nerve course
• Facial nerve bony ca nal is prominent
• Geniculate gang lio n is posterior to normal
IIM AGIN G FINDINGS lo catio n
General Features MR Find ings

• Best d iagn o stic clue : Absence o f en tire in ner earl • Tl WI: Mem bra nous labyrin t h not seen o n T1 due to
including coc h lea, vestib ule and sem icircular ca na ls air & bone of tempor al bone
• Locati on : In n er car • T2W I
• Morphology: Labyrinth fails to d evelop, so amorph ou s o No normal high signa l int ensity fluid in
hon e in place of coc h lea, vestibule & semici rcular membran ou s labyr in th o n h igh-resolution T2WI
canals o Sagittal T2W I th rough lAC show norma l CN ? &
with abse n t coch lear-vestibular co m plex
CT Findin gs
• NECT
Imagin g Recommendations
o Spec t ru m o f 'l-bone CT finding s d epends o n severity • Bone CT is sing le best diagn ostic m odality
of ano ma ly • If MR d one first, CT usually necessar y to con firm
o Mild form of labyrin t h ine ap lasia di agn osis
• Petr ou s hone lacks coc h lea, vest ibule &
sem icircu lar cana ls
• lAC sma ll but p resen t
DDx: Inn er Ear Lesions
Cochlear Aplasia CCVA LESA Labyrinthine Ossiiicans

LABYRINTHINE APLASIA
Key Facts
Termino logy To p Differ ential Diagnoses
• Old syno n ym: Michel deform ity • Coc hlear apla sia
• Co mplete absen ce of cochlea, vestibu le & • Co mmo n cavity deformity
semicircular canals • Cystic cochleovestib ular ano ma ly (CCVA)
• Large endolymp hatic sac ano maly (LESA)
Imaging Findings • Labyri n th ine ossificatio n, ohlite rative type 2
• No normal high signal int en sity fluid in membra nous
lab yrin th o n hig h-resol ut ion T2W l Clinical Issues 97
• Bone c r is sing le best d iagno stic modality • SNHL from birth
IDIFFERENTIAL DIAGNOSIS Dem ographi cs

• Age: Co ngenital, present at birt h
Cochlea r apla sia
• Late 3rd week arrest with absent coc hlea, dysmorphic Natural History & Prognosis
vestib ule &: sem icircular canals • Affected ear will never hear
Co mmo n cavity deformity Treatm en t
• 4th week arrest wit h ov oid si ng le i nner ear cavity only • If unilateral, n o treat men t ind icated
• If bilat eral, pro found deafn ess is present
Cystic coc hleovestibular a no ma ly (CCVA) o Cochlear implantation not an option
• 5t h week arrest with "figu re 8" cystic coch lea &:
vestibule with sem icircular ca nal dyspla sia
l arge e ndolympha tic sac ano ma ly (lESA) IDIAGNOSTIC CHECKLIST
• 7th week in ner ear arrest with large end o lymp hatic Co nside r
sac, coch lear dysplasia
• Labyrin thi ne aplasia if hony lab yrinth is featu reless,
labyrinthine ossificati on , obliterative type wit h no coc hlea, vestib ule o r sem icircular canals
• Post-men ingiti s inner ear o ssificatio n • Lateral wall of otic capsule is r LA'I~ as o pposed to
co n vex
IPATHOLOGY
Gen eral Features l. Scnnaroglu L et al: /\ new class lflcatlo n fo r
• Gene ral path co m me n ts coc hleovest ibula r malfor matio ns, Lary ngoscope.
o Emb ryo logy 112:2230- 4 1, 21X)2
• Arrest o f otic placode developmen t at J rd 2. Sch uknec ht IIF: Pat ho logy of th e Ea r, 2nd cd . Phllad clp hln:
gestati on al week Lea and Feblger, Vo l. t HO.I , 1993
• Genetics: Autosom al dominant inh erit an ce 3. j ackler R ct al: Co ngenita l ma lformati o n of the In ne r ca r.
Laryn goscope. 97:2- 14, 1987
• Etio logy: Cause of arre st at J rd gesta tio n week
un known
• Epidemio logy
o Extremely rare IIM AG E GALL ERY
o Acco unti ng for < I1r6 o f all co ngen ital inner ear
ma lfor matio ns
• Associa ted abno rmalities: Seen in Klippel-Fell
synd rome &. t halidom ide exposure
Gross Pathologic & Surgica l Features
• Failu re o f bony &. me m brano us labyrin t h forma tio n
• Inner car st ructu res not presen t
ICLIN ICA L ISSUES

(l.ej l) Axial l -bone C1" shows absence of inner {'Jr Wuctures
Presentati on consistent with labyrinthine aplasia. N ote middle car ;s clysmorphic
• Most co mmo n signs/symptoms (arrow) with tym panic CN 7 seen (open arrow ). Small lAC (curve-d
o SNHL from birt h arrow). (Righi) Conmsl Fbone CT reveals d normal external ,w ditory
o Extreme ly rare canal & midd/(~ caf, /J ut comp lete lack of formation of otic cepsule
(arrow) . COCh/t->ol, v('stibu/(' amI w micircutor canal.~ , Iff' .lbWIl/.
COCHLEAR APLASIA, INNER EAR
2
98
Axial graphic depicts classic example of C()(""t W 1v. i.]1 booc a shoo.''5 C:OCh/(',lf '1JJ/.l.~ i..l J.5 ab!il'1ltcoc/Jk'il
apl<lsi.J. The drawing 5/IO'tVS an alN'flt cocllk'a with (arrows ) with mildly dy¥>1.btic \'f!'5tib ule (open .1ffoov).
(YJ/.l fgt'C- f vestibule & dysplastic ~("JJidfCUI. 1f ("arMIs. Nott' l.lbyrinthilH:' eN 7 (cu(\,('d ,Irra-v) is posterolateral
Note <l rnffl<"e of cochlear nerve. lO rKXmJl location.
• La byrinthi ne, ge n icu late ga nglio n & an te rio r

ITERM INO LO GY tym pan ic po rtio ns o f facial ner ve occu py site
Abbreviatio ns and Syno nyms wh ere coch lea sh ou ld he
• Absen t coch lea • EAC. mi d d le ea r, o ssicu lar ch ain, bo n y vesti b ula r
aq ued uct & en dolym ph atic du ct are no rm al size
Definiti ons
• No co ch lea is presen t b u t vesti b u le, sem icircula r canals
MR Findings
&: in ter n al aud itory can al (lAC) a rc p resen t in so me • T2WI
for m o Th in -sectio n T2 MH shows absen ce o f coc h lea
o Ob liq ue sagi ttal T2 shows sm all lAC with n o
coch lear nerve
IIM AGING FINDING S o Marrow o r co rtica l ho ne signal int ensity re places
coch lea site
Ge ne ra l Feat ures o Vestibule, sem icircula r ca nals I< lAC are va riab ly
• Best dia gn o stic clue: Absen t coc h lea, m ost co m mo n ly affected
assoc iated wit h dysmorph ic ves tib u le N. semici rcu lar Imaging Recom me nd atio ns
canals
• If using high-resolutio n M R as 1st di agn o st ic to ol ,
• Locati on : Anteri or membranou s labyrinth
• Mo rp holog y: Featureless an te rior bo ny la byrin th
recom me n d foll owing wit h T-ho ne to m ore cr
precisely defin e o t he r in ne r ea r ano ma lies
CT Findings
• NECf
o Co m plete abse nce of coch lea o n T-bone cr, may be IDIFFERENTIAL DIAG NOSI S
bilatera l
• Vestibule, sem ici rcu lar ca nals & lAC ma y be
l abyrinthin e a plas ia
n ormal, hypoplastic o r dil at ed (cystic) • Clin ica l p rese n ta tio n: Co n gen ital senso rine ura l
• Coch lea r promonto ry is flat , instead o f n ormal hear in g loss (SNIIL)
lateral co n vex ity when coch lea is p resent • Imaging: Coch lea. vesti b u le & semicircu lar ca na ls
abse nt
DDx: Inn e r Ear l esions
Labyrinthine Aplasia Common Cavity CCVA Lab. Ossiticen »

COCHLEAR APLASIA, INNER EAR
Key Facts
Terminol ogy • If using high-resolution MR as 1st diagn ostic tool,
• No cochlea is present hut vestibul e, sem icircular recommend following with T-bon e CT to more
cana ls « in te rn al aud itory ca na l (lAC) are prese nt in precisely defin e othe r inner ea r anomalies
some form Top Differential Diagn os es
Imaging Findings • Lab yrinthine ap lasia
• Co m mon cavity deform ity
2
• Com plete abse nce of coc hlea o n T-bone Cl , may be
bilat eral • Cystic coc h leovestibu lar anomaly (CCVA) 99
• Thin-section T2 MR shows absen ce of coc h lea • Laby rinthine ossificans (Lab. ossificans)
• Em bryoge nes is: Developmen tal arres t, 3rd gesta tional

week Natural History & Progn osis
• Involved ear w ill ne ver hear
Co mmo n cavity d eformity
• Clinic al present atio n: Co ngen ita l SNfl L Treatm en t
• Imaging: Coa lesced cystic coc h lea & vestib u le form a • If co ntra late ral car is n o rmal, patien t will hear
com mo n cavity • Coc h lear im plant is co nt raindicat ed as th ere is n o
• Embryogenes is: Develo pm en tal arrest in 4th week coc h lea to im plant
Cystic coc hleovestib ular a no ma ly (CCVA)

• Clin ical presentatio n: Co ngen ital SN H L IDIAGNOSTIC CHECKLIST
• Imagin g: Coc h lea & ves t ibule are cystic with n o
int ern al arch itecture Co nsid e r
• Emb ryogenesis: Develop men ta l arrest, 5th wee k • Coc h lear aplasia if n o coch lea is seen o n c r or T2 M R.
but rest of memb ran ous labyr in th presen t
Labyrinthine ossifican s (La b. os sificans) • Be su re to d ist in guish from o blite rative labyrinth ine
• Clin ical present ation: Acq uired SN H L, usually ossifica ns
following meningitis
• Imaging: Dense bone fills mem bran o us labyrin th
including coc h lea ISELECTE D REFERENCES
• Normal lateral hu lge of prom onto ry p resen t
I. Sen nar og lu L et al: A new classifica tio n for
coc h leovestibular ma lformat ions. La ryngosco pe.
IPATHOLOGY 2.
112 :223 0· 41 , 2002
Schuknech t HF: Pathology of the ea r. 2 nd ed. Philadelph ia:
Lea and Febig c r, Vol. 180-1, 1993
Ge ne ral Features 3. jacklcr R et al : Co ngeni tal malfo rma tions of th e inner ca r.
• Ge ne ral path co m me n ts laryngoscope. 97:2-1-t, 1987
o Em bryo logy
• Arrest of o tic placode deve lop me nt at late 3rd
gesta tio na l week IIM AG E GALLERY
• Etiology: Un known
• Epidemiology
o Extreme ly rare inner ear anomaly
o < 1% of all in ne r ea r co nge ni ta l lesio ns
• Associa ted abnorma lities : Vestibule & lAC may be
dilated
Gross Path ologic & Su rgica l Features
• Absen t coc h lea, rem ainder of inner ca r also usuall y
abnorma l bu t pr esen t
ICLIN ICA L ISSUES

(I .e! J) Axial bone CT sho ws coc btes r absence (aplasia). Vestibule is
Presentati on cli/<lted &- spvt' reJy dysmorphic (arro w) . The genicu /at(' ganglion
• Most co m mo n signs/sy m pto ms (ope n arrow) is seen in normal location of upper coc"'ea . (Righ ,)
o Co n gen ital SNHI., usually bilateral Axial bone CT r('vc'Jls dilatpd vestibule (open arrow) ,Ii: postoriot
o Co m plete SNHL from birt h wmkirculer canal but complete lack or il cochlea (arrow ). MidriJp
ear is norm al; lAC is sma ll due to ebscn! coc hlear nerve.
Demographi cs
• Age: Co nge n ital, present at birth
COMMON CAVITY, INNER EAR
2
100
A'J(;al graphic roy<; feallJR'S 0'- common cavity Axial bono cr ~h()W5 coclJlecl ~\Z veslilw/l' as common,
111.lltOm Mt ;O{}. N ote ('rx-h1eJ "X H")/ ih u/e art' mddl'd int o f(,<lturP/('s.~
cavity wilhou l differentiation. lAC is small,
one commoll c~ t. 51'1nicircu!.lf cd lJ<1 /~ .lJl.' f10 t distinct semid rcul.l r canals .l /)s{·nt, middf(' ('ar & ma~ t()id.~
(rom cystic V{'5tib u/.l( componenl. wpll-form('d.
• Small lAC seen when co mm on cavity is small

ITE RM INO LOGY • lAC commonly ent ers cavity at cente r po rtio n &
Abb reviatio ns and Syno nyms fun du s defective
• Cystic co m mon cavity o Middle ear structures, includin g ossicles, &
vestibula r aq ued uct are no rm al
Definiti o ns
• Co m mo n cystic cavity represen ti ng rudim en tary
MR Fi nd ings
coc h lea & vestibule • T2WI
o High signa l inte ns ity fluid with in featureless cystic
cochlea &. vestibu le &. absen t modi olus
IIM AGING FIND ING S o SCC usually absen t bu t may be nor m al or d yspla stic
o lAC about 50% no rmal size d ue to small o r ab sen t
Genera l Fe atures CN8 components & normal CN?
• Best d iagno stic clue: Feat ureless co m mo n cav ity Imaging Re commendat ions
represen ti ng rud iment ary coc hlea, vestibu le &
• Bone CT of tem poral bone is sing le best d iagn ostic
sem icircular canals (Se C)
mod ality
• Locatio n : Inn er ear memb ran ou s labyrinth
• '1'2 oblique sagitta l 11,11{ th rough lAC used to assess
• Size: Co m mo n cavity may be small (proba bly earlier
presence of coc hlear nerve
develop mental arrest) O [ large
• Morphology: Ovo id cyst
CT Fi nd ings ID IFFE RENTIAL DIAGNO SIS
• NECf Cochlea r ap lasia
o Coch lea, vestibule & sec for m com mon cavity o f
variable size • Imagin g: Absent coch lea, vestibu le ma y be no rmal or
o Sem icircular cana ls usually ab sent hypo plastic
• May be norm al o r d ysplastic • Em bryoge nesis : Developmen ta l arrest lat e 3ed
o lAC always abno rma l gestatio nal week
• Large lAC fo un d wit h large co m mo n cav ity
DDx: Inner Ea r Co nge nita l Lesion s
Cochlear Aplasia CCV Anom aly CCV Anomaly U SA

COMMON CAVITY, INNER EAR
Key Facts
Terminol ogy Top Differ ential Diagn oses
• Co mmon cystic cav ity representing rudim entar y • Coch lear ap lasia
coc h lea &: ves ti bule • Cys t ic coch leovest ibula r an omaly (CCVAj
(incom plete partition, type 1)
Imaging Findings • Large en dolym phatic sac anomaly (LESA) (incomplete
• Coc h lea, vest ibu le & SCC fo rm co m mo n ca vity of partition, type 2) 2
variable size
• Semicircu lar canals usually abse n t Pathology 101
• Rar e, < l l}(}of a ll co nge n ita l in ner ear m alfo rm ations
Cystic coc hleovest ibu lar anoma ly (CCVA) Natural Hi story & Progn osis
(inco mp lete partiti on , type 1) • Successfu l co ch lear impla nta tio n (C I) has been
• Imaging: Coch lea & ves tibule are norma l size, bu t perf ormed in co m m o n ca vity d efo rm it y, sugges ting
cystic witho u t inter nal architecture so me coch lea r nerve flbc rs pr esent
• Embryogenes is: 5th gesta tio na l week develo pm en ta l Treatm ent
arrest • If bil ateral , SNIiL is co m plete; CI m ay be co ns ide red
Large end o ly m p hat ic sac anomaly (LESA)
(inco mplete par t iti on , type 2) IDIAGNOSTIC CHECKLIST
• Imaging: Bilateral large endo lymp ha tic sacs + coc h lear
d ysplasia Co nside r
• Emb ryogenesis: Develo pmental a rres t in 7t h • Com mon cavity w he n coc h lea &: vestib u le fo rm sing le
gestationa l week cavity wit h out differen tiati on
Im age Interpretation Pearl s
[PATH O LO GY • "1'2 sagitt a l MR im ages th rou gh lAC necessar y to
determi ne pr esen ce o f coc h lea r nerve if CI p lan ne d
Gene ral Features
• General path com men ts
o Com mo n cystic cav ity represen ting coc hlea and ISELECTED REFERENCES
vestibule, withou t internal archit ectu re I. Sen naroglu L et al: A ne w cla ssifica tion for
o Em bryology coc h leovestib ula r ma lfo rm ation s. La ryngosco pe. 112:
• Arrest o f development at -lth gestati ona l week, 2230-4 1, 200 2
af ter d ifferentiati o n of o tic (aud ito ry) placode int o 2. Sch u kn ech t HF: Path ology o f th e Ear, 2 nd cd . Phil adelphia:
otocyst Lea an d Febiger, Vol. 180-1, 1993
• Etiology : Unk no wn 3. ja ckler R ct al: Congen ita l malfor mati on o f t he in n er ea r.
• Epide m iology La ryn go sco pe. 9 7:2- 14, 198 7
o Rare, < l'MI of a ll co nge n ita l inn er ea r mal for ma tio ns
o 25% of a ll coc h lea r m a lformat ions
IIMAGE GALLERY
Gross Path ol ogic & Su rgical Feat ures
• Average size of cyst: 7 X 10 m m
Microscop ic Features
• May be so me different iation of o rga n o f Co rt i, bu t
n eu ral populations absen t or low
ICLIN ICA L ISSUES

Presentation
• Most co m mo n signs / sy m pto ms : Co nge n ita l
sen so rin eu ral h earin g loss
Demograph ics (I.eJI) Axial N fC T !ihOW5 fpature/C's5 cochtos. vestibule (~ sem icircular
canals as Single cystic stru c fUre. Ossicular chain, middle ( '<If S
• Age: Hea rin g loss present at b irt h
mastoids air cells are normet. (l lilfhO Axial bone CT shows lAC
entering anterior aspe ct o j co mm on cavity (arro w). Middle ear .':
m astoids form laler, d uring 2nd trimester & are normal. Tiny po sterior
sem icircular canal (open arrow).
CYSTIC COCHLEOVESTIBULAR ANOMALY
Key Facts
Termi nology Top Differential Diagn ose s
• CCVA: Arrest o f inner ea r development at 5t h week • Coch lea r aplasia
o f em bryogenesis • Common cavity deformity
• Larg e endo lym phatic sac ano maly (I.ESAj
Imaging Find ings
• Morphology: Coc h lea « vestibule hav e ' figure 8" Clinical Issues 2
co nto ur with no internal features • Most co m mo n signs /sy m pto ms: Co ngen ita l SNIIL
• lAC nearl y normal in size, with normal eN7, but from birth 103
coc h leovestibular nerves deficient or absent • Coc h lear implant relative co n traindication
Co mmo n cavity d efo rmity Trea tm ent

• Clinica l presentati on: Co ngen ital SNHL • If co nt ralate ral ea r is normal , pati ent will hear & no
• Imag ing : Cystic coc h lea &. vesti bule form a co m mo n treatmen t indi cated
cavity • Coc h lear im pla n t relative con t raindica tion
• Emb ryoge nes is: Developme n ta l a rrest in -lth
gesta tio na l week
IDI AG NOSTIC CHECK LIST
Lar ge e ndolymphatic sac ano maly (LESA)
• Clin ical presentation : Cascad ing bilateral SNI IL in 1st Co nside r
yea rs o f life • CCVA diagnosed if coch lea & vestibul e have
• Imaging: Bilat eral large endo lym ph atic du ct &. sac rud iment ary co n tours, but no int ern al arch itecture
wit h mild coch lea r dy srnorp h ism
• Embryogenesis: Developmen ta l arrest in 7th week Image Inter pre tation Pearls
• '1'2 MR sh ows fu ll exte n t of lesion, incl udi ng status of
CN? & CNS in lAC
I PATH OLOGY
Ge nera l Fea tures ISELECTED REFERENC ES
• Ge ne ral path comments I. Scn na rogl u L et al: A new cla wl flcat lon for
o Embryol ogy coc h leovesti bu lar m alfo rm at ion s. Laryngo scope.
• Arrest of otic placod e developme nt at - 5th J 12:2230-4 1. 2002
gestatio nal wee k 2. Sch ukn ech t HF: Path ology o f th e ear, 2n d cd . Phil adelphia:
• Etiology: Un kno wn Leu and Fcbiger, Vo l. 180- 1, 199 3
• Epide miology :1. j ackler Ret al: Co nge n ital mal fo nnat lon s o f t he in ner ear.
o Rare in ner ea r a no maly Lar yn go sco pe. 9 7:2- 14, 1987
o CCVA accounts for < 2% o f all conge n ita l
labyrin th in e lesion s
IIM AG E GALLERY
Gross Patho logic & Surgica l Featu res
• Dime nsio ns o f coch lea & vestibule n orm al, but
in tern al archi tecture lacking
Micro scopic Fe atu res
• Coc h leo vestibula r nerv es deficient
• Int ernal arch itectu re o f coc h lea & vestibu le lack
n orm al st ructu res

Presentati on
• Most Co m mo n signs/ sym ptoms: Congen ital SNHL (I.e/I) Axial bono CT in cochlro W !>libu/a, anom.l/y shows ditstod
fro m birth CO( "'t'.1 & yestibule with 110 intt'm.ll 5(ruclUn'S viMblt'. Nolf' norm JI
ossictes & middte mr with incident dl high-riding jugular bulb (arrow) .
Demograph ics (RighI) Axial bone C I shO\.vs - figun' 8 - appt.>ar,lnct> of
• Age: SNHL usuall y detect ed in first several years o f life cochlroyestibu/ar anomaly. Appeersnce is rim' to dilated but wp"',ltt'
• Gender: Lesion is ext remely rare, bu t no known coc btes &. vestlbute . Note sbsen t modiolus. Normot osstctes & middle
gender predlsposi tion ('a' prosom.
Natural History & Progn o sis

• Ear will never hea r
SEMICIRCULAR CANAL DYSPLASIA
2
104
Axial Wclphic depicts severe, syndramie type of Axial T-bone a shows dilated vestibule fused to lateral
semicircular canal dy~plasiil with complete Jb~'nce of semicircular canal. Note lack of cone is/and in centra!
,111semicirwlar canals, coc"h/('ar dysplasia g dysmorphic portion of VBtibufe. f'mf erior semicircular canal is
small vestibule (etrow ), hypoplastic (aff()1,v).
• Cen tral bo ne island in lat eral SCC normally 3.7

ITERM IN O l O GY rnm, < 3.3 mm with dysp lasia
Abbreviations a nd Syno nyms • Posterior & sup erior SCC m ay be normal, dil ated
• Abbreviati on: Sem icircular canal dysplasia (SCC D) or hypoplastic
• Syn onym : Sem icircular ca na l-vestibule dy splasia • Coc h lea can be norma l or in complet e apical Sr
middle turn partition
Definitions • Midd le ea r & mastoids variable, ran gin g from
• SCCD: Malformati on, hypop lasia or ap lasia of one or normal to ossicular anoma lies
all of sem icircu lar ca nals (SCC) • Oval wind ow atr esia co mmo nly associa ted
o Next most common is SeCD assoc iated with
CHARGE syndrome
IIM AGING FINDINGS • All SCCs absen t in both ears
• Vestibule sma ll &: dysmorphic
Gene ral Features • Oval window atresia always present
• Rest diagn ostic clu e: Spec trum o f anomalies, with • Tym pa nic segme n t of facial nerve may be found
sho rt, dila ted lateral SCC I< vestib u le form ing sing le overlying atretic oval window
cavity most com mo n appearance • Coc hlear anoma lies usually associ ated
• Locati on : Posterior membran ou s labyrin th • Most com mo n coc hlear a no ma ly: "Isolated
• Size: Varies fro m aplastic SCCs to en larged & dil ated coch lea" with lack of coch lear a perture
vest ibule-late ral SCC o SCC dysplasia or aplasia may he assoc iated with
• Morphology: SCCs may be sho rt I< dilated , wide or labyrinthine aplasia, coch lear hypopl asia or
aplastic co mmon cav ity deformit y
• Othe r more mild forms of coc h lear dysplasia also
CT Findin gs possible
• NECT
o Most common, least severe appearance of SeeD is MR Findin gs
dilated iat eral SCC forming sing le cavity wit h • T1WI: Labyrinthine structures poorly visualized by TI
vestibule MR seq ue nce s
• T2WI
DD x: Inn er Ear l esions
Common Cavity CCVAnomaly LES Anomaly Labyrinthine Ossificans

Key Facts
Termino logy Top Differe nt ial Diagn oses
• seeD: Malform ation , hypopla sia or aplasia of one or • Common cavity deformity
all o f sem icircular canals (SeC) • Cystic coc hleovestibular (CCV) anomaly (II' type I)
• Large endolymphatic sac (LES) anoma ly (II' type II)
Imaging Findin gs • Labyrint hine ossificans
• Most commo n, least severe appearan ce of seeD is 2
dilated lat eral s c e forming single cavity with Patho logy
vestib ule • see aplasia or h ypopl asia may be part o f genet ic 105
• Posterior & superior sec ma y be normal, dilated or syn dromes
hyp opl astic • Etiology: 6-8 week gestatio nal arrest or in sult
• Next mo st commo n is SeeD associat ed with
CHARGE syndrome Clinica l Issues
• All s e c s abse n t in both ears • Sens orineura l hearing loss (SNHL)
• Oval window atresia always presen t • Conductive hearing loss oft en pr esent due to oval
• Most com mo n cochlear anoma ly: "Iso lated cochlea I! window atresia & ossicular cha in ano ma lies
with lack of coch lear aperture • Bilateral syndromic SeeD ma y ben efit from coch lear
implantation
o Sporad ic Se e D • Midd le &. apica l cochlear tur ns fo rm commo n cavity &:

• Com mon sac formed by dilated vestibule & sho rt, associated dila ted vest ibule
Wide, dysmorphic lateral sce • s ees usuall y normal
• Posterior & lateral se cs usua lly normally Of
mildly dysplastic Lab yrinthin e oss ifica ns
o Syndrom ic SCCD • Bony repl acem en t of any porti on of mem bran ou s
• All high signal from late ral, posterior &: superior labyrin t h
SCCs absen t in bo th ears • Wh en affects SCCs, ma y mim ic SeCD
• Small, dysmorph ic high signa l vestibule ap paren t • History of profound sen sorineural heari ng loss after
• "Isolated coch lea" wit h ap lastic aperture episode of meningitis
• If "iso lated coc hlea" present (black bon y bar across
cochlear base), cochlear nerve absent
IPATHOLOGY
Imaging Recommen dati ons
• Best ima gin g to ol: T-bone CT single best stu dy to Gen eral Features
delin eate dysplastic o r aplastic SCC co mpo ne n ts • Gene ral path co mme nts
• Protocol ad vice o Wh en lesion not associa ted wit h congenita l
o Axial images best show lateral &. poster ior SCC syndro me, lateral sec most often invo lved
lesions • Often associated with abno rmal vestibule
o Coronal images, eithe r dir ect o r reform att ed from • Gen etic s
axial dat a set, best show superior sec o Embryology
o Combination of 2 planes needed to con firm oval • Membranous labyrinth comprised of par s su perior
windo w at resia &: inferior
• Pars superior, phylogen et ically o lder st ruct ure,
gives rise to SCCs &: utricle
IDIFFERENTIAL DIAGNOSIS • Because lateral SCC last to for m, more susceptible
to deve lopmen tal anomalies
Co m mo n cavity d eformity • Pars inferior gives rise to cochlea & saccule
• Coch lea &: vestibule fused int o single cavity o Specific genetic mutations link ed to SCC ano ma lies
• sees ma y be normal or absen t in mi ce
• Detection of similar mutations in human s may
Cystic coc hleovest ib ular (CCV) a no ma ly (lP guide gene tic co unseling in fut ure
typ e I) o SCC ap lasia or h ypopl asia may be part of genetic
• Coch lea &: vestibule separate but featureless, with a syndro mes
"figure 8" appea rance • CHARGE associat ion (colobo ma, heart disease,
• SCCs variable & may be norm al, dil ated &: atresia of nasal choana, mental o r grow th
dysmo rphic, or absent retardation , genital hypoplasia, ear an omali es):
Com plete s c e aplasia or isolated lateral canal
Large endolym pha tic sac (LES) ano ma ly (IP ap lasia usually seen
typ e II ) • Alagille synd rome (arteriohe patic dysplasia):
• Vestibu lar aquedu ct great er than 1.5 mm on T..bon e Posterior SCC hypoplasia seen
CT
• Endo lymp ha tic du ct & sac dilated on '1'2 MR
• Waardenburg sy n d ro m e (hy pe rtc lo rtsm, iris
hetero chromia, w hi te for elock): Poste rio r see
aplasia see n Consider
• Crouzon sy nd ro me (cran iofacial dy sostosi s): Large • If o ne or all o f sees are d yspl astic o r mi ssing, SeCD is
vestibule & sho rt lat eral s e e present
• Apert synd ro me (ac rocephalosynd acty lism type I): • If ch ild has e HARGE sy nd ro me , lo o k forf -bone c r
Large vestibule & shor t lat eral s e e
2 • Etio logy: 6-8 wee k gesta tio nal arrest o r insult
findings o f severe Se CD
• Ep ide m io logy Imag e Interpretati on Pearl s
106 o Rare in n er ea r anoma ly • \loth axi al & coro na l 'l-bo nc c r plane s reco m mend ed
o Late ral s e e d yspl asia m o re co m mo n than o the r to assess o ssic u la r cha in & ova l win d ow
sec d ysplasia varia n ts
• Associa ted ab no rma lities: Co m mo n & var ied with
synd ro m ic s e c d ysplasia ISELECTED REFERENCES
Gross Pathologic & Surgical Features 1. Yu KK ct al: Mo lecu lar genet ic adva nces in sem icircula r
cana l ab norma lities a nd se nsor ineur al hear ing loss: a
• sce s either co m plete ly absen t, rud imen tary or dilated repor t o f 16 cases. O tu laryng o l ll cad Neck Su rg.
& dysrno rph ic 129(6):6] 7-4 6, 2003
• Tympanic po rti on o f facia l nerve ca n al o fte n dehisccnt 2. Satar II ct <11: Co ngen ital ap lasia of t he semicircular cana ls.
& in ferio rly di spl aced 0 101 Neur ot o l. 24(3 ):43 7·46, 20<H
• Ova l win dow at resia co m mon ly a ssoci at ed 3. Pu rcell J) ct a l: Est abli sh m ent o f norm at ive coc h lea r an d
• Coc h lea , e nd o lym ph a t ic d uc t & sac var iable vest ibular m easur em en ts to a id in t he d ragn osts o f i nner
car m alform atio ns. O tolaryngol Head Nec k Surg .
Micro scopic Features 12H(1):7H-H7, 2IXH
• Sa ccu le N utr icle m ay be norma l 4. C u reog!u S et al: Scheibe d ysp lasia . O to l Ncurotol.
24 (1): 125-6, 2003
5. Sen naroglu I. ct al: A new classificatio n for
coc hleo vesti b ular mal fo rm at ion s. Laryngosco pe .
ICLINICAL ISSUES 1 12(12 ):2230-4 1, 2002
6, Glasto n b ur y eM et al : Im ag ing findi ngs of coc h lea r nerve
Presentati on d eficiency. AJN R Am J Neu ro radio l. 23 (4) :635·43, 2002
• Most co m mo n signs/sy m ptoms 7. Scholtz AW N al: Goldc n har's sy nd ro me : co ng en ita l
o Se nsor ine ural he aring loss (SNHL) hearing d eficit o f co nd uctive or sensor ineu ral ori gin '?
• Co m mo n eve n when co ch lea appears n ormal a ll Tem pora l bo ne hi st op atho logic st udy. 0 101 Ne u ro to l.
imag ing 22(4):5 0 1-5,200 1
o Co n d uc tive h ea rin g loss ofte n p rese nt d ue to oval H. Lem merlln g MM et al: Un ilatera l sem icircula r ca na l ap lasia
window at resia & oss icu la r cha in a no m a lies in Golde n h ar's synd ro me . Ajf'o: lt Am J Neu ro radi ol.
2 1(7): t:l3 4-6, 2000
o Other signs /sym pto ms 9, Ben to n C ct al: Imag ing o f co nge n ita l ano malies o f t he
• Vestibular fu nctio n va riab le, eve n in ch ild ren with t empor al bone . Neu roiruuglng Clin N Am. 10(1):3 5·53,
s c e aplasia vii-viii,2(X)()
• Ca lo ric responses m ay be absen t 10 . Ba rniou DE ct ul: Un ilater al sensor ine ur al h ea ring loss a nd
• Clin ical p rofil e its aetiology in chi ldh ood: t he co ntrib utio n o f
o Depe n ds o n w het he r SCC a no m a ly sporad ic o r com p ute rised tomog raphy in ac t tologica l d iagnosis a nd
a ssocia ted with co nge n ita l synd ro me m an agemen t. lnt J Pedi atr Ot or hinolaryngol. 5 1(2):9 1·9 ,
o Spora d ic Se e D: Ran ge fro m mild to profound SNII L 19 99
I I. w rcncr-vacticr SR et al: Vest ib ular fu nc tio n in ch ild ren
o Syndro mic Se e D: Usually profound SNII L wit h
with th e C HARG E associa tio n . Arch Otolaryngol Head
o th e r associa ted anoma lies Nec k Surg. 12 5(3 ):342-7, 1999
12 . Ad m iraa l RJ ct al: Vestib ula r a reflexia as a ca use of d elayed
Demographi cs mo to r skill d evelo pm ent in chi ld ren wit h the CHARGE
• Age: Co nge n ita l associa tion . In t J Ped lat r O tor h inolary ngol. 39 (31:205- 22,
199 7
Natural History & Prognosis 13. Fu jimo to M ct .11: Co mp uter gen erated th ree -d im en sion al
• Mild , spo rad ic SeCD may have m in imal clinical recon str uct io n of th e bo ny labyrin t h in Mondln l's
impact d ysplasia . Acta Med Okaya ma . 48 (1):5 7· 6 1, 1994
• In syn d ro m ic Se C D a ffec ted ear will nev er h ea r 14. Mizun o M et <1 1: Lab yrint h ine a no m alie s wit h nor m al
coc h lear fu nct ion . ORLJ Ot orhinolar yn go l Relat Spec.
Treatm ent 54(5) :2 78- 8 1, 1992
• Unila t eral spo rad ic SeeD requ ire s no treatment 15. Nager GT et al: Ana to m ic variat io ns a nd ano m alies
• Bilateral sy nd ro m ic SeeD may benefit fro m coc h lear Involving t he facial ca nal. O tolaryngol C lin Nor th Am .
implanta ti on 24( 3) :53 1-53, 199 1
16. Parn es L et al: Bilatera l sem icircul ar cana l a plasia with
• High -resolu tio n o bliq ue sagittal T2 MR through IAe
ncar-n o rm a! coch lea r d evelo pmen t. An n Oto l Rhin o!
re com m ended prio r to coc h lea r implantati on to LaryngoL 99:957 -59 , 199 0
co n firm p resen ce o f co ch lea r n erve
IIM AG E GALLERY
(1.£11) Axial T-bone C T of

right ear shows com plete
tack o f sco . small
rlysmorphic vesub otc
2
(arro w ), ~~ "iso lated
cochlea ~ in lh i_~ child w ith 107
SN I fL. No te bo ny oar across
coch lear b ase (ope n arro w ).
(Rigll/ ) Ax;al12 WI M R
shows dilalt'd vestibule &.
prom inent lateral sec.
VC'stibule 1.1Ch centrel bon y
is/.m d, resulting in feal ur(>les.~
cavity. t'osterior sec is
for{' s!JorICf)("C) (arro w ). lAC &
co chlea are norm al.
(I.e/' ) A xial righ t ( '<If I-bo ne

C1" reveals complete absence
of 5eCs wit h smdll vesti b ule
(," fOW) . No t(' bon e at oval
wine/ow, consistent with oval
window atresia. Middle e.lf,
m astoids & ossictos normal.
(Right ) Coronal N ECT sho ws
absence of secs.
Typ ical
oval window a tr('~ia , with
smil ff win dow cove red by
bo ne . Fad al nerve ca nal
(arrow) norma l, but more
nwdi,l/ lh.ln umal. Ossicular
chJin ncmnsl.
(Left ) Axial T-bone CT shows

severe inne r ear anomalies,
w il /) absen ce o f secs, sma/J
vestibule (arrow) & abse nt
cochtoer ape rture (open
.1rm w) . Small ear cav ity.
d ysmorp/Jic osstcles.
Cl IA RC f sync/romp baby.
(Right) Coronal T-bon e CT
shows absence 0; SCCs &
hone cOVl'ri ng ova l window.
No te chers ctetistic leck of
lenticu lar process o f incus
(arrow ), thic k stape s (open
arrow ), in this pali('nt with
CHA RGE syndrom e.
LARGE ENDOLYMPHATIC SAC ANOMALY
2
108
Axial graphic of left inner ear .~ ho~V5 thf> J.)(gt, Ih ial T1WI M R .~ hows magnified vkw of left inner ear
f'lJdolymp hatic sac intradural (arrow) & in"JosS('(){J.~ willi large t'n dolympllJlic sac (arrow), a~ymmt'lrically
(open arrow) com ponents. Notice also tho coch lea is large sc.l /a vesribuli (open arrow ) and modiolar
mildly dy~pliWic (cu rved arrow). deficiency.
o Bo ne Cf: Large hon y vestibula r aqu ed uct

ITERMINOLOGY a '1'2 Mil: Large cn dolym pha tlc sac
Abbreviatio ns and Syno nyms • Locati on: Po sterior wall o f te m po ral bo n e in foveal
• Larg e en do lym p h atlc sac a no ma ly (LESAl; '1'2 MR term a rea
• La rge ves tib u lar aq ue d uct syn drome (LVAS); T-bo n e • Size
CT term o Axial bone CT: Bony vestib ular aq ued uct tran sver se
• Incom plete parti tion type 2 d ime nsio n half way up from o pen ing > 1.5 mm
o Sen naroglu et al prop osed th is term in 2002 o Axial T2 MR: Visible sac in fovea is abnormal
La ryngoscope article • Morphology
a Defined as "cochlea with I. S turns (m id d le I'< a p ica l a Axial bone CT: "V-shap ed " e n larged bo ny vestibu lar
tur ns coa lesce to form a cystic apex) accom pan ied aque d uct
by dil ated vestibu le I'< e n larged vestibu la r aq u ed uct " o Axia l '1'2 Mil: Elo n gated sac a lo ng posterior wa ll
o Th is n ewest te rm awaits widesp read acce p ta nce 'l-bone
• Large vestibu lar aq ued uc t syn d ro me: Describes bon y CT Findin gs
a no ma ly seen o n 'f-bo n c CT
• NECT
• Mon d in i mal fo rm ati on : Obsolete term wit h mu lt ip le o En la rged bon y vestibu lar aq ued uct
con fusing defi nit io ns o > 1.5 mm , mid-bon y vestibu lar aq ued uc t
Definitions o Bony ves tibular aq ued uct di amet er > po ste rio r
• LESA/LVAS: Arrested developmen t o f inner ea r leaves sem icircu la r ca na l di am eter
large endo lym pha tic sac associa ted with coch lear a Coc h lear dy sp lasia wit h a pical turn dysmorph lsm &
d ysp lasia mo diola r d eficien cy
MR Findings
• Tl W I: Low to inte rm ed iat e signa l sac visib le along
IIM AGING FINDINGS po ster ior wa ll o f T-bon e
Gene ra l Fe ature s • T2WI
a l ligh signa l en larged en do lym p hati c sac ob vious
• Best di agn ost ic clu e
DD x: Inner Ear Anom alie s
Cochlear Ap lasia Common Cavity Cystic CV Anomaly Coch lear I fypoplasia

Key Facts
Termin olo gy • Coch lea r hypoplasia
• Large endolym ph atic sac a noma ly (I.ESA); '1'2 MR Pathol ogy
term • Famili al lesion with au toso ma l recessive in he rita nce
• Large vestibular aqued uct synd rome (LVAS); T-bo ne • LESA is result of a rrest of develo pm ent of in ner ear at
cr term
• I.ESA/I.VAS: Arreste d development of in ne r ear leaves
a pprox ima tely 7t h wee k of feta l develo pment
• lIest h ypothesis fo r etio logy SNIIL: Coc h lea is "fragile"
2
large en dolym phatic sac associa ted with coc h lea r &: susceptible to in jury from mild trauma as a resu lt 109
dy sp lasia o f microsco pic infrastru ctu ral deficie ncies
Imaging Fi ndi ngs • I.F5A is most com mo n congen ita l a no ma ly of inne r
• Bon e c r: Large bo ny vesti bul ar aq ueduct ear fou nd by imagin g
• '1'2 MR: Large en do lym pha tic sac Clinica l Issu es
Top Diffe re ntial Diagn o ses • Clin ical pro file: Chil d with bilate ral SNII L becomes
• Coc h lea aplasia profou n dly dea f ove r 1st mo nth s to years of life
• Co m mo n cavity • Coc h lear imp lan tation now used wh en bilateral
• Cystic coc h leovestibular (CC V) an om aly profound sensorine u ral deafness occ u rs
o Associated coc h lear d yspl asia findi ngs ma y he subtle o Anatom ic co m me nts
o r obv io us • En do lym pha tic sac h as intrad ural por tio n (la rger
o Coc h lea r turn d ysm orphism seen as bu lbous apical par t) & intraosscou s por tio n
tu rn (more severe d ysm orphism somc ttmcs present) • Delin eati on betwee n intrad ura l & int raosseou s
o M od iol ar deficiency m ost co m mon (vs no rm al or po rtions is by htstop ath ology in sac wall
absen t) • End olym phatic du ct is sho rt con nec tio n between
o Coc h lea r d yspl asia most co m mon ly subtle crus co m m un is & In traosseo us sac
o Sca lar cha m be r asymm etry with mor e ant er ior sca la • No rma l en do lym ph atic sac & du ct is barely vislhle
vcstibu li larger th an more posterio r scala tym panI o n t hi n-sectio n high -resolution T2 MR
• Gene tics
Imaging Re commendation s o fam ilial lesion wit h au toso ma l recessive in he rita nce
• Th in-secti on T-bo ne CT (1 m m) ca n make d iagn osis o f o Pen d red syn d rome mut ation s may lead to Pcn d red
I.VAS rou tin ely syndrome I< LFSA
o Must look for &. find large ho ny vestibular aq ued uct • Pendr ed syn dro me: Severe sensorine ural h ea ring
o Diagnosis easily missed if t h is speci fic visua l sea rch lo ss with th yroid path o logy
no t com p leted • Etio logy
o CT less sens itive for associat ed coc h lea r a noma lies o LESA is result of a rrest of developmen t of in ner ear
• Thin-secti on high -resolu tio n T2 ~1R (0.8-1 mm slice at approximately 7th wee k of feta l developme n t
th ickness) best imag ing too l in experienced ha n ds • Large endolympha tic sac & coc h lea r dys plasia
o Can eas ily iden tify L r~~ A wit h la rge sac« coc hlea r results
dys plasia o Etiology o f sensorineura l hea ring loss (SNHI.)
• SNIIL often p resents following head trau ma
• May displ ay cascading yet fluctuati ng patt ern over
ID IFFE RENTIAL DIAGNOSIS 1st decade
• lIest hy pot hesis for etio logy SNIIL: Coc h lea is
Cochlea ap lasia ' fragtle' & susce ptible to inj u ry from mild trau ma
• Absent cochlea with vestigial vestib ule & semicircular as a result of microscopic inf rastructu ral
cana ls deficle ncies
Common cavity • Ot her hy po thesis: Protei n-ric h endolymp h
• Coc h lea, vestibule & semici rcular ca na ls seen as sing le refluxes into coc h lea & vestibu le
cyst • Epidem iology
o LESA is most co m mo n co nge n ital anoma ly o f inn er
Cystic cochleovestib ular (CCV) a no ma ly ea r fou nd by imag ing
• Bilobed cystic coc h lea & cystic vest ibul e o lIi1ateral an omaly (90%)
• Associa ted abnorma lities
Cochlea r hypo plasia o Associated coc h lear dysplasia (;, 75%)
• Mod iol ar deficie ncy, coc h lear ape rture large o Associat ed vestibular a nd/or sem icircu lar cana l
anomaly (50%)
o Distal renal tubular acidosis ra rely assoc iated
IPATHOLOGY • Ch aracte rized by a defect in ur in a ry acid ification
wit h vario us d egrees of meta bo lic acidos is
Ge nera l Features o Brach io-ot o-rcn al synd ro me ra rely associa ted
• Gene ral pa th co m me n ts
Gross Path ologic & Surgica l Features Image Interpr etation Pearls
• Enla rged en do lym p h at ic sac is found in dural sleeve in • Wh en LESA/LVAS dia gn osed , re me m be r to q uan tify
fovea in posteri or wall of T-bone seve rity o f coch lea r d ysplasia
a Coch lear implan t su rgeon may wa n t to im plan t side
Staging, Grading o r Classificatio n Crite ria o f most no rma l coc h lea
• In n er ca r anoma lies (grad ing from severe to m ild )
2 o In ner ea r ap lasia (Mich el d eform ity): 3rd week arrest
o Coch lear apl asia: l.ate 3rd week arrest ISELECTED REFERENCES
110 o Co m mon cavity : 4th week arrest
I. Yan g WY ct a1: (C linical ana lysis o f 95 pa tien ts with large
o Cystic coch leo vest ibula r m alform at ion (in complete
vestib ular aq ued uct synd ro me ) Zhong h ua Er Hi Va ll H U ll
part ilio n t ype 1) : 5th wee k a rrest Kc Za Zil i. 3 8(3 ): 19 1-4, 2003
o Coch lea r h ypo plasia: 6t h week a rrest 2. Naga nawa S et al: Serial MR im aging st ud ies in en larged
o La rge e n dolym ph at ic sac a no maly (inc o m plete endolymphat ic d uel and sac synd ro me. Eur Radi ol . 12
partit io n typ e 2): 7t h wee k a rrest Su pp l J :S114-7, 2002
J. Sen naroglu L et al: A new cla ssificat io n for
coc h leovest ibular ma lfor m at io ns . La ryn gosco pe.
IC LIN ICA L ISSUES I 12( 12):22JO-4 I, 2002
4. Miya mot o RT e t a l: Cochlear impla nt atio n with large
Presen tation vestib ular aq ued uct synd ro me . Laryngoscope. 112 (7 Pt
I ): 1178-82, 200 2
• Most common signs/sym ptoms 5. Berretti n i S cr al: Distal renalt ubu lar ac idosis assoc iated
o Bilate ral SNHL in c h ild with iso lated lar ge vestib ular aqueduct and senso rineu ral
• Ch ild hears a t b irt h but h ea rin g de te rio rates over hear in g loss. An n O to l Rhino! La ryn gol. 11 1(5 Pt
ea rly yea rs of life 1):J85-9 I, 2002
o Ot h e r signs/sym pto ms 6. Bichcy lUi et al: C ha nges in qu ality o f life a nd t he
• Fluctuating o r "cascad ing " SNHL often wit h cos t-utlllty associated wit h cochlea r im plan ta tio n in
post-traumatic pot entiati on pati en ts with la rge ves tib ula r aq ued uct syndro me . 0101
• Clin ical profile: Ch ild wit h bila teral SNHL beco mes Nc ur otol . 23(3):323-7, 2002
7. Bc rrcttl nl S e t al: Lar ge vest ib ula r aq ue d uct in d ista l rena l
p rofo un d ly d eaf ove r 1st mon t hs to years o f life t ub ula r acidosis. High-reso luti o n MR in three cast's. Acta
De mograp hics Radio!. 42(3 ):J 20-2 , 200 1
8. Pyle G M: Ern b ryologlca l develo pment and large vestib ular
• Age aq ued uct sy nd rome. Laryng oscope. 110 (11): 18J 7-42, 2000
o Ped iatric o nset most co m mo n 9. Gov ae rts I'J et ul: Aud iologica l find in gs in large vest ibu lar
o Most present < 10 yea rs o f age aq ued uct syndrome . In t J l'cdi atr Otorhl nolary ngu l.
• Ge n de r: 2: I, M:F 5 1(3):157-64, 1999
10. Naganawa S ct al: MR imag ing of t he cochlea r mod iolus:
Nat ural Histor y & Prognosis a rea m easu remen t in h ealt hy su bjec ts a nd in pat ien ts wit h
• If bilateral . in evita bly leads to profo und SNII L a large cndolym p hat ic d uct and sac. Rad io lo gy.
• SNfl L develop s wit h varia ble speed 2 13(3 1:8 19-23, 1999
o Hearin g loss ma y not be present u n til early ad ult life 11. Davidson He ct a l: MR evalu atio n of vcstihulocochlear
anoma lies associ ated wit h large cndolym ph at lc d uct an d
• Progn osis is best in those where hearing loss is eithe r
sac. i\j NR AmJ Nc u roradi ol . 20(8):14J5A I, 1999
un ilat eral o r d elayed into early ad ult life 12. Phelps PD et a l: Rad iologica l ma lfor m atio ns of till' ea r in
Treatment l'c nd red synd ro me . Clin Rad io!. 5J(4):2 68 -73 , 1998
13. To ng KA ct al: La rge vesti b ular aq ued uct sy nd ro me : a
• Avo idance o f co n tact spo rts o r other activities that gene t ic disease? AJR Am J Ro en tgenol . 168(4 ): 10 9 7- 10 1,
ma y lead to head trau m a is essen tia l 199 7
• Coch lear im plantation now used wh en bilateral 14, Dah len RT ct al: Overlapping t hi n-section fast spin-ec ho
profo und senso rine u ral d eafness occu rs ~fR o f t he large vest ibu lar aq ued uct syndrome. Ajl'\R Am J
o Postlin gua l d eafn ess gro up does best with coc h lear Ncu ro radiol . 18 (1):67· 75, 199 7
im plant atio n 15. O ku m ur a T ct al: Magnetic reso nan ce im agin g of patien ts
o Ini tial fears regardin g LESA/LVAS an d co ch lear wit h large vest ib ular aqued ucts. Eur Arch
O to rh inola ryngo!. 253(7):425-8, 199 6
im plan ta tio n d ispelled
16. Harn sbe rgcr HR et a l: Adva nced techniques in mag netic
o No incr ease in coc hlear im plant co m plicatio ns reso na nce im aging in the eva lua tio n o f the large
o Sign ificant improvem en t in qua lity o f life re ported endoly m phatic d uc t and sac synd ro me. Laryngosco pe.
JO.I( IIl):1037-4 2, 199 5
17. Zalzal Gi l et a l: Enlarged vest ibula r aq ued uct and
IDIAGNOSTIC CHECKLIST sensor ineura l hea ring: loss in chil d hood. Arch Oto laryngol
flead Ne ck Surg. 121 ( I ):23-8, 199 5
Co nsider 1M. Bagger-Sjoback I) : Su rgical a nat omy of t he end olym ph atic
• Usc h igh -resolu tio n 1'2 MR as modali ty o f cho ice to sac. Am) 01 01. 14(6):576-9, 1993
19 . j acklcr RK ct a l: Th e large ves tibula r aq uedu ct syndro me.
mak e & refin e dia gnosis
Lary ng oscope. 99( 12):1238· 42; d iscussion 1242-3, 1989
• No relationship betwee n size of endo lym phatic sac &
severity o f SNl tL
IIM AG E GALLERY
(I.e/') A xial l-boo e CT

reveels (.>nla rgl-d bony
w stibular <1Clurouct (iJff OW)
on posterior wall. The
2
vestibufe (open .mow ) &.
1.11C'(.11 semicircular .1((' 111
normal. Cu(w d ,U fOW soovs
norm.l! slJOrt t>nc1o/ympholtic
duct (Rigid ) A\iJI T-hone
CT shows mild cochfe.1f
d ysplJ5ia in t!lis pat/t'nl ~\';th
U SA. N o tice Ih(' m rxJioJus is
deficit.·ot (,mow ) .:lnd tbe
apical turn bulbous dod
- /Jil Tf'- (open arro w) .
Typi cal
(I.£/I) Axi,JI TlW/ MR in
patient wit h bi latf>f.lllarge
('fldolymp /ldtic 5,1(' ,Jnom aly
shows l OIJSP ;Cuo us large
. .an (a r(()\V.~) p.1falll'ling thl'
! )/,s/erio r W.ll/ o f the
/('m porallx mes. (Rig" ') Ax,iJI
T2WI M R of /('[t inner ear
demonstrates large
l' lldolymphatic Sole (arrow)
associnod wilh mild
cochiesr d ysplasia. Modiolel'
<!t'[ic;en cy (open .JffO\v ).t
bulbom .lpiC.ll turn (curw d
arrow) .1((' (·vi(/pnl.
Variant
I (Lef t) Ax.ia1 11WI MR re vedls
bilalerallarge endol ymphatic
sac anomaly to be prewrn.
Ri~hl sse is isointen St:>
(arrow) whi le 1/.'[t SJC is
hypt'rint('n5p topon .mow )
com pared to Jdja renr brdin.
(llil: "') Axial TIWI AIR
shO\ ~ bilJt('r.ll U SA Righ t
sac is isointl'me (.u row) to
brain. " yperinICf)S e Il'''' sac
is highly unusu.ll topen
.m ow) w llow ioceosiry foci
(cur ved arrow) within
fCourl C'sy K. RC'ml('y. MO J.
LABYRI NTH ITIS
2
1 12
Axial bo ne a .~ hO\ys infectious (/<.'b' ;5 ;n middle ear A xial T1 C+ M R shows rymp<lnogeflic labyrinthili.~ as
(arrow) com p ncsux t by localized dcostruclion of bo ny enhancing infecred m iddle edr 1;5sues (arrO\v) .~preading
labyrinth around oval window & po:,lt-r;or semicircular kuo bony labyrinth (open arrow) end nwmbrsnous
c,ln,11 (01')('/1J rro w ). I<lh yrinth (cur\w / .l rm\v).
• Hlla tera l « m en ln gogenic etiology (c h ild hood

!TE RM INO l O GY m e n ingitis is usual cu lpr it)
Abbreviatio ns and Syno nyms • Area s of ossi fica t io n cr uci a l to id entify whe n
• Su bac ute labyrin th itis plann in g co ch lea r implantati on
Definitions MR Findin gs
• Subacute in flam ma to ry o r infecti ou s di sease of fluid • TlWI
filled sp aces o f inner ear, espe cia lly t he membranous o Signa l of te n n ormal
labyr in t h o In seve re, d iffuse m em branous la hyrin t h itis, m ay
show su btle 1 in sign al
o If intralabyrinth in e h emorrhage h as occu rred, h igh
IIM AGING FINDINGS signa l
• TZW I
Genera l Features o O fte n so m ewh a t bri ghte r tha n no rm al bu t usu ally
• Best d iagn ost ic cl ue: Tl c+ MR shows faint to n o t di agn ost ic
m od erat e e n ha nce me n t wit hi n normall y flu id -filled • FArly ch ro n ic stage: Diminish ed sign al but c r still
spaces of inner cal' no rm al
• Locati on : Diffuse o r seg men ta l • Late c h ro n ic stag e (labyri nth itis ossificans):
Dim inl shcd sign a l wit h CT abnor ma l
CT Findin gs o Ca n be used to diffe re nt iate in t rala by rin t h ine
• No ne in ac ute & subacu te phase o f labyrinthitis sc hwa n nom a from membranou s lab yrinthit is
• Fluid-filled lab yrinthine spaces m ay beco me oss ified in • In t ra labyrin th ine schwa n noma: Tu mor is lower
late chro nic phas e = lahyrin thitis oss ificans signa l than surrou ndi n g in ne r ea r fluid
o Especia lly true in supp u rative membranou s • TI C+
Iabyrin th ili s o Faint to m oderate e n ha ncemen t w ith in n orma lly
o Seg me n ta l o r di ffuse fluid-fi lled spaces o f coc hlea, vesti bu le I'<
o Ossi ficatio n of n o rm all y fluid-filled spaces of inner se m ici rcu lar ca na ls
e"r • Segmen ta l or d iffu se
DDx: Inner Ear Lesions
IL H emorrh age IL Schwannoma Cochlear Otosclerosis O tosypb ilis

LABYRI NTH ITiS
Key Facts
Imaging Findin gs Clinical Issue s
• Best d iagnostic clue: T1 C+ MR sho ws fain t to • Tympa no genic memb ranou s labyrin thi tis: Histo ry o f
mod erate en ha nce me n t within normally fluid-filled acute or ch ron ic oto mas to id itis
spaces of in ner ear • Meningogenic memb ran ou s labyrin thitis: Histo ry o f
• Early ch ronic stage: Dim in ished signal hut CT still bact erial meningitis
no rmal • Hemat ogenic mem branou s labyrin thitis: History of 2
• Late ch ronic stage (labyrin thitis ossificans): viral infec tio n
11,
Diminished signal with C l' ab nor mal • Bilateral, severe d isease lead ing to profo und deafn ess
• If classic clinical presentation , no imag ing necessary is treat ed wit h coch lear im plantation
• Atyp ical presen ta tion: Thin-section 1'1 C- &; T1 C+
MR are key Diagnosti c Checklist
• If focal en ha ncement seen, T2 high -resolu tion • Avoid internal audi to ry canal "tu n nel vision" when
imagin g d ifferentiates labyrint hit is from evaluating pat ien ts with SNHL
in tralabyrin th ine sch wannoma • Visually in terrogate in ner ear structu res also
Imaging Re commendati ons IPATHOLOGY

• If classic clini cal presen tation, no imaging necessary
• Atypical presentation : Th in-section T l C- & T I C+ MR Gen e ral Features
are key • Gene ral path co mme nts
• If focal en ha ncemen t seen, T2 high -resolution imaging o Nonspecific infl ammation of membran ou s labyrin th
differentiat es labyrinthitis from intralabyrinthi ne o Etiology most com mo nly viral
schwan no ma o Bacterial disease mu ch mo re d ang erou s
c Rc-em crgcn ce of o tosyph ilis is occu rring (IIIV)
• Etiology
ID IFFERENTIAL DIAGNOSIS a Serous or su ppu rative lahyrinthitis possible
o Etiology of in ner ear en hanceme n t
Intral abyrinthin e (Ill hemorrhage • Labyrinthine vascu lature breakdo wn with leakage
• Pre-contrast '1' 1 high signal of MR con trast & subseque n t neovascularizat ion
• Patient will usually have und erlying co nd itio n such as of intralabyrinth ine me mb ranes
coag ulo pa th y • Begin s in su bacute ph ase, persists for several
mon ths
Intralabyrinthine (Ill schwa nnom a • If inadeq uate ly treated , progression to ch ronic
• Tl c+ MR: Focal intralabyrinth ine en hancemen t d isease may ensue
o Such en hancemen t mo re int en se and localized than • Epidemi ology: Commo n clin ical problem that rarely
labyrin th itis co mes to ima gin g
• T2 high-resolution MR: Focal area o f decreased signal
• Sch wan noma involves bot h lAC and vestibule or Microscopi c Features
coc hlea = d umbbe ll lesion • Early ch ron ic findings: Fibrous stage sho ws fibrobl ast
proliferati on
Intralabyrinthin e meningiom a • Late ch ronic find ings: Ossific stage sho ws labyrin thin e
• Rare ossificatio n
• May appear iden tical to intralabyrin thine o Seco nda ry to ab no rmal proli feration of
schwan no ma un differentiat ed mesen chymal cells in endoste um &
• Associat ed with adj acent o tic capsule deminerali zation mod iolus basilar mem brane resu lting in formation
o f osteo blasts
Cochlea r otos cle ros is o On ly seen in severe, su ppurative cases o f
• Characteristic areas of demineralization identified at labyrin thitis
CT
• '1'1 C+ en ha ncement if present, in bony labyrinth
O tosyphilis ICLIN ICA L ISSUES
• Clinical history is crucial Presentati on
• Tl C+ en hanceme nt does occu r in labyrin thitis phase
• Most co mmo n sign s/symptom s
• Facial palsy occu rs co mmo n ly (TI C+ en hancemen t of
o Senso rineur al hearing loss (SNIi L)
nerve)
o Vertigo &. tinnitus
o Any o r all may be of sudde n o nset
• Membran ous labyrin thitis: Classified by etiology
LABYRINTHITIS
o Tympan ogeni c membranou s labyrin t hi ti s: Histo ry of
ac u te or chro nic o to masto id itis Im age Inter pret ati on Pearl s
• Usua lly unil at eral • Avo id inte rna l aud itory ca nal "t un nel vision" w he n
• Pathoge n s e n te r throug h round wi n dow into sca la eva luat in g patie n t s wit h SNHL
ty mpa ni of ba sila r coc h lear tu rn • Visually interrogate inn er ear structures also
o Men in gogen ic m embran ou s labyr inth itis: Histo ry of
bacte ria l me ni ng itis
2 • Usua lly bilat era l ISELECTE D REF EREN CES
• Streptococcus pneumo nlae is most common 1. Abdull ah A ct a l: Preoperati ve high resol ution CT and MR
1 14 path ogen ima ging in coch lear implantation. Ann Acad M ed
• Path ogen s usua lly en te r via lAC, rarely via Sing apore :12( 4): 44 2-5, 20m
cochlear aqueduct 2. Aho TR ct al: Intra labyrin th ine Men ingiom a. AJ NR.
24 : 1642 · 1645,2IXl3
• Most co m mo n cause o f acq uired chil dhood
:l . Hegart y J1. ct al: The value of en ha nce d magn etic
deafness resonan ce imagin g in th e evaluatio n o f endococh lear
o Hematogen ic m embranous labyrin th it is: Histo ry of d isease. Laryngoscope 112: 8-17, 2002
vi ral infecti on 4. Smit h JI. ct a1: Radiology q uiz case 1. Temporally separated
• Usua lly bilateral; rar e bilatera llabyrinthitis an d se n so ri ne ur al hearing loss. Arch
• Blood -borne process Oto laryngo l Head Neck Surg 128 : 80-82, 2002
• Ofte n measles, m umps S. Ha rt n ick C] et al: Prevent ing labyrinth itis oss lflca ns: t he
o Post t rau m ati c role of steroid s. Arch Otolar yn gol Head Neck Surg
12 7:180 -3, 200 1
• Un ilateral fract ure
6. Annesley-Willia ms DJ et al: Magnetic reson ance imaging in
• Often with per ilymp hatic fistul a the investiga tion of sensorine ural hearing loss: is cont rast
o Post su rgica l en hance me nt stil l necessa ry? J Laryngol Otol 115:14-2 1,
• Un ila te ra l 200 1
• Post-sta pedec tomy o r othe r inner ear surge ry 7. Aferzon Met al:Laby rin t h itis ossificans. Ear Nose Throat J
• Labyrinth in e ossificat ion always occurs after 80 :700 -1, 200 t
ob literativ e labyrinthecto my 8 . Palacio s E et al: Hem orr hagic labyrinth itis. Ear Nose Throat
• Mem brano us labyrinthitis: Classified by agen t J 79:80 , 2000
o Viral, bacterial , au to im m une 9 . Phelps PO ct al: Imaging fo r cochl ear implan ts. J Laryngol
Olol Suppl 24: 2 1-3, 1999
• Hearing loss may correspo nd to invo lved po rtion of 10. Ba m iou DE et al: Un ilatera l sensorine ural h earing loss an d
coc h lea its aetiology in ch ildhood: the contribution of
o Exam ple: High frequ enc y hearin g loss presen t when co m pu te rized tomograp hy in actio logical diagnosis an d
basilar turn of coch lea en hances management. ln t J Pcdiatr Oto rh inolaryngol S'lv l -v, 1999
11. Sch indler JS et al: Tra nsverse temp oral bone fractu res (left)
Demographics with subseq uent progressive SNHL, cons iste n t wit h
• Age syrnpat hctlc coc h lcolabyrint h itis. Arch Otol aryngol Head
o All age s Neck Surg 1 24 : 81 6 ~ 8 ) 1998
o Me n ingogeni c labyrin th itis is d isea se of ch ild hood 12. Sto kroos RJ et al: Magn et ic reson ance imagin g of th e inner
car in pati ent s with idiopathi c sudden sen sorineural
Natural Hi st o ry & Prog nosis hear ing loss. Eur Arch Otor h ino lary ngo l 2S S: 4 3 3~ 6 , 1998
• Full recovery do es occ ur in some patien ts 13. Strupp M et al: High resolution G d ~DT I'A MH imaging of
the in ner ear in 60 pa tients with idiopat hic vestibular
• Persisten t unil ateral deafness common
neur itis: no evidence for con trast en ha nceme nt of th e
Trea t m ent labyrinth or vestibular ncrvc.} vc sub Res 8:427-33, 1998
14. O'Keeffe LJ et al: Primary tumours of th e vestib ule and
• Viral etio logy: Ste roids, vest ibular su ppres sants & in ner ear. J La ryn gol Otol 111 :709~ 14, 199 7
vestibular exercises 15. Weissm an JL: Imagin g of Men iere's disease. Otol aryngol
• Bacteria l: To pical & IV an tlblotics: surgical Clin Nort h Am 30:1105-16, 1997
in terventio n when severe 16. Mafee MF: MR imaging of int ralabyrint h ine schwa nnoma,
• Chronic lab yrinthitis ma y ult imately require labyrin th itis, a nd ot he r labyrin th ine pathology.
la byr inthect omy Otolary ngo l Clin Nort h Am 28:407-::W, 1995
• Bilat eral, severe di sease leading to profound deafn ess is 17, Mark ASet al: Se nsor ine ura l he aring loss: more thanm eets
treated with coch lear impla ntation the eye" AJNR Am ) NeuroradioI 14:37-4S, 1993
18. Casselman JW et al: Path o logy of the mem branous
labyrinth: Com parison of T'l- and T2-weighted ami
gado lin ium-en hanced spin-echo and 3DFT-C ISS im agin g.
IDIAGNOSTIC CHECKLIST AjN R 14:59-69 , t 993
19. Seltzer Setal: Con trast enh ancement of th e labyrin th on
Co nside r MR sca ns in patients with sudden hearin g loss and vertigo:
• ln tralabyrinthine schwannoma differen tial diagn osis ev idence of labyrint hine di sease. AJNR 12:13-6, 1991
o En ha ncemen t typicall y m uc h mor e in te nse
o Low signal m ass seen on 1'2 MR
• Intralabyrinth ine hemorrhage differential diagn osis
o T1 C- hyperintense sign a l
LABYRINTHITIS
IIM AG E GALLERY
( l~fl) Axial T1 C• •\'IR shows

msuokiectom v dt'(ect with
residual e nhancing
granula tion in masroid b owl
2
(arrow). Enhanc ing foci in
cochlea (open ,mow) 115
seco nd ary to tym p anogcnic
subacute lahyrinthitis.
(Righi ) Axial T2WI MR
shows bony replacement o f
bestler turn of cocbtee
(arrow) sl>co nd,lf y to
chronic lab yrinthitis. The
apica l and middle turns
topon arrow) are of normal
signal intemity
(Left ) A xial t iw: MR in

pa tient with subacute
mem brano us labyrinth itis
reveals d iffuse faint
enbsncement of the cochled
(arro w) . The vestibule and
sem icirc ular cenels do /lot
l 'llhance . (Righi ) Axi.ll T l C+
MR shows faint b ut d early
defina ble pathologic
enhancement of the cochlear
rums (arrow) and vestibule
(open arrow ) in parient with
acute ons et of H>rtigo and
hearing 1055.
• •
(I A!f l) Coronel bone CT
d('monstrates ossification of
.r:' -.·~I
'~."~~
basi',lT tu m of cochlea
(erro w ), There is d radic<ll
msstokiectcmv de{('ct with
..
extensive debris. Ih is
: ~'
indicates a tvmpe nogenk:
~l\
etio logy to lab yrinthit is.
(Right) Axial bone C T reveals
.. ~
ossification tocstired to
basilar tum of cochlt>a
(cUHlftl .1(fOw)
'~,. ""~tfI!.
. "
post-pro sthetic
stapedectom y.
Tympanogen ic (iatrogen ic)
:t"x- labyrinthitis resulted in
lab yrinthine ossiiicsns.
OTOSYPHILIS
2
116
Coronal l-bone CT sha.vs subtle permeative A>.ial T1 C+ MR reveals pathologic enhancement of

demineralization of otic capsule (arrows) from leptomeninges within internal auditory canals (arrows)
ol osyp hilis. Small foci .lfe different (rom larger cochlear and coc bleer membranous labyrinth (open arrO\vs) in
Dtuse/prosis 1,/aC/Lle-like demin('ralization. ocosyphilis.
o Laby rin thi tis: Higher signa l of in ner ea r fluid

ITERM INO l O GY possible
Abb reviat ions and Synonyms o lAC meningeal infecti o n: Thi cken ed CN7-H
• Lue tic lahyr in th itis, oste it is, m eningitis • TJ C+
o Osteitis: If seve re, patch y en ha nc ing fo ci in o tic
Definitions ca psule
• Sexua lly tra nsm itt ed inner ea r disease caused by o Labyrin th it is: Enhancement o f flu id filled spaces of
ba cter ium spirochete Treponema pallidu rn inner ea r
o lAC meningiti s: En ha nceme nt of leptom en inges
wit hi n lAC includi ng C N7 I< C NH
IIM AGING FIND INGS Imaging Recom me ndatio ns
Ge ne ral Features • Os te itis: Axial &: coro na l 'l-bo ne CT
• Rest d iagnostic cl ue: Perm eative d emineralization o f • Labyri n th it is & lAC m eningiti s: TJ C+ MR
o tic capsule
• Locati o n : Otic capsule, lAC & lAC meninges
CT Find ings
IDIFFERENTIAL DI AGNOSI S
• NECT Cochlea r oto sclerosis
o Oste itis: "Mo t h-ea te n" perm eati ve o tic ca psule • Clin ical: Mixed hea rin g loss
d estr uct ion • Imaging: Radio lucent foci th ro ughout o tic capsule
o Laby rin th itis: No t seen o n 'I-bone CT
• Cr.CT: Con trast not useful or reco mm end ed
Os teogenesis im pe rfecta ta rda
• Clin ical: Ch ild ren with britt le bo nes & blue sclera
MR Findi ngs • Imaging: Exac t coc h lear o tosclerosis mi m ic exce pt
• 1'1WI: Osteitis: Pat ch y areas of in term ed iate signal usually mo re severe
• T2W I Paget disease
o Oste itis: If severe, pat ch y h igh signal in o tic capsule
• Clin ical: Affects th e eld erly
DDx: Otic Capsule l esion s
CachoOtosclerosis Os teog enesis Imperf. Paget Disease Fibrous Dysplasia

OTOSYPHILIS
Key Facts
Terminology • Diagn osis mad e wh en oto logic sym pto ms are p resen t
• Sexu ally tran smitted inner ea r d isease caused by with positi ve sero logy
bact erium spiroche te Tre po ne ma pallidum Diagnostic Checklist
Clinica l Issues • Perm eative deminerali zati on of o tic capsu le
• Hearin g loss & vertigo : Oft en acu te & fluctuating (syph ilitic osteitis)
• T1 C+ MR en ha nce me n t o f CN7 I'< CNS in lAC ±
2
• Sim ulates Men lere disease
• Facial palsy; meningeal signs membranous la byrin t h (syph ilitic 11 7
labyrinth itis-m en in gi ti s)
• Imaging: Otic ca psule demineralizati o n is diff use,

invo lves en tire sku ll base Treatm ent
• Antibiotics &. co rticoste roid s
Fibrous d ysplasia
• Clin ical: Un de r 30 yea r o ld patien t gro u p
• Imaging: Gro u nd -glass expa ns ile bo ne wit h relative
sparing oti c capsule
IDIAG NOSTIC CHE CKLI ST
Con sid er
• Sarcoid os is &. metasta tic d isease in pati en t wit h
IPATHOLOGY lep to meni ngea l en ha nceme n t
Ge ne ral Features Image Inte rpretatio n Pearls

• Etio logy: Spiroc h ete Trep o nema pa llid u m; sexually • Perm eati ve dcm inerali zatlon of o tic ca psu le (syp h ilitic
t ransm itt ed ost eitis)
• Epidemiology • TI C+ MR en ha nce me n t of CN7 I'< CNS in lAC ±
o In ciden ce began to I in 19HOs d ue to AIDS memb ranous lahyrin th (syph ilitic
o Ma jor ity o f cases in so ut hern USA labyri n t h itis-mcningitis)
• - 15 cases o f syph ilis per I OO,IX)O
Gro ss Path ologic & Surgical Featu res ISELECTED REFERENCES

• End o lym pha tic duct rarely obst ructed by gu m ma I. Rucken stein MJ et a l: Im m un ologic and sero log ic tesu n g in
Micro scopic Features pat ien ts with Meniere's di sease. 01 0 1 Neurotol. -
23 (4 ):5 t 7-20 , 2002
• Osteitis: In flammato ry resorptive os te itis 2. Smit h ~{ M ct a l: Neuro syph ilis a\ a GW~ for facial a nd
• Labyri nt hi tls: Oblite rative enda rteritis vcsub ulococ hle ar ne rve dysf unc tio n: ~IR imaging featu res.
AJNR. 2 1:1673-5 , 2lKXl
3. Lint h icu m fit Temporal ho ne h isto pathology case of t he
ICLIN ICA L ISSU ES mon th : Otosyphi lis. Am J 0 101. 20:259-60, 1999
~. Gleich L1 , e t ..I: Oto syp hili s: A d iagnostic and t herapeu tic
Prese ntation d ilem ma, Laryng osco pe. 102: 1255·9, 1992
• Most co mmo n signs/sym p to ms
o Il earin g loss &. vertigo: Often acute & fluctu ating
• Hearin g loss (HOIX,) IIM AG E GALLERY
• Sim ulates Meni crc disease
o Facial pa lsy; men ingeal signs
o Diagn osis mad e when o to logic sy mp to ms are
presen t with positi ve sero logy
o Oto syp h ilis is lat e man ifestation
Demogra phi cs
• Age: Older patients
• Gender: M > F
• Et h n icity : Africa n-Americans > Caucasians
Natural History & Prognosis
• 25 1}() h earing loss im prov es after th erap y (fL II) Coronell t-bon e a of right ear sho\\'s se vere otosyp/Jilis as
• 70 1}() tinnitus & vert igo im p roves after th erap y permea tive (Iemineraliza tion of otic capsule (Jrrows) .
• Best response wh en sym pto ms are fluct uating, hearin g Otosclerosis-like p laf/ue on coc hlear p romontor y (op en Jrro w )
loss is < 5 years durati o n &. pa tient is < 60 yea rs o f age (Courtt:'sy M. 5.1ntllin, MDj. t Right) l\'I(ial T-lxm e CT of 'e(/ ear sholVs
ot05 YfJhili~ ,] ~ permeeuvc demi neralizat ion of otic cap sule in region of
b<lsal turn of coc hlea (arro w ) & roun d window (op en <m ow ).
(Courtesy D. You5em, M O).
LABYRINTHINE OSSIFICANS
2
118
Ih ial T-hone CT shows St-'veft" labyrinthim· o5sifiGlIJ.' Avi.JI 12 W / MR in p.lt;etJt w ith ~('V(.,(' righ f infl('f ('<1r to
with ossific replacement of most 0; cochlP.u f{'\'t'<!/s a cochle ar not ve entering the cochlear <1p(.>r1llr('
fTJt>mb'.l/) ou.\ lab yr in th (arro w). N o/t ' !xJ/le kxi w ith in (a rrow ) wilh IJO Ilorm al coc hlt'Jf fluid si~/ J.l/. R('sidtJ.l1
\'e ,tibu /t> (open ,mow ). vesti lw !e (open arrow ).
ITE RM INO LO GY CT Findin gs

Abbreviation s a nd Syno nyms • NECT
o Mild LO: Flbro-o sseou s c ha n ges seen as h azy
• Labyr in thin e oss ifica tio n (LO) in cr ease in d ens ity wi thi n fluid space s o f
• Lab yrint h ine ossi ficatio n, os sifyi ng la byrin th itis, membran ou s la byrin t h & p rom in ent mod io lus
ch ro n ic lab yri nthiti s o Mod erat e La: Focal ar eas o f bon y enc roachm en t on
Def inition s fluid spaces of membra n ou s labyrin th
• LO : Mem bra n ou s lab yrinth ossi fica tio n as h ealin g • May he coc h lea r o r no n -cochl ea r or both
response to in fect ious, inflammatory, tr aum atic or o Severe LO : Memb ran ous labyrin th is co m p le tely
surgical in sult to inner ear ob lite rated by bone re place me n t o f its flui d spaces
• C ECf: Has no ro le in making LO d iagno sis
MR Find ings
IIM AGING FINDINGS • T2 WI
General Fe atures o Mild LO : In term ed iate &: low sig na l fibro-o sseou s
mat er ial parti ally rep laces hig h sign al fluid spaces of
• Best di agn osti c clu e membranou s labyrin t h associat ed wit h ap pa ren t
o 'I-Bo n e CT: High densit y bone depositi on with in
"en large me n t" o f modio lus
memb ran ous laby rin t h
o Mod erat e La: Fo cal areas o f low sign al bone
o '1'2 MR: Low intensity foci wit hi n hi gh signa l fluid o f
encroach in g on h igh sig na l fluid spaces of
inner ear
m embranou s laby rin th
• Locati on • May be coc h lea r o r n on-cochl ear o r bot h
o Memb ran ou s labyrin t h fluid spaces
o Sever e La : High sign al membranou s lab yrinth is
o Coch lea r LO: Fluid spaces o f cochlea itself affecte d
ab sent as it is co m pletely repl aced by lo w sign al
o Non-coc h lear: Fluid spaces of semici rcu lar ca na ls o r
bone
vestibu le affecte d
• Co ch lea r n er ve o fte n severe ly at rop h ied
• Morphology: Focal oss ific plaques vs diffo se
ossification of m embra n ou s labyrinth • Tl C+
DD x: Inn er Ear Lesions
Labyrinth ine Ap lasia Vestibular lipoma C. Otosclerosis Inner Ear Schwannoma

Key Facts
Termino logy Path ology
• LO: Membra nous lab yrinth ossificati on as h ealin g • Suppurative m embran ou s lab yrinthit is sets lip
respon se to in fectio us, in flamma to ry, trau mat ic or cascad ing in flam mato ry respon se in membrano us
surgical insult to inn er ca r labyrinth
Imagin g Find ings
• Begi ns wit h fibros is, progresses to oss ifica tio n (as
ea rly as 2 mon th s) 2
• 1 111m thi ck ax ial & coro na l Tsbo ne c r easiest • Mcn tng ogc nt c labyrin thi tis is m ost co m mo n cause or
im agin g too l to usc in d iagn osing LO 119
acq uired ch ild hoo d deafness
Top Differential Diagnoses Clinica l Issue s
• Labyrin th ine or coc h lear a plasia • Clin ica l profil e: Bilatera l SNtlL in ch ild 2-1B m onths
• Inlravest ihu lar lipom a after acute men ingit is episode
• Coc h lear otos cle rosis
• Labyrint h ine sch wa n noma Diagn o stic Checklist
• In pre-coch lear im plant eva lua tion of 'l-bo ne in
ch ild ren, loo k for LO &: inner ea r co nge n ita l
a no ma lies
o Membran ou s labyrin th itis seco nda ry to in fect ion is

usual precu rsor to La Cochlea r o toscle ros is
• In th is prc-LO pha se, membra nous labyrin th • Clin ica l: Disease of yo ung ad ults
en hances sign ifying act ive lahyrin t h itis • Im aging: Radi olu cent foci in vol ves bon y labyrin t h on
• Enha ncement ma y be holo -Iabyri nt hine or T-bon e cr
segmental o Docs not enc roach on membranous lab yrint h eve n
• En h a nce me n t ma y persist in ossifying stages of La in healin g phase
o Differential dia gn osis o f e n han cing m embra nous Lab yrinthin e schwa nnoma
lab yrinth is lab yrinthi tis vs intra labyrin th in e
• Clin ica l: Protract ed hi stor y of slowly progressive
schwa n nom a
uni latera l SN HL
• T2 MR used to different iat e lahyrint hit is from
• Imaging: f oca l tntra lab yrlnth in e en ha nce me n t o n 1" 1
int ralabyrint h in c sch wa n no ma
C+ M R
• Lab yrinth itis shows high intra lab yrinthinc signa l a '1'2 MR sh ows tissue in ten sit y mat erial wit h in
wh erea s ln trnlab yrinth inc schwa n nom a ha s tissue
portion of mem bra nous labyrin t h
inten sity tum or repl acing hig h signa l fluid in
membranou s labyrin th
Imaging Re commen dations IPATHOLOGY
• 1 mrn t h ick axia l & coro na l T-bon e c r eas iest im aging
too l to usc in dia gnosin g La
Ge ne ral Featur es
• Gene ral path co m me n ts
• High -resolutio n th in-section T2 MR imaging ma kes
o Labyrinth ltls progresses to lahyrint h ine osslfica ns
diagnosis
whe n su ppu rat ive
o Ca refu l in spection for absen ce of in ne r ea r fluid
a 1.0 seen as ea rly as 2 mo nths aft er ep isode o f
spaces critic al
o '1'2 MR imagin g ca n sh ow fibro us oblite ratio n of men ing it is on T-bone CT
membranou s labyrin th wh ereas CT ca n no t • Etio logy
o Su pp u rat ive membra nou s lab yrin th iti s sets up
o T I C+ MR VERY usefu l in showin g en hancing inner
ca r in pre- I.O p hase wit h labyrin th itis casca d ing in fla m matory respon se in me mbra no us
la byrin th
o Begin s wit h fibro sis, progresses to oss ificat ion (as
ea rly as 2 mo nths)
IDIFFERENTIAL DIAGNOSI S a 1.0 a rises from suppurati ve labyrin t h it is from
La byrinthin e or cochlea r aplasia m ultiple sources
• Clin ica l: Sensorineura l hearin g loss (SNI11.) present • wh en seco nda ry to m eningiti s: Men ingogeni c LO;
from birth hilat eral
• Imaging: In ner ca r or coc h lea r ed ifice is absen t • \Vhen seco ndary to midd le ca r infection:
o lAC may be small Tym panogen ic LO; unilateral
• Wh en from blood -borne infect ion such as measles
Intr ave sti bul ar lipoma o r m umps: Hemat ogen ic La ; bilatera l
• Clin ica l: Mild, h igh frequenc y SNIIL ofte n present o 1.0 may also ari se aft er severe tra uma or temporal
• Imagin g: '1'1 MH shows h igh signa l foci in vestibu le o f bon e surgery
in n er ea r • Epidem iol ogy
a CPA lipoma may be associa ted
o Meningoge n ic labyrinthitis is most co m mo n ca use o Scala vestibuli inse rtio n an alternative
or acq uired ch ild h ood d eafness • I.abyrinthect o my used in cases of in trac table vert igo
• M ost com monly from Streptococcus pneurn onla e
o r Hem ophilus lnflue nzae
o have so me degree o f hea rin g loss follow ing
6 -3()l )() ID IAGNOSTIC CHECKLIST
meningitis
Co nside r
2 Gross Pathologic & Surgical Features
• Bivalvin g in ne r car o f pat ient with LO sho ws new
• In p re-cochlear impla nt eva lua tio n o f T-bo ne in
ch ild ren, loo k for LO &. inner ear co nge nita l a no malies
120 bone formati on in membran ous lab yrinth • Both th ese d iagn oses will o fte n force su rgica l plan to
• At su rgery for coc h lear implantation, bon y obstruc tio n be individualized
to implant en t ry through ro und win dow nich e is • LO may co n traind icate o r co mplica te coc h lea r
observed im plan ta tio n
Microsco pic Features Image Interpretation Pearls
• Fibrous stage: Fibrobl ast proliferati on • Rad iol ogi st shou ld d escribe LO as "COCf Il.EAR" or
• Ossific stage: Osteoblasts forming ab no rmal bon y "NON-COC HLEAR"
trabecul ae with membranous labyrinthine spaces o Ju st descnbmg LO o f inner car d oes no t help
o Scala tympani in ba sal turn most frequen t area o f coc hlear impla n t su rgeo n decid e what ca n he done
ossification in LO o f all causes • Coc hlear LO makes im plan t prohl em ati c
o Meningitis -- sup pu rative laby rinthitis associat ed o Be specific about wh at non-cochl ear portions o f
with greatest amou n t o f oss ificatio n membranous labyrin th are inv olved
IClI N ICA l lSSU ES ISELECTED REFERENCES

Presentation 1. Berrett m i S ct ill: Scala vcst lbuf co chlea r lmpla ntatlc n in
pa tient s with pa rt ially ossifit'd co ch lcas. } Laryngol Ot o.
• Most com mo n signs/sy mpto ms 116:94 6·50 , 2002
o Bi lat e ral SNHL 2. Thoma s J et ul: Evalua tio n o f r O\.' h lca r im plantatio n in
o Other signs/sym pto m s po st-meningitic adu lt s.) l.aryngo l Otol . 113:27·33, 1999
• Severe vertigo is infreq uen t but d evastating 3. Mu ren C et <II: I'ostmc n tng tttc lahyrfnt hlne ovsifi cation
sym pto m prima rily affect ing the sem icircul ar ca nals . Eur Radiol.
• Verti go may be serious enoug h to req uire 7:208 -13, 199 7
labyrin t h ecto m y 4. Silbe rma n B et ill: Roll, o f modern il11 a ~i ll ~ tec h nology in
• Clin ical p rofile: Bilat era l SNHL in ch ild 2- 18 months the implementat ion o f pediat ric cochlear im pla nts. An n
Oto l Rh inol La ryngol. 104(1 ):42-6, 199 5
after acut e meningitis episode 5. Jo hnson Mil et a t: cr of posunc ntngtt tc deafness:
• Ot her poss ible pati ent hi stor ies o bse rva t io ns a n d pr edict ive va lue of coc h lear implants in
o Su ppu rative mid dl e ear in fect ion (ty rnpanogcnlc children. AJNR. 16(1 ):10:1-9 , 199 5
La) 6. deSou Zil C et al: Pathology of labyrl nthl ne ossification. J
o Severe bou t o f mumps, measles o r o the r viral illn ess Laryngol 0101. 105(8 ):621 -4, 1991
(hem atogenlc LO ) 7. Stee nerson RLet al: SCala vest rbull coc h lear im pla ntation
o Pro found head & skull base trauma (pos t-trau matic for lahyrin th ine ossifi canon . Am J Otol. 11(5 ):360 -3, 1990
H. Novak ,.fA et a t: Labyrin thin e ossificatio n after me n ingit is:
1.0)
Its im plicatio ns for coch lear impla ntat ion. Otolarvngol
o Prev ious tempor al bone o peratio n (pos t-su rgica l LO ) Head Nec k Surg. 103 (3) :351 -6, 1990
Dem ographi cs 9. Hamsberger HR et al: Cochlear implant candidates:
assess ment with c r and ,.f R imaging. Radiology.
• Age: Ped iat ric malad y 164(1):53-7, 198 7
10. Kotzias SA et a l: Labyri nt hine ossif ica tio n: differences
Natural History & Prognosis be twee n two types of ec to pic bo ne. Am J Ot o l. 6(6 ):490-4,
• Grad ua l det erio rat ion of hearing following ea r 198 5
in fectio n (u nilateral) o r men ingit is, blood-borne I I. Swartz JD et al: Lahyrln thinc ossifi ca t ion: etiologi es and cr
inf ecti o n, head trauma o r f -bone surger y (b ilate ral) findi ngs. Radi ology. 15 7(2 ):39 5-8 , 19 85
• Progn osis for SNHL is defin ed by response to coc h lear 12. Bec ker TS ct al: Labyr inthi ne ossification seco nda ry to
im p lan ta tio n ch ildhood bacterial me ningi tis: implicat ions for coch lear
im plant surgl'ry. A.lNR Am J Neuroradlo l. 5(6 ):739-4 1, 1984
Treatm ent 13. Ibrahim RA et 'II: Labyrinthine ossifl cans and coch lear
• Coc h lear implantation used for SNHL co rrectio n, if im plan ts. Arch O tol aryn gol. 106(2): 111- 3, 1980
coc h lear nerve still present 14. Hoffma n RA ct OI l: Radiologic di agn osis of labyrin th itis
ossmca ns. An n O to l Rhin o ll ..a ryngol . 88(2 Pt 1):253·7,
o Bilateral co chlea r LO is serious de trimen t to coch lear
19 79
imp lanta tio n 15. Suga Fct OIl: Laby rint hitis osstttcans. An n Ot ol Rhinol
• PRE-su rgical iden ti fication o f coch lear LO is key! Laryngo!. 86(1 1'1 1): 17-29, 19 77
• Allows plan n ing for "drill-out" of obstruc ted
coch lea &. modi ficat io ns o f im plant device
• "Drill-o ut," newer coc hlear im plant d evices ava ilahlc
for obs t ruc ted coc h lea
IIM AG E GALLERY
Typ ical
(u Jt) Axial right t -bone CT
demons/rates mild coch/car
labyrint hine ossific.ln5. M ild
encroachment ts vi.~ibJ(~
2
along the posterior basal tum
of cochlea. (arrows). (RighI) 12 1
Axial T2WI ~,,\ in right inner
car with mild La sholVs
bony enc ros cbmont on basal
turn of cochlea (arrow) .
Notice associate d "large
modiolus " (op ell arrow ) =
iibro-oswc us cochlear
involveme nt
Typica l
(I.ef l) Axial left T-hone C T
reVf'.lI.~ a foca l i O plaque
fillin g the prOXima l basal tum
of cocblea (arro w) . Notice
th(' round window niche
(open arrow ) is occluded as
1Ve'1I. (Right) Axial T2Wt MR
shows J focallahyrinthine
cssittcens plaque .lS a
bvpcinten sc band across
proximal b,IS.l1 /lJrn of
cochlea (arrow ). Cochlear
impl.mratic)fJ of this cochlea
co uld be problemetic.
Typi cal
(ILf t) A xial left I-bene CT
reves ls severe cochlear
labyrinthine ossiiicens. The
basal ttl m L~ hyp('rclf..'llSC! hu t
slill visible (arrow ). The 2nd
turn can no longer he
discerned from bon y
lah yrinth (open arruw ).
(Rigllt) Axial T2W I MR of Il'ft
inne r ear shows severe
lab yrinthine ossiticens. No
coc hlear turn tJuid is see n
(arrow) . Vestibule fluid is
partially see n (open arr o w ) .
Curved arrow : Atrophic
cocbtes r nerve.
INTRALABYRINTHINE SCHWANNOMA
2
122
I
.1
A\.;.IJ r 1 C+ MR shows cJ.mic (,~<1mpJl' of Axial TlWI ,\ IR rf'WtJ1,~ typic.l1 fine/inn!; of

int,.lJ.Jbyrinthine sd nvannomd. Nolice b ion ;s .1 \'t~tibul(xochlcJr schwarmomol. Foeoll tissue Sij;n.11
\~t;bul()nJ( Nt'o1' type w ith kx st t'nh.l (}(WrH-nt of IhP tumor is sem repl,King perilymph,llk l1uid both in
\'t' \t if>ult> (.1rrcllv) S, coch lea (open .lrrow ). \'('Sti!>ulc (arron') iJnd cochk'a (o(J(·f1 .l rr o w).
• Tran sm acu lar = sch wa n n o m a c ross ing fro m

ITE RM INO l O GY vestibule in to fundus o f lAC
Abb reviations and Synonym s • Transotic = sch wa nnoma c rosses e n t ire inner ear
• In tralabyr luth in e schwa n nom a (1L5) from fu ndu s of lAC to m iddl e ear
• Inn e r ea r sc h wan nom a • Size
o Usua lly rema in s in m illimet er range w it h in
Defin itions m emb ran ous labyri n th
• IL'i: Benig n t u m or a risin g fro m Sch wa nn ce lls within o Large r lesions ex te nd ex t ra labyrin t h ine
st ruct u res o f membran ous labyrinth • Mo rp hology
o Early, sma ll lesions are ovo id to round
o O lde r, larg e r lesions take on sha pe of portion of
IIM AGING FINDINGS membranous labyrin th affecte d
General Fea tu res CT Findin gs

• Best di ag no stic cl ue • N hC'l'
0 '1'1 C+ Mil : Foca l e n h a nc ing ma ss in m e mbran ous o Bone Cf
labyrint h • No rm a l is ru le unless m ass project s in to m iddl e
o High-resolution '1'2 Mil : "Fill in g defect " within ea r t h ro ugh rou nd window nich e
h yp er intense perilymph • In very la rge lesio n s (t ra n sm odiola r, tra nsmacular,
• Locati on tr ansotlc), bon y ero sions ma y be visible
o Foca l intralahyrinthin e m ass named by location • 1I0ne Cf u sua lly n ot helpful in ma kin g this
• Intravestibular = sch wa n n oma within vest ibul e o f di agnosis
II inner ea r • C ECf: ll.S n ot vis ibl e o n C liCf eve n if thin -section s
(I
• Intra cochlcar = schwan n oma within coch lea ar e o bta ined
" • Vestibulocochlear = sch wan noma inv o lves both MR Findin gs
vest ibule & coc h lea
• Transmodi olar = sc h wa n nom a cross ing m odiol us • T1WI
o Tissue in ten sit y ma ter ial in inne r ca r
from coch lea to fu nd ", o f lAC
DD x: Inn e r Ear l esions
Lahyrimhitis Lab. Ossiiicens Labyrinth I l emorrhage Dchiscent FNS

Key Facts
Termin ol ogy • Tran sm acu lar = sch wan no m a cro ssing from vestibule
• IlS : Benign tumor ari sing from Schwa n n cells wit hi n in to fu nd us of lAC
structu res o f m em bra no us labyrin th • Tran soti c = schwan norna crosses e n tire inner ea r from
fund us of lAC to mid dle ea r
Imagin g Findin gs
• Tl C+ MR: Foca l en h a nc ing m ass in memb ran ous
Top Differential Diagnoses
• Labyrint hiti s
2
labyrinth
• High-r esolution T2 MR: "Filling defect " wit h in • Labyrinth in e ossificans 123
hyperintense perilymp h • In tra lab yrinth in e h emo rrh age
• In travesti bu la r = sch wan n o m a with in vest ibu le of • Facial nerve sch wan no m a O:NS) wit h deh isce nce into
inner ea r in n er ea r
• In tracochlear = schwa nnoma wit hi n coc h lea Diagn ost ic Checklist
• Vestibu locochlear = schwa n n o ma in vol ves bo th • Wh en visua lly in te rrogat ing MR im ages to "ru le o ut
vestibu le & coch lea acoust ic schwa n n om a", reme m ber to ca refu lly
• Tran smod iola r = schwa n nom a cross ing mo di ol us eva luat e in ner ca r flu id spaces for I1..S
fro m coch lea to fun d us of lAC
o Not see n u n less la rger lesion is presen t & thinner o T l C+ ~ fH : Minimal or no inn er ea r en ha nce me n t
sections a re ob ta ined o High-resol u tion T2 M k : Fo cal low signa l a reas
• T2WI : High-resolut ion '1'2 shows focal low signa l mass with in hi gh sign al in ner ea r fluid; whe n
wit h in h igh signa l fluid s of m em bran ous lab yrinth fib re-osseous, m ay m im ic ILS
• T1 C+
o T l C+ shows focal en ha n ceme n t o f ILS Intralabyrinthin e hem or rh age
o 11$ may p ro ject m ult ipl e d irections fro m in ne r ear • Clin ica l: Un ilate ra l sudde n Oil set SNHI.
• Th ro ugh ro u nd wind ow in to m iddl e ea r • Im agin g
• Alo ng vestibu lar nerve bran ch es in to fundu s o f o T l MR: lIi gh sign a l fluid wit h in memb ra no us
lAC =lran sm acu la r II.S la byrin th
• Thro ugh mod iolus N coc h lea r ap erture int o lAC = Facial nerve schwa nno ma (FNS) with
tra n sm od iola r ILS
dehiscen ce into inn er ea r
Imagin g Recommendati ons • Clin ica l: SNHL wit h assoc ia ted facia l neuro pathy
• Use focu sed T l C+ o r hi gh -resolu tion '1'2 imaging o f • Imagin g
CPA-lAC to m a ke di agnosis o f ILS o Bone CT: Sm ooth en large me n t o f in t ratem por al
• Ca refu l exa m in at io n o f a ll "ru le o ut aco ustic" MR sca ns facial ne rve ca na l
for presen ce o f int rala byrint h ine ma ss is crit ica l o Tl C+ MR: En ha nci ng tubu lar ma ss follow s co urse o f
• Ob serve precise locat io n o f tu m or int ratem po ral facial n erve ca n al
o Co ns ider if it invo lves vest ibu le, coch lea o r both o In vo lvem ent o f in ner ea r is secondary find ing
o Co n side r if it project s int o mi dd le ea r o r lAC fun d us
• All pa tients un d ergo in g su rge ry fo r Mcnlcr c di sease
shou ld undergo preo perativ e focused MR imaging to IPATHOLOGY
exclude Il S
Gen eral Fe ature s
• Gene ral pa t h co m me n ts: Same pa thology as ot her
IDIFFERENTIAL DIAGNOSIS sch wan nomas in h um an bod y
• Etio logy
l ab yrinthiti s o Tum o r arises from Sch wa rm cells wra ppin g dista l
• Clin ical: Acute o nse t senso rineu ral hea ring loss vesti bu lar or coc h lear n erve axo n s wit hi n
(SNHL) ± vert igo & facia l n europa th y m em b ra n ous lab yrint h
• Imagin g o Seco n da ry e ndolym p hatic hyd ro ps exp lain s Meniere
o T l C+ MR: En h an ceme n t of m o st o r a ll o f sym ptoms
m emb ra n ou s labyrint h • Epidem iology
o Il igh -resol utio n '1'2 MH.: No t issue in tensit y mass o Ra re lesio n
seen wit h in h igh sign a l in n er ea r fluid o Perh a ps 1/1 00t h as co m m o n as acoust ic
schwarmo rna of CPA· IAC
l ab yrinthin e ossifica ns
• Clin ica l: Histo ry of p revio us m en ingitis o r suppu ra tive Gross Path ologic & Surgica l Features
m idd le ea r-m as to idi t is • Ta n -gray, encapsu lated m ass fou nd wit h in lab yrin th
• Imagin g
o Bo ne CT: l.n croach mcn t on fluid o f membranous Microscopi c Fe ature s
labyrin th by bone • Differen tiat ed n eo plast ic Sc hwa nn cells
• Areas of co m pac t, elo nga ted cel ls = Anton i A
INTRALABYRINTHINE SCHWAN NOMA
• Othe r areas less den sely cellular with tu mo r loosely
ar ra ng ed , ± cl usters of lip id-l aden cells = An to n i B Image Interpretati on Pearls
• St rong, d iffuse expression o f 5-100 prot ein • W h en visua lly int errogati ng MR im age s to "rule out
aco ustic sch wa nnom a", re m ember to carefully evaluate
inner ea r flu id spaces for ILS
IC LIN ICA L ISSUES o Un less rad io logists specifica lly loo k at in ne r car for
focal lesions, Il.5 will be mi ssed
2 Prese ntation • Once ILS is sus pected , u sc hi gh -reso lut io n T2 MR to
di ffere n tia te 11.'; fro m lahyrint h itis
12 4 o Un ilateral sensori ne u ral hearin g loss o 1l.5 will a ppear as tiss ue inte n sity lesion wit h in h igh
o Tum or location-specific symp to ms signa l in n er ea r fluid
• Wh en in vestibule: Tinni tu s, episod ic ve rt igo with o Labyrin t h itis will show no suc h foca l mas s wit h in
na usea &. vom iting, mixed hearin g loss (tumo r h igh signa l inner car flu id
imp edes stapes footp late, creating an element of
co nd uctive hearing loss)
• Wh en in coc hl ea : Slo wly p rogressive SNIIL ISELECTED REFERENCES
• Clin ical p rofile: Un ilate ra l SNI II. th at de velops ove r I. Jackson LE et al: lnt ralah yrm t h lnc sch wan no m a: subtl e
decades d ifferen tiat ing sym p to m at o lo gy. Otolaryng o l Head Neck
Su rg. 129(4):4 39-40, 2003
Dem o graph ics 2. Falcion i M ct ai: Inner ear ex t en sio n o f vestibu lar
• Age: Adults o ver 40 years of age schv v a n n o m ns. Lary ngosco pe . 113(9 ): I 60 S-H. 20 0:{
:J. Neff BA ('I al: In tralabyrtn t hine sch wanuomas. Ot o l
Natu ral History & Progn osis Neurotol. 24(2):299-30 7, 20 03
• Very slow -growing , benign tu mor o f mem branous 4. Hegart y JI. et a l: The valu e o f en ha nced magneti c
la byrin th resonance im aging in till' eva lua tion o f endococh lear
• Ili sto ry of progressive h ea ring loss m ay da te hack 20 di sease. Laryngo sco pe. 112 (l ):H- 17, ZOOZ
years s. Green JD j r ct <I I: Dtagn ovtv an d manageme n t of
in t ra labyri n th ine schwan nomas. Laryngoscope.
• Ofte n grows to fill in ne r ea r, t hen stops growing 109 (1O):1626':J1, 1999
• To tal dea fness in ear will result eve n t ua lly if left alone 6. Kro n enbcrg ] et al: lntracoc hle a r schwanno ma and
• Deafn ess certa in if tum or removed coc h lea r im pla n tatio n . Ann Ot o l Rhinol Laryn go l. 108(7 1't
1):659-60 , 1999
Treatment 7. Fitzgerald DC ct al: lntrala byrinthinc sch wa n no mas. Am J
• Co nse rvati ve ma nage m en t O IUI. 20(:J):3Ht ·5, 1999
o Watchf u l wa iti ng 8. Deu x JF ct al: Slow -grow ing labyrin th ine m asses:
o Applied wh en sym pto ms a re m in or (se rv iceab le co n trib utio n of MRI to diagnosis, foll ow -u p and treat ment .
hea rin g ma in tain ed ) & tu mo r is co n fin ed to in n er Ncu ro radi ol ogy. 40( 10):684 -9, 1998
ca r 9. Zhar RI et al : In visible culp rit: in t ral ahyr in t lline
schc....an no ma s th at do not ap pea r on en hanced magn etic
• Surgica l remova l
resonan ce imagi ng . Ann Oto l Rhino ll.ary ng o l.
o Trans laby rin thi ne su rge ry rem oves tu mor in 106(9):7:19 ·42, 199 7
vesti bu le 10. M afcc M F: M ft im agin g o f in tral abyrtn th ine schwa unom a,
o Tran so ttc su rgery co m pleted for tu m ors invo lvin g labyrin t h itis, and o the r labyrin t hine path o logy.
coc h lea o r midd le ca r Otolar yng o l Clin Nor th Am . 28 (3):40 7-30 , 1995
o Completed if sym pto m s a re di sab lin g I I. Saeed SR ct al: ln tralabyrtn thine sch wa n no ma shown hy
• Usua lly whe n th ere is in t ractah le ve rtig o magnetic reson ance imaging. Ncu ro radiology. :-i6( 1):63-4,
o If tran smo d iolar or tra nsm acu lar exten sion is 1994
1Z. Ozluo glu L ct al: ln t rulabyrin t hi ne sch warm o mn. Arch
sign ifica n t, m idd le cranial fossa a pp roach ma y be
O lo lary ngo l Head Neck Su rg. IZO(l 2): 140 4-6, 1994
u sed 13. Doyle KJ et al: ln t ralaby n n thi ne sch wan nom as.
O lOlaryngo l llead :"Jcck Surg. J 1O(6):51 7-Z3, 1994
14 . O ht an i I ct 31: Tempor al bo ne pa t ho lo gy in in t racochlear
IDIAGNOSTIC CHECKLIST sch wan no ma wit h profou nd hear ing loss. i\uris Nasus
Laryn x. 17(1 ): 17-22, 19 90
Co nside r 15. M afee M F ct <I I: CT and l\.1R imaging o f Int ralabyrtnthine
• ILS is m issed d iagn osis by exce llen t rad io log ists schwan noma: report of two cases a nd rev iew o f the
beca use th ey are not awa re of its exis te nce literatur e. Rad io logy. J 74(2):395-400, 1990
16. Sataloff HTct al: ln t ralabyrin thi ne schwan noma . Am J
• Mor e ILS n ow being d iagn ose d wit h hig h-reso lution
0101. 9(4):323·6, 1988
T2 im agi ng th an befo re wh e n on ly T l C+ Mit being
perfo rm ed
o Increased d iagn osis in par t secon dary to , awareness
of this lesion
o Also a resu lt of fact that so me ILS do n ot e n ha nce
rob ust ly hu t ca n be see n on h igh-resolut ion T2 MR
IIM AG E GALLE RY
(Lef t) A xial 1'1 c+ MR
de m onst rates /('(t inner ear
inlravestibu/.l( SCh W d flTU ) l1Ja
(arrow) as an ,1((',1 oi tocst
2
enhancement filling the
Vt'stibulC'. Note normsl 125
vnbe ncemem of tymp anic
facia l nerve (ope n arrow).
(RighI) ;\x ial T1 C+ MR
sho ws right inner edr
inlracochle ar sc bwe nnoma
(arr o w ). Notice "djacent
v<,s tihuh.' is fluid signal
w i/h oul evide nce (or
t'nhancement (ope n a"olv).
(I.ef l ) Axial T2Wf M R of left

inner ear r('veals tissue
in ten .~ ity foc; within the
\I('.~ til){l1f· (arro w) and
co cllk',l (open arrow). The
correc t term for this lesion is
vf'slibulocochlf'af
(Right) A xial
SclnVJ IJIlOI1Ja.
T 1 C+ MR. of right inner car
cU Pa shows ly pical pxample
of tr;IIJ,m lOd io l,lr
schwannoma. Enhancing
tumor fiJls coch/e,If basal
turn (arrow ), then extends
across modiolus in to lAC
fundus (oIX'n arrow).
Typical
(I.eJI) A xial T2WI MR reveals
J sub tle example of a
trammJcula r scbwonnome,
Notice the slip of !lImor
alo ng tile cln tpriur wa ll of the
vestibule (arro w). 7(lI1lOr
extends in /a fu ndus of lA C
(op m arrow ). «<;1:"1) Axial
T I C+ MR of left inner m r
area .~ h ow~ example of w ry
rare tr<1 /J.\otic sc/nv.lnn oma .
[n hancing tum or ex/e(1</'''
from CPA through lAC
(arro w ), inner ear (op en
arrow) into middfe eer
(cu rved arrow).
ENDOLYMPHATIC SAC TUMOR
2
-,-- -'
L
I
Axial grdph ic of T-bwlt., i/!usrralL->5 typical apfX\1f.lnce of Axial t-bone C1 of /ell ear rew al') smaller f IST
lIS1 ImIK)rl.ltJt fe ,ll u fC>S include its VJ .<;CV I.lf nature, permeating the hone of the posterior peuous ridh't.·
ll 'm ft..t lcy fa fiswliLC innt'f l'ar (arrow ) & bon e fragmcnts (arrows). Nolin ' illvo/vt'menl of po sll'rior m argin of
within tumor m atrix. inlerm l .w dilory canal (OfX'n arrow ).
o > 5 e m in max imu m di am e ter co m mo n

ITE RM INO LO GY • M orp hology
Abb rev iat io ns a nd Synonyms o Tumo r e n la rges to in vol ve en ti re posteri o r wall of
• Abb reviati o n : En do lym ph a tic sac tumor (ELST) T-bo ne , C PA cist ern &. jugu lar fora men
• Sy non ym : Adenomatou s tumo r o f c n dolym p ha tic sac o Margi n s may appea r dest ruct ive &. agg ressive
Defin itions CT Findi ngs

• E1.51': Papillar y cystadc noma to us tum or of • NECT
cndo lym pha tic sac o Aggressive, so ft t issu e mass that e rod es posterio r wall
o f '[',bon e
• Po sterol at eral jug u lar forame n wa ll may be
d estro yed '
IIM AGING FINDINGS
• Po sterio r wa ll o f lAC m ay be destroyed
Genera l Fea ture s • Tu mo r may permea te in n er ea r &. m idd le ea r
• Best di agn ostic cl ue o Ce n t ral sp icu la ted calc ifica t io ns wit h in tu mo r
o Bone CT: In tra tu mo ra l ho n e spicu les m atr i x ( 100%)
o M il : HI GH SIGN Al. FO CI wit h i n tu m or m atr ix on T1 o Th in rim o f calcifica tio n a long po sterior ma rgin of
MR t u mo r co m mon
• Locat ion • CECT: ln h o m oge n cou sly en h a nci ng tum or
o Distinctive rctrolab yrint h in e locatio n • C l'A
o Ce n te red in fov ea of en do lym ph at ic sac in o En la rged d ist al vessels feedi n g vascu la r tum or
presigmoid . post erior su rface of petr o us T-bo ne a feeding vessels most co m mo n ly in clude ascendin g
o L1Q~ l' lesions (> :1 e m) sp read to in vol ve middl e ca r, p h a ryng eal a rte ry &. occi pi tal ar tery bran ch es of
CPA cistern &. jugu lar fora men exte rnal ca ro tid arter y
• Spread to m iddl e ea r from e n dolym ph at ic sac MR Findings
th rou gh inner ea r
• TlWI
• Size o HYPERINT EN SE foci wit h in tu m or on T1 Mil
o Usually la rge at prese nta tio n
• High sign al alo ng tumor ma rgin whe n < 3 em
DDx: l esions Around Endolym p ha tic Sac Area

~~
Cho/. Granuloma PA Glomus lugulere Sch\vannoma IF M eningioma CPA

Key Facts
Termin ol ogy • 80'Ml o f ELST h ave th ese foc i of in creased sign a l
• Abb reviatio n: Endolym pha tic sac tumor (ELST) intensit y
• Synonym: Ade norna to us tum or o f endolympha tic sac Top Differential Diagn o se s I
• ELST: Pap illar y cys tade n o rn a to us t um o r of • Ch o lestero l gr a n u loma of pet rous a pex (PA)
endolym phatic sac •
•
Glomu s [ugulare pa raga nglio ma
Schwan nom a of jug ular fo ramen UF)
2
Imagin g Find ings
• Bone CT: Int ratumo ral bo ne spicules • Men ing ioma of jugular foram en-CPA cistern 12
• MR: IIIGH SIGNAL FOC I with in tu mor m at rix o n T1 Pathol ogy
MR • If ELST is BILATERAL, vo n llippe l-Lindau d isea se is
• Centered in fovea of endo lyrn phatic sac in presen t
pr esigrnoi d, po sterior su rface of petro us T-bo nc
• IIYPERINTENSE foci wit h in t u m or on Tl MR Clinica l Issu es
• High signal alon g tumor ma rgin wh en < 3 em • Sensori ne ural hearin g loss (100?,,",)
• High signa l foci wit hi n tum or m atri x w h en > 3 em • Age: Average age at init ial su rgical resection = 36
yea rs
• High sign a l fo ci with in tum or ma t rix w h en > 3 em a Bo ne CT sho w m ultilobul ar, smoot h ex pa nsile
• 80 1M, of El.ST ha ve these foci o f increa sed sign a l margins
in te n sity o High signa l on T l MR inv o lves entire lesion
o Flow vo id s (foca i low sign a l areas o n T1 MR) whe n
tumor s > 2 em G lo m us ju gulare paragan glioma
• T2W I
• Clin ica l: Pulsatile tin nitu s with vascular retrotym panic
o luhom ogenco us signal co m mo n mass
o Bone fragme nt s low signal • Locati on : In volve jugu lar forame n , spread ing u p &
o lli gh protein-o ld hem o rrh age are as very high signal lat era lly in to middl e ea r
• '1' 1 C+: Het erogeneous en hanceme nt o n T 1 C+ MR o Rarely invo lves retr o labyrin thin c T-bo nc
images • Imaging
• MRA: Ext erna l caro tid bran ches feed lesio n o Bone CT shows permeative-destruc tive ho ne
• MRV: Larger tum o rs occlude sigmo id sin us invasio n
o High signal foci on '1'1 MR rare; high velo city flow
Angiogra phic Findi ngs vo ids present
• Sm a ll tum o rs « 3 ern ) supp lied by bra n ch es of
ex te rna l carotid ar te ry (ECA) o n ly Schwa nno ma o f jugul a r fo ram e n OF)
• La rger tu m ors (> 3 em) supp lied by ECA bra n ch es ± • Clin ica l: Cra n ial n eu ro pa t hy (CN9- 12 possible)
interna l ca ro tid arter y (ICA) ± b ranches from pos terio r • Locat io n : Cen te red in jugu lar foram en
circu lat ion o On ly involves ret rolabyrin th inc 'l- bo nc when large
• EC,\ branch es serving t umo r • Imaging
o Ascend ing ph aryn geal, sty lomasto id & pet rosal o Bone (.1 shows smoo th en largeme n t of jugular
branch o f mid dle meningeal ar te ries foramen
• Hypervascu lar tum or blush is no rm wit ho ut ea rly a '1'1 C+ MR reveals unifo rm enha ncement; intramu ral
ven ous retu rn cysts possibl e
Imaging Re commen dati ons Meningioma of jugular fo ra me n-C PA ciste rn

• Both bo n e CT I< T 1 C+ MR a re nece ssar y to fu lly work • Clinical: Sensorine ural hea ring loss or inci de ntal
up thi s lesion find ing
o Larger th e tumor, the mo re importan t it is to use • I.ocatio n: Lesio n follo ws d ura l surfaces
bot h mo da liti es • Imaging
• MRA I< MRV he lp in defi n in g vasc ula r rela t ionsh ips o Bo ne C l' shows permeative-sclero tic margins
• Larger lesio ns will be nefit fro m preop erative o En han cing m a ss OIl TI C+ MR; lacks flow vo ids
angiog raph y with embo lization
IPATHOLOGY
ID IFFE RENTIA L DIAGNOSIS Gene ra l Fe ature s
Cho leste ro l granuloma of petrou s a pex (PA) • General path co mments: Benig n tumo r wit h variable
• Clin ical: I.ong term seq uelae of ch ro nic otit is media papill a ry growt h patte rn s I< foca lly cys ti c co m p o ne n ts
• Location : Centered in petr ous apex • Genetics
• Imagin g a Most ELST are spo radic
o Mu tations I'< allelic deletio ns of von Hippel-I.ind au • If spo radic ELST, loo k for clin ical I'< molecular
tumo r sup pressor gene play a role in generating evide nce of von Hlppel-Lindau disease
th ese sporadic tu mo rs
o 7% of vo n Hippel-I.indau pati en ts will de velop ELST
o If ELST is BILATERAL, vo n lI ippel-Lindau d isease is IDIAGNOSTIC CHECKLIST
pr esen t
• Etiology: Slow-grow ing tu mo r arisi ng from cells lin ing Image Interpretat ion Pearls
2 endo lym phatic sac • Lob ulated tum or of posterior wall of T-ho ne wit h Tl
• Epidemi ology: Rare tumo r of tem poral hon e MR h igh signal foci is EI.ST until proven o therw ise
12 8 • Associated abno rmalities
o vo n Hippel-l.indau di sease
• Ce rebe llar & optic nerv e h em ang ioblastom a, ren al ISELECTED REFERENCES
cel l carcino ma, ph eochrom ocysto ma I. Richard s PS ct OI l: Endolym phatic sal' tumours. J Laryngol
• Kid ne y & pancreas cysts Otol. 117(H):666-9, 20m
2. Co he n JE ct a l: Endolym pha t ic sac t u mor : stagl'd
Gross Path ologic & Surgical Features cndo vascula r-nc u ros u rgjca l approach . Ncurol nc s.
• Hea ped up tu mor o n po sterio r wall o f tempor al bo ne 25 (3):2:17-40 .200J
with foci o f o ld hemorrhage wit h in substa nce of :I. Fer reira MA et al: End o lympha ttc sac tu mor: un iq ue
tu m o r feature s o f two cases and revi ew o f t he literat u re. Ac ta
• At surgical ex ploratio n sma ller tumo rs see n wit hin . eurochi r (w tcm . 144(1O):1047-5J. 2002
4. l .uff LJA ct al: End c lyrnphatlc sac tumours. J Lar yngol 0101.
endolympha tic sac
116(5 ):398-401 , 200 2
Microsco pic Featur es s. Baltacl ogf u F ct al: M R im aging. C I ~ ami angi ograp hy
features of endolym phatic sac tumors: report of two cases.
• Histologic va riabil ity is co mmo n Ne u roradiology. 44( 1):9 1.6. 2002
• EI..ST is th erefo re referr ed to as ade no ma, 6. Murph y RA et al: Cyto logy o f e n d ol y m p h a tlc sac tumor.
adenocarcino ma, & adc noma to us o r carcinoid t um or s Mod I'athol. 14(9 ):9 20-4, 20tH
• Co m plex, interdi gitating papill ary pro cesses th at 7. Ho rigu ch i H et al: End olymp hali c sal' tu mor associated
infiltrat e surro unding co nnec tive tissue &: bo ne with a vo n Hip pel- Ltnda u di sease pa tient : nu
o Pap illary pro cesses em bed de d in sheets o f de nse Immunohlsto ch cmical stud y. Mod Path ol. 14(7):72 7-32,
fibrou s tissue with evide nce of recent & prev ious 200 1
hem orrhage H. Richard S ct al: Cc-n tral ner vous system
hem a ngio blasto mas. cndolym pha tic sal' tu mo rs, and von
o Papil lary pro cesses lined with a single layer of Hippe l-Lind au disease. Nc u ros u rg Rev. 23( 1): 1·22;
cubo ida l epithelial cells th at resem ble cells th at lin e discussio n 23 ·4 , 2()(XJ
endo lym pha tic sac 9. Gaeta M et al : Sud de n u n ila teral dcafm..oss with
• Lining cells are low co lu m nar to cuboi da l wit h end o lym p hat ic sac adenoca rcin o ma : ~ I R I . Ncuroradiology,
deepl y eos ino philic homogen eo us cyto plasm 41 (10 ):799-801 ,1999
• Cells also have sma ll, centrally placed , o vo id 10. Kcmper mann G ct al: Endolym phatic sac tu mo u rs.
nuclei Histopathology. J J (I ):2- 10, 199 H
• Pleomo rphis m minimal, mit o tic act ivity &. I I. Roche PH ct al : En d olym p ha nc sac tumors: report o f th ree
cases. Neur osurgery. 42(4):92 7·32, 199 8
necrosis very rare 12. Ste ndel R ct a l: Neo plasm of c ndol ym phatic sac ori gin:
• PAS-positi ve, colloi d-like lumi nal ma teria l abu nd ant clinica l. rad iologica l and pat ho logica l feat u res. Act a
• Rich ly vascular stroma present be low epithe lial lin ing Neur oc hir {Wlen) . 140 (10 ): 108 3.. 7, 1998
of tu mor U. Ti bbs REJ r e t a l: Should e n d olymp h at ic sac tu mor s be
co nside red pa rt o f t he von Hippcl-Lindau co mplex?
Pat ho logy case rep or t. Neu rosu rgery. 40( 4 ) :84K~5 5 ;
IC LIN ICA L ISSUES d tsr usston K55. 199 7
14. Mukh er ]i SK et al: Pap illa ry endolym p ha t ic sac tum ors: cr.
Presentati on MR im agin g, a nd an glographtc fin d ings in 20 pa tien ts.
Iladiology. 202(:l I:HO!-H. 199 7
• Most co mmo n signs/sy m pto ms I S. Levin HJ et al: Aggress ive papillary tu mo rs o f th e tem poral
o Sensori neural hear ing loss (100% ) bo ne: an im mu nohistoc hem ica l a na lysis in tls..ue culture.
o Othe r signs/sy m pto ms Laryngoscope. 106(21 )t 1): 144 · 7, 199 6
• Facial nerve pal sy (60%) 16. 110 VI' et al: Low-grade ad enocarcino ma o f p robable
• Pulsatil e tin n itus (SO%) em Jolym p halic sac o rigin : Cf and MR appea rance. AJNR
• Vertigo (2(1*,) AmJ Neuroradiol. 17(1 ):16 8-70 , 1996
17. Feghali JG et al: Aggressive papillary tu m o rs of the
Demograph ics endolym p hat ic sac: clin ica l a nd tissue cult ure
• Age: Average age at init ial su rgical resection = 36 yea rs characteristics. Am J Otol. 16(6):77 H-H2, 1995
1H. Po llak A et al: Are pa pi llary ade nomas c ndo ly mphatic sac
Natura l History & Prognosis tu mo rs? An n O tol Rh inol Laryngo l. 10 4(8):6 13·9, 1995
• Prog nosis is excellent if co m plete surgical resecti o n 19 . Megen an Ct\ ct al: Endolym pha tic sac tu mor s:
ach ieved hi st opath o logic confirma tio n, clin ical cha racterization,
and implicatio n in von Hippcl -Lindau d isease .
• Late recurrence possible (slow grow th rate of tum or) La ryngoscope. 105(81'1 I): HO I -8, 1995
Treatment 20 . 1.0 WW ct a l: Endoly mpha tic sac tumor s: radiologic
appearance. Radi o logy. 189 (1 ): 199 -204 , 1993
• Co m plete surgical resection with wide margin s
IIM AG E GALLERY
(Lef t) Axial l -bone CT of

right ear shows typical
permeative bone changes of
fLST. Ibls lesion involves
2
otic cap sule (arrow) <.': round
window niche. Note its 129
penetration iruo middle' ear
(ope n ,mow). (/UghlJ A xial
T1W I M R revea/~ the ctsssic
posterior pfOtroU5 ridgp
location g high signal IOc; in
an £LST matrix (arr o ws). The
intemal auditory canal is
involved (opon arrow ) by
Wm or.
Vari an t
(l£ f " Axial bone CT reveels
bilateral f'ndolymphatic sac
wmOTS (arro w s) in this
pa tienl with von -t tippe!
Lindau syndrome. Tumors
Wl-'ff> small ,H a resutt o f
being (ound on a sc nsmmg
exeminetion . (Rig lrl) A xial T1
C+ MR with (at-salUrall'on
s/Jo lVS lindings of von -t-tippel
Lin dau syndrome. These
include ph eochromocytoma
farr o w), p an creatic cySt5
(open arrows) & spinal
hemangioblastoma (curved
arrow).
Variant
(l.£f t) 1\ )(;.11 T2WI MR in
p atien t with large n ST
sho ws replacement of right
tempo ral hone hy tumor.
Notice fluid-fluid level seen
in mC'di.11 margin (arro w)
from intratlllJ)or.ll
IwmorrlIJg{'. (Hi!:",) tator.a 1
common carotid angiogram
in patient with massive HS1
rvveels tilt' iflteme c,lp illiJfy
Mush (arrows) olten s('('n
wilh this tumor.
Pre-operative embolization
may he lp with hemcHiJsis
;S qlf'.~ .
COCHLEAR IMPLANT
2
130
Modifit"Ci 5tenvffs view of the right temporal bone CexonaJ oblique reiormattro bone a c.kn)()()strates
5ho.vs cochlear implam enlf?ting arm of round windo» axhlt.'af implant ffill'(;ng the round winch.., (arran')
(arrow), then spiraling josie/f-' the coc btoe (O(X'fl o.rrO\\'). and spiraling up the cochlearturns (opm .l(fo. V).
• Held in place by magnet in subcutaneous receiver

ITERM IN O LO GY o Receiver
Abbrev iations and Syno nyms • Th in, subcuta neous compone n t that resides
• Coc h lear implant (CI) behind ear
• Coc h lear electro de • Surgically impl ant ed
• Co nverts magn etic im pu lses from tran smitter to
Definitio ns electrical signal for stimulato r wire
• CI: Multi-compone nt electron ic device th at provides o Stim ulato r
auditory in formati on by directly stimulating aud itor y • Wire placed inside coc h lea d irectly stim u lates
fibers in coch lea spiral gang lion cells and coc hlear axo ns
o Microphone • Sti mulator wire en ters coc h lea via round window
• External com ponen t that resides beh ind ear • Array of electrodes along wire appear as tin y
• Receives sound from environme n t bump s radiographi cally
• Transforms sound to electr ical impulse
• Trans mits impu lse to speech processor
o Speec h processor IIM AG IN G FIN D INGS
• External com po ne n t that may be attac hed to
mi crophone or worn sepa rately in clot hin g General Feat ures
• Custo m programmed computer that emphasizes • Best diagnostic clue
speech ove r other sounds o Thin metallic wire (stimu lator) with tiny beads
• Digita lly encodes sounds from frequ ency range of (electrod es) exte ndi ng into cochlea
human speec h o Stim ulato r wire is con nected to subcu taneous
• Encoding strategy de pen ds on manufacturer receiver beh ind ear
o Trans mitte r • Locati on
• Exte rna l compo nen t that resides behi nd ear, ato p o Stim ulato r wire sho uld be in basa l turn of cochlea,
subcuta neous receiver some times int o 2nd turn
• Transcutaneo usly sends magn etic impulses from o En ters coc h lea via rou nd window
speech processo r to receiver • Size: Sub-millimeter thickness
DDx: Co ntraindicatio ns to Co nve nt ional Cochlea r Impl an tati on
Absent Cochlear Nerve Labyrinthine Aplasia Cochlear Aplasia Leb. Ossiticens

COCHLEAR IMPLANT
Key Facts
Te rmino logy • Key pre-operative contraindication on MR: Absen ce
• C I: Mu lti. -component elect ron. ic devic.
e that .
provides of cochlear nerve
auditory information by directly stim ulating aud ito ry Clinica l Issu es
fibers in coc h lea • Torque experien ced by C I in 1.5T MR is sufficien t to
• Stimulat or wire en te rs coc h lea via round window cause im pla n t movement; C I patients sho uld n ot
underg o 1.5T MR
2
Imaging Findi ngs
• Sti m u lato r wire shou ld he in basal turn of coc h lea, • CI is effecti ve rebabilitation meth od for profoundl y 13 1
so metimes into 2n d turn hearing impaired pati ents wh o d o n ot ben efit fro m
• Key pre-operative ab solute co n t raind ication: Coc h lea r hearing aid s
ap lasia alone o r in labyrinthine aplasia Diagn ostic Checklist
• Key pre-operative relative co n traind icatio ns : • Are there a ny co n traind ica tio ns to C I placem ent ?
Lahyrinthi tis os sificans, other inner ear d yspl asias • Are there a ny find in gs that mi ght co m plicate
• Key post-up com plica t io n: Misplaced wire (no t in su rgery?
coc h lea ) • Which side would be easi er for su rgeo n?
• Morph ology: Cu rviline a r with small bead s o n • Ipsilate ral brain stem in fa rct
in tracoch lca r stim u lato r wir e • Supe rficial side rosis
o Post-ope rative sett in g: Trad itio na l C I arc no t
Radi ographi c Findin gs considered safe fo r 1.5T MR
• Modi fied Ste nv c rs view o f te m poral bone shows CI • Magn eti c torq ue ma y d islodge C I
best • Embedded magnet ca uses ma rked field dist ortio n
o Head rot ated 45 degrees from d irect AI', away fro m
im p la n ted ea r Imaging Re commenda tions
o Sligh t head flexion • Req uires h igh -reso luti on T..bo n e c r o r h igh-resolution
T2 MR
CT Fi nd ings • Pre-operative eva lua tio n
• NECf o Te m poral bo ne CT
o Pre-im pla nt eva lua tio n : Absol ute &. relative • Ade qua te ly eva luates ro un d window pat ency
co n t raln dica rio ns to im pla n tation • Iden ti fies bony phase of labyr in th itis os sifica ns in
• Key pre-o pe rative a bsolute co n train dicatio n : coc h lea
Coc h lear ap lasia alo ne o r in labyrint h in e a p lasia • Shows in ner a no malies & ana tomi c va ria nts
• Key pre-operative relat ive co n train dic atio ns : o Tem poral bo ne Mil
Labyri n t hitis oss ificans , o the r in ner ea r dyspl asias • ld en tifies bot h fibro us « o ssific obs truc tions
o Pre-im plant CT: Find ings th a t ma y co m plica te wit h in coc h lea
su rgery • Ca n see a bse n t o r hypo p lastic coch lea r n erve
• Hypo plastic masto id process • Posto pera ttvc eva luatio n
• Abe rrant facial ner ve co ur se o Modified Ste nvers view o f te m po ral bo n e sh ows CI
• Ot omastoi d itis mi splacem ent
• Fenes tra l ± coc h lea r otoscleros is o High -resolutio n T-bo ne c r now su perior too l
• Persist ent stapedial ar ter y
• Dch isce nt jug u lar bul b
• Aberra nt interna l carotid a rtery ID IFFE RENTIA L DI AGNOSI S
• Enlarged en d o lym ph atic sac & d uct
o Post-operative searc h fo r co mplications Maj o r lesio ns to ident ify o n p re-opera tive CI
• Key po st-op co m p licatio n: Mispla ced wire (n ot in ca nd id ate
coc h lea)
• Abso lute con train d ica tion d iagn oses
• \ Vire pen et rat es o n ly pa rt way into coc h lea
o Abse nt coc h lea r nerv e
• Brok en wi re o Atre tic coc h lea (laby rint h ine a plasia, coc hl ear
• Wi re penetrati on o ut o f inn er ca r
a plasia)
MR Find ings • Relative co n train d ication di agn o ses
• TZW I o Dysplastic coch lea (co m mo n cavity, cystic
o MR mu st in clud e h igh-reso lu tion flui d seq uenc e co ch leovest lbula r a nomaly)
o Pre-op erati ve sett ing : Look for a bso lu te &. relative o Large c nd olym p ha tic sac a nomaly
co n t rain d icatio ns o Labyrint hi n e o ssiflca ns
• Key p re-o pe rative con traindi ca tio n o n MR: Absent cochle a r nerve
Absence o f coc h lear ner ve
• Imaging: Absen t co ch lea r ner ve wit h small lAC
• Absence o f flu id in coch lea (e.g., laby rin th itis
(co nge n ital type)
oss tficans)
COCHLEAR IMPLANT
• Embryogenesis: Coc h lear nerv e fails to for m o Must also sho w no ben efit from conventional
hearing aids
Labyrinthine aplasia
• Imaging: No coc h lea o r vestibule present Demographics
• Embryoge nes is: Developmental arres t, 3rd gesta tiona l • Age: Cand idates m ust he > 2 yea rs old
week
Cochlear a plasia • Postlingually deafened pa tie nts (those wh o ha ve
• Imagin g: No coc h lea present already learned to spea k, usually > 5 yo) have best CI
132 • Embryoge nes is: Deve lopmental arre st, late 3rd o utco me
gestational week • Postoperative co m plications (5%)
o Transient eN? pare sis, imbalance , perilymph fistula,
Common cavity deformity ha rdware failure &. skin flap probl ems
• Im aging: Co alesced cystic coc h lea &. vestibule form a • 901}f 1 of CI patients rep ort basic sen te nce
com mon cavity und erstand ing afte r 6 mont hs
• Embryogenes is: Developmental arrest in 4th • Torq ue expe rience d by CI in 1.5T MR is sufficien t 10
gestationa l week cause im plan t m ovem ent; CI pa tie nts should not
Cystic co chleovestibular anom aly undergo 1.ST MR
o MR-compatible CI are now ava ilable
(inco mplete partition type 1) o External co m po ne n ts sho uld be remo ved in all cases
• Imaging: Coc h lea &. vestibule cystic with no internal
arch itect ure Treatm ent
• Em bryogenes is: Developmental arrest, 5th gestat ional • CI is effective reh abilitati on method fo r profoundly
week hearing im pai red patients who do not benefit from
hear ing aids
Lar ge endolymphatic sac anomaly • CI users should return to cli nic at least o nce a year for
(inco mplete partition type 2) speech processo r ad justme n ts
• Imaging: Larg e end o lym phatic du ct &. sac wit h mild • Postoperative result s depend o n number of
coc hlea r dy splasia in tracoch lear electrodes
• Em bryogenes is: Developmental arre st in 7th week • Alternative hear ing augme ntatio n o ptio ns
o Hearing aid
Labyrinthin e oss ificans o Ossicu lar p rosth esis
• Clin ical presentauon: Sensorine ura l hear ing loss, o Audi to ry brainstem implant
usually foll owin g meningitis
• Imaging: Dense bo ne fills part s of membra nous
lab yrinth
• If coc hlea spa red, CI still possible
• If both cochlea signifi ca ntly affected , relative Co nsider
co n traind icatio n to CI • Are there any contra ind icati ons to CI pla cement?
• Are there any findings th at might com plicate surgery?
• \Vhich side wo uld be easier for surgeo n?
IPATHOLOGY • Post-op pa tie n ts: Is CI in approp riate location (basal
tu rn of coc h lea)"!
Gen eral Features
• Etio logy: Prim ar y causes of hea ring loss = co ngen ital,
infection ISE LECTED REFER ENCES
• Epide m iolo gy: > 28 mi llio n America ns with hearing
1. Witt e RJ ct .11: Pedia tric a nd adu lt coc hlear implan tation.
loss in 1993 Rad iographies. 2:1(5):1IR5-20 0, 2003
Gross Pathologic & Surgical Features 2. Lo WW: Im agtn g o f coc h lea r and a ud itory brain stem
im plan tatio n . AjNR. 19(6 ):11-1 7-54, 199 K
• Placement of CI req uires parti al mastoidecto m y 3. Tcissl C ct .11 : Coc h lear im plan ts: in vit ro inves tigatio n of
electrom agnetic in terfere nc e at ~tR imaging-ccom pati hility
Microscopic Features and sa fety aspe cts. Rad io logy. 208(3 ):7(lO-K, 1998
• Beaded ap pea rance of sti m ula tor wire represen ts 4. Sh plzner BA ct .11: Postoperative imaging of the
ind ividu al sti mula ting electrodes mult ich annel o..x h tear im plan t. J\ j NR Am J Neu ro radiol.
16(7):15 17-24 ,1995
5. j ohnson MH ct .11: c r o f posunen tngn tc dca fncs..:
ICLIN ICA L ISSU ES o bserva t ions and p red ictive va lue for coc h lea r implan ts in
children. AjNR Am j Neuroradiol. 16(1):10:1-9, 1995
Presentation 6. Mukher]i SK ct .11: cr o f th e te m pora l bone: fin d ings after
mastoid ectomy, oss icu lar recons t ructi on , and cochlear
• Most co m mon signs/sy m pto m s: Seve re to profound
implantation. AjR Am j Rocntgenot. 163(6):146 7-71, 19 9~
bilat eral sen sorineural hearing loss (SNHI.) 7. Har n sbcrger HR et .11: Coc h lear im plan t ca nd ida tes:
• Clinica l profile -:·.·, . ~~ . ' ; :. assessm ent wit h cr and MR im agin g. Rad iolo gy.
o CI ca nd ida tes (n ust lie:> 2 yo wit h bilate ral severe 164(1 ):53-7, 1987
SNIIL ., ;. .
COCHLEAR IMPLANT
IIM AG E GAL LE RY
(I.ef l) Axial /C·ft ear I-bono

CT shows cochtesr implant
wire entering the round
window (arrow) at a normal
2
clngl/:'o Thfl .~ tinwl, l tor wire'
colltinucs up 'hl.' bcl .~ ,)/llJrn 13 3
to the reach the second turn
(oPPI1 arrow). (HiJ,lhl)
Co ro nal left eaf t-txoo CT
revea ls cochlear imp/'lIll
entf:'ring the round window
niche (arrow ). (rom tllp/{'
the stim ul,Jto r w ire pa sses up
the basal tum in to ttw
second /Urn ill nom",!
situation.
Variant
(Lef t ) Axial T-bone CT shows
C1has traversed ba sal turn to
wco nd tum (arro w).
Malfunction of C1is
!jL'(;OlJ(/ilry to break ill
mastoid portion of stinw tmor
wire (Opf'fl arrow ). Curved
arrow shows otosclerosis.
(RighI) AxiJlletl ear l -b one
C1 shows misplaced
CCJChfP,lf impl ant extending
into E u~ ta ch ia n tub o (arrow ).
8,H.1/lUfIJ of ("()chlpd (open
drrolV) is em pty as implant
has missed round window
niche (curved arrow).
Variant
(I.ef l ) Modified Stetwers
view of (('ft T-hone shows
redundan!, m isplaced
eac h/ear implant ex rending
into hypotympanic recess
(arrow). Receiver (open
arrow ) and magnet (curved
arrow ) are shown. (Highl)
Coronal maximum inten sity
projection bam! CT shows
misplaced cochlear implant
extending through
hypotympanum to
petroclival synchondrosis
(arrow). Inner ear
labyrinthine ossifican s is
present.
IMAGE"l POR RESONANGIA

MAGNn ICA, SA DE C.V
IRM 94~ 3 24 f-L5

::.-.. ._._--'" .--' ."
INTRALABYRINTHINE HEMORRHAGE
2
134
Axi.ll 11 WI , "fR !>IJO\VS p.1Cho/ogic hypcvinr('f}.~ signal Coroo.l1 T1\\1 MR shows p.,,/)oJogic h)'pt'fintf'n.'Oiry
wirh in \'C'Scibuk (am " v) comr..tt'l ll with ILl /. There was within fluid (jlbl ~ of tlk> 1.Ju,'rinlh consi~ll"'fll with
no (1t:~('f11ibk enruncC'fflt'flt III I on right sid<> (arrow ). I be Sll/X'rior .~ IdlC'fal
~('mk:irclll. )( camls can be !if 'W>() .
ITE RM IN O LO GY MR Findin gs
Abbrev iations a nd Syno nyms • TlWI
o Hlgh signal wit h in n orma lly fluid -filled space o f
• In t ral ab y rin t h in c he m o rrh age (11.1 1) labyrinth o n TI C·
• In n er ear h em orr ha ge, membrano us labyrin th ine • In ner ear normally low signal (fluid in tensity)
hemor rhage, pe rilymph at ic labyrin th in e hemorrhage
• T2W I
Definitions o High signal loo ks n orma l
• 1II0od within nor mally fluid -filled spaces o f la byrint h o Inner ear hemo rrh age no t visib le o n rh in-s ec tion
• Sudd en hearing loss: llearing loss tha t ha s evolved h igh-resolutio n '1'2 imag ing
over hou rs to days • TI C+
o At least a 30 d ecibel 1 in thresh old in 3 co n tiguous o High signal alrea dy p resen t
test freq uen cies over 24-72 h our pe riod o If pre-contrast ima ging not done, may be mistaken
for inner ear en ha ncemen t
Imagin g Re commen d ation s
IIM AGING FIN D IN GS • Include at least o ne T l C- seq uen ce in all inn er ca r
Ge nera l Fe atures p rotocols
• Best diagn osti c c lue
o Brigh t signa l o n T l C- t hi n -sect io n MR images
• In ner ea r flu id normall y iso ln tensc wit h CSF
ID IFFERENTIAl DIAGNOS IS
• llighl y protclnaccous in ner ear co n ten ts Illay h ave Subac u te la byrinth itis
ident ical a pp ea ra nc e • TI c+ MR high signa l (en ha nce men t)
• Locati on : Memb ra n ous labyrin t h o f in ner ea r o Focal o r diff use, usuall y faint
• Size: May be d ifrusl' o r segmen tal with in in ne r ea r • T1 C~ usu ally n ormal
spaces
• Mor phology: Co n fo rms toinner ear sha pe
DDx: Inner Ear Abno rmal Signa l
Labyrinthitis C- Labyrinthitis C+ Fibrous Tissue Scln vannoma

INTRALABYRINTHINE HEMORRHAGE
Key Facts
Termin ology Path ology
• Blood wi th in normall y flu id-filled spaces of laby rint h • Ge ne ral pat h co m me n ts : Shorte ne d '1'1 relaxat io n
time ca use d by intra- o r ex t race llular meth emoglo bin
Imaging Findings
• Hig h signa l wit h in n or m ally fluid-fill ed space of Clinica l Issues
labyr inth o n TI C- • Clin ical profile: Pa tie nt w ith h istor y o f a nt icoa gul ant 2
• Inclu de at least one '1'1 C- seq ue nce in a ll in n er ear the ra py, sickle ce ll di sease or traum a
protocols • Hearing mayor m ay not ret urn 135
Increased signal in associa tion with acoust ic Tre atm ent

sc hwanno ma • No ne , unless unde rlying co nd itio n
• Intralab yrinthin e h igh sign al on '1' 1 C- MR pr ob ab ly • Treat und erl yin g co nd itio n
seco n dar y to hi gh protein co n te nt
o Of len post-operative
Intralabyrinthin e sc hwa nno ma
• T1 C+ MR high signa l (foca l intense en ha nce m en t) Co nsider
• '1'1 C- h righ t signal on ly possibl e if lesio n hemorr hagic • ln travcsttbul ar lip oma in d iffer ential di agn osis
= rar e Image Interpretati on Pearls
Intralab yrinthin e lipoma • Always perform Tl C- & eva lua te for evi de n ce of
• '1'1 C- bright signal ma y ap pea r identical lnt ralab yrmth inc h igh signa l
• No t ty pica lly asso cia ted wit h sudde n hearing loss
ISELECTE D REFERENCES
IPATHOLOGY I. Hegar ty J L ct al: The val ue of en hanced magnetic
resonance imaging in the evaluation o f endococ hlea r
Ge ne ra l Features disea se. Laryn gosco pe . 112(1): 8-17, 2002
• Gen eral path co m me nts : Sh or tene d T l relaxatio n t im e 2. Schick Bet al: Magnetic reso nance imaging in patients
caused by int ra- o r ex tracellu lar m ethemo globin wit h sud de n he ar in g lo ss, tinnitu s and ver tigo , Oto1
Ncurotol. 22 (6):80H- 12, 200 1
• Etio logy
3. Sh inoh ara S etal : Clin ica l fea tures o f sudd en h ea ring loss
o In tralahyrin th ine hem orrhage in cit in g eve n ts
associated with u high signtl1 ill thvla bynnth 011
• Trauma un enhan ccd Tl -wcigh tcd m agn eti c resonan ce imaging, Eur
• Anticoa gu lant th er ap y Arch Otorhtnoluryngol. 257(9): 480-4, 2000
• Hemat ol ogi c lesions: Leu kem ia, sickle cell a ne m ia 4. Whi tehea d RE ct al: Spon ta neo us labyrin thi ne hemor rha ge
& o the r h yp crvi scostt y sy nd ro me s in sid le cell disease. AJ NR. 19:1437-40, 1998
• Neo plasm
IIMAGE GALLERY
ICLINICAL ISSUES
Pre sen tati on
• Most co m m on Signs / sym pto m s
o Acute o ns et unil at eral SNHL
o Other sym pto m s: Vertigo, tinnitus
• Clin ica l profil e : Pati ent w it h h istor y of an ticoag ulan t
the ra py, sickle ce ll di sea se o r trauma
Demographics
• Age: All
• Eth n icity
o Spo n taneo us ILH m ore co m m o n in
Africa n-Am eri cans du e to in cr eased in cidence in (I.e/I)Axial T1WI AIR shows pathologiC high Signal within the hasilar
sickle cell di sease coc hlear turn (arrow) on the right consistent with intraJahyrinthine
o Post tr aum ati c lU I = n o differ en ce hem orrhage (Tl C-J, (RighI) Axial T1 C+ AIR shows high .~ ig ll a l in
'Vestibule (arrow) & laleral semicircular canal (open ,m ows ). Signal
Natural History & Progn osis present on pre-contrast images, making diagnosis intral.lbyrinthinc
• Hea ring may o r may n ot return he morrhage, no t schwannoma.
SEMICIRCULAR CANAL DEHISCENCE
2
136
Corona! graphic illustrates thf:' principal findings of Coronal T-bnne CT shows absence of bony covpring on
superkx spmicircular canal clphiscence, absence of cephalad mergin of superior semicircular canal (arrow)
bone ow rlying sse (arrow) l~ JS5OCiatro thinning of d iagnostic of sec deh isC(·n ce. IK>\Vf. ,(/, thin t('gmf'fl
tegmen tympani (open arrow ). tympani a.~socia red (open arrow).
ITE RM INO l OGY MR Findin gs

Abbreviati on s and Syno nyms • T2W I
o Thin-sect ion hig h- resolu tio n T2 MR
• Semic ircu lar cana l dehiscen ce (SCD) • Coro na l: Absen ce of arcua te em ine nce bone
Definition s covering SSC
• se D: An extreme thinning or abse nce of hony roo f • Axial: Rest shows segme nta l abse nce of superficial
ove r superior or poster ior sem icircular cana l wall of PSC
• Tl C+: Look for aco ustic schwa n no ma as a lternative
explana tio n for vertigo
IIM AGING FINDINGS Imagin g Recomm end ati on s
General Features • Axial & corona l 'f-bo ne CT best tes t
o None nha nced, bone algo rith m, h igh -resolution (1
• Rest diagnos tic clue: T-bone CT sho ws dehi scence of
mm) CT
bone cove ring sup erior (SSC) or posterior (PSC)
o Oblique recons tructions in plan e of SSC o r I'SC
sem icircular ca na l
shows scope of dehi scen ce
• l.ocation : May be bilat eral
• Size: 2-4 mm dehi scent segme n t
CT Findings IDIFFERENTIAL DIAGNOSIS
• NECT Normal thinning of SSC or PSC wall
o Corona l 'l-bone C l'
• ,, 2 rnm de h iscence of roof of SSC • Asym ptomat ic thi nn ing bony cover of SSC or I'SC
• Extreme thinn ing of tegm en tympani ma y be occurs
associa ted • Usually seen o n o nly one coro na l or axial CT image
o Axial T-bo ne c r
• z 2 m m dehi scen ce of superficial bony wall of
I'SC
DDx: N ormal Vari ati on s in Arcuate Eminence (AE) Thi ckn ess
Normal AE Bone fCT) Normal AE Bone (M RJ Thin AE Bone (CT) Thin A E Bone (M R)
SEMICIRCULAR CANAL DEHISCENCE
Key Facts
Terminology Clini cal Issues
• Semicircular ca na l dehi scence (SCD) • Sou nd ± pressure-induced vestibu lar sym pto ms ± eye
• SeD : An ext reme th inning o r absen ce of bon y roo f mov em en ts
over sup erio r or posterior sem icircular ca na l • Tu llio ph enomen on: Vertigo ± n ystagmus relat ed to
sound
Pathology • Age: Mean age: SO j ran ge from 20-70 yea rs 2
• Un known, most likely a developmen tal anoma ly • Treat ab le form of vestibu lar disturbance
13 7
IPAT H O LO GY Treatm ent

Ge ne ral Feat ures • Treat abl e form of vestibular di sturbance
• Earp lugs & avo idin g pr ovoki ng stimu li
• Gene ral pat h co mme n ts: Similar clinical findin gs • Surgical resur facing of affected semicircular canal
describ ed with ch o lestea to mas erod in g horizontal beneficial
sem icircular can al
• Etiology
o Un know n, most likely a developmental an omaly
• Head injury or change in int racranial pressur e
(barotra uma) ma y fracture th in bone or desta bilize Co nside r
dura over pre-existent dehiscen ce • Vestibu lar symp to ms + positive Cl' = sem icircu lar
o Best h ypothesis for clinical findings: Open ing in canal dehiscen ce syn d rome
bone ove rlying se creates "Srd mobil e win d ow" into
in ner ca r th at allows canal to respond to sound & Im age Interpretation Pearl s
pressur e cha nge s in membran ou s labyrin th • Since usuall y u ni lat eral, use op posite sse as baseline
• Moti on at ova l win dow (from loud n oises) or 1 n or mal to co m pare suspicio us side with
intracranial pressure ma y th en cause bowing of
thin cov er over SSC or PSC
• Result s in "u np hys iolog ical moti on" of en dolym ph ISELECTED REFERENCES
in affect ed semicircu lar cana l J. Kromb ach GA ct <:1[ : Semicircular cana l dehiscen ce:
• Ep idem iology comparison of T2-weightt..-d tu rbo spin-echo MRI and CT.
o - 2% of populati on h ave thinn ing o r dehi scence of Neuroradio logy. 46 :326-3 1, 2004
bone over sse o n a uto psy ; 509·6 bilat eral 2. Minor LB ct al: Superior ca na l deh iscence syn d rome. Am J
o sse deh iscen ce sligh tly more com mo n than pse 0 '01. 2 1(1),9- 19, 2000
3. Mo n g A et al: Sound- an d p ressure-induced ve rtigo
G ross Path ol ogi c & Surg ica l Features associated wtih dehiscen ce o f the roof of th e superior
• Su rgical view shows absen t bony cover sse o r rse sem icircular ca na l. AjNR. 20 :19 7:{-7S, 1999
ICLIN ICA L ISSUES IIM AG E GALLERY

Presentation
• Most co m mon sign s/symp to ms
o So un d ± pr essur e-induced vest ib ula r sym pto ms ± eye
mov em ents
o Oth er sym pto rns & signs
• C h ron ic diseq uilibrium ma y be debilitating
• Oscillopsia (oscilla ting vision)
• Tullio ph enomenon: Verti go ± n ysta gmus relat ed
to so un d ·
• Hcn nebert Sign : Vertigo , n ystagmus,
disequ ilib rium ± nausea in response to
pne uma-otoscopy
(Left ) Coronal T-bone CT slw ws unroofed superior semicircular canJI
Dem o graphi cs (arrow) with thinnf' d lcgmen tympani (open arrow) diagnostic of
• Age: Mean age: SO; ran ge from 20- 70 years superior semicircuier canal dehiscence. (RiKht) Coronal T2WI MR of
T-bone reveals cort ical hon e covering SSC is gone (arrow).
Natural Hi story & Progno sis
• Slow ly progressive sym pto ms
FENESTRAL OTOSCLEROSIS
2
138
Comn..11 Br.Jphic illusl.ratt"5 J -donut· oI05pongiotic CorollJl right T-bO(}f-> a mvs ({'f)(>5tral Olcxc!erosis
,N,IC/LIf.· (.lrrO\vsJ Ihlt surrounm lilt.· ~ r.Jpes fOOlpLll(' in invol'lirw ,111 "lalgjtJ~ oIlhe OVJI window (arrow ). TIx-
the oval w;fl(b .... Tilt.' cri5pfTJdfgim of rhe oval w1rwv IK"l effect. is to efl.'die a blurring and disappearance 01
drL' obscUH'f! by p/.HJLX!. lh t' OV.1J winchv niche.
ITE RM INO LO GY CT Find ings

Abbrevia tions a nd Syno nyms • NECI'
o F."ly bo ne c r findings: Radiolucent locu s seen at
• Abb reviatio n: Fenestral o toscl ero sis (l-Oto) an terior margin , oval wi ndow
• Syno nyms: O tospo ng rosis, fenestr al oto spo ng losis • Spread s to involve a ll margin s of oval &. ro und
Def init ion s wind ows
• rOta : Pathol ogic co nd it ion o f peri fen estra l hony • In mo re severe cases of FOto, m ultiple radi olu cent
lahyrinth of unknown cause wh ere spo ngy ho ne foci foci present
appear • If spread s to inner ea r o tic capsule, bo th FOw I<
• Fissula an te fenestra m: Cleft o f fibrocartilaginous coch lear o toscleros is (COto) diagn osed
tissue betwee n inner & mid dle ears just a nterio r to o Late, ch ronic bone cr findin gs: Heal ing pha se
oval wind ow sho ws heap ed up new bo ne alo ng ova l &. rou nd
window ma rgin s
• Oval ± round win dow may becom e occluded by
IIM AGING FIN D IN GS healed plaqu e
• CECr: No role for CECr in di agn osis o f FO to
Gen e ra l Featu res M R Fi nd ings
• Rest diagnostic clue: Bone CT: Lytic (o tospo ngiot ic)
foci on an ter io r marg in of oval wind ow • T2WI
o Thi n-secti o n high-resolutio n '1'2 m ay not visua lize of
• Locati on FOlo, even wh en ex ten sive
o Initial locati on : Anterior margin oval wi ndow
o Large plaq ues visible ou t su btle
(fissula ante Icn estram)
o May invo lve any bo ne along med ial wall of m idd le • T1 C+
o Shows en hanci ng pun ctate foci in media l wall of
ear
mid dl e ea r
• Size: Millim et er punctate perifenestral foci may
• Most o bvio us whe n FOto &. COto co m bined
beco me con fl uent
o Ch ro nic, severe cases sho w multiple en ha nci ng foci
• Morpho logy: Ovoid plaqu es most co m mo n
along margins of oval &. round windows
D Dx: Abnormal Bone Around Oval Window
Bony Labyrinthitis Tynipenosclerosis Fibrous Dysplasia Radiation ClJ.1nge

Key Facts
Terminology • Late, chronic bone CT findin gs: Healing ph ase shows
• Syno ny ms : Ot ospon glosts, fen est ral o tos po ngiosis h eap ed up new bon e alo ng oval & roun d window
• FOlo: Path ologic co nd ition o f peri fen estral bony margins
labyrin th of unknown cause wh ere spo ngy bon e foci • Shows enhancing punctate foci in med ial wall o f
appear middle ear
• Fissul a ante fen estr am : Cleft of flbror artil agln ou s Top Differ ential Diagnoses
2
tissue between lnner Sr middl e ears just anterior to • Bon y lab yrint h infect ion
oval win dow 139
• Tympan osclero sis. post- infl ammatory n ew bone
Imaging Findings deposition
• Best d iagn ost ic clu e: Bone CT: Lytic (otos po ngiotic) • Paget disea se
foci on anterior margin of ov al window • Fibrous dysplasia
• Early bon e CT findings: Radiolu cent focus seen at • Osteo rad ionec rosis
a n te rio r margin , ov al window Diagnosti c Chec klist
• If COto presen t, FOto also is present, loo k for it!
o En hanced MR sensitivity to FOto un kn ow n • Clin ical: History of sku ll base o r na sophar yn geal
rad iation the rapy
Imaging Recommendati on s
• T..bon e c r best imagin g too l for di agn osin g FOto
• High -reso lut ion T2 MR may miss l'Oto
• T1 C+ MR shows en ha ncing foci in active pha se of
IPATHOLOGY
FOt o Ge ne ral Features
• Gen eral path com me n ts
o Bony o tic ca psule developme n t: 3 layers
IDIFFERENTIAL DIAGNOSIS • Th in inner endostea l layer
• Middl e layer of co m bined e ndoc ho nd ral &
Bon y lab yrinth infecti on intrach on dr al bo ne (FOto occ u rs here)
• Imaging: Destructi ve foci in bo ny labyrin t h • Outer periosteal layer
• Clin ical: Acute o to mas to id itis o "Otos pong iosis" is better term t han oto sclerosis
Tympa nosclerosis, post-infl amm ator y new • Describes acti ve d isease process, no t ch ro nic ,
h ealing ph ase of FOto
bon e deposition o Norma l FOt o progression
• Imagin g: Post-inflammat ory new bone deposition is • FOto begin s at fissul a ante fenest ram
not lim ited to oval & round win dow s as wit h most • Disea se spreads from fissula an te fenc st ram
FOto posterio rly alo ng oval wi ndow ma rgins to round
o Seen in tympan ic membra ne (TM), middl e ear, window
ossicles an d mastoids • Con tin ued act ive d isease spreads to otic ca psu le
o New bone deposition is irregul ar, n ot smoot h in (bot h FOlD & COto presen t)
oval wind ow a rea o Active FOto fixes sta pes footplate in oval wind ow
• Clin ical: Obvio us ch ron ic middle ca r-ma sto id ni ch e
in flammato ry d isease • Th is "d on ut" FOto a n kyloses stapes footplate
Paget disease • Patho ph ysio logy o f con ductive hearin g loss
• Gene tics: Spo radic or auto som al do m ina nt gene
• Imagin g: Diffuse skull base in volvem ent is rul e
t ran smi ssion
o Diffuse in vol vement of bony labyrinth, n ot
• Etiology: FOto etiology unkn own
confined to lateral wall
• Ep ide mio logy
o Usually seen as a d iffuse tem poral bon e o Occur s in JlMI of pop u lation
"co t to n-woo l" a ppea rance
o Most co m mo n type of otosclerosis is FOto (85911) vs
• Clin ical: Bon e disease o f o ld age (> 50 year o lds) COto (15%)
Fi brou s dysp lasia o In ad ult patien ts wit h co nd uctive h ea rin g loss, FOto
• Imagin g: Inv olves all par ts of te m por al bo ne respo nsible in - 9{)lKI
o Relat ive spa ring of inner ea r is rul e • Associa ted ab no rma liti es: COto presen t in 5 159-(1 o f
o Usually scle rotic, grou nd glass in ap pear a nce cases of FOto
• Clin ical: Bo n e disease o f yo ung « 30 yea r o lds) Gross Path ologic & Surgical Features
Os teo radione crosis • Otoscopic vascu lar hu e behind tym pani c mem bran e =
• Imaging: CT shows diffu se, permeative lucen cies of Sch wa rtze sign
otic capsu le
o Rep resen ts active o tos cle ro tic areas alo ng marg in s o f o Term "coc h lea r cleft" has been used to d escribe
oval &. rou nd windows fissula an te fenestrarn
• Bon y a nkylosis 01stapes lootplat e is reflect ed as sta pes • 3T MR w ill m ake otos clerosis mor e appa ren t o n Tl C+
immohilizati on when pulled o n by sur geo n MR im ages
Microscopic Features Image Interpretation Pearls
• Spongy, vascular, d ecalcifi ed , irregula r bo ne fo rmatio n • Find ova l wind o w o n axia l bone cr « int errogate area
2 a In enchondr al layer 01 labyri nth alo ng m argi ns o f
ov al &. round windows
fo r rad ioluce n t plaque
• If p resen t, check area aro u nd rou nd win dow « o tic
140 • Three path o logic phases FO to ca psule
o Acute phase: Depo siti on of islets 01 os teo id tissue o If rad io luce nt areas arou nd o tic ca psule, CO to also
o Subacu te p hase: Spo ngiotlc remodeling w ith presen t
osteoclasts ca using focal bone resorption
o Ch ro nic-sclero tic p hase: O steoblasts crea te new
bo ne with irregular featu res th at resem ble a mo saic ISElECTED REFERENCES
• May he h isto logicall y ind isti nguishable lro m Paget I. ChadwellJil et al: The cochlear cleft. AJf';RAm J
di sease Ncuroradiol . 25( 1):2 1-4, 2004
2. Pckkol a J et 011: Locali zed pe rlcoc h lear hypoattcn uat lng fod
at tempo ral-bone uu n-scc tton CT in pedi atri c pa tient s:
ICLl N ICA l lSSUES nonp athologtc d ifferen t ial diagnostic enti ty? Radio logy.
2:1O( 1):1l8-92 , 2004
Presentation :i. Ko mlinl-Gilli E et 011: IOtosl..- lt'[llsls surgtcal tech n tqucs and
result s in ISO pa tie n ts] An n Otolary ngo l C h ir Ccrvico fac.
• Most co m mo n signs/sym p to ms
119 (4):227-:n , 2002
o Bilateral p rogressive co nd uctive hearing lo ss
4. Slim mer I I et al: Magn etic resona nce imagin g and
o Ot her signs /sym p to m s high -resolut io n co m puted to mogra phy in t he
• Tin ni t us (ringing in ears) otospo ng iotic phase of o tos cle rosis . O RL J
• O toscopy: Vascular hu e behind tym pan ic Oto rh tnclary ngcl Relat Spec 64(6 ):45 1-3, 2002
membran e = Sch wartze sign 5. Chole RA et 011: Path oph ysio lo gy of o to scle rosis. O tol
• Clinical pr ofil e: Young ad ult p resen ts with Ne urOIO!. 22(2):249·57 , 200 1
u nexplained hilat eral p rogressive conductive hearin g 6. Sh in YJ et al: Sensor ineura l hearing los s a nd o tos clerosis: a
lo ss clin ical and rad io log ic survey of 43 7 cases. Acta
Oto laryngo !. 12 1(2):21Xl-4, 200 1
Demographics 7. Sh in YJ et 011: Cor rela tio ns be twee n co m puted to mograph)'
find ings and fam ily h isto ry in o tosc lero tic patien ts. Otol
• Age: Appears in 2nd to 3 rd deca d es o f life
Neurotol. 22 (4):46 1-4, 200 1
• Gende r: M:F = 1:2 8. Rucken stein MJ ct al: Ma na gem ent of fa r ad vanc ed
Natural History & Prognosis o tos clerosis in the era o f coch lea r implanta tio n . O to l
Neuro tol . 22( 4):47 1-4, 2001
• Co nd uc tive hearin g loss is p rogressive 9. Veillo n F et a l: Im aging of the windows of t he tem por al
• If round windo w is obliterated , stapedecto m y will fail bo ne. Sem in Ultrasou nd cr MR. 22(3):27 1-80, 200 1
• If round window oblitera tio n present bil at erall y, 10. Niyazov D et a l: Fenest ratio n surgery for o tosclerosis: CT
coc hlea r im plan ta tio n may be mor e cha llengi ng find ings of an o ld su rgica l proc edure. AJf\' R Am J
• Fluoride tr eat me nt ea rly in d isease may stabilize Neuroradlol . 2 f (9): 16 70 -2, 2()()(J
hearing loss I I. Ziye h S et .11: Mltl -visible pe rlcoc hl ear lesio n s in
os teo ge nesis Im perfecta type I. Eu r Radi al. 1O( IOJ:1675·7,
Treatment 2000
• Staped ec to my follow ed by p rosthesis ins ert io n 12. Zlyeh S et al: MRI of ac t ive o tosc lerosis. xcurc rac nology.
39 (6):453 -7, 199 7
o Resu lts negat ively impact ed by co nc u rren t COta
B, Mturu M et <1 1: Co m puted to mographic Image analysts of
• Co ch lear implantat io n ca rs wit h o tosclero sis. ORt. J O to rh ino la ryngo l Relat Spec.
o Used whe n severe FOt o I< CO to presen t bilat erally 58(4):2IXI-3, 1996
resu lti ng in p rofo und m ixed hearin g lo ss I ·t v atvcssort GE: Imagin g o f o tos cle ros is. O tul aryngol Clin
• Fluorid e t reatmen t used il FOto &. COto bo t h p resent Nor t h Am. 26 (3):35 9· 71, 1993
IS. Wi lbrand It F: Radi oan at omy of coc h lea r and stapedial
o tos cle ros is. Scnnd Audiol Su ppl . 30 : 18 1-3, 1988
IDIAGNOSTIC CHECKLIST 16. Mafcc MJ= ct a l: Use o f CT in stapedia l o toscl eros is.
Radi o log y, 156(3 ):709 · 14. 19115
Con sider 17. Swar tz J1) et a l: Fenestra l and coch lea r o tosclerosis:
co m puted to mogra phic ev al ua tio n . Am J 0 101.6 (6):476-81,
• II COto pr esent, FOlD a lso is p resen t, loo k for it ! 1985
• In cr eva lua tio n o f co nd uctive hearin g lo ss, must
always look at a n te rio r o val window margin lor FOt o
o II this area is no t carefully eva luated, rad io logist will
m iss diagnosis as CT find ings may be subtle
• Multidet ecto r cr so meti me s shows normal fissu la an te
fen estr arn o n ped iat ric T-bo ne exa ms as focal
rad io lucency
IIMAGE GALLERY
Typi cal
( IL f l) Axial 1-bone ( 1
demonstrate s .1 clessk:
o to sp o ngio tic p laqu e (arrow )
as an extra lucen t foci on the
2
anterior margin o f the oval
window (fiH u /a .l l1le 14 1
tenesuem location ). One
caps ule otherwise spared.
(Right) Coronal bon e CT
shows patient that has
undergone stapedectomy to,
fenestral oto.~c1er(b;5 .
J\·fctallic stapt's prosthesis
can be see n. Also not e
otospongiotic plaqu£> iu.~ t
anterior to oval window
(arrow).
(l.£f l ) Axial bone ( 1 ShOWS.1

mixed ar rive S healing
fenestral otosclerotic plaqu{'
(arrow) at the expe cted
location of the fis5u/a ante
tenestrem on the anterior
margin o f the ova! window.
(Rig ht ) Coronal bone in a
patient with long history o f
conductive hearing 10 55
reveets a "heal Jf..' d up ·
p laque just anterior to o val
window on high cochlear
promontory (arrow) .
Variant
(Lef l) Axial bone CT
dem on strm es severe case of
combined ff'n estral (arrO\\I)
& cochlear (open arrow)
otosclerosis. Schws n ze sign
was clC'arly s{'('n on
otoscopic exe mkuuion.
(Righi) A~;.11 bo ne CT in
palienl wi th bOlh fenestral
o tosclerosis (arrow) /:..
coc bles r otosclerosis (0/X'n
.1rrow) . Tht' IAC ma rgin
coctitcsr otosctcrottc plaque
is an alypical location.
5tapt's prmtht'sis pre~{'n l.
COCHLEAR OTOSCLEROSIS
2
142
AxiJ f Waphic illustrates cLl.~.~ic example of cochlear Axial T-bon e CT shows COlo as osteolytiC foci (arrow)
olOSCkY05is. Notice ol~pongioric plaques in halo forming ('xtra ·cochlear tum ". f O to is.'iffY ) as uJnfluent
aroun d coch /(oa (arrow s). A lso no te concurrent [cnC'stral di.<>ei15e along cocbtesr pr omontory from iissula ante
oIo .;cIPfo!>is topon arrow ). fenefotram (open arfO\YSJ.
• Bony pericochlear lab yrin th may take o n "ha lo"

ITERM INO l O GY ap pea rance wh en involvem en t diffu se
Abbre viations a nd Syno nyms • As d isease progresses, may spread to an y portion
• Abbreviati on: Coch lea r o tos clerosis (CO lO) o f bony lab yrinth includ ing lat eral wa lls of
• Syno nyms: Coch lea r o tos po ngtos ts, retrofenest ral intern al aud ito ry cana l
o tos clerosis, Beetho ven ma lad y o Ch ro nic COto : Mixed rad iolucc nt- rad tode nse foci
present in bon y labyrinth
Definitio ns o Perifen estral (ova l & round window s) foci indi cate
• COlo: Primary lyti c disease o f encho nd ral layer of conc u rren t fene stra l otoscleros is (FOto )
bony labyrin th of unknown caus e • Healing! hea ped u p new bo ne suggests chro nic
ph ase r Oto has begun
• CECT: No role for CECf in diag nosis of COto
IIM AGING FINDINGS MR Find ings
Gene ra l Features • Tl WI: Ring of int ermediat e signal in pericoch lear &
• Best diagnostic clue: Focal lyt ic plaq ues in perico ch lea r perilabyrinth ine regions
bon y labyrinth on T-bone Cf • T2Wl
• l.ocati on : Pericochl ear bon y labyr in t h o Thin-section h igh-resolution T2 may not visualize
• Size: Pun cta te o r linear millimeter foci COto, especially wh en mild to mod erat e in severity
• Morphology: Ovo id to linear (con fluen t foci) o Even large COto foci may o nly show as subtle
peri coch lear high signa l
CT Findi ngs • T1 C+
• NECf o COto sho ws as enhancing perico chl ear foci marking
o Active COto : T-bone CT shows rad iolucent foci in lesions in encho ndral layer of bon y labyrinth
pericoc hlear distributi on o In more severe cases, en hanc ing lesio ns may be seen
• Term "focal dem ineralization" ha s been applied to an ywhe re in bony labyrinth
radi olu cent foci
• when severe, "do uble-ring sign " appears as low
de nsity ring su rrounding cochlea
DDx: Inn e r Ea r l esions
Osteogen. tmperiects PJgel D isease Fib rous Dysplasia Ost eoradion ecro sis
Key Facts
Te rmin ology • Osteo rad ionecrosis
• Abbreviatio n: Coc h lear otos clerosis (CO to) • Ot osyphilis
• Synon yms: Coch lear oto spo ng tosts. ret rofen est ral Pathology
otosclerosis, Beethoven mal ad y • CO to bil a tera lly sym me t ric in 8S(11, o f cases
• COto: Primary lyt ic d isease of enc h o ndral layer of
bon y labyri n th of unknown cause Clinical Issu es 2
• Clin ical profile: Young ad ult pr esents w ith
Imaging Findings unex pla ined bi lat era l, p rogressive, m ixed hearin g loss
14 3
• Best dia gn ostic clu e: Focal lytic plaq ues in
pcrlcoch lea r bon y labyrin th o n 'l-bo nc c r Diagnostic Checklist
• Axial & co ron al T-bone CT is best imaging too l fo r • Watch for punctate foci of en hanceme n t in bony
d iagnos ing COto and FOto labyrinth in pati ents un dergo ing en h anced MR to
"ru le o ut acoustic schwan no ma"
Top Differ ential Diagnoses • If hon y encroa ch me n t o n memb ranous labyrint h is
• Osteogenesis im perfecta tarda present , radi olo gist is lookin g at labyrinth ine
• Paget disease os slftra n s, not coch lear oto sclerosis
• Fib rous d yspl asia
Imaging Recommendation s IPATHOLOGY

• Axial &. coro na l T-hn ne CT is best imaging tool for
d iagno sing CO to and l'O to
Gen eral Features
• High-reso luti o n '1'2 ~m may n ot iden tify COto • Genera l path com me nts
o T2 MR is n ot a good mod ality choice if t h is o "Otospo ngiosis" is bette r term th an o toscleros is
d iagnosis is suspec ted • Describes active d isease process, no t h ealing ph ase
• T J C+ MR n ecessar y to delin eat e act ive COto foci • Note: Lite rat ure uses term otosclerosis n ot
o No st udy yet to indi cat e sensitiv ity of en ha nced MR o tos pangiosisx
to presen ce of COlo o COto bilat era lly sym met ric in H S l ~'h of cases
• Ge ne tics: Spo rad ic O f au toso mal dominan t gen e
transmissio n
IDIFFERENTIAL DIAGNOSIS • Etio logy
o COto etio logy un known
Osteogen esis imp erfecta tarda o SNHL in COto etiology h ypotheses
• Clin ical: Ch ild ren wit h b rittl e bones &. blue sclerae • Best hypo th esis is SNIIL resu lts from co m prom ise
• Imaging: Find ings arc in distingu isha ble from CO ta of spiral ligamen t
o Looks like very seve re form of COlo with more • Secondary h ypothesis suggests th at to xic p ro tcases
gene ralized demineral izat ion of bony labyrint h affect nerve cells of coc h lea
• Epid emiology
Paget disease o Occur s in pj(, o f popu lation
• Clin ical: Elderly pati ent s o Two types o f otos clero sis
• Imaging: Mo re di ffuse invo lvement o f ca lvarium &. • Fenestral otosclero sis (HSlY6)
sku ll base th an CO to • Coch lear otos cleros is (I Sl}h)
o Diffuse bon y labyr in th in volvemen t no t co nfined to • Associated abn or malities
enc ho n d ral layer o w heu COto is presen t, FOt o gen era lly also present
o Diffuse bony en large me nt is rule • FOto o ften ex ists wit hout CO to
o Usually see n as a d iffuse, "cotto n-wool" appearance
Gross Pathologic & Surgical Features
Fibrou s dysplasia • Otoscopic vascular h ue beh ind tym pan ic membrane is
• Clin ical: Bon y disease o f yo ung « 30 years old ) referred to <IS Sch wartze sign
• Imaging: Invo lves all part s o f tem poral bo ne o Sch wa rtze sign represen ts active otos clero tic area
o Relati ve sparing of o tic ca psule is rule just ben eath surface of coc h lear pro mon to ry
o Usually sclero tic, grou nd glass in ap pearance • Co ncu rren t FOto may ankylose stapes foo tplat e
o Cause o f sta pes im mob ilization when pulled a ll by
O steoradion ecrosis su rgeo n
• Clin ical: Histo ry of brain, sku ll base o r naso pharyngeal
rad iatio n th erapy Microscopic Fea tures
• Imaging: Cf reveals d iffuse, perm eati ve rad iol ucency Encho nd ral layer o f bo n y lab yrin th d isp lays spo ngy,
o f o tic ca psule vascu lar, decalcified, irregula r focal bo ne forma tio n
• Three path olog ic phases COto
O tosyphilis o Acu te p hase: Deposition o f islets o f o steoid tissue
• Clin ical: Adul ts with system ic syp hilitic infect ion ; rare
• Imagin g: Lyt ic lesion s o f otic ca psu le may m im ic COto
o Subacu te ph ase: Spo ngiotic rem odeli n g with
os tcoclasts causing resorpt ion of bo n e & creating Im age Interpretatio n Pearl s
large cavities • CO to is di sease o f bony labyrinth , n ot m em b ran ous
• Ot o spo ng iosis see n as foca l demineralizatio n in labyrin th
otic ca psu le • If bony en croach m en t o n m em b ranous labyrinth is
o Ch ro n ic-sclero tic pha se: Osteoh lasts crea te new presen t, rad io logist is lookin g at labyrin th in e
ho ne with irregu lar features resem blin g a mosaic ossifica ns, no t coch lea r otosclerosis
2 • Lesions poorly seen in otic capsule becau se already
most den se bone in hu m an body
144 ISELECTED REFERENCES
I. Goh JP et al: :ViRl of cochlear otosclerosis. Br J Rad iol.
ICLINICAL ISSUES 2.
75(89 4):502-5, 2002
Cl1OI<.- RA et al : Pathoph ysio logy of otosclerosis. Otol
Prese ntation Neuroto l. 22 :249-5 7, ZOO I
• Most com mon signs/sy mpto ms 3. Declau F ct al: Prevalence of otosclerosis in an unselcc ted
o Bilat eral, pro gressive, mixed h ear ing loss series o f temporal bones. 0 101 Nc u ro tol. 22: 596 -602, 200 1
4. Shin YJ et al : Sensorineural hearing lo ss and o to scle rosis: a
• Mixed heari n g lo ss means bot h co nd uctive clinical and radiologic sur vey of 43 7 cases. Acta
hearin g loss (CHL) &. senso rine ura l hearing loss Ot o raryngol. 12t (2):2(XI-4, 200 1
(SNHL) co m po ne n ts p resen t S. De rks W cr al: Fluorid e therapy for cochlea r otosclerosis?
• CHI. fro m FO to an aud iometric an d compute rized to mography evaluation.
• SNIIL from COto Acta Otolaryngol. 12 1(2):174-7, ZlX»)
o O the r signs/sym pto ms 6. Salvinelli F et a1: Otosclerosis and coc h lear otosclerosis: a
• Tin n it us (ringi ng in ea rs) pos t mortem study o n tem por al bones. Eur R l' V Mcd
l'harm acol Sci. 3(4):179·82, 1999
• Vertigo 7. Lippy W H et a l: O to scle ro sis in th e 19 60s, 1970 s, 1980s,
• May become wo rse du rin g pr egn an cy o r lactatio n an d 1990 5. La ryn goscope . 109(8 ):130 7-9 , 1999
• Otosc o pic vasc u lar hue beh ind tym pan ic X. Hua ng TS ct al: Coc h lear implantation in a patient with
m em b ran e = Sch wartze sign osteogen esis im perfccta and otospongiosis. Am J
• Clin ica l pro file: You ng adu lt p resen ts wit h o toraryngot. 19(3):209-12, t998
unexplai n ed b ilat e ral , progressive, m ixed hear in g loss 9. Ramsde n It ('I al: Cochlear implantation in otosclerosis: a
unique posit ioni ng and programmlng problem. J Laryngol
Demographics 0 101. 111(:1 1:262-5, t997
• Age : Beco m es sy m pto m atic ill Zn d & 3 rd decad e 10. G u n e rt EA ct al: High-resolution computed tomographic
• Ge n de r: M:F = 1:2 evaluation of the cochl ear capsule in otos clerosis:
relationship between densitomet ry and sensorineural
Natural Hi story & Prognosis heari n g loss. Ann 0 101 Rhinol Laryn gol. 105(8):659-6-1,
• Un trea ted CO to will gradua lly wo rsen t owa rds t996
II . Vartt al n e n E ct al: Value of computed tomography (Cl) in
pro found h ea ring loss
the diagnosis of cochlear otosclerosis. Clln Otolaryngol.
• Fluoride t reat m en t ca n a rrest pr ogre ssio n of SNHL 18(6):462-4, 1993
o PAu ly di agno sis p rio r to o n set o f p ro found SNHL 12. v atvasso rt (jE: Imaging of otosclerosis. Otolaryngol Clio
cr itica l Nort h Am . 26 (3):359- 71, 1993
13 . Huc b MM et al: Otosclerosis: the University of Mi n nesota
Trea t me nt temporal hone collection. Otolaryngol Head Neck Surg.
• CO lo treated ea rly with flu o ride the rap y 105(:1):396 -405, t99 I
o Lo n g term fluo rid e admin ist ra tion n ot fou nd to he 14 . Hav rilia k D e t al: Cochlear otosclerosis presenting in
bett er tha n shorter ( 1-2 yea r) co urse children: a case report. Am J 010 1. 12(1):61-:i, 1991
• Wh e n CO to is bila teral &. seve re, ma y lea d to need for 15. Wilhrand HF: Radioanatom y o f cochlear and stapedial
coc h lea r imp la n tatio n otosclerosis. Scnnd Audiol Suppl. :iO: 181 ·3, 19M8
16 . Hino josa Rei al: Otosclerosis and sensorineural hearing
o If FOto ha s o bscu red rou nd win dow wit h h eaped u p,
loss: a histopatho logic study. Am J Otolaryngol.
h eal ed bo ne, access to coc h lea r basal turn m ay he 8(SI:296 -:10 7, 198 7
blocked 17. Fo rquerun c t ill: Coc h h..-a r o toscle ro sis: a revi ew of
• For Ci ll . assoc iated wit h FOt o. sta pedecto my fo llow ed audiometric findings in 150 cases. Am J Otol. 8(1):1-4,
by p rost hesis in se rtio n 198 7
1R. Swartz J D et al: Fenestral and cochlear otosclerosis:
computed tomograph ic evaluation. Am J Otol. 6(6):476-81,
IDIAGNOSTIC CHECKLIST 1985
Co nside r
• If yo u find CO to o n T-bo ne C'l, loo k for associat ed
FOto in ova l window ar ea
• Watc h fo r punc tat e foci o f e n h ancemen t in bon y
lab yrint h in pati e nt s u nd ergoi n g en ha nc ed MR to
"ru le o ut a co ustic sc hwa n nom a"
• Expect th at T1 C+ images o n 3T Mil will creat e greater
co ns picu ity o f enha nci n g CO to lesions
IIMAGE GALLERY
fu l ' } Axial T-bone CT of
right ear shows mild mixed
coc hlea! o tO!ocll'fOSis (drrm v)
end (PIJ('strJI otosclerosis
2
topen arrow). N o tice f O W
pl aque L~ in toc suion of 14 5
(is .~u'a JfJIC km ostrern .
(RighI) A xial 't2WI MR
</PfJlOflSlrdles o tospongiotic
plaque in pat ient w ith
cocbtesr o toSdf:'TOSis as J
high signal crescen tic foci
ju st medial ro b,Hdl turn of
cocbtoe (arro w).
t/. t'/tJ A xial bone CT reveels

bilate ral cochlear
o tosclerosis (arrow s) .15 h" los
of lucent foci 5Urrowu!ing
both cochleas. rOro is
diegnosod by fk su/a ante
tenestrem active
o tospongiotic rod (op !..'11
arrows ). (Rig hI) Ax i,)1 T1 C+
MR shows hi/a INa/ seven"
cCKN e,Jr ouncterosis. Ibe
imaging diJgoosi" is made
b.1Sro on curvilineJr
enhancement ~(-'t'n
surrounding both cccblcs
(arrow s). Opf:'n arrows:
Bi/Jte r.l1 row.
Varian t
(/~fl) Coro nal l -bon e CT of
right ('Jr revests TO RP stap es
prosthesis (arro w) li d S been
used 10 treat t Cno . Coc hlosr
otosdprosis is also prt'wnt as
lucent irony lab yrinth ksci
(open arrow). (Right) AX;.ll
t -booe CT of le ft ear rt:!vt'iJ/s
com bined COto (arrow) '"'"
r O lo (open arrow ) de<lfne.~ ",
h clS been treated with a
cochkdr;mpMn tdevke
(cu r ved arrow).
ASYMMETRIC MARROW, PETROUS APEX
2
146
Axial Tl Wf MR. shows mmp;cuous blight "lesio/l n in Axial boo o CT .~ hows asymmetric marraw in ceuoos
right petrous apex (arrow) suspicious for · cho/esterol apex (arrow) . Notice op posite parous apex is
W.lllulot1J.1 ". CT l\ far-saturation 11 MR rpvpak>d it to 1Jc. pnew Jl.ltizPd (open arrow), A~ymmel ric {,my m,1!ro\V
J.~ ymll l{'tr;( · ';ltfy marrO\\I. .~/ )tl ce'i may aplx',lr quite conspk uous on r, MR.
• T2WI: W h en fatty, AM-PA will fo llow su bcu ta ne ou s
ITE RM INO l O GY fat with J signa l
Abb reviations a nd Syno nyms • T l C+: Fat-sat u rated seq uences co n firm d iagn oses of
• Asy m m etric m a rrow, petr o us a pe x (AM-PA) n ormal AM-I'A
• Pet ro us apex pscudo lesio n Imaging Recommen dati ons
Definitio ns • "Lesio n" is 1st suspected o n brain M R cxa rn lna tion
• AM-PA: Asym m etric ae ra tio n o f PA w it h without fat- saturati on
no n -pneumati zed m a rrow space in o ppos ite PA • Fat-satu ratio n seq uence co n firms "leave-me-alone"
sim ula ti ng m ass lesion nature
• Most co m mo n ly an in cid ental find ing on brai n MR
o 'l-bone cr reco m mend ed to assess su rrou nd ing
IIM AGING FINDINGS anato m ic lan dscap e
• CT with bone a lgo rit h m can ensu re no wo rrisome
Ge nera l Features cha nges to trabecu lae &. lack of ho ny ero sions
• Best di agn o st ic cl ue: Asymm etric aerate d PA across
from opposit e norm al PA bon e m arrow in absence o f
cx pansile ch anges ID IFFERENTIA l DIAGNOSI S
CT Fi nd ings Co ngenita l choles teato ma , PA
• NECT: ROl1e algo rit h m im agin g shows norm al PA a ir • C l: Smo oth , expansllc lesio n
cells ac ro ss from n o rm al PA marrow space • MR: Low TL, high '1'2 signal mimics mu cocele
• CECT: No abnorma l en hanc eme n t is seen
Trapped fluid, PA
M R Find ings • C I: Non-expansilc, opacified PA a ir cell'
• Tl W I • MR: Low Tl , hi gh T2 signa l in most cases
o No n -p neu m at ized PA shows h yp er intense TI
n o rm al fatty marro w
Apical petro sitis
o If red ma rrow, may be o f in termed iate signa l • C f: Destructiv e lesion with trabecu la r &. co rtical loss
DD x: Petro us Apex l esions
Cholesteatoma, PA Trapped Fluid Apical Petrositis Cholesterol Granuloma

ASYMMETRIC MARROW, PETROUS APEX
Key Facts
Termin ology Top Differential Diagnoses
• AM-PA: Asym metric aeration o f PA with • Co nge n ita l cho lestea to ma , PA
no n -pn eum at ized marrow space in opposite PA • Trapped fluid, PA
sim ulating ma ss lesio n • Ap ica l pet rosit is
Imaging Findings
• Cho leste ro l gran uloma, PA
2
• Best di agno st ic clue: Asymmetric ae rated PA acro ss Diagn ostic Checklist
from o pposite norma l PA hone marrow i n absence of • AM·PA is com mo n in cidenta l fin din g o n bra in MR 147
cx panstle cha nge s
• MR: Th ick c n ha nci ng wa lls wit h focal flu id ; du ra l

e n ha nce me nt
Mu co cele, PA Co ns ider
• CT: Smo o th, cx pa nsile lesion • AM-PA is co m mon in ciden tal finding o n hrain !\IR
• MR: Lo w '1'1, hi gh '1'2 sign a l • May he misd iagn osed as PA c ho lestero l gra n ulo ma or
• May exactly mimic c hole stea to ma of I'A o the r PA lesio n
• O ne o f "leave-m e-al one' lesions o f PA
Cho leste ro l gran ulo ma, PA o Trap ped fluid , PA is o the r main "leav e-me-alo ne"
• CT: Smo ot h cxp a nsilc mass lesio n
• Mit: High sig nal o n T1 «'1'2 • If misdiagn osed, call elicit u n necessar y medi cal or
su rgical th erap y
IPATHOLOGY
Ge ne ral Fe atures I. Leo nett i J P et al: Incid en ta l pe lreu s apex findi n gs o n
• Gen eral pa t h com men ts magn et ic reson an ce imaging. E...ar Nose Th roa t J.
o Norm al marro w space m istaken for pat ho logy 80(4):200- 6, 2fKJI
o Em bryo logy-an ato my 2. Moo re KH et al: "Leave me alo ne" lesio ns o f til l' pctro us
• 33% o f peo ple have pn eumati zed pct rous a pices apex. AJN R. 19:733-8, 1998
• PA pn eumati zati on degree co rrelates wit h degree 3. Virapo ngse C et al : Co mp uted tomog raphy o f tem por al
of mastoid aeratio n bo ne pn eu ma tizatio n, 1: Nor m al patt ern an d morpho logy.
AJR. 145:473-81, 1985
• Etiol ogy: Co nge n ital n or mal var ia n t in PA
pn eumat izati o n-marrow space
• Epidemiology: I(JI}{1 o f bra in rvt R reveal asym me tric PA
fatt y hone mar row IIM AG E GALLERY
ICLI N ICA L ISSUES

Pre sentation
• M ost co m m o n signs /sym pto ms: Asymp to ma tic by
definit ion
o Pat ient unde rgoing brai n MR for u nrelated
symproms
o Inciden tal MR find ing
o AM-PA describ ed as "susp icio us for cho leste rol
g ra n ulo m a" in radi o logy report
o Patient is referred fo r su rgical assessmen t (/ .t10 Axial f ' WI Mf.:. revesl» .1 high ~;gnd ' " /t>~ iulI~ in l ighl fJt'fru m
apex (.lrrmv). F.l l ty marrow \If'I .~U 5 t-.lfly d)(}/t'~ tf'f()I gr.lnU/om.l
o T-ho ne c r reveals normal PA air cells across fro m
("omi!{pI"pd . C T _~ h (Jwf'd on ly swnune uic ':l lty marrow in IlJis
o ppos ttc normal PA hon e ma rrow lo(",ttioll. (RiKItt) A xi.lI TI C+ MR w illI t:ll -s.ll{/ra tiorJ ill fJa lient w ilh
Natural History & Pro gn osis suspicious high siRnal tcsknv on TI Mf.:. in righ t l'l\ sholVs
d isapp t'.l ra net' of toston in dicating (.1" 0 \\,) il W.l ~ .Hymnw lr ic ':Jll)'
• Remains u nch an ged th rough out life of pati ent mdrlOW cct iecticn.
• Incidental fin d in g
• Mult ip le po ssib le mo rbidit ies from treatm ent of thi s
"leave-me-alon e" lesion o f PA
Treatme nt
• Req uir es n o treatm ent or fo llow-up
SUBARCUATE ARTERY PSEUDOLESION
2
14H
A\i. 11 l -fKJf1P CT oi an .Idull IPtr f'.lf show~ .1 normJI CorOllJ! It." P." l-bonto CT in .m .J(/ult rp\'t-'d/s d f'/(J{trJ./I
QIIJ.IfUMtf' Jfl<'f Y c;m.,J (Ju a,,"' pdssifl1: t;OItJ m('(kJI c urvilir"l4 '.U sul:"lrcUdlt> ..u lery c.ln••1 (J r(()l.v) p.Js);ng
pCffutJ" ric4:,l(' 1Ifl( Jt.r the SUflt'fior 5C'fflidrwl. lf c.m.ll /x>llf'.lth Ill(' superiorsemicircu/.lr c.m.l l (OfX"I .lfmwJ.
(or )('O .lrrows}.
ITE RM INO LOGY MR Findin gs

Abbreviations a nd Syno nyms • T2 WI
o High· rcso)ut io n T2 in in fan t
• Suba rcua te a rte ry ca na l lSAC, • CSF in ten sity e n tering subarcuate fossa, th en
• Suba rcuate ch a n ne l o r tract , pet romastoid ca n al pa ssing ben ea th sse
Defin itio ns • In volu tes in l st 2 yea rs of life usuall y
• SAC: No rm a l tem po ral ho ne osseo us ca na l th at passes o High-reso luti on T2, ad ult: SAC n ot visi b le
through a rch of su pe rio r sem icircular cana l; co nv eys Imaging Re commendations
blood suppl y to otic ca psule • Axial t h in-sect io n tempo ral hone CT withou t co ntrast
bes t sh ow s SAC
• SAC may n ot be seen if sect io n th ickn ess > 1.0 111 111
IIM AGING FINDINGS due to vo lume ave ragi ng
Gen eral Features
,".
• Best di agn ostic clu e
, o Osseous can al passing th rou gh su per io r se m icircular
ca na l (SSC)
c' Prominent ve stibular aqueduct
• Infant: Tubu lar with CSF d en sit y/int en sity o n • May he m istaken fo r inner ea r fractu re o r SAC
I'
,I CT/MR • C 1': Co n nects c nd ol ymp ha ti r sac fovea to n us
• Adu lt: Thin lin ear a ppeara nce wit h scle ro tic co m m u n is (co m mo n n us )
margin s o n CT • Usually in conspi cuous in ad u lt inn er ea r
,
'" CT Fi ndi ngs Pro minent cochlear aqued uct
• NECT • May he mi staken fo r fractu re o r SAC
o In fa n t: SA C passin g under sse wit h can a l • C'l: Para lle l &: inferio r to lAC
cross -sect ion a l m easurem ent 2-] ti m es th at of sse
o Adul t: SAC seen as "d ark lin e' passin g u nde r SSC Temporal bone fra ctu re
wit h cross-sect io na l measu re me n t s that o f sse • Clin ica l h istory o f signi fica n t head traum a
DDx: Adult Subarcuate Artery Canal Mimi cs
Vestibular Aqueduct Cochlear IIqu educl t-bone Fracture

SUBARCUATE ARTERY PSEUDOLESION
Key Facts
Terminology To p Diffe rential Diagn oses
• SAC: Normal temporal bone osseous ca nal th at passes • Prominent vestibular aq ued uct
through arch of superior sem icircular canal; conveys • Prominent coc h lear aq ued uc t
blood supply to olic ca psule • Temporal bone fracture
Imaging Findin gs Clinical Issu es 2
• In fan t: Tub ular with CSF den slry/inten sity o n CT/MR • SAC may be mi staken for pat ho logy
• Adult : Th in linea r a ppea ran ce wit h sclerotic margins • Infant: In ner ea r anoma ly 149
o n CT • Ad ult: 'l- bo ne fract ure
• c r: Lacks sclero tic margins o f subarcuate ca nal

• Other c r finding" Usually wit h air -fluid levels in Natural History & Progn osis
midd le ea r cavity ± mastoid ai r cells • Norma l ana to mic va rian t
• Pot ential route o f spread of infec tious processes
bet wee n masto id an tru m &. cra nia l cavity
IPATHOLOGY • Norma l pet ro us temporal bo ne cana l
Ge ne ral Features Trea tm ent

• General pa th co m me n ts • None needed, normal ana to mic st ruc tu re
o Embryology-anato my
• 2 1st week o f embryonal develo pmen t, max im um
size o f subarcuate sin us ID IAG N O ST IC CHECK LIST
• The n 1 in size to for m suba rcua te fossa &. ca nal Co nsider
• Dur ing 2nd postnata l year, fossa is nea rly
• SAC = po tential route o f spread of infectio n
obliterated to form a shallow dep ression o r slit 0 11
• SAC ca n be m isdiagn osed as co ngenital in ner ca r
posterio r 'l- bo nc I< variab le SAC size whic h Ih en
lesio n in infan t
co n tains subarcuate artery & vein
• SAC can be m isdiagn osed as fractu re in ad ult
• Visibility o f SAC dependen t o n degree of
ossificatio n o f labyrinth & masto id
pneu mat izati o n
• Suba rcuate arte ry arises from labyrint hi ne artery
ISELECTED REFERE N C ES
med ial to lAC I. Tckdcmtr I et at: The subarcuate canaliculus and ih artery:
• Usua lly arises med ial to lAC, th en en te rs A radl oanato m ical study. Anat Anz. 18 )( 2):207·11 . 1999
2. Wilbrand H ct al: The subarcuat e fossa and ch an nel. A
suba rcuate fossa (on pos te rior wall of pe rrous
radioanatomic invest igatio n. Acta Radio) Diagn. tStockh )
temp o ral bo ne) 27(6):637 -H , 1986
• Artery then passes th rou gh bone, in SAC enclosed 3. Mazzon i A: The suba rcuate artery in man. La ryngoscope,
by supe rior semicircular canal , runn ing relativ ely 80( 1):69 · 79. t9 70
st raigh t, in a lat eral, posterosu perior direction
• Supplies otic capsule, scm lclrcular cana ls &.
posterior wall vest ibu le
• Dista l branches anasto mose wit h bran ches from
IIM AG E GALLERY
su perficial petrosal artery, stylo masto id, po sterio r
meni ngea l & occip ital arter ies
• Mean length of SAC = 10.5 mm
• Approx ima te ly 501)() o f canals have wid th betw een
0.5 mm -l .O m rn

Prese nt at ion
• Most co m mo n signs/sympto ms: Asym pto ma tic
nor mal varian t
• Clinical profil e (IL/ t) Axi,]1 left cor l-bone CT in infant shows normal c<lO,11 of
o Incid ent ally seen during wo rk-u p for o the r clinical subarcuate <]f(t>ry as a tubular lucency (arrow ) passing lJt>nealh the
find ings arch of the superior semicircular canal (ope n arrows). (Right) Axial
o SAC ma y be mista ken for path olo gy T2W/ /\ IR dcm onstretes conspicuous high Signal within .:l norma l
• In fan t: In ner ear anomaly sub.uc uate artery canal (arrow ) p.Hsing beneath thp super ior
• Adult: 'l-bone fracture semici rcular c.lOal (open arrows ) in an infant.
CHOLESTEATOMA, PETROUS APEX
2
f'- - --i
150
I
"
Axi,ll graphic depict!; t}'Picai fA cOI"h'PIJit,J! ,h ;.l l 11 C+ .\1/\ f,}1(J\Vl; ,I brj.,'t' H-\ ('ho/(-steatoma
cllok'Stmtoma. Notice the benign e\p..m s.ik· I M /Uft' 0'- (arrow} with mifJimdl rim-t·nh.IIJCf·mt·nr, II x' 1I-'5ioll i.~
fA bono as. it respond s. to growing chok'5tt'Jlom .l. impinging Oil tilt· intt'ftJ.l1Juditory { ',In,11 ( Op t >fl .l ffOW) It
1for;zont,ll (X'lroos leA involwn)f-'fll (ilrr(1w} . ;\ I{-'( /.. t>1 caw (con n } ,1f(O"'}.
ITE RM INO LO GY • Size

o Ma y become very large befor e d iscove red
Abb rev iatio ns a nd Syno nyms o 2 ~ 10 cen time te rs in maximu m diamete r
• C ho lestea to ma. petro us apex (Cllo l-r1\) • M orph ology
• Co ngen ital ch o lesteat oma, epiderm o id o r epide rmo id o Ovo id to rou nd
cys t of pet rou s apex (I'1\) o Wh en in vo lves m iddle car o r mastoid as well as PA,
may have dumbbell morph ology
Definitions
• C ho l-PA: Petro us apex focus of cho lestea to ma du e to
CT Findin gs
ep ithelial rest o f emb ryo nal o rigin or acq uired • NECT
c ho leste ato ma exte nd ing fro m m idd le ea r into pelrou s o Th in-sectio n 'l-bo nc c r
apex • Sho ws sm oo t h, ex pansilc lesion o f PA
• If co ngen ital varian t, may simultan eously affect
mastoid area
IIMAGING FINDINGS • If acq uired Cho l-!'A, may sim ultaneously affect
midd le ea r area
" Ge ne ra l Features o Larger lesions erod e regionall y
.' • Best d iagn osti c clu e • Il o rizontal petrous le A
.'
.',
I
o lIo ne C l: Expansilc mass with smooth-lo bular bon e • Otic capsule
• In ternal aud ito ry cana l
,I rem odeling
o M R: Expans ile rA lesion low T1, high T2 signa l bu t • Jugular fo ramen
.' wit ho ut en hanc eme n t • CEcr: Chol-Pa will NOT en hance
"
. • Locati on
o Petrous apex
MR Findi ngs
o Co ngenital va ria n t: Inv o lved masto id & PA • T1 WI
o Low T I signal
sim ulta ne o usly
o May he homogen eous or heterogen eous
o Acq uired c hol-Pa : Midd le ear filled with extens io n
• T2WI: High T2 signa l
in to PA
• fLAIR
DDx: Non-Tumor Petro us Apex l esions

\ -'
. --..
"1
. f:c" .-
/~ _.~
, \ ',:
'\ .
Trapped Fluid Apical Petrositis Cholesterol Granuloma PA !\m.' urysm
Key Facts
Te rm ino logy • Mucocele of PA
• Cholestea to ma , petr o us a pex (Cho l-Pa) • Petrou s l e A a ne u rysm
• Chol-PA: Petrous apex focus of cho lesteatoma du e to Path o logy
epithe lial rest of em bryo na l origin o r acquired • Very rare PA lesion « 11M) of PA lesio ns)
cholesteato ma exte nd ing from middle ea r in to
petr ou s apex Clinical Issues 2
• Senso rine ural h ea rin g loss
Imaging Fi nd ings 151
• Bone CT: Expansile ma ss with smoot h-lobular bone Diagnost ic Checklist
remo de ling • On ce di scovery o f petr ou s apex ex panstle lesion
• MR: Expa ns ile PA lesion low Tl , hi gh T2 signal but occ urs, sor t in to benign ex pa ns ile and invasive
wit ho ut en ha nce me n t expansilc gro ups
• Benign ex pa ns ile PA gro up includ es cho lesteato ma,
Top Differe nt ial Diagnoses cholesterol granu loma, mu cocele &. petr ou s l eA
• Trapped fluid , pet ro us a pex a n eu rysm
• Apica l petrositis
• Ch o lestero l gra n ulo ma o f PA
o Does not a tten ua te on FLAIR • MR: Thi ck en ha ncing walls wit h focal fl uid : dura l
o Parti al atten uatio n (m ixed int erm ed iat e-lo w sign al) th ickening &. en h an cemen t
may be seen
• DWI
Cho lestero l granulo ma of PA
o RESTRICrED DIFFUSION (hig h sign al o n IJWI) is • Clin ical: Previou s history o f ch ro n ic oto ma sto id it is
characte ristic com mon
o Same as co ngen ital cho lesteato ma in CPA • Bo ne C I: Smoot h , lobu lar ex pansilc mass
(epide rmo id cyst) • Mit: ll igh signal o n TI I'< T2
• TI C+ Muco ce le o f PA
o Chol-I'A will NOT en ha nce
• Bon e CT: Smoot h cxpa nslle lesio n
o Mild rlm -enhan cern cn t possib le
• Mit: Low TI , h igh '1'2 signa l
• MRA : Lar ge lesion s may cause mass effect o n
• May exact ly m im ic choleste ato ma o f I'A
ho rizontal petro us in te rn al ca rotid a rtery
o Exce pt NO dif fusion rest ricti on see n on DWllvt R
• MRV: Large lesion s may co m press sigmoid sin us ±
seq ue nce
jugular fo ra men
Petrous ICA a ne urysm
Angiograp hic Find ings
• Clin ical : Sku ll base trau ma h istory may be present
• Avascu lar pctrous ap ex mass lesion
• Bon e Cf: f usiform o r focal ex pans io n cen tered in
Imaging Re co mm endatio ns hori zontal pet rou s le A ca nal
• Thi n -section (I 111111 co n tiguous) bon e CT th roug h • MR: Co m plex signal ovoid to fusiform ma ss
skull base &. Ttbon e in ax ial &. co rona l pla nes is best inse parable from horizo ntal petr ous l e A
in itial exa m
• Th in-sectio n (3 mill co n tiguous) MR in ax ial &. co ron al
plan es used to confir m di agn osis &. obtai n so ft tissue IPATHOLOGY
road map for su rge ry
o Especially u seful in large lesio n s
Ge nera l Feat ures
o '1'1 C+ MR co n firms lack of en ha nce me n t • Gene ral pa th co m me n ts
o Use DWI seq uen ce to co n firm di agn osis o Most PA cho lesteato ma co ngen ital
o I.ess co m mon ly PA cholesteato ma fro m ex te nsion of
acq uired m idd le ea r cholestea to ma
IDIFFERENTIAL DIAGNOSIS o Em bryology-a na to m y
• Rest s of e pit helial tissue can occu r in m ulti ple
Trapped fluid, petro us a pex locatio ns in & aro und tempora l bo ne
• Clin ical: Asym pto mat ic incid en tal find ing o n T2 MR • Midd le ear > CPA > ma sto id > perrous apex
• Bon e CT: No n-expa ns ile, o pac ified PA air ce lls • Etiology: Aberra n t e pithe lial rest in pet rou s apex o f
• MH: Lo w T l , high T2 signa l in most cases ex foliated kerat in wit h in stra tified sq uamo us
ep it he lium
Apica l petrositis • Epidem iology
• C lin ical: Septic pa tie nt un less already pa rti ally treat ed o Very rare PA lesio n « }lYiI of PA lesio n s)
wit h a nti b iotics • Tra pp ed flu id > > apica l pet rositis, cho leste rol
• Ho nc CT: Dest ructive lesio n wit h trabecu lar &. co rt ical gra nu loma, meta stases > C ho l-PA
loss
• Tl HIGH, T2 high
Gross Path ologic & Surgical Features o Mucocele of petrous apex
• Pearl y-white tissue within eggs he ll bone • T1 lo w, '1'2 hi gh
Microsco pic Features • DWI shows no restri ct ed d iffus ion
o Petrous l e A a n e u rysm
• Sh eet s of st ratified , keratinizing, sq uamous epitheliu m
o No evide nc e o f ab norma l mitosis pre sent • Mil seq ue n ces show COMPLEX SIGN AL mass
cen te red o n h orizon ta l pe trou s ICA
2 o Gra n u latio n tissue a n d fib rosis o fte n su rro u nd t hem
• Rich in cholestero l crystals
• C o m p le x sig n a l du e to va rio u s a ges of blood in
lu mi n al clot Sr t urb ulen t flow
152
[CLINICAL ISSUES ISELECTED REFERENCES

Presen tation I. Mattox DE: End osco py-assisted surgery o f the petro us apex.
• Most co m mo n signs/sym pto ms Otola ryngol ll ead Neck Surg. 130(2):229-4 1, 2004
o Sensorine u ral hearing lo ss 2. Shea ha n I) et a l: Supralabyrinthi ne appro ac h to petro sal
o Othe r signs /sym pto ms cho lestea toma. J Laryngo l O tol. 117(7):558-60, 2003
3. t'tsanesch! MJ et al: Co ngen ital ch olestea to ma and
• Peri pheral facial ne rve par alysis
cholesterol gran uloma of th e tem po ral bo ne: ro le of
• Abducen s nerve para lysis magne tic reso n an ce imagin g. Top Magn Reson Ima gin g.
• Headach e 11(2 ):H7-97, 2000
o Acq u ired c h o lestea to ma exte nd in g in to PA 4. Profan t M ct a l: I'etrou s apex cho lesteat o ma. Acta
• IIisto ry o f ty mpa n ic mem brane Otolaryngn l. 120(2):164-7, 2000
retract lo n-perforat ion S. Muck le RP et <II: rc rrous apex lesion s. Am .l O to l.
• Prev ious surgery o n middle ear cho lesteato ma 19(2 ):2 19-25, 199H
exte nsion co mmo n 6. Cha ng P ct al: Imagin g destruct ive lesions of th e pc tr ou s
apex. Lar yngoscope. 108(4 Pt 1):599-60-1 , 199M
• Persist ent ea r pain & otorrh ea despi te apparent
7. Rob ert Y et a l: I 'et rou s ho ne ex te ns ion o f m idd le-car
su rgica l cu re acquired cholesteat o ma . Acta Radi ol. J7(2):166-70, 1996
• Clin ical profile: 3 5 yea r o ld adu lt with unil a te ral 8. Mafee l\IF et al: Epide rmo id cyst (cholestea to ma) a nd
sen sorine u ral h earing loss ch o lestero l gran uloma of the tem poral bon e and
epidermo id cysts affecting th e brain . Neuro im aging Cli n N
Dem ographics Am. 4(3):561-7H, 1994
• Age: 20-50 yea r o ld 9. Kikuchi S et a l: Co nge n ita l cholesteatoma of t he petrous
pyram id. ORLj O tor hi nolaryngo ! Relat Spec. 55( 4):236· 9,
Natur al History & Prognosis 1993
• Very slow gro wing lesio n 10. Mafce MF: M RI a nd C I' in th e evalua tio n of ac q u ired and
• Co m plete surgical removal arrests sym pto m co ngen ital ch olesteato mas of the tem poral bo n e. J
p rogression Otol aryngo l. 22(4):239-4H, 1993
11. j ackler RK et al: Radiogra phi c d ifferen tia l d iagnosis of
Treatment petrou s a pex lesion s. Am J 0 101. 13(6):56 1-74, 1992
• Su rgica l a pp roac he s 12. Atlas MD et al: Petrou s apex choles teatoma: d iagn ostic and
o Rem ova l via transpetrou s approach treatmen t d ilemmas. Laryn go sco pe. 102( 12 Pt 1):1363-8,
1992
a Middle fossa approa ch also used 13. Ish ii K et al: Midd le ear ch olesteato ma exten d ing into the
pctrous a pex: eva luat io n by cr an d MR imagin g. Aj NR Am
J Neuroradiol. 12(4):719-24,1 991
[DIAGNOSTIC CHECKLIST 14. Arriaga MA et a l: Differenti al di agnos is o f prima ry pct rous
a pex lesio ns. Am) Otol. 12(61:470-4, 199 1
Co nside r 15. Glasscoc k ME 3rd et a l: Pet rou s apex ch olesteat oma .
• O nce discov er y o f petro us a pex ex pa n sile lesio n Otol aryn gol Clin Nor t h Am . 22(5) :9HI-l 00 2, 19H9
o ccu rs, so rt in to benign cxpa n sile an d in vasive 16. Charachou It et al: Tumours of the pet ro us a pex: a clin ical
expa ns lle gro u ps series. j Neurorad iol. 15(2):186·20 1, 19RR
17. Rose nber g RA et al: Cho lesteato ma vs. cholesterol
• Invasive ex pa ns ile PA group in clude s ap ical petrositis, gran uloma o f th e pet rous apex. Otula ryngol Head Neck
metastases & La ngerh an s ce ll h istiocytosis Surg. 94(3):322 -7, 1986
• Benign cx pa ns ilc PA group in clu d es chol es tea to ma, 18. Ho rn KL ct al: Co nge n ita l cholestea to ma o f the petrous
choleste rol gra nu loma, mu cocele & petrous l e A pyramid . Arch Oto 1aryngol. 111(9):62 1-2, 1985
an eur ysm 19. Latack ]'I' et at: Epid ermoi do mas of th e cerebel lopo n tinc
a ngle and tem po ra l bo ne: CT ami MR aspects. Radiology.
Image Interpret ation Pearls 157(2):36 1-6,1 9HS
• Sorti ng t hroug h radi o logic ch a racte ristics o f bo n e CT 20. McDon a ld 'I) et a l: Conge n ital cho lestea to ma of t he ear.
ba sed be ni gn expa ns ile PA gro up with MR key An n Otal Rhin o! Laryn go t. 93 (6 Pt 1):637-40, 1984
a Ch o lesteato ma o f pe t rous a pex
• T1 low, '1'2 hi gh
• T1 C+ MR shows no enh ance me n t
• FLAIR sho ws partial or ab sent attenuatio n
• DWI sh ows RESTR ICTED DIFFUSION
o Ch o lestero l gra n u lo ma o f pet rous a pex
IIM AG E GALLERY
(/.£/1) A xial TlWI AIR shows

ovoid. exp.ln sile
inhomogeneous m j),oo
low-intermediate sinnal
2
petrous ape x cho/csteacom.J
(J u ow). Tile lesion h.u 15 ~
t"xp,mded p05lerol,uc'f all y
into inlernal Ju ditory (,iJlM I
(open arrow). (Right) Ax;'"
T2WI MR reveals ovoid high
signal PA choleSlt'a(Oma
(arrow ) on the left. Medial
expe nsion causes le sion to
abut basi/ar artC'ry (open
,mow) with posle(o/.Jlt"fdJ
invo/\'l'ment of lAC Icurvvd
,UfOW ) .
Typical
(/ .{'fl) Axial OWl MR
demonstrates diffusion
restriclion in this left petfOu _~
epox congenital
<- hO/('Sft'<l to m, l (a rrow ) .
Notin ' tlun tilt> If'~i()n .1/50
has infero/.tter,ll ('\(('1I5;on
(01)1.' 11 ,mow ). (Rig"lJ AX;,JI
I·hom· CT {{-'w'.l/ .. p revious
m.bto i(/("( lo my (or dcqu;rf!d
choJeS(t'dloma. lesion has
/ >.l5Sed from middle e,Jf to
jJelrous apex (arrow) where
it again e xpanded (open
arrows J. Petro us leA :
Cur ved <!r row.
Variant
(/-t'f t) A xial l-bone C T of
right ear shows mastoid
(,}frow ) & petrous ap ex
(op en arrow) cornponerns of
hi/abed congenital
choles teatoma. erosion into
f:'xlernaf ear (c u f\,(>(! ,}ff()w)
i5 source of air. ( 1(;/: 111) A'i.J1
Tl C+ MR dem o ns/h Ut'S .l
dumbb<4'-s/t.1/)t!(/ molstoid
(arrow) &. pt'troU5 J(K"
(open arro w ) congl'nitill
c ho lC'stm tom.J. Note the'
nonenhancing low siRnal
bulk of the lesion h.1S
minim al rim -f'nham -f:'m en t.
PETROUS APEX CEPHALOCELE
2
154
"h i,11J.:f.lphic illll'.tr.Jl('5 hemi,,'ioll of.l ('pl,.,h. (.!t. (rom ,\\i.11 TlW/ MR !>hows the d.h.~ic ,l/ l' )( '<lf,m H ' or ,I 11,\C
All'( 1..('/ (',1\1' into the p('CrOl.15 Jrx'\ (.l m l\ v) . A p C)Ttio fl o( with direct comm unication hd\\1'( '(l Afl'ckf'/ ( .we
tlK' trigc'tllitl.ll g.mg!io/J ;.\ pt ultUding imo 'he (.I(( o w) .md (h(' CSF ill/(,Il~ify f l·"hl/C)( t'/t· m tl w
c('I'1l.,kx ('k~ (op t "fl.lrro wJ . ,m tf" ;OI '>l ·IUJU.~ olpt' ); (0, )(-'/1 . ll lO lV}.
o Lo wer bon y crescen tic ma rgin o f porus trig em in us is

ITERM IN O LO GY en larged
Ab b re viatio ns a nd Syno nyms • CECr
• Abbreviati o ns: Pct rous a pe x ce ph aloce le (PAC) o No nc n ha nc ing lesion
• Syno ny ms: Pri m us ap ex arach noid cys t, a pical o No adj acen t me ni ngeal en h anc em en t
men in gocele • c r Cisternogra p hy
o Used ill rare cases wh en d iagn osis ca n not be made
Definitions co n fiden t ly wit h co nve n tio na l im agin g
• PAC: Cong enital or acqui red hern iati on of o Rad iopaq ue co n t rast mat erial will fill in cephalocele
po stero lateral wall o f Meckel ca ve (MC) in to 1'/\ defect in 1'/\
o In rare cases with CSF o to rrhea, may d em onstrate
co m mu n ica tion between PA &. m iddle ea r
IIM AG ING FINDINGS MR Find ings
Gene ra l Featu res • TlWI
• Rest di agno stic cl ue: C_'iF d ensity/ inten sity lesion o f PA o Low T l signa l, isoi n tense to l _() F
wh ich di rectl y co m m u n icates wi th ~I C a Ap pears to sp ill o u t of pa tu lo us, ipsilateral MC
• I.ocation • T2 W I
o An te ro med ial aspect o f PA d irectl y ad jace n t to Me o Bright ova l area CSF int en sity w it h in PA
• Cen ter of lesio n m ay he outside of I'A o Coro nal T2 im ages best shows con nect io n to \fC
o Usua lly unilateral , ra rely bilateral • f LAIR: fluid in ce p ha loce le attenuat es wi t h CSF
• Morpho logy: Rou nd, well-ci rcumscr lbed withi n Pi\ • TI C+
wit h M e co n nectio n o Usually sh o ws n o en ha nc eme n t ve rsus m ild rim
en ha nce me n t
CT Findi ngs o If gasserian gang lio n within ce pha loce le, will appear
• NECf as "en ha nc ing com po ne n t" with in ovo id
o Smoot h , n oni nvasive bo ny excav ati on of P,\ nonenhan cin g lesion
o Ex pa ns ile wit h ben ign , smoot h mar gi ns • Perl-ga nglio n veno us plexus ac tua lly is en ha ncing
tissue
DD x: Petrous Apex l esions
Cholesteetoms PA Tr"pped Fluid PA Trigem . Schwannoma MlIcOCL'/e PA

Key Facts
Te rminol ogy • Apical pet rositi s
• Abbreviati on" Pet rol" a pex cep ha locele (PAC) • Mucocele in PA
• Syn on yms: Petrou s a pex a rac h n oi d cyst, api ca l • Choleste ro l gra n u lo ma in PA
men ingocele • Meckel cave trigeminal schwan no ma
• PAC: Co n ge n ita l o r acq u ired hernia tio n o f
pos tero lateral wa ll o f Mec kel cave (MC) into PA
Pathology
• On e or all th ree layers of meninges may be present in
2
Imaging Findings PA defect 155
• Best dl agn osti c cl ue : CSF de n sity/in tensity lesio n o f Clinical Issues
PA wh ich d irectl y co m m un icate, wit h MC
• Most com mo n presenting sym pto m: In ciden tal
• Smoo th, noni nvasive bony excava tion of PA ASYMPTOM ATIC Mit brai n fin di ng
• Best imaging tool: Th in-sectio n, high-r esolut ion , • No treatmen t in avera ge case
mul ti pla n a r T2 M It m ak e, dlagnosis
• Treatment requ ired on ly in comp licated cases with
Top Differe ntial Diagnoses recurre n t m eningitis, cra n ial n europat hy, o r
• Co n ge n ital cho lestea to ma in PA persiste nt CSF leak
• Trapped fluid in PA
• If ce pha loce le diag no sis no t ap p recia ted, th is • Mit: T1 I< T2 signal hi gh

e n ha ncemen t ma y suggest tu m o r to unwa ry • In tern al co n te n ts d o not su pp ress o n FLAIR
rad io logist
Meckel cave trigeminal schwannoma
Imaging Re comm endati ons • c r: Sm oo th ex pa ns io n o f PA I< MC
• Best im aging t oo l: Th in-sectio n , h igh -resolu t io n , • Mit: T1 I< T2 interm ed iate signa l
m u ltiplan ar T2 MR m ak es d iag nosis • '1'1 C+ MR: Dense e n ha n ceme n t o f m ass lesio n
• Th in-sec tion T-bo ne CT co n firm s impressio n
• If still co nc e rn ed Cl, clste rn ogra phy is dia gn osti c
o Reserved for pati ents with CSF o to rrh ea IPATHOLOGY
Gen eral Feat ures
IDIFF ERENTIAL DIAGNOSIS • Ge ne ral pat h co m me n ts
o Explanat io n for h ern iatio n of poste rol ateral M C into
Co ngen ita l cho lestea toma in PA PA h as va ried fro m a rac h noid cys t to m enin goc ele
• Cf: Sm oo th , expa nsile PA mass o One o r a ll th ree laye rs of m eninges m a y be presen t
• MR: '1' 1 MR signal is low; n o m enin geal e n ha nceme n t in I'A defect
see n o In acq u ired cases pne u m atized PA is requi red
• May h av e d est ructi ve feat ur es; n o co n nection to M C • 33<)(, o f popu lat io n h as pneuma tized PA
• Et iology
Trapp ed fluid in PA o Co n ge n ita l h ypo th esis
• 0: PA air cell tra becu lae m aintai n ed ; no e xpa ns io n o f • Devel o pme ntal an oma ly results in deficient d ur a l
PA a ir ce ll area & o sseo us co vering o f PA
• MI{: T l signa l usuall y low (ca n be intermed iat e o r • Defect a llow s Me "he rn ial io n " into petro us apex
rarel y h igh ) with T2 sign a l h igh o Acq uired hypothesis
• All su rro un d ing co rtic al ma rgin s are in tact • Ch ro n ic CSF pu lsa tions ag ain st th in a nt eri o r wa ll
Apical petrositis o f a pne uma tized PA result s in dehi scen ce
• C f: Perm ea t ive, d est ruct ive cha nges o f PA co rte x No • Arachnoid 'gra n u latio n adj acent to a n terio r PA
m a y accelera te d ehiscen ce
t rabecu lae
• Mit: Low T l , h igh T2 signa l • Eventual pro lapse o f me ninges in to PA defect
• Ep ide m io logy
• n C+ MR: Th ick, e n h anci ng rim: m enin ges t hick I< o Unco m mon inci de n ta l lesion o n b rain MR
e n ha nci ng
o Less common th a n tr apped flu id in I'A
• Clin k a l sett ing o f o to m astot di tis or
o l.ess th an 30 cases descr ibed in literature
post-mastoi dectomy
• Most of these, in surgica l literat ur e, focused on
Mucoce le in PA cases requiri n g su rgica l in te rven tion before advent
• Cf: Smoo th, ex pa ns ile PA mass o f ad va nc ed imaging
• Mit : T1 sign al lo w, T2 sign a l high • No w co ns ide red muc h m ore co m mo n as
• Mimics co ngen ital cho lestea to ma ; ve ry ra re lesio n inc ide n ta l fin d in g
Cho lestero l granuloma in PA Gross Path ologic & Surgical Features

• C J': Trab ecu lar br eakd own &. co rt ica l • If un co m plica ted . shou ld nev er be hiopsied
expa nsio n- th iru u ng in PA • If b lop sied . no rm a l m en ing es & gasseria n ga ng lio n
• Co n nectio n bet we en PA &: Me is a lways eviden t if
Microscopic Features so ug h t o n a ll MR pl an es
• No tu mor o r inflam ma to ry cells present
ICLINICAL ISSUES
I. Bolger WE ct a l: Tempo rallobe encepha locele appea ring as
a lytic lesio n o f th e skull base and pte rygoid p roc('~s. F.3r
2 Presentati on
• Most common signs/sympt om s
Nose Throat J. 82(4):269-72, 274·5, 2lXl3
2. Kasugat Set al: A case of mcningocn ccp ha llc hern iation of
156 o Mo st co m m o n pre se n t ing sym p to m: Incidental the te mpora l bo ne . " uris Nas us Larynx. 30 Sup pl:SI03-.5,
AS YMIYIU MATIC MR bra in findin g 21Xl:!
o If co m p licated, present ing sym ptoms & signs :I. Batra A et al: Pct ro u s a vex a rach noid cyst exte nding in to
• Trigemin al n europa t h y o r trigem in a l neura lgia Meckel's cave . Aus tratas Radiol . 4( 0 ):295-8, Z00 2
• CSF otorr hea 4. Leo netti j P et .11: Incidental petrou s apex fin dings o n
• Recurr ent meningitis, part icularly l'neu mococca l magnetic reso na nce imagi ng. Ear Nose Th roat J.
80(4):200-2,205-6,200 1
m en in git is
5. vergc n! G et al: Spon taneous cvrvbrospinnl flu id
• Clinical profile rhi no rrhoea in nntcro m edl al tem poral occu lt
o In cid ental MR o bserva tion in a pati ent im aged fo r ence ph alocele. Br j Ncuros urg, 15(ZI:156-8. ZOO I
n o n specific brain ind ica tio n 6. Moore KR ct a l: Pe tro us apex cephalocclcs . AjNR .
o Ch ild with recur rent Pn eumoc occal m eningitls 22:1867.71,200 1
o Adult with spo n taneo us CSF o to rrhea o r insidi o us 7. Muckle RP ct a l: l'et rou s apex lesions. Am J Otol.
trigeminal neuralgia 19(2):219-25, 199M
8. Mulcah y M ~'( et a t: Co nge n ita l encepha locele of t he medial
Demographics skull base. Laryngoscope. 107(7):9 10· 4, 199 7
• No kn own age, gen der or et h nic predi sposit ion 9. Cheun g SW et al: l'ct ro us alll'x arac hnoid cyst :
Rad iographi c co nf usio n wi th pri m ary cholest eat o ma. Am J
Natural Hist or y & Progno sis Oto l. 16:690-4, 1995
• Excellen t if avo id mi sdi agn o sis 10. Cur t in 11 0 et al: Th e petrous a pex . Oto laryngo l Clin Korth
• In co mplicated cases sym pto ms resolve after successful Am. 28(3):473-96, 1995
11. A et al: Petro us apex m ucocel e: h igh reso lutio n cr.
M (, lI1 i ~
su rgica l repa ir of PAC Ncuro radiology . 36 (8):63Z-3, 1994
Treatm ent 12. La rso n Tl.: Pct rou s apex a nd cave rnous sin us: Ana tomy and
patho logy. Sctu lu Ult raso und c r MRt. 14:232-46, 1993
• No t reat m ent in av erage case 13 . Wilki ns RII ct al: Spo ntaneous te mpora l encephalocele.
• A bias towa rd ' ...·atch fu l wa iti ng justi fied Case repo rt .} Neurcsurg . 78(3):49 2-8, 1993
• Su rgical treatme n t 14. j ackler RK ct <\1: Radiograph ic differential diagnosis of
o Treat me n t required o n ly in com plica ted ca ses wit h petr ous apex lesio ns . Am J Otol. U (6):S6 1-74, 1992
recurrent m e n ing iti s, cra n ial n eu ropathy, o r IS. Leblanc Ret al : Deve lo pmen tal untcrobasa l tem poral
persi ste n t CSF lea k ence phalocele and tempora l lo be epi lep sy, j Neurosurg.
o Mo st critica l ste p is to m a ke co rrect d iagn o sis o n 74(6):93:1-9, 199 1
16. Beaumo nt GO c t al: Enceph alocele Inv ol vin g the petrous
ini tia l pre-operat ive im agi ng to av o id u nnecessa ry o r
ho ne. Neuro radio logy. 32(6):533-4, 1990
im pro per tr eatm ent 17. Frank lin Dl et <I I: Man agemen t o f petr o us apex lesio ns.
o Midd le cra n ia l fossa ex t rad u ral a p proac h wit h repair i\r(h Oto luryngol Hcad Neck Surg. 115(9 ):1 121 -5, 1989
o f d ural d efect I< o bl ite ra tio n o f I'A defect with
m uscle I'< fa t
Co nside r
• Co ns ider PAC a "leave-me-alo ne" lesio n of PA a lo ng
wit h trapped flui d in PA a ir ce lls &: 1'/\ asym me tr ic
m arro w
• Requ ires no further wo rk-up o r su rgica l in te rven tio n
unless a ty p ica l im agin g features o r co m plica ted
clin ica l co urse arc p rese nt
• Be very ca refu l to m at ch o b jecti ve sym ptom s to thi s
lesio n befo re in sti t ut in g an y treatm e n t
• By im agi ng , st ro ng ly mim ics PA c ho lestea to ma o r PA
mu co cele if co n n ec tio n to MC is no t o bse rved
Image Interpretation Pearls
• PAC is o n ly lesion of I'A which ma y be ce n tered
o utside o f PA &: is co n tig uo us wit h Me
• Le sio n wit h in PA whi ch fo llo ws CSF sig na l o n a ll
seq uences
IIM AGE GALLERY
(lLft) Corona! TlWI MR

sho ws ,1 n ·ph .lfo('t·/e
demon strating homog eneous
high signal. Trigf'min.l l m vw-
2
fibers (arrow J can be 5('('0
coursing through this CSF 157
.\ / M Cf' !Jt'(O(C' ente ring
,\ II ·cl..('/ cave. (Rigltl ) CCJmlJJI
TIWI AtR f(' vt'a/~ I'/K
(arro w) w ith d ',If.K IP';stic
low '1Mgn.11 follow ing CSF.
Notf' connection with
,\ I('Ckt·/ cave topen arrow ).
1't\ C ,./ways haw
Imm ogl'IIl'(Ju5 low signal on
II AIR.
Typical
(l LII ) A x;.l! T2 WI M R sho ws
high T2 signal in right PAC.
No te that there is thillning of
1/1l.' J IJ{I?{ior co rtic al m .lfgim
of (hI' ,1p ('X (arr o w) but thl'
n '/Hd ilJdp{ or (1Je cortical
ma rx ins afe in tact. (Right)
1\\; .11 CT cistem ogrsm shm v5
co ntr,N opscitics ticn 0; lilt'
def('(', in l ht.' petrou s , J/ Jf.-'X
(.1rrow ) proving
communica tion wah tho
subsrsc hnoid 5fJdU·. Note
the co m m unication w ith
M e(J,.t'I caw' (open .11101").
Variant
(I-,/t) , h idl lxmt' Cl (( ·" ( '.I'S
bit.tlN al de fect s in Ihe
Pt't10U_~ apin '5 « t r1OIV~),
Nou- tiw .~ m o()th
no n-,1J.:gn'ssin· m.lrt:in.~ of
tbese lesiom .'\ thp ' 055 of
cor tical m.lrg im .m /e1io rly in
the wg iol1 of M (xJ"eJ ca ve.
(HiglJt) A xial 12 WI MR
shows bi lateral petrous apex
n"pl M/ocd cs as CSf intensity
ht>m i.lt;ons from M ffke/
C.IVf' int o tht' pt'lf()U_~ .lp ices
(arrow s). Bi/atetJ /ity;5 an
unu sual imagi ng variant.
APICAL PETROSITIS
2
158
Axial graphic of left petm u5 apex show.s "confll/ent !l xial T-bolle CTshows opacification of lell pctrous Jpt'x
apical r)('lr~ iti~ " with PA ab:-onos5 (ormation. Pus & middle CJ(-m.lstoid. No tice PA cortical erosion
sUf/nufJ(Js Iht! Mh cranial ne rve (opon ,1f(uw) S thickens (.1ffon ....) diagno"tic of <lpica/ JX>lroMti.~ . Open arrow:
adjan 'nl meninges (arrow ). Nor mally aerall;'c1 /cfl /~.
ITE RM INO LO GY CT Find ings

Abb rev iatio ns a nd Syno nyms • NECT
o Opacificatio n o f PA ai r ce lls
• Abbr ev iatio n : Apica l pet rositis (AP); pet rous a pex (I'A) o Middl e ear &: masto id also usuall y opacifi ed
• Synonyms: Co nfluen t ap ical pet rositis, petrou s a pici tis o COAI£ \C ENCE o f PA air cells seco nda ry to lysis of
Defi nitio ns intern al hony trab eculae
• AP: Extens io n o f in fectio n th ro ugh masto id air cells o Perm eative co rtical erosio n & fistulizati on to
t racts in to pne uma tized PA wit h trab ecul ar labyrin t h in adva nce d di sease
disin tegra tion & meningeal in vo lvem en t • CECT
o Peripherall y en ha n cing flu id (pu s) in pet rou s apex
o Th icken ed & en h an cing m en inges
IIM AGING FINDING S o Peri p hera lly en ha nci ng epid ura l ab scess in adva nced
cases
Ge ne ral Featu res MR Findi ngs
• Best d iagn osti c clu e: Trab ecul ar breakdown in
• '1' 1\VI: Asym me t ric in termed iate signa l ill PA most
o pa cified I'A air ce lls
co m mo n
• Locat ion
o Rot h ma stoid an d petrous ai r cell s in vol ved • T2WI
o High signa l within air cells o f pet ro-masto id
sim u lta neo usly
co m plex
• Early fin d ings includ e PA wit h po ssible me nin geal o High signa l focus cen tered in PA wh ere focal abscess
in vol vem en t
may occu r
• Late findings sh ow sp read to men in ges, skull base,
a Ad va nce d di sease may cause venou s t h ro m bosis
Meck el cave & caverno us sin us
with associated b rainstem ± cerebellar isch em ia
• Morph ology: Irregular ph legm onou s mass co nfine d to
PA until co rtical breakth ro ugh & meningeal • TI C+
o Pcrlp herally en ha ncing flu id filled I'A
invo lveme n t occu rs
o Men ingea l th ickening wit h av id en ha n cement
DDx: Petrou s Apex Lesions

}
~
CongoCholesteatoma Trapped Fluid Cl1OI. G ra n u lom a C h o n drosar co ma

APICAL PETROSITIS
Key Facts
Terminology • Prim a ry m alignancy of PA area (ch o n d ros arco m a,
• Synony ms : Co n fluen t apical pe tros itis, petro us chordoma)
a picit is • Me ta sta tic di sease o f PA area (metas tas is,
• AP: Exte nsion of in fection th rough ma stoid air cell s No n- Hodg kin lymph oma )
tract s into pne uma t ized PA w it h trabecular
d isint egration & meningeal involvement
Path ology
• Acut e o r chron ic suppu rative in fecti on spreads via air
2
Imaging Findin gs ce lls or venous cha n ne ls to PA 159
• Best d iagnostic clue: Trabecular br eakdo wn in • In fect io n of PA air cells causes coa lesce nce wit h
opacified PA ai r cells breakd own o f trab ecu lae
• COALESC ENC E o f I'A a ir ce lls seco nda ry to lysis of Diagn osti c Chec klist
in ternal bo n y t rab eculae • Co ns ider in it ial im agin g wit h a thin-section
Top Differential Diagnoses nonenh an ced T-bo ne CT
• Co ngen ita l cho lestea to ma in PA • MR wit h multi plan ar, fat -satu rated / en ha nced im ages
• Trapped flui d in I'A a re most e ffecti ve way to evaluate for intra cra n ial
• Cholestero l gran ulo ma in PA co m pl icat io ns
o Asymm etric en h anc e men t of effected cra n ial ner ves,

Mecke l cave & ca ve rn o us sin us Primar y malign an cy of PA are a
o Tl C+ MR best ldcntlfles in tracrani al co m p licatio ns (cho nd rosa rco ma, chor do ma>
• Epid ural abscess • Clin ical: Lacks ac ute infectious sym pto ms
• Parenchyma l br ain abscess • Cf: Destru ctive mass of clivus, pet ro -occipital fissure
• Skull ba se oste o m yelitis see n as en ha n cing o r I'A
m arro w in ad jace n t sku ll base • MR: In filt rative inhomogeneous en h a nci ng ma ss o f
• MRA cl ivus, pctro-occipital fissu re o r PA
o Severe lesio ns ca n invo lve adj acent sku ll base
a rte ries Metastatic disease of PA a rea (metas tas is,
• In tern a l ca rotid a rte ritis Non-Hodgk in lymph oma)
• Petrous caro tid pscud oancurysm • Clin ica l: Lacks ac ute infect iou s sym pto m s; syste m ic
• MRV mali gn an cy known
o Seve re dis ease may ca use dural ven ous sin us • C I': Perm eative-destructive m ass of PA
t h ro m bophlebilis • MR: Infiltrative inhomogeneou s en ha nci ng PA mass
o Petrosal sin us, sig mo id sin us, jug ular bulb-vein
thrombosis pos sib le
Nuclear Medicine Findin gs IPATHOLOGY
• Bone Sca n : Asymmet ric upt ake in PA o n Tc bo ne scan Gen eral Features
o r Gallium sca n • Ge ne ral path co m me n ts
Imagin g Recommendations o Em bryology-an ato my
• Init ial d iagn osis best m ad e wit h thin-section 'l-bo ne • Pneumatized petro us ap ex present ~ 33 1Ml
CT • PA pn eumati zation requi red for AP to occu r in
• '1'1 C+ a xial & co ro na l MR wit h fat- sat u ratio n most cases
includ ing PA, sku ll ba se & caverno us sinus important • In no n-pneu matized PA, spread via fascial planes,
in eva luating intracrania l co m plicat io n s va scu lar cha n ne ls o r directl y th rou gh
os teo mye litic bone
• Etio logy
IDIFFERENTIAL DIAGNOSIS o Acute o r ch ro n ic su ppu rati ve infect ion spreads via
a ir ce lls or ve no us cha n ne ls to PA
Co ngenita l cho lesteato ma in PA o Infection o f PA a ir ce lls causes coa lesce nc e wi t h
• CT: Smoot h , ex pans lle PA mass brea kdo wn of t rab ecu lae
• MR: Lo w 1"1 MR signa l; no men ingeal en h anc e me n t o Th ro m bo ph lebit is or di rect extension to ad jace n t
st ruc tu res including meninges, Meckel (a ve &
Trapped fluid in PA ca verno us sin us
• CT: PA ai r ce ll trabeculae m a intain ed ; n onexpan sile • Epide m iology: Rare in post-antibiotic era
• MR: Usually low T I MR signa l; h igh '1'2 signa l; n o
meningea l enha ncement Gross Pathologic & Surg ical Features
• Soft os teo m ye lit ic bon e wit h pocket s o f p uru le n t
Cho leste ro l gra nuloma in PA ma ter ial wit hi n co n flue n t PA air ce lls
• C I"; Trab ecular br ea kdo wn & co rt ical ex pa n sio n in PA Air ce ll track s fro m ma stoid to PA filled with pu s and
• MR: '1'1 & '1'2 sign a l hig h gran ula tion tissue
APICAL PETROSITIS
• Phl egmon thicken s & infl ames adj acent meninges
Micro scopic Features • T-bo ne CT to eva lua te for su btle co rt ical erosio n &.
• Offendi n g o rgan ism o fte n n ot cu ltu red secon d ary to invo lvem ent o f mid d le an d inner ea r
pr eoperative broad spec t ru m antihiotics • To d ifferentiate from other PA lesio n s, loo k for
o Flo ra o f acut e AP sim ilar to oto ma sto id itis: S. peripheral I'Alm enillgeal en ha ncemen t & co rrelate
pneumoniae, H. influcnza c with clin ical h isto ry
2 o Ch ro n ic AP associate d wit h ch ro n ic su p pu rative
ot omastoiditis: P. acru ginosa, Pro teus
• Eva luate vascu lar structu res ad jacen t to PA fo r
invol vem ent: ICA, dural ven ous sin uses, cavern ous
160 sin us

Pre sentati on Par k SN ct a l: Ca vernous sin us throm bo phleb it is secondary
L
• Most com mon signs/sym pto ms: Otorrhea associated to petr ous api citis: a case rep ort. O lo la ryn go l Head Neck
with deep fac ial, ear or retro-o rbital pain Su rg. 128 (2 ): 284 ·6 , 2003
• Clin ical p rofile 2, Math ew L ct al: G radcnigo's synd ro me: findin gs o n
o Symptoms va ria ble; ma y be sub tle a ppea ring co mp uted tomo graph y a nd mag neti c TeSOJ1<1 nce imaging. J
grad ua lly or acutely Postgratl Mt.'d . 4R( 4 ): ] 14·6, 2002
• Acut e onse t of deep facial pain & oto rrhea 3, Price T ct al: Abd ucen s n erv e pa lsy as t he so le presen ting
foll owing acu te o to masto id itis sy m pto m o f petr o us a picit is. J La ryng o l O to l. 116(9):
726·9, 2002
• Insid iou s o nset of cran ial n europathy (especia lly 4, j agadc csan P et al: Grnde nigo's sy nd ro m e.. a rare
CNS) & otor rh ea wit h chroni c su pp ura tive ear co m plica tio n of o t it is m edi a. J Ind ia n Med Assoc. 100(11):
• Ot her cran ial n europathies (CN6, 7 & 8) 669 -70, 2002
• Fever, h earing lo ss &. d iplopia 5, Price T ct a l: Abd ucens nerve palsy as t he so le presen ting
• Co m plete clinica l syn drome = GRALJEN IGO sym pto m o f pet rou s ap icitis. J Lary ngol O tol. 116(9):
SYN DROME, rare presentation 726·9, 20 02
o Acu te oto ma sto id itis, deep facia l pain (CNS) & CN6 6, Damrose EJ et al: Radi ology fo ru m : qu iz case 2. Diagnosis:
pal sy pctrous ap iciti s with seconda ry abducens nerve palsy, Arch
O tol ary ngol Head Nec k Su rg. 127(6):7I S-7, 200 1
o Class ic clinical tri ad associat ed with a pica l pet rositi s
7. So mers 'IJ et al: C hron ic pet ro us a picit is with pertcarottd
• Oto rnastoi d itls, d eep facial pain (5t h n erve palsy), ex tens io n i nto t h e n eck in a chi ld . An n 0 101 Rhi nol
an d lateral rectus palsy (6th nerve pa lsy ) Laryngo l. 11O(10): 988 -9 1, 20 0 1
8. Dave AV et al: C lin ical and m agn eti c resonan ce imaging
Demographi cs fea ture s o f G rad cnl go syndrome. Am J O ph t ha lmo l. 124(4):
• Age 568-70, 199 7
o C h ild o r ad olesce n t with acu te o to masto id itis 9, Mu rakami T LIt a1 : Graden tgo's syndrome : CT and MRI
o Ad u lt with ch ro nic su p purative ear or followi n g find ing s. l'ed iatr Radi o l. 26(9) : 68 -1 -5, 199 6
m astoidectom y 10. ll ardj asud arma M et al: Magn eti c reso nance im agin g
features o f G rad en tgo's syn drome. Am J O tola ryngol. 16(4):
Natural History & Progno sis 247 ·S0, 1995
• Progresses to o bt undatlon &: death if untreat ed 11. Tutuncuo glu Set ill: G rad cni go synd ro me: a case report.
(co m mo n in pr e-antibiot ic era) Ped lat r Radinl . 23(7): 55 6, 1993
12. Frates MC et a l: Pctr o us apicit is: eva lua tio n by hon e SPEl.
• Progn osis excellen t give n ade q ua te surgical d rainage &: and m agn eti c resonan ce im aging. Cli n Nuc l Moo. 15(5):
agg ressive an tibio tics 29:1-4, 1990
Treatm ent 13. d e Graaf J et al: Graden tgo's syn d ro me: a rar e co mplication
o f o titis m ed ia. Clin Ne ural Neu rus u rg. 90 (3 ): 237-9, 1988
• An tibiotics alo ne possible bu t su rgical drainage is 14. Con rrucci RB et (11 : Petro us api citi s. Ear Nose Th roat J.
o ften req 1I ired 64(9): 427-3 1, 1985
• Aggressive su rgica l interven tio n wit h masto idectom y 15. Kea rns DR et al: Tu be rculous pctrous a picit is. Arch
foll owin g ai r ce ll tracks to PA usually pr ovid es O to la ryngol . 111(6): 406-8, 198 5
ad eq uate dr ain age 16. C ho le RA: Pet rou s ap ici tis: surg ica l an ato m y. An n Oto l
• Mu ltiple surgical o ptio ns have been described Rhinol Laryngol . 94 (3): 251·7, 1985
o Sim ple vs rad ical ma sto idect om y &. midd le cran ial 17. C ho le RA et al: Pet ro us apici tis. C linica l considera tions.
An n O to l Rh inol Lary ngol . 92( 6 l't 1): 5 44·5 1, 1983
fossa ap pr oach 1H. A ll am AF et <I I: Pat hology of pe tro sitis. Lary ngoscope.
78: 18 13-32, 19 68
Co nside r
• Cons id er ini tial im agin g with a thin -sect ion
n onenhanced 'l-bo ne CT
• MR wit h m ul tip lanar, fat-saturat ed , en ha nc ed im ages
are most effective way to evaluate for intracranial
co m plic atio ns
APICAL PETROSITIS
IIM AG E GALLERY
(l£j l) A~;Jl I-bone Cl of

rig ht ear {ew.lls
in flam mation o f middle
('ar-mastoid. Apical petrositis 2
is diagnmt'd because of PA
opJcification with trs becuter 161
/)rmJ.down &. erosive co n test
changes (arrow s). (NiKllI)
A'(;al ll C. AIR show s .1p iedl
p('lfm;tis wieh infectiom
ennsncemeat of PA Notice
pnh.mct>mffit invo/H!5
mf'ning«~ (arr ow), ,\ It''Ckel
C,W(', div.lf nM"O'o\' space
(o p t.· n dHOW ) &. Cd ve fOOUS
ICA (cur vro ,m ow ).
(/.('/1) A";,ll T1 C. AIR

ff·W'I I.~
otom ,woiditis
(enha ncing middle
ear-n lJ 5to id ) with ap ical
pet rositis. Apical pe trositis is
st'l'n as enhancing PI\ Jn d
adjacen l meningt'.\ (arro \v).
No lr' lAC ('nhann-mC'nt
(OpPfI .ITroW). (Rig" " Axial
T2W/ AIR demons trates fluid
signal in right mastoid
(arrow ) with connueru high
signal in the right petrous
,lfX'X (ope n arrow). No te
normally aerated lpit PA
(curved arrow) with low 12
~ign a /.
(l Lfi ) A:~;dl TI C+ MR shrnvs

,1 seve re ca st" o f .1pical
/X'trositis with nononbsn cing
t1uid (p u s) in e'(p. m dt'( ! PA
(arrow) and euension into
the CJ \ '('fnOUS sinus (0/X' n
.1,,0\'1), Curved .1rrO\v:
A-'.1stoiditis. ( /( ig 1ll) Corona!
11 C+ AIR shows Sf'\'t'rt' caw
o f co nt1uent ap ic.ll f)f't m.~ it;s
with focal PA dbsu'ss
(a rrow) ,~ meningeal
thickening of lA C (op{'n
Jrrow). Curved .uro", merks
/,)(('(.11diva! conte s t
des truction.
TRAPPED FLUID, PETROUS APEX
2
162
Axial graphic or Icft temporal bone demonstrales or

Axial bone CT left temporal bone shO\vs the cJas.'iic
fluid-tilled peuous apex air cells (arrow). Notice that appearance of trapped t1uid in the PA (arrow). The
trapped fluid of the peuoos apex has no expansion Of lesion is non-expansile end the Ihin inlcrnal hony
trobec..ular breakdo wn. trabeculae are intact.
• No expa ns ile co m po nen t to lesio n

ITE RM INO LO GY • Air ce ll margin s may be sclerotic from prev ious
Abb reviatio ns and Syno ny ms inflammation
• Abbr evi ati on : Trapped fluid , pet rou s a pex (TF-I'A) • CECT: No en ha n cemen t of PA or adjacen t m eninges
• Synony ms: Petrous ap ex effu sion; "leave-me-alone" MR Findi ngs
lesion o f I'A
• TlWI
Definitions o Signa l in PA air cells ma y var y from low to h igh
• TF·PA: Sterile residual fluid co llect ion in PA air ce lls (inc rea sed p rot ein conte n t o f fluid )
probably left behind aft er remote oto mastoid it is • Low '1'1 sign al most co m mon
• Less co m mo n sign a l is interm ed ia te in inten sity
• Rarel y TI signal in te ns ity will be hi gh
IIM AGING FINDING S o Uncom mo n ly h et ero gen eou s Tl signal may be seen
• Protein con ten t of ad jace nt ai r cells m ay va ry
Ge neral Features • T2WI: High T2 sign al in otherwise n ormal-appearing
• Best di agnosti c clu e: Low '1'1 , hi gh '1'2 signa l "lesio n " of petrou s apex
I'A on MR w he re bone CT shows opacified I'A air cells • T l C+: No enhancement of PA o r adj acent m eni nges
wit hout trabecu lar loss &: wit hou t ex pa n sion sho u ld he present
• Locatio n : Un ilate ra l "lesion" of PA air cells Im agin g Reco mmen dati on s
• Morpho logy: Co n fi ned to PA wit ho ut abno rma lity in
• Imagin g recomm endati o n s in sus pected TF-l'A are not
ad jace nt me ninges
we ll est ablishe d
CT Findings • TF· PA co m mon ly 1st det ect ed o n rou ti ne bra in MR for
• NEeI' unrelat ed indicati ons
o non e CT • Hon e CT t hroug h 'I-bo ne a rea used to so rt o ut rare
• Un ilate ral opacification o f PA a ir ce lls in ab sence surg ica l lesio n s of PA fro m far more co m mon but
of m id d le ear or mast oid in fla m ma to ry cha nges in cidenta l TF-I'A
• No ev idence o f corti ca l erosion o r trab ecu lar o Bone CT done if any q ues t io n abou t et io logy of PA
destructi on wit h in PA lesio n ex ists
DDx: PA Non-N eopl asti c Lesion s
Cholesteatoma PA Mucocele 1'/1. Cbo! Granuloma Ceph alocele PA

Key Facts
Termino logy • Mucocele, PA
• Abb reviation: Trapped flu id, petr ou s apex (TF. PA) • Choleste rol gran uloma, PA
• Synony ms : Petrous a pex effusio n; "leave-me -alo ne" • Apic al pet ro sitis
lesio n o f PA • Ce pha locele, PA
• TF-PA: Ste rile res id ua l fluid co llec tio n in PA air cells
probab ly left behind afte r rem ote ot orn asto iditi s
Pathol ogy
• TF-PA is resu lt of extensio n of fluid from midd le
2
Imaging Findin gs ear-mas to id to PA d uri ng rem ote oto mas to idi tis 163
• Rest di agn ost ic clue: Lo w 1'1, hi gh 1'2 signa l "lesio n " • Residu al fluid in PA air cells is presen t in - 191, of all
of PA on MR where bon e Cf shows o pacified PA air head MR
ce lls w ithout tr ab ecu lar loss & with out ex pa ns io n • TF-PA is most com mo n lesion found in PA
• Bon e CT t hrough 1'-ho l1earea used to sort out rare Clinica l Issu es
sur gica l lesio ns of PA fro m fa r mo re co mmon but • Patien t is un dergo ing brain M it for un related
inciden tal TF-PA sym ptoms
Top Differential Diagnoses • No th erapy o r follow-up is warra nt ed for classic
• Co nge n ita l cho lesteato ma, PA trap ped fluid in pet rous apex
o Always do ne if n eu ro-o tol ogtcal sy m pto ms o Loss of cor tical margin wit h con nect ion to
att ributa ble to side of TF-PA presen t subarach no id space
• Remo te ( ;~ year) follow-up bon e cr is recommended • Mil: csr sign al (low Tl , high 1'2)
for TF-PA lesion where cr shows no rmal PA air cells o Co ntiguous wit h Meckel cave
but h igh '1'1 signa l o n Mit
o Th is follow-up c r d on e to exclude remot e
possibility of tran sform ati on in to cho lesterol IPATHOLOGY
granu loma
o Docu men tatio n o f case whe re TF-PA has been noted Ge ne ral Features
to tra nsform in to cho leste ro l gran ulo ma has not • General path com men ts
occ u rred o Em bryo logy-anatom y
• PA pn eum atization is requ ired fo r Tf~ PA to occur
• 33% o f people have pneumati zed petro us apices
IDIFFERENTIAL DIAGNOSIS • 5% o f th ese are asymm etrica lly pn eum atized
o Air cell tracks co n nect mastoid &. middle ear with PA
Co nge nita l cho lesteato ma, PA • Tracks allov.... spread o f infectio n or infl ammat ion
• Bo ne CT: Smooth, cx puns ilc lesion of PA air cells fro m midd le ea r-mas to id to PA
• MR: '1'1 C+ MR sh ows low signal lesion wit h • Tracks also pr ovide su rgical access to PA
rim -en ha ncemen t • l'eritubal, posteromedial &.
o No men ingeal en ha nceme nt present posterosuperior-subarcuate tracks have been
described
Mucocele, PA • Etio logy
• Bon e c r: Smo o th, expansile lesion of PA air cells o TF-PA is resu lt o f extension of fluid fro m midd le
• MR: Low Tl MR sign a l, h igh T2 signa l ea r-mas to id to PA during remot e ot ornasto tdit ts
o Rlm -cn ha ncement 0 11 T l C+ MR o Air cell tracks con necting PA to middl e ear-mas toid
• May mimic co ngen ital cholesteato ma of PA becom e o bst ructed followi ng ho ut o f oto masto iditis
Cho lestero l granuloma, PA o Sterile fluid becomes trap ped in PA air cells
• 130n e Cl: Expansile lesion of PA air cells • Epidem iology
o Residua l flu id in PA air cells is present in - 1% of all
o Lo ss o f PA bo ny t rabecul ae
h ead MR
• lIi gh Tl and hi gh T2 MR signa l
o Tf-PA is most co m mo n lesion found in PA
Apica l petrositis o TF-PA: Cho lesterol gran uloma of PA ratio is - 500: 1
• Bo ne C I': Destruction of trabecu lae an d co rtical Gross Pathologic & Surgica l Features
eros io n
• Clea r to xan th och rom ic fluid d iscovered in PA air cells
• MR: l.ow T I signal, high T2 signal
with in in tact hon y trabeculae
o '1' 1 C+ MR sho ws th ickened , e n ha n cing me n inges
• Adjacen t co rtical bone is no t so ft &. meni nges a rc not
wit h spread to ad jacen t struc tures
th ickened
• Clin ical setti ng o f oto masto idi tis o r
post-mastoidecto my Micro scopi c Features
Ce p ha loce le, PA • Ster ile fluid
• No microo rgan isms o r tumo r cells present
• Bon e CT: Fluid att en ua tio n with in PA
• Careful evalua tio n o f all im aging in co mbina tio n with
IC LIN ICA L ISSUES revi ew o f clinical fin d ings sh o u ld re sult in co rrect
Pre sentati on d iagn osis in nearl y all cases
• Most co m mo n signs/sy m pto ms
o Principa l pr esenting sym ptom : NONE!
o Ca n po se a clinical dil emma in so me patient s ISELECTED REFERENCES
2 • If pati ent has unrela ted but co n fusin g
neuro-oto logi c sy m pto ms , co nfusio n ca n result
I. Pa lac ios E et al: 'Don't tou ch me' lesio ns o f th e petrous
apex. Ear Nose Thro al .l. 80(3):140, 2(Xl!
• Clin ical profil e 2. Le on ett i J P ct al: Incid en tal pct ro us apex find ings on
164 magn et ic reson ance im aging . Ear Nose Throat J.
o Pati ent is und ergoing br ain MR for u n related
80(4):200-2,205-6,200 1
sy m pto m s 3. Profant M ct a l: Pet rous a pex cho lestea to ma . Acta
o In c idental MR findin g o f PA lesion d escribed as Otolaryngol. 120(2):164-7, 2000
"suspic io us for cholestero l gran ulo ma o r apical 4. Muckle RP ct a l: Pet rou s apex lesio ns. Am J Otol.
petro siti s" in radi ology rep ort 19(2):219-25, 1998
o Pati ent is referr ed fo r cl inica l assessm ent &: po ssib le 5. aw cn gen DF: Surgical an atomy of th e tran stemp oral
su rgical assessm ent approaches to th e pctrou s apex. Am J Oto l. 1 9 (2 ) : 2 4 B~9,
1998
Dem ographi cs 6. Cha ng P et al: Imagin g destru cti ve lesions of the petrous
• Age: All ages a ffected apex. Laryngoscope. 108(4 Pt 1):599·604, 1998
7. Moore KR ct af: "Leav e me a lone " lesion s of th e pctrous
Natural History & Prognosis apex. A.lNR. 19:733-8, 1998
• Tra pped fluid in pe trou s a pex will rem a in unchanged R. Gad rc AK et al: Ven ous ch an nels o f th e pet reu s apex: their
p resence and clin ical im po rtan ce. Ot olaryngol Head Neck
t hroug ho ut pa tient's life
Surg. 116(2):168-74, 1997
• Th eor etical possib ility that one of rare intermed iate o r 9. Casse lma n JW: Te mporal bon e im agin g. Neuro imaging
hi gh T1 sig na l lesions will tran sform into ch o les tero l Clin N Am. 6(2):265-89, 1996
gran u loma exists 10. de Jesu s 0 : Pet rous apex regio n . Surg ica l an ato my, lesions,
• No case report o r series has been publish ed as yet a nd o pera tive techn iqu es. P R Healt h SdJ . 15(2):107-12,
show ing this t ra nsfor ma tio n can occur 1996
11. Curti n HD ct al : The pet rou s ap ex. Otclaryngo l Clin North
Treatment Am. 28(3):473·96, 1995
• No therap y o r foll ow -up is warranted for classic J 2. Mem ts A e t a l: I'etrous apex mu cocele: high resolution CT.
tr apped fluid in pet rous apex Neuroradiology. 36(8):632-3, 1994
13. Larson TI. ct a1: Prima ry m ucocele of the pct rous apex: MR
appearance. A.lNRAm ] Neuroradiol. U(I ):203-4, 1992
IDIAGNOSTIC CHECKLIST 14. j ncklc r RK ct <I I: Radi ograph ic diff eren tial d iagno sis of
petro us apex lesions. Am J Oto l. B (6):56 J·74, 1992
15. Arriaga MA et al: Differen tial d iagn osis of primar y petrous
Consider apex lesion s. Am ] 0 101. 12(6):470-4, 1991
• TF-PA as proba b le d iagn osis a ny tim e lesion in I'A 16. Franklin DJ ct a l: Managemen t of petro us ap ex lesions.
sus pe cte d ba sed o n MR findin g Arch Ololaryngo l Head Neck 5urg. 115(9):112 1-5, 1989
o TF-PA is ve ry co m mon incidental find in g on hrain 17. Floo d LM et a l: Th e in vestigation and ma nagem ent of
MR co m plete d for o t h er indicati on pet rous apex erosion . J Laryngo l Otol. 99:439-50, 1985
o O ften mi sdiagn osed as cho leste ro l gran ulo ma o r 18 . v trapo ngsc C et al: Co m puted tomogra phy of tem poral
bone pn eum at izati on , 1: Nor ma l patt ern a nd morphology.
apical pet rositi s
A.lR. 145:473-Rl, 1985
o Misguided aggressive med ical or su rgical therapy
may result
• Co ns id er thin -slice T-bone c r in cases wit h atypica l
findings o n MR
• Con trast-en hancem en t &. fat -saturation o n MR may
also be h elpful in differentiating T F-PA fro m o t her
lesions o f PA
Image Interpretati on Pearl s
• Non ex pa ns ile &. nondestru ctive lesions o n bone CT
with unifo rm lo w '1'1, hi gh T2 sign al o n MR requ ires
n o further work-up
• Nat ur a l hi st ory of T F-PA w it h a ty p ica l MR findi ngs
(h igh or intermediate '1'1 sig n a l) is n ot certain
o In th ese rare cases, w ith a normal T-bone C'I, fo llow
up cr in 3 years is recommen d ed
o Fo llow-up c r d one to exclu de ea rly I'A cholesterol
granuloma
• View any I'A expan sio n, co rtical irreg ula rity o r
ad jacen t meningeal en ha nce m en t-t h icken ing with
suspicio n
IIMAG E GALLE RY
(Lef t) A xial T2Wf MR sho lVs
signal within the '('it p etrous
ap ex (arrow) which is
isoinlense with the ( SF in
2
the <1dj.lcenl cistems . Internal
PA ho ny tr.llwcu/.le .w en .15 165
subtle low signal Jines arc
p reserved. (Rigltt) A xial
r I WI MR shows low signal
inWll sity in th l' {pft p e(rous
ape x n m sistt'llt witb fluid
(arro w) . Note tIlt>no rm al
hypNin lpfN ' Yf·/IolV marrow
!liMn ,]1in Ihe contratueret
petrous apex (op en .1ffO\\, ) .
JI~~ .~~.\: \
(i:>: _.:4~)f (l~ft) A xial T2WI MR sho ws
anoth er cla ssic case of
trapJX.>d fluid in right petrous
.
, r.-w1 apex (arro w ) ,H high PA
r: r.,. -:r _•••

I ; ' . "\1' .. "., ' , signal with p reserved inl f'ffMI
r '- :,r • " trabeculae . Nole ,Jt'ra /e (/
r, ~ ~ • ' . ' cOlltralateral petrous ap ex
" , i/.r ~
•J r' " ~ . , •",
\ (orw n arrow). (Ril: lIt) Axial
T1 C+ MR reveals low T1
... • .. 1~
..~.~
,/ "- , ~ ~. " ,
\.: i /lf( " 15it y 1)('lrou_~ apc>x l1ui rl
~
' , Oil tht' right (arr o w ) . Nolin'
), ." , . th e db w /l ce of t'fl hafK em ent
..,
.
r , in the typ ic al case of lrappf.'<./

flui d in the p otrou s apex .
.,,'~\
Varia nt
' \ .' ~.'
\. ,. . "~.: . - t j
(LRf l) Ax ial hon e CT

ell'm o nslrales Iyp ical
app(.'dran c(.' of tr apped fl uid
in the pctrou5 apex .
Non-exps nsite fl uid densit y
in right pt'If()IJ~ .1J>P.r. pre.~t'nl
witll p'esC'rvaliolJ of th e
imcmsl bony lr.1bpc{l/J('
(arrow ). (Rigllt) A xial ' 'WI
MR sho w5 an examp /(' of
increased T 1 signJI in
trap p('d fluid in PA (arrow).
C T showt'd fl uid in air cells
in sbsence of eX!J.lmio n or
trabecutar b n'akdown. J
year C T follow -up
rec ommended.
CHOLESTEROL GRANULOMA, PETROUS APEX
2
166
Ax;al graphic ,~ h()ws a cholesterol granuloma of WI. The Axial T 1WI MN. reveals a homogeneously bright
lesion is f'Xflilmi!(' with air cell trabecular loss and cholesterol granuloma of /f.'ft F'l\ (arrow). Note mild
"c8!:-shefl" medial cortex (arrow) . Open arrow : lAC expansion with bowing of tho Ie,ti leA (opon arrow )
imvlw menl. an /prior/y.
o Well defin ed smo o th ly cx pans ne PA lesio n

ITERM INO LO GY o Trabecu lar breakdown with co rtical thinnin g of IJA
Abbreviations and Syno nyms ex pected
o Lu ger lesio ns will ha ve areas o f focal bo n y
• Cho lestero l gran u loma- l'A (CG-PA)
• Choleste rol cyst, "choco late" cyst, xan thoma deh iscenc e
o When large, erodes in to lateral clivus, jugular
Definitions tubercle, lAC ± petrous in ternal ca rot id canal
• CG-PA: Expa n sile m ass of PA resulting from a ir cell • CECf: Faint periph eral en h an cemen t may be present
iso lati on with infl ammat or y granu latio n ttssuc
respo nse to presence o f cho lestero l crysta ls
MR Findin gs
• TlW I
o High in tern al Tl signal
IIM AGING FINDINGS • Seco nd ary to pr esenc e of h em orrhage. blood
break-dow n p rodu cts & chole ste rol crysta ls
Ge ne ra l Fe ature s • Primary reaso n most likely presence o f
• Best d iagn ostic d ue: High Tl & T2 signa l in expans ile paramagn eti c intracellular methemoglob in
PA mass o Large lesio ns co n tain low signa l lin ear areas =
• Locati on septatio ns
o Ante ro me d ial PA • T2W I
o Wh en large, exte n ds int o su rroun di n g area o High in terna l 1'2 signal
• Medially into clivus, lateral to m idd le ea r, o Per ip heral d ark hem osiderin rin g
posterio r to CPA cisterns • flAIR: High T2 signal does not attenu at e (remains
• Size: Range fwm small lesio ns co nfine d to PA to large high )
lo bulated masses expand ing ad jacent areas • Tl C+
• Morph o logy: Smooth, shar p ly marginated , lobulated o Peripheral en hance me n t m ay be di fficu lt to
when large ap preciate adj ace n t to 1'1 bright lesio n
o No internal en ha ncemen t
CT Find ings • MRA
• N ECf
DDx : Petrous Ape x Lesions
Asym metri c Ma rrow Cholesteatoma, P;\ Pel ro us ICA Aneurysm Chondrosarcoma

Key Facts
Termino logy • Prim ary malign ancy o f PA a rea (cho rdom a,
• CG -PA: Expan silc mass o f PA resulting from a ir ce ll ch on drosa rco ma)
isolati on with infl ammator y gra n ulatio n tissue Patho logy
response to presen ce o f cho leste rol crysta ls • Gra nu lation t issue forms seco nda ry to re peated
Imaging Findi ngs hemo rrhage leadin g to cxpans tlc PA lesion 2
• Best d iagnostic clue : High '1' 1 &: 1'2 sign a l in cx pans ilc Clinica l Issues
PA m ass 1(,7
• Sen sor ineu ral h earing loss (SNHL)
• MR more sens it ive t h an CT for evalua tion o f • Exten ded m iddl e cra n ia l fossa approa ch wit h
recurren ce extradura l remova l o f CG- PA N oblite ra tio n of its
Top Differe ntia l Diagn ose s cavity
• Asym met ric fatty ma rro w, petro us apex Diag nostic Checklist
• Co ngen ita l cholesteato ma of PA • Co nside r CG-PA in a ny no n-aggresstve lesion o f PA
• Trapped fluid , pet ro us a pex wit h hig h '1' 1 & '1'2 sig na l
• Apical petrosit is
• le A aneurysm, pet rous apex
o Usefu l in su rgica l pla n n ing, assess for incasemcn t o f

pct rou s le A Apica l petrositis
o Bewa re: Lesio ns w it h hig h T J sig nal w ill appea r • CT: Permeative, destructi ve c hanges of cor tex &
br igh t o n ti m e of fligh t MR A; m im ics an eurysm t rabecul ae
• T1 MR: Low sig na l; '1'2 M R: lIi g h sig na l
Imaging Recommendations • T t C+ MR: Th ick, en ha ncing rim; men inges thi ck &
• Rest imaging tool en h anc ing
o Combi nation bon e CT &. M R
• 'l-bo ne CT eva lua tes bo ny erosio n & in vasion of
ICA ane u rysm, petro us apex
co n tiguous struc t ure s • CT: Smoot h ex pa nsion of pet ro us le A ca na l
• MR cha racte ristic lesion signal con firms d iagn osis • MR: Heterogen eous '1'1 & T2 with in ter na l flow vo id
• Co n trast is no t help ful in delin eati ng dia gn osis o f • T t C+ MR: Heterogen eou s int erna l enha ncem en t
C G~ PA
Primary ma lignan cy of PA area (cho rd o ma,
• MRA. to ev alua te for in vo lvem en t of petro us l e A in
la rge lesio n s cho ndrosa rco ma)
• Post-o pe rat ive im agin g for recu rrence • c r: Destructive lesion at clivus o r petro-occip ital
o MR m or e sens iti ve th an CT for evalua tion of fissure with cho ndroid matri x
recu rren ce • T I MR in ter med iate: T2 !vfR: High signa l
o Increa sing '1'1 signa l in po st-o perative PA = • T I C+ MR: Heterogen eous in tern al en ha nce me n t
recu rren ce
• Beware su rgica l fat packin g
IPATHOLOGY
IDI FFERENTIAL DI AGNOSIS Ge ne ral Feat ures
• Ge ne ral pat h co m me n ts
Asymmet ric fatty marrow, petro us apex o Embryol ogy-a na tom y
• c r: No n-cx pa nsl le fa t den sity • Pneum at ized PA ai r cells req u ired
• TI MR: High signal; '1'2 MR in termedi ate to h igh • PA pn eu ma ti zatio n occu rs nor m ally in 33% o f
• Su ppresses on fat -sat urated MR peo ple
• Etio logy
Co ngenita l cho lesteato ma of PA o Etio logy- pat hoge nes is (classic h ypot hesis)
• Cl: Smooth , cx pa ns t!c m argin s • Muco sa l o bst ructio n o f PA a ir cells spo n sor s
• T1 MR: Lo w to int erm ed iate sign al; '1'2 M R: developme n t o f a vacuu m
In h.'. rn H..-dia te to high • Vacuum p hen o mena leads to rupt ure of hlood
• '1' 1 C+ MR: Rim -en ha ncem en t vessels &. hem orrhage in PA a ir cells
• Anae ro bic degradat io n o f red b lood cell s fo rm s
Trap ped fluid, petrous ap e x cho lesterol crysta ls whic h inci te for eign bo dy
• Cf: Opacified a ir cells; no nexpa nsile: co rte x &. gia nt cell in filt rati on
trab ecu lae intact • Gra n ulatio n tissue fo rm s seco nda ry to repeat ed
• '1' 1 MR: Low-int erm ed iat e signa l (ra rely h igh signal); h em o rrh age leading to expa nsile PA lesio n
'1'2 Mit lIi gh o Etiology- pa thoge nesis (recen t hypot hesis)
• T I C+ ~vf R : No cont rast-enhan cem ent of lesion or
men inges
• Mucosal pen etrati on in to PA exposes marrow • CT, MR &: MRA in p re-op erati ve plan ning, par ticularly
wh ich leads to susta ined/ repea ted in large lesions
micr oh em orrhage • MR is most sen sitive stud y in eva luati ng for
• Epide miology post-op erat ive recurren ce
o Most co m mo n su rgica l lesion in PA
o Cholesterol granu loma of middle ea r-mastoid more Image Interpretati on Pearls
com mo n than CG-I'A • Characteristic appearance o f h igh T1 & T2 signal
2 Gross Path ologic & Surgical Features
without cen tra l en hancement d ifferentiat es from other
I'A lesion s
168 • Cystic mass wit hout epithe lial lining • c r most useful to evaluate bony des truct ion &
• Fibrous ca psule filled with brownish liqu id con tain ing inv ol vem ent of ad jacen t otic caps u le &: carotid canal
old bloo d & cholestero l crystals = "ch ocola te cyst " • Make sure to eva lua te for int ernal flow to avoid
• Fluid described as "crankcase oil" mi sd iagn osing a petrou s ICA aneu rysm
• RBe in variou s stages of degradation
• Multi n uclea ted gian t cells surro unding choleste rol
t. Sze C I et a l: Intracal vari al c ho lesterol
crystals em bedded in fibr ous co n nec tive tissue alo ng
gra nu lo mas--c1 inic o pat holog i<.: cor rela tes of three cases.
wit h hem osiderin -lad en macrophages, chron ic Clin Neuropathol. 22(1): 4 t-6, 20m
infl ammator y ce lls &: blood vessels 2. j ackler RK et al: A new th eor y to expla in th e gcnesis of
pct rou s 'lpex ch o lestero l gra n uloma. Otol Neurotol. 24(1):
96 -106; d iscussion 106, 20tH
ICLINICAL ISSU ES 3. Hun t JP ct a l: Cho lestero l gran ulo ma o f t he petrous apex,
EarNose Throat J. 81(4): 217-8, 2002
Presentation 4. Brack m ann DE ct a l: Sur gica l management of pet rou s apex
• Most co m mo n signs/sy m pto ms c holeste rol granu lo mas, Otol Neurotol. 2](4): 529·33, 2002
o Sensori n eural hearing loss (SNHL) 5. Bonneville r et al : Un usua l lesions of th e cc rcbcllo pontine
ang le: a segme n ta l approac h . Radiogra p h ies. 2 1(2): 419-38,
o Othe r presenting sym pto ms
200 1
• Tinn itus, hemifacial spasm, facial numbness, 6. Pisan esch l Mj et al: Congen ita l cho lestea to ma and
trigem in al neuralgia &: abd uce n s pa lsy cho leste rol gran ulo ma of th e tempora l hon e: role of
• Clinical profile magnetic resonan ce im agin g. To p Magn Resou Imaging.
o Ot oscopy is usually nor ma l, may appear as a blu e It (2): 87-97, 2000
ret rot ympan ic mass if has broken into middle ea r 7. Ch aljub G e t al: Magnet ic reso nance ima ging o f petrous tip
o Aud iometric studies de monst rate SNIIL or a mixed lesions . Am j Ot o lar yngo l. 20 :304· 13, 1999
pattern 8. Palacios E et al: Pet ro us ap ex lesion s: cholesterol
gra nu loma. Ear Nose Throat J. 78(4): 23 4, 1999
Demographi cs 9. Muckle RP et a l: Petrou s ap ex lesio ns . Am J Otol. 19(2):
219-25, 1998
• Age: Young to middl e-age adults
10. Ch an g r et al: Im agin g destru ctive lesio ns o f th e petrous
Natural Histor y & Prognosis ap ex . Lar yn goscope. 108(4 I't 1): 599· 604, 1998
11. Eisen be rg MB et a l: Pctrous a pex cho leste rol gran ulomas:
• Great variabi lity in rat e o f growth of CG-PA exists evo luti o n a nd managem ent . .J Neuros urg. 86($): 822-9,
• Depe nds o n freque ncy & severity o f micro 1997
hemorrhages 12. Isaacso n J E et al: Ch oleste rol gran ulo ma cyst o f th e petrous
• Most take decad es to grow ap ex . Ear Nose Throa t J. 75(7): 425-9, 199 6
o Symp to ms show up years after initial bou t of 13. Morriso n GA e t a l: Cho lestero l cyst and c holesterol
chron ic ot itis medi a gra nu loma of th e pct rous bon e. .f Laryngol 0 101. 106(5):
• If adeq ua tely dra in ed , exce llen t pro gn osis 465-7, t992
14. Clifto n AG et 01 1: Cho leste rol gran u loma of t he petrous
Treatment apex. llr J Radio!. 63(753): 724-6, 1990
• Pati en ts who have minima l or no sym pto ms ca n be 15. Grccnbcrg j ] et al: Ch olesterol gra n ulo ma o f th e pctrous
ap ex: MR and CT evalua tio n. AjNR. 9:1205-14, 1988
safely followed wit h ima ging
16. Amed ee RG ct 01 1: Cho lestero l gran uloma of t he pet rous
• Tradition al treatmen t : Drainage & ste n t placem ent to apex. Am J Otol. 8(1): 48-55, 1987
re-establish I'A aera t ion via tran stem poral approach 17. Griffin C c t at: MR a nd CT co rrela tion o f chol esterol cysts
o 'Reported recurren ce rat e as high as 60% of the pctro us ho ne. AjNR Am J Neu rorad io l. 8(5): 825-9,
• Extended middle cra n ial fossa approach with 1987
extrad ural rem oval of CG-I'A & ob lite ration of its 18. 1.0 WW ct a l: Cho leste ro l gra nu lo ma o f th e petrous apex:
cav ity CT d iagn os is. Rad iology. 153:705-1 1, 1984
o Signi ficant decrease in recurrence rat es
Co nside r
• Co nside r CG-PA in any non-aggressive lesion of PA
with h igh T1 & T2 signal
IIM AG E GALLERY
Typical
(Left) Axial ho ne C T shows a
smoo thly exp onsile lesion of
the left PA Co-ncot
dehi scence o f the medial PA 2
border (arro w) allo ws {or
(' XpiUJ.';O/l of th l! nJ.HS in to 164
the n 'l"cb cll opontinc angle.
(Right) Axiel bon e Cl show s
non-agw essive expansilc
(m((If{'sof CC -PA 1.0 55 of
cortical contiguity
w /m aintenilflce of a th in rim
o f co rtex in so me locations
(arrow ) indicates a slowly
enlarging ben ign process.
Typical
(lLJI) Ax ial T 1WI MR shows
a classic t'xample of CG·PA
wit h b omogonoou s high
signal wit h w eI/-defined
margins. This lesion expands
the PA ml>rlially with mass
effect on the cerebetter
peduncle (arrow). (Rig" l)
Ax ial T2WI MR reveals a
!JO/l}ogC/lrous int em cll high
signal in a CG - /~\. Naif;' the
dark p rriph (>ral ring .1fOund
f h i~ le.~ i()n (iJ{{ow) con~;stt'nt
with hem m id('rin c/e,XJsition.
Va riant
(Lejn Ax ial Tf C+ MR shows
n large, complex CC· PA with
h f!t/;:lfOge lJ(!()U ~· internal T1
Signal. Internal septaliolJs
(arrow) arc atypical. Mass
(lIJJ'1rj; es tn to sphenOid sinus
(op en arr ow ) & lAC (cu rve d
arrow ). (Hi£" I) Axial Tl WI
MR clenuJ(1stratcs at ypical
heteroge neous signal within
this CC-PA (arlOw ).
Com plex signal within thcsc
lesions is most likely due to
various ages of internal
blood product s.
leA ANEURYSM, PETROUS APEX
2
I 170
'\
Axial gr.lphic 51lm V'i foc-.ll ;!flPurymJ.11 dilation (arrow) of Axial TtWI MR ~h()IVS c1 comp lex sign.l1mass in (X·trous
horizontal petrow; inlt'fflcll carotid artery. Note apex (arrow ). Posteromedial bright Tt 5i~na 1 (open
proxim it)' to tht· lTigw Jim l nerve (open arrow ) and arrow ) rep'f!!;enl5 (ally marrow in PA Iligh sign.ll in
abducens n f" \'{' (curved arrow). am' urysm waif is throm bus.
a When foca l; post-trau ma tlc, pos t-in fectious or

ITERM IN O LO GY at herosclerotic eti olog y
Abb rev iatio ns a nd Syno nyms CT Findin gs
• Internal carotid artery an eury sm, petr o us ap ex • NEcr
(ICi\ i\-I'A) a Fo cal o r fusiform enlarge me nt o f pct rous l e A canal
• l'etro us caro tid aneu rysm ; aneurysm of int rape tro us • Increased att enuatio n int ernally seco nda ry to
int ernal caro tid arte ry (ICA) thro mb us
Definitions • Cu rvilinear calcifica tio n s in an eurysm wa ll
• ICAA-PA: Rare co ngenital o r acq uir ed ane u rysm of • May appear destr uctive with extension int o
petrous ICA ad jacen t struct u res
• CECT: Well d em arcated expa nsile lesio n with ,
, att enua tio n & en hance me n t involving pet rou s le A
,• IIM AG ING FINDING S • C rA
• a CTA is d iag nost ic
""
II Ge nera l Features o Shows an eu rysm al dila tion of petrous l e A
,
",.
.,. • Best dia g nostic clue: Co mplex cx pans ile ma ss o f MR Fi ndi ngs
petrous le A canal dem o nstra ting in ter nal flow o n
r: MltA, C rA o r DSA angio • T1 WI
I' o Ovoid or fusiform co m plex signa l mass
,I • Locat ion predominant ly h yperinten se o n TI
.
.,
o May he found an ywh ere from entrance o f skull base
to cavern ous sin us
• Mu ltiple ages of in tralu mi nal clo t & flow
ph eno m en on
,,
.. o Most co mmo nly in h orizontal pctr ou s le A segment
• Size: Varies from small focal an eur ysm to diff use • T2W I
o CO MPLEX SIGNAL MASS with periphera l
fusifo rm d ilati o n of pet rous le A
hemosiderin
• Mo rp ho logy o In ter na l flow voi ds produce swirl patt ern
o Most are fusifor m wit h in tral u m inal throm hu s
• T2* GRE: I.uminal clot may show blooming
• T1 C+: Diffusely en ha ncing co m plex mass within PA
DD x: T-bone Vascul ar-Appear ing l esion s
Aberrent leA Chol. Granuloma Glomus lugulare

Key Facts
Termin ology • Best ima ging tool: MR wit h MRA delin eat es size,
• IeAA -!'A: Rare co nge n ita l o r acq uired aneu rysm o f sha pe & site of a ne urys m; exte n t of intraluminal
petr o us ICA th rombu s; pat en cy o f petrous ICA
Imaging Find ings Top Diffe rentia l Diagn ose s

• Best d iagn ostic clu e: Co m plex ex pa ns ile m ass of • Abe rra n t in terna l carot id a rtery (l e A)
• Dehi srent jug ula r bulb
2
petrous l eA ca na l demonstrating interna l flow on
MitA, CTA or DSA a ngio • Persistent staped ial a rtery 171
• CECT: Well demarcated ex pans ile lesion wit h I • Ch olestero l gran u lo ma, PA or m idd le ear (M E)
atte n ua tio n &. e n ha nce me nt in vo lving petrous le A • Glomus tympan icum paragangliom a
• Ovoid o r fusiform co m plex signa l mass • Glom us [ug ula re paraga ng lio m a
pred ominant ly h yperin ten se on T l • Schwa n nom a o f ju gular foramen OF)
• COMPLEX SIGNAL MASS with periphera l Clinical Issues
hemosiderin • Clin ical presen tation ranges fro m insid io us cran ial
neu ro path y to life threat ening epistax is o r oto rrhagia
• MRA • Mit: Co m plex PA or ME mass with 1 T1 I< '1'2 signa l

o Enlarged, irregula r area along pet rous l e A
• Oft e n sm aller than act ual aneury sm since o n ly Glom us tym pa nicum pa raganglioma
lum en with fl owing blood is seen • CT: Foca l mass o n coc h lea r promo ntory; not tubular
• Bewar e: Any lesio n with T l sho rtening will be • '1' 1 C+ MH: Enhancin g co chl ear promontor y mass
seen o n time of flight MRA; can m imic aneu rysm Glom us jugul ar e pa raganglioma
Angiog rap hic Find ings • c r: Perm ea tive-dest ructi ve bon y c ha nges on
• Aneurysma l dil atation of pet rou s l e A su pe ro late ra l margin o f jugula r fo ram en
• Size of ane urys m as see n o n an giog raphic imag e o ften • T I C+ MR: Enhancin g m ass exte nd ing up fro m jugul ar
m uch sm aller than act ua l an eurysm for am en into m iddl e ear cavit y
• In tral um in a l clot may obscure aclua i ICAA·PA size Schwa nno ma of jugul ar fo ra me n (JF)
Imaging Recom mendatio ns • CT: Smooth en largement of JF wit h ex pa ns ion int o
• Best im aging tool: ~'fR with MRA delin eates size, sha pe ad jacent st ruct ures
&. site of a ne ury sm; extent of intra lumina l t hrombus; • T 1 C+ MR : Diffusely en h anc ing tu bula r mass foll ows
pa te ncy o f pct rous le A cran ia l nerv e co u rse
• Thin-sectio n bone-o nly cr confirms locati on a long
ca rot id can a l
• Angiograp hy for en dovasc ula r th erapy, not diagn osis IPATHOLOGY
Ge nera l Featur es
IDIFFERENTIAL DIAGNOSIS • Genera l path co m me n ts
o False an eu rysm (acq u ired) in vo lves a ll three vessel
Abe rrant int e rna l ca ro tid a rtery (leA) wa ll laye rs
• CT: Tubula r ma ss crosses mid dle ear cav ity to rejoin • False an eurysm = pseudoa neurys m
ho rizo n tal petrous ICA o Tru e aneurysm (congen ita l) fro m weakness of sin gle
• MRA: Artery e nte rs m or e postero latera lly t ha n vessel layer .
oppo site norma l side • Etio logy
o Co nge n ita l a neurysm m ost com m on
De hisce nt jug ular bul b • Aneur ysm forms in a rea of co ngen ita l weakness at
• CT: Foca l ab sence o f sigm o id plate co n nects jugula r origin of obliterat ed e mb ryo log ic vessel
hu lh to midd le ear "m ass" <caro ticotym pa nic)
• MRV: Coro n al repro ject ion shows "IJUd" off o Acquired pscudoaneurysm less co m mo n
supe ro late ra l jugul ar bulb • In ju ry to vessel wa ll results in ext ravascu lar space
su rro u nde d by co n nec t ive tissue
Pe rsiste nt stapedial ar te ry • Post-tra umati c or post-in fecti ou s
• C f: Sub tle en largeme n t of an terior ty mpani c segme n t • Ath ero sclerot ic etio logy ver y u n usual in thi s
facial n erve with a bse n t forame n spinos urn location
Choles tero l gra nulo ma, PA or middl e ea r • Epid emio logy
o Very rare lesion makes erro rs in dia gn o sis co m mo n
(M E) o Co nge n ita l an eur ysm m ore co m mo n th an acqu ired
• C l: Smoot hly margina ted m ass in PA or ME wit h a neurysm
cortical b reakthrough • Associated abnormalit ies
o Co ngen ita l ane u rysm associ ated wit h mult iple
add itional in t racranial aneurysms
IDIAGNOSTIC CHEC KLIST
• Rare associa tion with neu rofib rom at osis &: Co nside r
co n nective tissue disord ers • Co nsid er ICAA-PA in cases with en h anc ing co m plex
Gross Pathologic & Surgica l Features ma ss n ear PA or in vo lvin g pet rou s ICA
• Focal o r d iffusely en larged pet ro us le A with • M R wit h MRA most effective way to demonstrate flow
2 int ralum inal clo t wit h in aneurysm I'< relat ion shi p to pet ro us ICA
Microscopic Features Image Inter pretatio n Pea rls

172
• Co ngen ital: Med ial aplasia & in tern al elastic lami na • Bo ne cr demo nst rates co rtical eros io n an d
degen erat ion invo lvement o f co n tiguo us spaces
• Acq uired : Co n nective tissue pseudo-wall formed • Be carefu l to evaluate for pr esence o f flow wit hi n an y
around area of full th ickn ess arteria l in jury PA mass
o Misdiagno sis can result in d evastating co nse q uences
• If un certain afte r ini tial imaging , ang iograp h y is
IC LIN ICA L ISSU ES d iagno stic and assists in pret reat men t plan n in g
Presentation
• Most co m mo n signs/sym pto ms ISELECTED REF ERENC ES
o Ipsilateral eNS dy sfunc t io n (sensorine u ral hea rin g I. Depa uw P et al: Endo vascular trea tm e nt of a gian t pct rous
loss) in ternal caro tid a rtery a ne ur ysm. Case repo rt and review of
o Ot her signs /sy m p to ms th e literature. Min im Invasive Ncu rosurg. 46(-1 ):250-3,
• Pulsa tile tinnit us 200J
2. Hwa ng Cl ct al: Bilat era l pctrou s in tern al carotid artery
• Headach es pscudoaneurysm s presen tin g wit h sen sor ineura l hea ring
• Othe r CN neuropat h ies (CN S-I J) loss. AJI"'R Am J Neurorediol . Z4(6): 1139·4 1, Z003
• Sudden o nset severe o to rrhagia o r epistaxis (blood J. Eliaso n JI. et <II: Skull bast.' resectio n with
d own eustach ian tu be) cervical- to -petro us carot id art ery bypass to facilitat e repair
• Sympto ms related to em bo lic st rokes of d istal intern al carotid ar tery lesio ns. Ca rdio vasc Surg.
• Clinical p rofile to:31 -7, 2002
o Clin ical presentat ion ran ges from in sid iou s cran ial 4. Vasama JP ct al: l'et ro us inter na l carotid artery
neu ropath y to life thre ate n ing e pista xis or pseudoaneurysm du e to gu nsho t in jury. An n Oto l Rhinol
o to rrhagia Laryngol. I to(5 PI I ): 49 1-3, 200 1
o Will not presen t with subarac h n oid h em orrh age as
s. Red ek o p G ct al: Treat men t of tr aumati c a ne u rysma l and
arte rioveno us fistu las o f th e skull ba se hy using
petrous ICA is ex trad u ral endo vascular stc nts. J Ncurosurg. 95 :4 1Z·9, ZOO I
o If large, o to sco py revea ls red pul sati n g 6. Forsh a w MA ct al : Rupture of an intern a l carotid artery
retro tym pa nic mass an eurys m in the pet rou s tem por a l bon e. Br J Neurosurg.
• Med ical h istor y 14(5): 4 79-82, 2000
o Majo r h ead t rau ma with fractur e of sku ll 7. Reece PH et a l: An a m'ur ysm of th e pct ro us in tern al carotid
base-tem poral bo ne artery. J I..a ryngo l Otol. 113(1): 55- 7, 1999
o Imm u n oco m prom ised ho st wit h lo ng h istor y of 8. Zander DR ct al: An eurysm of t he in trapct ro us in te rnal
caro tid art ery presen ting as isolated Ho rner's synd rome:
sku ll base- tem po ral bo ne in fectio n case report. Can Assoc Radi ol J. 49(1 ): 4 6 ~ 8 , 1998
De mo grap hics 9. Tanaka 1I ct a l: Pseudo ancurysm of t he pct rou s in tern al
caro tid artery after skull base in fection an d prevert ehral
• Age abscess d rainage. AJNR Am J Neuro radiol, 19(3 ): 50 Z·4,
o Co nge n ital fusiform ty pe p rese nt s in ch ildh ood o r 1998
ado lescen ce to . Coley SC et al: Gia nt ane ur ysm of t he petrou s in ternal
o Acqui red ICAA· PA presents at an y age ca rotid artery: d iagnosis a nd treatment. J Laryngol Oto l.
112 (2 ): t 9 6-8. 199 R
Natural Histor y & Prognosi s I I. Pa trlck jf Magn etic reso nan ce imaging of pct rou s carotid
• Grad ua l en largeme n t and progressive risk of life aneurysms. J Neuroimagtn g. 6(3): 177·9, 1996
threat en ing ru pt u re 12. 1.0\'(' MH et 3 1: Case repo rt: gia nt a neurys m o f th e
• Emho lic o r occlusive st roke possible if left alone lnt rapetro us caro tid artery presen ti ng as a cerebell o po nti ne
an gle ma ss. Clin Rildiol. 5 1(8): 58 7-8, 1996
• Excellent p rogn osis if t reated successfu lly
13. Cross 1)'1' 3rd et a l: Endo vasculn r t reat m ent o f epistaxis in a
Treatment pa tien t wit h tuberculosis and a giant pct ro us caro tid
psc u doa n eu rysm . A] NR Am J Ncuro radiol. 16(5): 1084-6,
• En dovasrular th erap y 1995
o Allows fo r pretreat men t ICA occlusio n t rial 14, Umezu II et al: Ane urysm arising from t he pc tro us po rtion
o Balloon trap ping o r aneu rysm al oblite ratio n wit h of the in ternal caro tid arte ry: case report . Radi at Med.
le A preservatio n 11(6): 25 1-5, t 993
o Endovascu lar ste n t placement 'Kross aneu rysm 15. Halbach V ct al: Ane ur ysm s of the pet rous po rtio n of th e
viable o p tio n intern a l carotid artery: Results o f treatm en t wit h
• Su rg ical t hera py n o longer p referred 1st approa ch e ndo vascula r or surgica l occlusio n . AJN R. 11:Z53· 7, 1990
o Wh en n ecessar y incl ud es ICA sac rifice wit h o r
wit hout ECA-ICA bypass
IIM AG E GALLERY
Typical
(1~JI) Axial lx)fJe CT ';!JO W5
de structiv e ma ss in righl
pctrou s apex . Note
1,1mioared calcified walls 0'-
2
Ihis aneurysm (arrow) and
ero sion o r th e antt'f ior wall 1 73
of the in (ern a/ .lud itor y cs net
(ope n arrow). (RighI) A'(jal
11 C+ AIR {('\'(,iJIs right
pcuoos apex 10\ .1Of:'Urysm
(arro w ) with irr pgu/.lf
lumina l enhann-'nlf.-'1U S,
"sw irting " flow void. Note
('n r m ar hnu'nl O f} an f(', ;or
m¥gin ifllt'fIlcll ,w dilory
canal (o p t ' /1 d f f U IV } .
(Lej t) A\ ;aI1 2WI AIR show:>

a complex signa l ma ss
involvin g th t:> left petrous
spex (,,,,O\v). Thrombus in
<1 /1etJr y:>m fl·ve.l/s bright
$ign.1/ (0 1'<'/1 arr ow) with
adja n·tlt .m -'J :> of flow vo id
(c ur vL'u ,mow ). (Rig" ' )
Oblique .ln t('(o pos te(ior ICA
angiogram ~ho\Vs an
;"<'8 (11.11 JOl'ur ysm of
pr oxim .}1 hO';7ontal petrous
intertM/ caro lid arlery.
Frond·like supe rior
projections (arro ws) aft'
particularly p rone 10 ruptur e.
Variant
- ----,,--,--.,-- -,,..- - --- (I .e!t ) A nt l"roposferio r ICA
,mgiogram shows a
pwuc1cxmeur ysm (.mow)
from lhf' petrou s portion of
,10 aberrant intem.l/ cs rona
drrer y. This .Jneur ysm
rosutted from .1 hiopsy d uring
a m yrinJ:ofom y. (Right)
Lateral radiography eliter
betloon trapping of
pseudoeneur ysm of middle
ear portion of ebensm ICA.
Sacrifice' of /CA was
completed. Proximal (arrow J
S dist.J/ balloons (open
arrow' .
PETROUS APEX METASTASIS
2
174
Al(ial fight ('at l-bo,.,(' CT sho" ruhtk' hUI Ck'oldy Alii.l1 T1 C+- ,\ IR tc...'m/~ .1n .1ft>,) 01 ,l~ymnlf'tric
rlpfin.lhlt·dt..,tftKt;\,(> k~ioo of lx " rous .1/)(" (af/uw ). TI t 'lJ!J.m( I'tJJ l'llt (.l m ,.,y) in tht· r~"l / )('f!f XI\ " /)( >,\
C+ AIR 1lf.'td"l:J kIf confirm, UKNl 0; Ihi\ findln~ .15 ' 11J(('X'illing J mel.bt.1f;c It~i()n. Nolicr- ,l<lj,ICl'fIl
/ ,,'t rou, " I JP,\ .hymlJlt"lry is commoll. l'll h..mcillg {a t ill tIlt' di'il1<; (OP(,(1 .lrfOw !o).
ITERM INO LO GY MR Findin gs

• T 1\VI: No ns pcctf!c. low-in termed ia te signa l
Definitions
• T2W I
• Hema togen o us OC direct spread fro m p ri mary o No n spcc tflc, ma y he h yper in te nse o r hyp oint cnse
neo plasm to pet rous a pe x (PAl • Depen d s upo n cellula rity of p rimary lesion
• T1 C+
o Asymm etric pa t ho logic e n ha nce me n t
IIM AGING FINDINGS o Virtua lly alwa ys en ha nces th ough in ten sity may
Ge nera l Fea tures var y
o Fa t-saturatio n essen tia l to ex clude fatt y marrow
• Best diagn ostic clu e: Bone cr shows foca l destructive
o w hen large, adj ace n t m eninges m ay thicken &.
lesio n o f ho ne at PA
e n ha nce
• Loca tio n : Pctro us apex
• Size: Variabl e, 2-8 centimete rs Angiographi c Findings
• Mor ph ol ogy: In filt rating margins • May a pp reciat e blush or pa t hol ogic vessels (renal,
thy roid )
CT Findin gs
• NEeI' Nucl ear Medicin e Findin gs
o Lytic o r permeative. rarely blasti c lesion • Bone Scan
• Dissoluti on of normal archi tecture o Asy m metri c increased uptake o f radl onuclide
• O vteobla stic metasta ses less co m mon ; ma y mimi c o Abno rma l hut no nspecific
benign Hb ro-ossco us lesio n • PET
o PA bone cortex is dcst roved o Positive a t th is loca tion a nd usuall y elsewhere
o 1'/\ mar row space o r ai r ~ell trabec ulae in vaded wit h o Posit ive if lesio n large eno ug h (> I o n )
focal destructi o n seen
o May he subtle as pne u matiza tion is hi ghl y variable Imaging Re commendati ons
in P,\ c r &. en hanced MR required
• Ho th
• CECf: En hances sign ifica n tly in most cases
DD x: Destructive Petrous Ape x l e sions
Ap;call )l'trositis Cholesterol Granuloma LClI, I~ I'IJsmJcytoma, PA

PETROUS APEX METASTASIS
Key Facts
Imaging Find ings • Plasm acyt oma, PA
• nest diagno stic clu e: Bone c r sho ws focal destructive Path ology
lesion o f bone at PA • Etiology: Marr ow-filled PA may predi spose to
• Both CT & en ha nced MR required metastases
Top Differential Diagnoses Diagnosti c Checklist 2
• Apical pet rositis, co nfluent • In fection (co n flue n t ap ical petrositi s) in ap propr iate
• Cholesterol gran ulo ma, PA 175
clinica l sit ua tio n
• Langerh ans cell h istio cyt o sis (LCH), PA
• Fat-satu ratio n lessen s co nfus ion from region al fatty • Gen de r: M < F (bre ast)
marrow
• Poor, sa me as fo r pr im ar y tum or
IDIFFERENTIAL DIAGNOSIS Treatment
Apical pet rositis, co nflue nt • Palliat ive, same as that for und erlyin g co nd ition
• Clinical: In fect ious sympto ms
• Imaging: Destructiv e lesion o f PA + m eningeal
th icken ing [DIAGNOSTIC CHECKLIST
Cho leste ro l gran ulo ma, PA Consider
• Clin ical: Histor y of ch ro nic otitis media • In fection (co n flue n t ap ical petro sitis) in a ppropriate
• Ima gin g: Exp ans ilc PA lesion; '1'1 h igh .sign al clinical sit ua tio n
Langerhans ce ll histiocytosis (LCH), PA Image Interpretati on Pearls

• Clin ical: Ped iat ric patient • Remember background PA asy m me t ric m arr ow
• Ima ging: Destru ct ive PA lesio n commo n whe n reviewin g a rea for m etastases
Plasmacytom a, PA
• Clin ical: 50C ~h wit h multip le m yelom a ISELECTED REFERENCES
• Im agin g: Isolated destructive PA or with ot he r m ar row l. Gloria-Cruz TI et al: Metastases to tempo ral bones fro m
space lesions primary no nsystemic malignan t neoplasms. Arch
Otolaryngol Head Neck Surg . 126:209-14, 2000
2. Moore KR, el al: "Leave me alone" lesions of the pc tr ou s
IPATHOLOGY 3.
apex . M NR. 19:733-H, 19lJH
Muckle RPct al: Pctrous apex lesions. Alii J 0 101.
General Features 19:219·25,1 998
• Ge ne ra l path co m me n ts: Direct ex te n sion from
nasopharyn gea l ca rcino m a must be exclud ed
• Etio logy: Mar row-filled PA may predispose to IIM AG E GAllERY
met astases
• Epide miology : Brea st m align an cy is m ost co m mo n
Gross Path ologic & Surgical Features I -\-. (.~:
• Surge ry rare ly performed
• Makes di agn osis of speci fic tumor typ e
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Stag ing, Grading o r Classification Crite ria
• Per o nco logis t (dep ends on primary lesion)
ICLI N ICA L ISSU ES (1.£11) Axial right es r T·bone CT clemofl w ates sub tle, di{(uw! blsstic
Presentati on metsststic dlses se second,Jfy to prostate carcinoma with involvement
of petrous apex (arrows). (RighI) Axial right ear T-bone CT reveals
• Most co m mon signs/sy m pto ms : 6t h n erve pa lsy
lytic, destructive metastasis o f right petrous ape x (arrows) ill patient
• Clin ical profile: Oft en h istory of tumor (not always!) with known ovarian carcinoma. Loss o f cortical margins is key
Demographics observation.
• Age: Olde r pati ents
FACIAL NERVE ENHANCEMENT, INTRATEMPORAL
2
176
A\ia/ 11 c+ MR .d JOWS normal ('f} hdnC(.'mt:>tl t of Axial T1 C+ MR at J T revl' il /s eve n mo re consp icuous
inlrc1tf'mpora/ (acial nerve. On left, gmictJlalc ganglion is geniculate ganglion m hanc('f1lf'nt (arrow) as a normdl
(arrow) . On right. tympanic segment CN 7 visihle
S<'l"fl finding. Witl1 in Cfeasin~ Held strength. normal
as enhancing line (ope n arrow). enhancement of eN7 eVt'1J more visible.
ITERM INO LO GY Ima gin g Recommendations

• No rmal en ha nc eme n t seen best o n thiu-sect lon axial
D efiniti on s & corona l T1 C+ MR o f T-ho ne
• Nor ma l en hancemen t on Tl C+ MR along co u rse o f • Be certa in to acq uire pre-contrast images, to ensure no
intrat empo ral facial nerv e (FN) wit ho u t abno rm al false-posi tive en ha nce ment along FN co u rse
bon y cha nges • CT used if co n tin ued doubt abo ut FN normalcy
IIM AG ING FINDINGS ID IFFERENTIAL DIAGNOSIS

General Features Bell pal sy
• Best di agn osti c clu e: Tl c+ MR en h anceme n t alo ng FN • Clin ical: Acute o nset unila teral per ipheral f N paralysis
ge n icu late ga ng lio n & anteri or tympan ic segmen t o n ly • T1 C+ MR: Int en se en ha nce men t of int ratem poral FN
without bon y FN cana l cha nges o "Tuft" o f en ha nc ement within lAC fundus highly
• Locati on: Ge nic ulate gang lio n & tympanic segmen t sugge stive
en h anceme n t m ost co m mo n • Bone CT: FN bon y ca na l normal
CT Findings Facial ner ve (FN) schwa nnoma
• NECT: Nor ma l bo ny in tra tempor a l FN cana l • Clinical: Hearing loss ± g rad ua l o nset peripheral FN
MR Findin gs paresis
• Tl W I: No rma l FN will prod uce increased signa l o n Tl • Most freq uently fo und in ge nic ulate fossa
C- co m pa red to su rro u nd ing osseous struct u res • Tl C+ MR: Abnorma l focal en hanci ng mass along FN
co u rse
• T1 C+
o En ha nce me n t is no rma l along portions of FN, most • Bone CT: Focal enla rgeme n t of in trat ernpo ral FN
comm only at gen iculate ga ng lio n & tym panic Facial nerve (FN) hem angioma
segme n t . • Clin ical: 1~1r l y, unil at eral FN paralysis
o Enha nceme n t usually asym me tric • Mos t frequ ently locati on = ge nic ulate fossa
DDx: Intratemporal Facial Nerve Enhancing Lesions
Bell Palsy FN Schwannoma FN t temengic ma PN 7; l -Bone CN7

FACIAL NERVE ENHANCEMENT, INTRATEMPORAL
Key Facts
Terminology • NECr: Nor mal bon y intratemporal FN ca na l
• Normal en hanceme n t o n Tl C+ MR along cou rse o f • Enha nceme nt is normal along portions of FN, most
intrat emporal facial nerve (FN) without abn ormal co m mo nly at geniculate ganglio n & tym panic
bony cha nges segme nt
Imaging Findings Top Differ ential Diagnoses

• Bell palsy
2
• Best diagn ostic clu e: T1 C+ MR en hance me n t along
FN geniculate gan glion & anterior tympanic segme n t • Facial nerve (FN) schwan noma 177
only without bony FN cana l changes • Facial nerve (FN) hemangioma
• Perineu ral tumor (PNT), intratemporal CN?
• T1 C+ MR: Focal en hancing mass in en larged

geni cu late fossa Natu ral Histor y & Prognosis
• Bone Cf: "Ho neyco mb" bo ny changes - 5(1)(1; irregul ar • No rma l f N en hancemen t will not change over time
margin s co m mo n • Nor ma l Tl C+ MR finding
Perineu ral tu mo r (PNTj, intratempo ral CN7 Treatm ent

• Clinical: Parotid ma ss + facial nerve para lysis • No ne; do not mistake for Bell palsy
• Tl C+ MR: Abnormal focal en hanceme n t sp read ing • Bon e cr used whe n asym met ric en ha nce me n t is
from inva sive parotid ma ss int o mastoid FN segme n t marked to rule ou t underlyin g bon y cha nges
• Cf: Enlarged bony masto id segme n t o f FN ca na l
• Fat in stylo masto id foram en repla ced by tissue
ID IAG N O ST IC CHECKLIST
I PATHOLOGY Co nside r
• Enha nceme nt alo ng ciste rna l, lahyrin t h ine segmen t o r
Ge ne ral Features extracra nia l ma sto id FN segme n ts is NOT norma l
• Gene ral path co mmen ts • High er field st rength (31') makes normal en hancemen t
o Lush CIRCUMNEURA L ARTERIOVENOUS PLEXUS more co nspicuous
surrou nd s FN
o I.abyrin th ine segme n t is least well vascularized
o Emb ryo logy-anato my ISELECTED REFERENCES
• FN plexus ha s 3 co mpo nen ts I. Tahuchi T ct al: Vascu lar pe rmea bili ty to fluo rescen t
• Epidemiology substa nce in human crani al nerves. Ann O to l Rhi n o!
o 7S-981)() o f cases have enhancem ent along at least Laryngol. 111(8):736-7, 200 2
o ne segmen t o f int ratemporal FN 2. Martin -Duvern eull N et a\: Co ntras t en ha nce me nt of t he
o En ha nceme n t seen along FN segmen ts facial nerve o n MRI: No rma l or pa th ological ?
Neuro radiology, 39(3):207 -12, 199 7
• Gen iculate ganglio n: 97% .J. Gcba rskt SS et al: En ha ncem en t along th e nor mal facial
• Tym panic segm en t: 88% nerve in th e facial ca nal: MR imag ing and a na to m ic
• Masto id segme n t: 6 7')(, cor rela tio n. Rad iology. 183 (2):39 1-4, 1992
Gross Path ologic & Surgica l Featu res
• Arterioven ou s plexus consists of co mb ina tio n of
relatively large arteries &. vei ns in capillary plexu s IIMAGE GALLERY
• Den se FN circu m neural arterio veno us plexus is
predominantl y located in geniculate gang lio n,
tympanic &. masto id segmen ts ± proximal grea ter
supe rficia l pet rosal nerve
IC LIN ICA L ISSUES

Presenta tio n
• Most co mmo n signs/sympto ms: Patient is
asymptomat ic by definition
• Clin ical profile: FN norma l en hanceme n t see n (l~fl) Axial T1 C+ M R at J T shows p romi nent normal enhan cemen t
incidenta lly d u ring an T1 C+ MR imaging work-up fo r of geniculate ganglion (arrow) as well as the anterior tymp anic
segment (open <1rrow ) of inr ratempora l eN7. (Rig ht) Coronal T1 C+
unre late d clin ical fin din gs
MR at J T demo nstrates conspicuo us enhancement of the genicu late
ganglion f arrow). Compere degree of enhanceme nt to noneobsncing
coc hlea (op en arro w ) seen inferiorly.
PROLAPSING FACIAL NERVE, MIDDLE EAR
2
178
Coron..,1 T-/>OrJt> a
of fight ('.If !JJOW" '.oir t;SWt' "m.m- CoronJI T-hf:>tw a oj righ t (W S/ KJ\\!S normal fymfJ<lf1ic
in ava.1 window niclx' (.l rfOl,Y) ,)Iong Und<'f5Urf.K to of ~mefll of (.loaf nerve in Cflli S-Sf.>Cfio n (arrow) .1/0ng
llft.,,}1SCfnkircular (".In.)! in IocJtion of norm..l l l )'tJlP.IIlK· undersurf,xf;' of IJf(.,al 5f.midrwl.1r r.lfl.l/. Note suhll('
,:,ri.11 fl('fV(' foegment. !X)()(' cD\'('fjng.
o Axial bo ne C l' may show "h am mock-like" facial

ITE RM INO LO GY nerve span n ing m ldd le ear cavity u nder LSC
Abbreviations and Synon yms o Co nco mitan t a nomalies of sta pes may be present
• Protruding ln tra te rnpo ra l facial ne rve o Sim ple dehi scen ce can not he det ect ed un less nerve
is pro la psing th rough dehi scen ce
Defin itions
• Fari al nerve O-'N) protrud es th rou gh bony de h isce nce
MR Fi nd ings
as it courses alo ng un d ersu rface o f lateral sem icircu lar • No a bno rmality iden tified
ca na l (I.\ C) • T I C+ is normal exclud ing Iaclal nerve schwa n no ma
Imaging Recommendati on s
• Axial & co rona l th in-secti on bo ne CT images
IIM AGING FINDINGS o Prot ruding ner ve best see n o n coro na l images at
Gen eral Features level o f oval window
o Co n t rast not necessar y o r reco mmended
• Best diagn ostic clu e: Tubu lar so ft tissue ma ss
• T l C+ MR will di fferentiate fro m facia l nerve
pro lapsing from no rmal locati on o f tym pan ic segm ent
schwa nno ma
o f facial nerve
• Locatio n : \Vi th in oval w i ndow nich e alo ng
un dersu rface o f lateral semicircu lar ca na l (LSC)
• Size: Variable, may be su bt le o r a ppea r mas s-like (2-3
ID IFFE RENTIAL DIAGNOSIS
m m ) aro und oval window Oval wind ow atres ia
• • Morphology: Smoo th, tu bul ar appearance • \ Vilh o r without EAC atresia
CT Findings • Facial nerve tympanic segment "ecto pic"
• fo u nd medi al & inferi o r
• NEe I'
o Soft tissue "mass" in o val wid ow ni ch e Co ngenita l cho les teato ma of middl e ea r
• Along u n dersu rface of LSC • Squ am ou s foci may he anywhere in midd le ear
• Best seen on coro nal images • Rarely in area of facia l nerve tym pa n ic segment
• Co n tiguo us with mid-ty m pan ic segme n t o f CN7
DDx : Prolapsing Facial Nerve Mimics
Ova l Window A tresia CongoCholesteatom a FacialSchwa nnoma Glom us Tympanicum

PROLAPSING FACIAL NERVE, MIDDLE EAR
Key Facts
Imaging Findings • Prolap sin g FN = nerv e prot ruding thro ugh de hi scence
• Protrud ing n erve best seen 0 11 co ro na l images at level • Sim ple dehi scence wit ho ut p rotrusion occurs - 50%
o f oval windo w • Prolap sin g facia l n erve is relatively fa re (- liM)
• 1'1 C+ MR will differentiate from facial n erve Clinical Issu es
sch wan n oma • Usually is in ciden tal find ing
• Critical to co m municate its presen ce to surgeon prior
2
Path olo gy
• Facial nerve d ehi scen ce: Refers only to th e segmen tal to m idd le ear explo ration 179
absence of bon y covering of 7th nerve • Easy to in jure facia l n erve d u ring sta pe decto my if f N
pro lap se is present!
Facial nerve sc hwan noma Natural History & Progn osis

• '1' 1 C+ ?\ IR shows en h a nc ing tubu la r mass • Excellen t if left alone
• Most co m mo n locatio n = genic u late gang lion, but • May cause co nd uctive hear ing deficit
m ay (KCUr i n tympan ic segme n t CN ?
• Reme mber: Man y facial nerve schwan no mas will no t Treatm en t
he associat ed wit h facial palsy • Carefu l avo id ance at time o f m iddle car surgery
Glom us tym pa nicum paragan glioma

• Usua lly arise a long coc h lear promo n to ry IDIAGNOSTIC CHECKLI ST
• Or igin in ova l win do w nich e ran.'
• Vascu lar rctroty m pa n tc mass at o to scopy Image Interpretat ion Pearls
• Prolapse of ten associ ated with ab sen ce of n otch de fect
along undersurface of LSC
IPATHOLOGY • If notch is see n, co ns ider alte rna tive expla natio n
Genera l Features
• Genera l pat h com ments ISElECTED REFERENCES
o Facial n erve deh iscence : Refers o n ly to th e 1. Blaser B et al: Stapes sur gery in a n omalies of t h e co u rse o f
segmen ta l abse nce o f bon y coveri n g o f 7th nerve th e facial ne rve. Schweiz Mcd wochc nschr. 116:9 7S·1005,
• Most co mmo n at level of tympani c segmen t 2000
o Prolapsing FN =ner ve p rotrud ing t hroug h 2. Ballester M eta l: Sta pcdo torn y and anatomical variatio ns
deh iscen ce of t he fac ial ne rve . I{ l'V Lary ng o t Ot o l Rhiuol . 12 1:181 -6.
• Etio logy: Co ngen ital/deve lo pme n tal 2lKlO
Swnrtz j n: Th e facial nerve cana l: CT ana lysis of t he
• Epid em io logy
pro trud ing tym panic segment. Radiology. IS3:4·U -7. 1984
o Simp le dehi scen ce wit hout p rotr usion occu rs - SOlMl
o Prolapsing facial ner ve is relatively rare (- 1%)
Gross Path ologic & Surgical Features IIM AG E GALLERY
• Id entified in ova l win dow n iche at tim e o f midd le ea r
explora tio n
ICLIN ICA L ISSUES

Prese ntatio n
• Most co mmo n signs/sym pto ms
o Asym pto ma tic
o Rarel y, co nduc tive deficit d ue to lm pln gemcnt up o n
sta pes
o If chro nic o titis media present , facial palsy mo re
like ly
• Clin ical pro file (I .l11) Axial bone CT of right ear rovenls "h.lm mex ·k" t }'mp .l nic
o Usua lly is in cid en ta l fin ding wgl1Jf:'nt of fadal ne rve SIfting J UOS5 micldlt' est cavuy (" rrow).
• C ritical to co mm un icate its presen ce to su rgeo n Mielelh." ear ma ~ 5 m .ly .It first be -" U_~Pl'ctl'cI in this f}'fX' of pro/.Jp sing
f.leia! ne rve . (RighI) Coronal right ear t -bone CI demOllslratC's focal
p rio r to midd le car explora tio n
ovoid "mass" i USI 5up eromecli.l / iO oval w indow (arrow ). l esioll is
• Easy to in jure facia l n erve d u ring stapedec to my if
p ro/" p s('c/ f"d,,1 nerve, n OI J m icldlt' ( ' , If m <I.~ S .
FN prolapse is present!
Demographi cs
• Age: All ages, co ngen ital lesion
BELL PALSY
2
180
Axial T1 C+ MR shows Bell p..)/sy in lett ear as Axial t t C+ M R reveels classic Bell. Note enIJann:'mt'tJl
compicuous enhancement of the g<>nicu/.lle ganglion 01" intra/wJ fXJral e N 7 inclu ding .:llltt:'fKK tympa nic
(.lfro w). labyrinthine- segment (open "rrow ) end the (arrow), b'f' oiwlate ganglion (open arrow ) &
fundal portion orCNl (curved arrow). labyrinthine 5f:'gmenfj (curvt.¥:! arrow) .
• In trat em po ral eN? bon y ca na l wit hout focal or

ITERM INO LO GY d iffuse en largem ent
Abbreviatio ns a nd Synonyms • If en largemen t presen t, not Bell pa lsy
• Abbreviation : Bell palsy (BsP) • CECT: No role for con trast-enha nce d c r in BsP
• Synonym : Herpetic facial paralysis MR Findi ngs
Definitio ns • Tl WI: lntratem poral facial nerve may be more
• BsP (origina l defini tion): Idiopat hic acu te onset lower pro min ent on side affected by BsP
motor neu ron facia l paralysis • T2 WI: Brain no rmal wit hout evide nce fo r high signal
• EsP (modern defin ition ): Herpetic facial para lysis lesions
seconda ry to herpes simplex virus • T1 C+
o T l C+ sh ows u nifor m , con tiguo us en hance ment of
facial nerv e
j lM AGING FINDING S • CN? is norm al in size
• CN? conspicuo us h igh signa l appears slightly
Gen eral Featu res en larged
• Best diagnost ic clue: Funda l ' tuft' & labyrinthine • En hanceme n t patte rn is linear, no t nodular
segment of facial nerve (eN?) in ten se asymmetr ic o Enhancem en t is usuall y presen t fro m di stal int ernal
en hanc eme nt on Tl c+ MR auditory artery (lAC) th rough labyrint hine segment,
• Locati on geni culate ganglion &. a n te rior tym pan ic segment
o Fun dal & labyrin thine segmen t CN? most • TUFT of en hance me nt in fundu s of lAC
com mo nly affected (prerneata l segm ent) along wit h en ha nce men t of
o May invo lve enti re intratem poral CN? labyrin thine segmen t of CN? are disti nctive MR
• Size: CN? not sign ificantly enlarged findings in IlsP
• lJistal in tratempor al facia l nerve en ha nces less
CT Findin gs frequ entl y
• NEcr o Holot ympani c CN? enha nce men t may be seen
o Bone cr normal in BsP
DD x: Intratem pora l En ha nci ng Facia l Nerve Lesions
NL Enhancement CNl FN Schwannoma FN Hemangioma PNT CN l

BELL PALSY
Key Facts
Termin ol ogy Top Di fferen tial Di agn oses
• Syn onym: Herpeti c facial para lysis • Normal (NL) enhancement of intratemporal facial
• BsP (mo de rn definiti on): Herpetic facial paralysis nerv e (CN?)
seco n da ry to h erpes sim plex virus • Facial nerv e (FN) sch wa n noma
Im aging Findings
• Facial nerv e (FN) hemangioma
• Perineural tumor (PNT) fro m pa rotid 2
• Best diagnost ic clu e: Fu nda l "t uft " & labyrinthine
segme n t o f facial nerve (CN?) intense asy m me t ric Pathol ogy 18 1
enhancement o n T1 C+ MR • Path ophysiology: Formati on o f intraneural ede ma in
• eN? is normal in size neuronal n erv e sheat hs caused by breakdown o f
• CN? conspicuou s high signal appears sligh tly blood-nerve barrier & by venous co ngestio n in
enlarged ep ine ura l & perin eura l ven ous plexus
• Enhancem ent pattern is lin ear, not nodular
• ATYPICAL Bell pals y requires sea rch for underl yin g Clinical Issues
lesio n • Most co mmo n signs/sym pto ms: Acute o nset
peripheral CN? paralysis (24-48 hour o nse t)
Imaging Recomm endati on s Fa cial nerve (FN) hemangi oma

• Classic rapid o nset RsP req u ires no ima gi ng in in itial • Clin ical: CN? pa ralysis occu rs whe n lesio n is still sma ll
stages • c r imagin g: Bone cr may show in tratu mora l bone
o 90f Ml reco ve r e N? fu ncti on spo n ta n eo usly < 2 spicules
mont hs • MR imagin g: Poo rly-circu mscribed , en ha n cing mass
o If deco m p ressive su rgery is a n ticipated , MR imagin g o n T l C+ most co m mo n ly fou nd in genic u late fossa
is warran ted to e nsu re t ha t no o th er lesio n is
causing facia l ner ve para lysis Perineural tum or (PNT) fro m parotid
• ATY PICAL Bell palsy req u ires sea rch for u nde rlying • Clin ical: Paro tid malign an cy us ually palpab le
lesio n • Imagin g: Invasive parotid mass is presen t
o Aty pical Bell pa lsy d efinitio n o Sty lomasto id fo ram en is tissue filled
• Slowly progressive CN? palsy o Facial n erv e is en larged fro m d ista l to proximal wit h
• Facial h ypcrfuncti on (spasm) precedi ng BsP mastoid air cell in vasion associ ated
• Recurrent eN? pa lsies
• Un us ual d egrees o f ea r pain
• RsP with a ny o th er associ ated cra n ial neu ropath ies I PATHO LOGY
• Peri pheral facial nerve pa ra lysis per sisti ng o r General Featu res
dee pe ni ng > 2 mon th s
• Th in-secti on (2-3 mill) T1 C+ MR Images th rough lAC • Ge ne ral path co m me n ts
&. temporal bo ne is imaging exa m ina tio n o f choice o ln tratempo ral CN? normal a na to my
• T..bo ne C l' has ro le in fur th er defi n ing lesion s fou nd • CN? norma lly e n ha nces at its ant er ior &. po sterior
o n e n ha nced MR imagi ng &. sea rch ing for subtle ge n us
in tra tempo ral hem an gio ma o f eN? • Th is e n h a nce me n t from rob ust circu m ne u ral
a rte riove nous plex us in th ese locat ions
• Radi ol ogist mu st be fam iliar wit h n ormal CN? T1
ID IFFERENTIA L DI AG N O SIS C+ MR en ha nceme nt
• Famil iarit y -with no rmal patt ern s of in tr atem poral
Norma l (N L) enhanceme nt of intratem p oral CN? enha ncemen t allows rad io logist to recogni ze
abnormal en ha nceme n t see n wit h BsP
facial nerve (CN?)
• Etiology
• Clin ical: No facial nerve para lysis o Etio logy-pat hogenes is (curren t h yp o the sis)
• 1'1 C+ MH: Mild, linear, d isco ntinuou s en ha nceme n t • Laten t he rpes simplex in fecti on o f geniculat e
of a n ter ior N poster ior genus o f intrat empo ral CN? gang lio n with reactivatio n &. sp read of
o Prem eatal & la byrin t h in e segme n ts o f CN? infl ammato ry process along p roxim al &. distal
u n in volved facial n erve fibers
Facial nerve (FN) schwa nno ma • Pathoph ysiology: Fo rma tio n o f intran eu ral ede ma
• Clin ical: Hearing loss mo re co m mo n t h an facial nerv e in neu ron al n erve she aths ca used by breakdown o f
palsy blood -nerv e barri er &. by ven ous co n gestio n in
• 1'1 c+ ~1 R : Well-circumscribed , tub u lar, en ha nc ing e pineu ral &. perineural veno us plexu s
ma ss wit h in e n la rged eN? ca n al most co m mo n ly • Epidemio logy: Herpeti c facial paralysis is respo nsible
cen te red o n ge nic u late ga nglio n for > S(Nft o f cases of pe ripheral facial n erve pa ralysis
BELL PALSY
Gross Path ologic & Surgica l Feat ures Image Interpretation Pea rls
• CN 7 ed ema peaks at 3 weeks foll ow in g o nset o f • 'Tuft" o f en ha nc eme nt in fu ndus o f lAC assoc iated
sym p to ms wit h en h anc ed labyri n th ine segmen t CN7 wit hout
enla rgement is highly suggestive o f Bell pa lsy
Microsco pic Feat ures
• Herp es sim plex DNA recov ered fro m BsP facia l nerve
2 specimens ISElECTED REFER ENCES
, Stag ing, Grading o r Class ifica tion Criteria I. Kress B et a l: Bell pal sy: q ua ntitat ive ana lysis of MR
I 18 2 • Ho use-ll rackrnann (HB) facial ner ve gradi ng syste m im agin g da ta as a met hod o f pred ict ing o ut com e.
.
'\ ,
' 2.
Radiology. 230(2) :504-9, 2004
Bccc lll R ei ill: Diagn os is o f Bell pals y wit h gado linium
..
., ,
IC LIN ICA l lSSUES m agn e tic resona nce imagin g. J Cra n lofac Surg. 14(1):51--1,
20o:!
3. Un lu Z ct al: Sero logic exa m ina tio ns of hcp atltts,
Presentati on cy to megalovirus, <ami rubella in patien ts wit h Ik'lI's palsy.
• Mo st co m mo n sign s/sym pt o ms: Acut e on set Am J I'hys Med Reh ahll . 82(1 ):28·32, 2003
peripheral CN7 paral ysis (24·48 h ou r onset) 4. Kress BP ct al : Bell's pa lsy: what is th e prognostic value of
• Clin ica l p rofile: Ot he rwise healt h y ad u lt wit h acute me asureme n ts o f sig nal intensi ty Increa ses ,..'lt h contrast
o nse t o f unilat eral pe rip heral CN7 paralysis en ha nc ement o n ~( RI ? Nc u ro radiology. 44(5 ):428-33. 2002
• Ot her signs/sym p to ms 5. Groga n PM et a l: Practice pa ra m eter: Steroi d.., acyclovir,
a nd surge ry for Hell 's palsy (an evidence- based review):
o Freq ue n tly a viral prod rom e is re po rted 7 days
Repo rt o f the Quality Standa rd, Subcomm ln ec of the
befor e BsP o nset Ame rica n Academ y o f . e uro logy. Neurology. 56:83()"6,
o 70% ha ve alte ratio ns in tas te d ays before facial 200 1
paralysis 6. Suz uki F et .11: Ilcrpe s vir us reacti vatio n and
o 50 l }'h h ave pain in o r aro un d ipsi lat eral ca r (n o t gadolin ium-e n ha nced ma gnetic resona nce imaging in
severe pain) pat ien ts wit h facial palsy. 0 101 Neu rotol . 22( 4):549-53,
o 20% ha ve nu mb ness in ipsila teral face 21m
7. j ackso n CG ct al: The facial n erve . Curren t trends in
Demograp hics d iag nosis. t reatm en t, a nd rc hahll itatlon . Med Clin North
• Age: All ages affected Am . 83(1 ): t 79-95, x, t 999
Rooh G et a l: Pe riph eral facial palsy: etio logy, d iagnosis
Natu ral Histo ry & Pro gn osis a nd tr eatm ent. Eur Neurol . 4 1(1):3-9, 1999
• > 90% o f patien ts spon taneous ly recover all o r part o f 9. Fu kazawa T ct .11: Facial palsy in m ultiple sclorosts. ]
'c urol. 2H ( 111):63 1-3, 1997
facial nerve fun cti on wit h ou t th erapy in first 2 months
10. Engstro m M vt al: Seria l gadolini um ....n ha nced magne tic
Treatm en t reso nance imaging a nd assess me n t of facia l nerve function
in Bell's palsy. Otola ryngol Head Neck Surg . 117tS):559·66,
• Med ical th erapy
,••,
1997
o Stero ids ± acycl ovir 11. Saatci I ct .11: MRI of the facial nerve in idiopathi c facial
• • Surgi ca l t h era py palsy. Eur Radiol. 6(5 ):63 1·6 , 1996
o Profo und d e nerva t io n (> 95% ) t reated wit h facial 12. Ca ta lano pJ er .11: Cra nia l neuro pathy seco ndary to
nerve d ecomp ression from lAC fundus to perin e ural spread of cu taneous ma lig nancies. Am J Otol.
stylo m asto id fo ramen 16(6):772-7, 1995
o Deco mpressio n m ust be performed wit h in 2 weeks 13. Jo nsso n Let al: Gd-DVfA en ha nced MRI in Bell's palsy and
h erpes zos te r o ticus: an ov erview a nd im plica tio ns for
o f o nset o f total paralysis for it to be maximally
future st ud ies. Acta Otolaryngol. 115(5):5 77-8-1, 1995
effective 14. Girard N ct OI l: 3 D-FT MRI of t he facia l n erve.
• In ten sit y, pattern ± locati o n of en h ance men t seen o n N cu roradi ology. 36(6):462 -8, 1994
T I C+ MR n ot helpfu l in pred ictin g o utco me for an IS. AdmIT KK: Medi cal ma na geme nt o f idio path ic (Hell's) palsy,
in d ivid ual patient Oto lary ngo l C lin No rt h Am. 24(3):663-7:'i. 199 1
• Olde r patients have lo wer l){) o f co m plete reco ver y o f 16. Mu rp hy TP: MRI o f t he facial nerve d u rin g pa ralysis.
CN7 fu n cti o n Otolaryngol Head N<'Ck Surg. 104(1):47-5 1, 199 1
17. Schwabcr MK e t al: Gad olin iu m-e n hanced magn etic
resonan ce ima ging in nell's palsy. La ry ngoscope.
IOO(t 2):t 264-9, 1990
ID IAGN O STI C CHECK LIST 18. Tl en R et .11: Co n t ras t-enha nce d MR imaging of th e facial
nerve in II patie n ts with Bell 's pal sy. AJ"'R Am J
Consider Neurorad iol. 11(4):7]5-01 1, 1990
• MR im agin g reser ved fo r ' at ypical Bell pa lsy' 19. Da niels DI. et a l: ~t R im aging of facial nerve e nha ncement
presentations o n ly in Be ll palsy o r afte r temporal bo ne surge ry, Rad iology.
• Abn o rma l facia l n er ve en ha n ceme n t may pe rsist well 17 1:807-9, t989
beyond clin ica l im p rovement o r full reco very 20. Ma tsu mot o Y et al: Facial nerve biopsy fo r etiologic
• No t all in t ratemporal facial ne rves en ha nce in BsP cla rificati on of Bell's pa lsy. An n Otol Rh inol La ryn gol
o Less th an 10 da ys foll owing o nse t of Bsl', CN 7 o ften Suppl. 137:22·7, 1988
n o rm al
BELL PALSY
IIM AG E GALLERY
(I ,eft ) Axial TI C+ /'viR sholYs

BsP unilateral intense
enhancement o f e N?
tympan ic segm cn t (arrow) &
geniculate ganglion. N ot e
2
"w it" of fundal eN? l En
(>/lhanceow ot (open arrow).
Normal op(X}site eN?
(curved arrow ), (Rig''' ) A~ia/
T1 C+ MR rev eals Be/l palsy
as uoilalera/t ympanic
segmen t o f inlracL'm p oral
eN? cll/JdflCemefl t (arr o w).
NOlin' only minimal
enhann'nwfll of CO fl / f Ol/d l pf.)1
lympanic .\ eg ml'fl t o f e N ?
(open arrow) .
(I.eft) Sagitral obliqut:' T1 C+

MR t!JmugIJ inlra cpmp o fal
fada l n erv e in Us!' patien t
51JO\V5 -!lolol ympanic ,r eN?
Pf}/JJIl CCnJenl irom anterior
g<>nicu/a/C' g.lngJion (arrow)
/0 stylomas/oid (oram('/l
(op en ilffm v). (R ig hI )
Coronal T1 C-+ MR with fat
q lmcltion demotwre tes
(>nh.JnCl'IJIL'nl o f entire
T1IJ5!oid seg men t o f facial
ner ve from posterior genu
(arro w) to ~ t ylomJs toid
tomnwn (0l'('n arrow) in 8(·11
flcll...y patient,
Variant
(l A.'jt) Aliial T1 C+ M R sbows
variant example Befl palsy
imaging finding.~ ,H fifl{\ )(
en han cement of entire
internal auditory canal
compoilcill of tilcial nerve
(arr o l\/s) alonf: with
gt'nicu/atl' ganglion ( 01' (' 1}
iI/fOW) . (Righi ) COfOll dl T1
C+ AtR with f,lt -S,il Ur<ll ioll
shows a patienl with I('(t !J('II
p alsy w here the ('xUacranial
p ro ximal fac ial nerve is . .con
to enhance (arrow), , his is
an unusual imaging find ing
in I3sP.
FACIAL NERVE SCHWAN NOMA, T-BONE
2
184
Axial graphk shO\vs a tubular (aci.l/lll 'fvt:> sd rw<1r1!lof17a Axial lefr ear T·lxme CT rew J/s a bcial nerve
involving the labytinrhint.· (arrow ), &'t'nim/ale gaflg!ic)f) K hw.1nnoma t>l1ldrging laJwrinthine (arrow), genicu/dte
(open arrow) and anterior tympanic (curved arrow) {ossa (open clrrows) & anterior tympanic (curved Jrrow)
~t'8ments of
tlw f.lcial oeve. sq:mcnt of fel dal nerve canal.
• Geniculate fossa: Ovoid

ITE RM INO LO GY • Greater superficial petrosal nerve: Ovoid, projects
Abb rev iatio ns and Syno nyms in to m idd le cra n ial fossa
• Facia l nerve sch wa n no ma (FNS) • Tympanic eN ? segme n t: Lobular
• Facial neu ro ma; facial neuril em mo ma • Masto id e N ? segme n t: Irregula r marg in if breaks
into su rrou nd ing air cells
Definitions • When spans mo re th an one int ratem por al e N?,
• FNS: Rare be nign tu mor o f Schwann cells tha t invest t ubu lar shape
peripheral facia l nerve (FN)
CT Findings
• NECT
IIM AGING FINDINGS o General T-bone Cl' ap pearanc es
• Tubular en largemen t of int ratem po ral eN?
Gene ral Features segme n t(s)
• Best di agnostic clu e • Bon y margin s are smooth, "benign -appearin g"
o 'f-bone Cf: Tubular ma ss following cou rse of o 'I-b on e CT appearan ce is d ictated by specific
in t ratem poral eN? with smoot h en largemen t of location of f NS alo ng CN ?
bo ny FN canal • Genic ulate gang lion FNS: Ovoid smoo th
o T1 C+ MR: Homogeneously en hancing tubula r mass en largeme n t o f genic ulate fossa with thi n bon y
• Locati on wa lls
o Most C0 l111110 n locati o n: Gen iculate gan glion • Tympanic segmen t FNS: Pedu ncu lated mass
o Often spans more than one intrate m poral e N? emanates from tym panic segme n t o f CN? int o
segmen t midd le ea r cavity
• Size • Mastoid segmen t FNS: Either t ubu lar with sharp
o O ften lo ng (multiple cen time ters) m argi ns or glo bular with i rregular m argi ns
o Cross-sectio nal measurement usua lly < 1 em d ependi ng on whet her FNS break s in to
• Morpho log y su rro und ing masto id air cells
o Locati on dependen t • CECT
o No ro le for CECT in thi s d iagnos is
DD x: Intralemporal Facial Nerve Lesionsr - . .....-----,
Norma' CN? TI C+ 8ell Palsy FN Hemangioma FN PN Paroti d Tumor

FACIAL NERVE SCHWANNOMA, T-BONE
Key Facts
Terminology • Mastoid seg ment FNS: Either tubu lar wit h sha rp
• FNS: Rare benign tum o r of Sch wa n n ce lls that invest margins or globular with irregular ma rgins depending
per ip heral facial ne rve (FN) o n wheth er FNS brea ks in to surrou nd ing m asto id air
cells
Imaging Findin gs
• T..bone CT: Tubula r m ass fo llowin g co u rse o f
Top Differential Diagn o ses
• Norm a l in trate m po ral facial nerve en h a nce me n t
2
in trat em por al e N ? wit h sm oo t h e n large me n t o f bony
FN ca na l • Bell pa lsy (her petic facial paralysis) 185
• T l C+ MR: Homogen eou sly en h an cing tubul ar ma ss • Faci al n erve (FN) h em an gioma
• 'l-bone CT ap peara nce is d ictat ed by specific locat ion • Facia l n erve (FN) pe rin eura l (PN) pa rotid m align an cy
of FNS a long CN? Diagnostic Che cklist
• Ge n icu late ga n glio n FNS: Ovo id sm oot h en la rgeme n t • Olde r pa tients with FNS of te n follo wed, n ot ope rated
of ge n icu late fossa with th in bony walls
• Tym pa n ic seg me n t FNS: Pedun cul ated m ass ema n ates
from tympanic segmen t o f CN? int o mi dd le ear
cavity
o Use en hanced MR in stead

Be ll pa lsy (he rpe tic facia l paralysis)
MR Find ings • Clin ical: Overni gh t appearance of perip heral FN
• T I WI: In term ed ia te to low sign a l lesio n pa ral ysis
• T2W l: High sign al lesio n • T..bone c r: No rm al in tra tem po ral FN cana l
• '1'1 C+ • T l C+ MR: Pro m ine n t en ha nce me n t of int ratcmpor al
o Ge n icu late ga nglion FNS: Ovoid , enhan cing m ass in CN ?
en lar ged ge n icu la te fossa
o Tympanic segme n t FNS: Peduncu lates into mi dd le Facial nerve (FN) hemangioma
ear cav ity • Clin ical: Rapid onset un ilate ral perip heral FN par alysis
o Masto id seg m en t FNS • T-hon e CT: In t ratum oral bon e sp icu les (- SocM') )
• Eit her tub u la r wit h sha rp m a rgin s o r glob u lar with • T1 C+ MR: Poo rly-circu m scribed , e n ha ncing mass
irregu la r ma rgins m ost co m m o n ly found in t he ge n icu late fossa
• Depends on whether it breaks in to su rro u nd in g Facial nerve (FN) perine ural (PN) parotid
m astoi d air cells
o Grea ter su perficial pet rosal nerve (GSPN) malign an cy
schwa n noma • Clin ical: Known o r recu rre n t pa rotid m alignan cy
• Diagn osed wh en e n ha ncing mass is see n in • T-bon e Cf: Facial n erv e ca nal e n la rged fro m di stal to
locat ion of GSI'N proximal
• Ju st a nterom ed ial to ge niculate fossa • T l C+ MR: Infil tr ating parot id m ass is prese nt
• Pro jects cep ha lad in to m idd le cra n ial fo ssa o Sty lo ma stoid fo ram en tissue filled
• May be d ifficult to esta blish ex t ra-ax ial n at ur e of
t hi s sch wan n o ma
[PATHO LO GY
Imaging Re commendati on s
• Sta rt wit h th in- sect ion '1'1 C+ fat -satu rat ed MR in axial Gen eral Features
& co ron al p lan e through lAC & 'l-bo ne • Ge ne tics: If multiple sch wa n nomas, me ni n giomas,
• If en h an cing ma ss is d iagno sed o n MR, T..bo ne CT think NF2 -
h elps d elineat e n atu re o f lesio n based o n bo ne • Etio logy : Slo wly-growi ng , benign tum or from
c ha nges Schw arm cells inv est ing int rat empo ral eN?
• Epidemio logy
o FNS is rar e tu mor « PM) of in t rap etro us tu m ors)
[D IFFERENTIA L DIAGNOSIS • Within 'I-bo ne > > C PA-lAC> in t raparotid
• Associa ted abn o rmalities
No rma l intratem po ral facia l ne rve o NF2
en ha ncement • Bilateral acoustic sch wa n no ma
• C lin ical: Asympto matic • Ot he r schwa n n orna, me n ingiom a also possib le
• T..bone CT: In t ratem po ral FN ca na l norm a l Gross Patho log ic & Su rgica l Features
• '1'1 C+ t\1H: Gen icu late ga ng lio n , an ter ior ty m pa n ic • Tan, ovo id -tubula r, en capsu lated mas s
segm en t n orma lly e n ha nce
• Arises fro m oute r n erve shea th layer o f CN?,
o Labyrinth ine seg me n t e N? does n ot n orm ally
expa nd ing ecce nt rically away fro m n erve
en ha n ce
FACIAL NERVE SCHWAN NOMA, T-BONE
..
·
• Beni gn enca psu lated tu mor made up o f hu nd les of
Image Interpretat ion Pearls
• CPA-lAC FNS: Exac tly m im ics acous t ic sch wa n no rna if
spind le-sha ped Schwa n n cells fo rm ing whor led n o exten sion int o labyrin th ine segm en t e N ? occurs
patt ern o If p rese nt , lahyrin t h ine segme n t "t ail" makes
• Cellular arch itectu re cons ists of de nsely cellula r im ag ing d iag n osis
1
(An to n i A) a reas ± lo o se, my xom atous (An to n i B) a reas • In tra tem por a l FNS: Segmen tal, tubu lar en largement of
t 2 • Ma y di spla y in tr a mu ral cys ti c c h a n ge CN? cana l
o Disti n cti ve im ag in g fi ndi ngs de pe n d in g o n segment
Je
I; 186 of CN ? invo lved
:".
•
IC LIN ICA l lSSU ES • In tr apa roti d FNS: Tu b ul a r m ass in pa rot id co urs ing
.
,". Prese ntation
• Most co m mo n sig ns /sy m p to m s
la te ral to rct ro ma n d lb u la r vein
o If p resent , m asto id segme n t "ta il" suggests d iagnosis
o Diffe re nt iat e fro m p e rin e u ral paro tid m ali gna ncy
o Hea rin g lo ss p resent in 70%
o Slow ly p ro gressive faci al n erve p ar alysis (- 50 l MI)
o Ear ± facial pain ISELECTED REFEREN C ES
o Hem ifacial spas m
I. Abd u llah A et al: The d ifferen t faces of facial ner ve
o Acu te o n se t Bell palsy-lik e faci al n e rve pa ra lysis ra re sc h wan no mas. Med J Mala ysia. 58(3 ):45(1-:{, 2003
o O th er sign s/s y m p ro m s b y lo ca tio n 2. Kim CS ct al : Man agemen t o f in tratcm poral Iactal nerve
• C PA-lAC FNS: Sen so rin e u ra l h ea rin g lo ss (SN HL), sch wa n noma. 0 101 Ncur o tol . 2·H2):] 12-6, 200 3
ver ti go &. tinn it u s :J. Kim .Ie ct al: Faci al nerve schwa nnom a. An n Oto l Rhinol
• Large r ty mpani c & ma stoid seg m e n ts FNS: L aryn gol . 112(2 ): 185-7, 20<U
Ava scu la r rc t ro ty mpa ni c m ass; co n d uc tive h ea rin g -I. Phi llips CD ct al: The faci al nerve: an at o m y and com mon
lo ss path o logy. Scmln Ult raso u nd CT 1\..(1{. 2](3 ):202 · 17, 2002
5. l.iu R ct al: Facial nerve sch wau nom a: surgi cal excision
Demographics ve rsus co ns ervative man agem ent . An n Oin t Rhi nol
l.aryngol. 110(11 ):) 025-9, zon t
• Age : Mea n ag e a t p resenta ti on = 35 years
6. Jagt'r L c t al: cr and ~ tn i m agi ng o f th e nor mal an d
Nat ural Histo ry & Prognosis pa th ologic co n dit io ns of t he facia l nerve. Eur J Radlol.
-10(21: 133--16, 2(0 )
• Slo w-grow in g be ni gn t u m or
7. Ker tesz TR e t a l: l n tra tcm pora l facia l n er ve n eu roma :
• will e ve n tua lly e n la rge su ffici e n tly to ca use h ea rin g anato mical loca tion an d radi ol ogical featu res.
lo ss N. o th er c ra n ia l n euro pa th y La r yn goscop e. I I I : 1250 ·1 256, zoot
• So me t u m o rs « 101M,) d o 110 t grow or becom e H. Salzma n KL e t al : Du m bbell sclrw an nomas of th e internal
sy m pto m a ti c auditory cana l. AJN R Am J Neurorad lol . 22 17): 1:{6H-76,
,' 200 1
• Treatme nt 9. Dcvi HI et al: FiH,: ial nerve neurinoma presen ting as middle
···,. ,'
• Co nserva t ive m an age men t
o If FN p ar a lysis a b se n t o r m ild whe n di ag n os ed ,
10.
cran ial fo ssa and cerebellopo n ti n c an gle mass: a cast'
repo rt. Ncu ro l In dia . 4HH ):JH5-7, 2000
Yoko ta N ct al : Pacin l n erve sch wanno ma in t he
surgi ca l cu re ca n be w o rse th a n d isease!
cerebcll opon t tnc ci stern. Fi n d i n ~s on h igh resolution cr
• In co m p let e recove ry o f fu ll FN fun cti on d esp it e
and MR cistem ograph y. IIr J Ncu ros u rg. U (5):5 12-5, 1999
su rg ica l res to ra tio n o f FN co n tin u it y 1ll.IY o ccu r 11. Ku mo n Y ct al: Grea ter superficia l petr osal ne rve
o Follo w u nti l e N? pa ral ysis d evel o ps neu rin o ma . Case rep o rt. J Neurosu rg. 9 1(4):69 1-6, 1999
o Trea tm e nt u sed in eld e rly pati en ts 12 . Ch ung SY ct al: Facial nerve sch wa n n omas: CT an d ~f R
• Su rg ica l t rea tm e n t findin gs. Yonsei Mt'd J. 39(2):14K-53, 199H
o G oa l = co m p le te re mova l of tu m o r w it h pr eserva tio n 13 . Zh ang Q ct al: Ou tgrowin g sch wa n n omas an stng trom
o f h ea rin g &. resto ra t io n o f FN fu nc ti o n tym panic segmen ts o f the facial nerve . Am J Otolaryngol.
o Size specific sur gi ca l tec h n iq u es 17:31 1-31 5,1 996
• Lar ge FNS: Remove tum or, t h e n FN ca b le g raf tin g 14. McMl'1I0 1l H..'y SO ct al: Facial nerve n eu romas presen ting as
aco ust ic tumors. Am J Oto l, 1 5 n ) : 3 0 7~ 12, 1994
• Sm all f NS « 1 e m ): FN tra n spo sit io n wit h p ri m ar y 15. Fagan 1·/\ et ill: Pactal n euroma o f the ccrcbcllopon ttne
an ast om o sis an gle am i th e int erna l auditory can al. Laryngoscope . 103(4
o Location sp eci fic su rge ry PI 1):44 2-6, 199 3
• Labyr in t h ln c o r ge n ic u late f NS : Midd le c ra n ia l 16 . Ma rtin I\' ct a l: Facial nerve n eur o mas: MR imagin g. Report
fo ssa « tran sm asto id a p p ro ac h es co m b in ed of fo u r cases . Ncu ro radi o logy . :H (I ):62-7, 1992
• Tym pan ic Ex m asto id FNS: Tran smastoid a pproac h 17. Ok nbc Y ct a l: lnt ratem por al facial nerve neur ino ma
alone without facial paralysis. Au rls Nasus La rynx. 19H J:223-7,
1992
18. Parnes 15 ct JI: M agn etic reson ance imaging of facial nerve
neuro mas. Laryngoscope . 10 I( I Pt 1):3 1-5, 1991
IDIAG NOSTIC CHEC KLIST 19 . Lido v M et al: Eccen t ric cys t ic facial sch wa nnoma: CT and
~I R fea l ure ~ . J CAT. 15: 1065-67, 199 1
Co nsider 20, Inoue Y ct al: Facial nerve n euro m as: CT findings. JCAT.
• O ld e r pa ti e n ts w ith f NS o fte n fo llowe d , no t o pe rat ed 11:9-1 2-7, 19H7
• Yo u n ge r pa tie nts w it h o u t FN pa ral ysis m a y a lso b e
fo llo wed
FACIAL NERVE SCHWANNOMA, T-BONE
I IMAGE GALLERY
(I.£j l ) Ccmnet l -bonc ci of

right earshows
benigrhlppe.lr in/? smo oth
cnlarW'I71l'nt of g{'nicu/.l t('
2
fossa (errows) by
ovoid-shaped facial nerve 187
scliwa nnofJlJ. Note th e roo;
or fmsd is dchisccllI (open
arrow). (Righi ) Co ronal 11
C+ M R rcveets an avidly
(~n han cing, ovoid facial
nerve sc/llvannoma (arrow)
('Il/argi ng the geniculate
ganglion. Notice cochlea just
Jx./o\\' tu m or (op en arro w ).
(Lef t) Coronal T·bone CT of

right ear demonstrates
lo bular m ass ema nating from
ao ('(io( tymp anic segment of
facial ner ve (arrow). This
facia l nerve sdnvannoma
plesefJWdas avas cular
(C'lrolymp.lnic mass . (Right)
Corollal left esr l -bcne C1
sho lVs mastoid facial nerve
scbws nnoms .10 irr l'gu/.1f/y
sh.l fJed ma ss. Notice the
tumor i~ eX/I'm /inE; into
adjacent ma ~/oid air ('('lIs
(arrow s),
Var iant
(/£}i) A xial T1 C+ M1<
reveets an enhallcing facial
nerve scbwermome involving
geniw /.lle ganglio n (arrow)
& lab yrinthine CN? ~egmenr
(op en arrow). Tu m or has
slso invadedlhe subjacent
coc llh'd (curved arrow ).
(RiK" t) Coronal rt C+ MR
reveals a grealer superficial
p et rosal nerve sch wannoma
p u.shing far up into th e
middle crani al (o.~sa . Note its
origin from grea te r superficid f
petrosal ne rve in potrous
apex (arrow) ,
FACIAL NERVE HEMANGIOMA, T-BONE
2
I llS
Axial graphic illustrates a classic example of c1 A xial I-bone CT slJOws large facial nerve hemangioma
medium- sized fa cial nC'(w hemangioma cenU'fffJ in the (.l rrO\\ls) centerro in Kpniculate fossa with characlerisric
W'lliculate (os_".1 of the l-bone. No lio:! the "honeycomb" bon y meuix So involvemC'n1 of
~ h( )/ I('yn Hl ,h~ bcme within the tumor rnauix. IJb yrinlhin<' .~ egm('rJ1 eNl (olJen arrow).
ITE RM INO LO GY CT Findings

Ab b rev iatio ns a nd Syno nyms • N ECT
o 'l -b on e CT
• Facial ner ve h eman gioma (FNII) • Amo rp hous "h o n eyco m b" bo n e ch a nges are
• In t rar em po ral benign vasc u lar tum or, o ssifyin g d istincti ve
h emangioma, vascu la r m alformati o n • See n in I OOIYtl o f la rger lesions
Definiti ons • Poorl y mar ginat ed lesio n of ge n icu late fossa
• FNI I: Benign vasc u la r tum or a rising fro m ca p illa ries • C EC I': No ro le fro m C ECr in m ak in g t h is radi ologic
ar o u n d facial n erve, mo st co m m o n ly in a rea o f di agnosis
gen icu la te fossa MR Findin gs
o Capillary, caverno us o r ossifying types defined • '1' 1WI : Mixed sig nal lesio n with fo ci of low sign al
wit h in lesion mat rix (oss ific matr ix)
• T2 WI: High sign al lesion '....it h foci o f low signal within
IIM AGING FINDINGS lesion m atrix
General Features • T1 C+
o Avid lesio n con t rast-en h a n ce me n t is rul e
• Best di agno sti c clue: Ossific "ho ne yco m b" mat rix o n
o Perineural sp read a long prox ima l facial n erve may
hon e CT h ighly cha racteristlc when present
be p resent
• Locati o n
• If present, o n ly ex ten ds a few mi llim et ers in either
o Site o f occurrence
directi on
• G ENIC ULATE FOSSA > > internal auditory ca n al
o If in fu n d us o f lAC, ma y exactly mimi c acoustic
(lAC ) fu n d us > post e rior ge n u facial n er ve
schwa n n o ma
• Size • Ovo id, well-de ma rcated , en ha n cing lAC mass
o Ra n ge: 2 m illimet ers to lip to 2 ce n time ters
o Sm all at prese nta tion , o fte n < I cm
• Mor pho logy: irregul ar, in vasive-appear in g mar gins
typi cal
DDx : Enhan cing Lesions of Intratemporal Facial Nerve
NL CN? TI c+ MR !Jell PJlsy r N Sclnvannoma Perineural Malignancy

Key Facts
Terminolo gy Top Differential Diagn o ses
• f NII: Ben ign vascul ar tum or a rising from ca pillaries • Nor mal (Nl.) in tr atempor al e N ? en h an ceme n t
arou nd facial nerve, mo st co m mon ly in a rea o f • Con gen ital cholestea to ma, midd le ear
genicu late fossa • Bell palsy (h erpet ic facial paralysis)
• Ca pilla ry, cavernous o r ossifying typ es defin ed • Facial nerve (FN) schwa n n oma, T-bon e
• Perineur al pa rot id malign an cy o n eN? 2
Imaging Findings
• Best diagnostic clu e: Ossific "h oneycom b" matri x o n Diagno stic Checklist 189
bone cr hi gh ly ch aracteristic wh en present • Earl y detectio n while still extra neural may save facial
• Start with th in -sect ion TI C+ MR imaging focused to nerve at surgery
cerebellopo n tin e angle-lAC-temporal bone in pati ents • Rem ember th at sma ll FNH may be diffi cul t to see o n
with per iph era l facial ne rve para lysis T1 C+ MR
• If MR n egati ve or sh ows equi vocal small areas o n • Use T-bone cr liberally in n egativ e o r equivocal MR
en ha ncemen t along intrat emporal CN?, recomme nd • Poorl y circumscribed, en ha nci ng mass in genic u late
'I-bo ne CT to look for very small FNH ganglio n in pati ent wit h facial nerve paralysis most
likely FNH
Imaging Re commendations Facial nerve (FN) schwa nno ma, T-bone

• Start wit h thin-section T1 C+ MR im agin g focused to • Clin ical: Hear in g loss ± grad ua l onse t of facial nerve
cerebellopo n tine angie-lAC-tempor al bon e in pati ents para lysis
with peripheral facial n erve paralysis • Imagin g: T I C+ MR reveal s tubular en ha nc ing mass,
o Rem ember to include parotid gland in scan area smoot h ly en la rging CN? ca na l on hon e CT
o Axial « co rona l plan es recom mended • Com me n t: Most co m mo n ly cen ter ed o n gen iculate
• If MR negative or shows eq u ivocal sma ll areas o n ga nglio n like r-NH
en ha nce me n t along lntrat empora l CN?, recommend
T-bone CT to loo k for very sma ll FNH Perineural parotid malignancy on CN?
o Bon e-on ly I mm thi ckn ess 'I-bon e c r in axi al &: • Clin ica l: Parotid malignancy in h istory, pa lpable or
coron al plan es subclinica l
o Inspect ln tratc m po ral facia l nerv e ca nal very • Imaging: T1 C+ MR sh ows inv asive pa rotid mas s
ca refu lly with eye o ut for 1-2 millimet er FNH o Stylomast oid fora men is tissue filled
o CN ? en large d & enhancing fro m dista l to proxim al
o e N? may be inv o lved tn CPA-lAC
IDIFFERENTIAL DIAGNOSIS o Mastoid air cell invasion also possible
• Com me n t: Con ti n uo us lin ea r nature diffe ren t from
Normal (NL) intrat emporal CN? focal r-NH
e n ha ncement
• C lin ica l: Asympt om atic
• hnaging: 1'1 C+ M R sh ows n ormal en hanceme n t of
IPATHOLOGY
geniculate ganglion, an te rio r tympa n ic CN? or Gen eral Features
ma stoid segme n t CN? • Etio logy: Be nign tumor arising out o f site s o f
• Co m me n t: Some times mistaken for facial n erve anas to moses between feeding a rte ries in temporal
pat h ology bone
Co nge nita l cho lesteato ma, middl e e ar • Epidemio logy
• C lin ical: Avasc u lar ma ss beh ind intact tympan ic o Ra re lesion
membrane ('I'M) • 0.?1)6 of all temporal bone tumors
• Imaging: TI C+ MR sh ows a no nenhancing m iddle ea r o Sligh t ly less co mmon t han facial nerve sch wan noma
ma ss tracking along facial nerv e cana l Gross Path ologic & Surgica l Features
• Com men t: Involvement of facia l n erve ca nal rare wit h • Rich ly vascu lar mass wit ho ut la rge feed ing vessels
thi s lesion
Bell palsy (he rpe t ic facial paral ysis) • No nen capsu la ted , hen ign tu mor co m posed of vascu lar
• Clin ical: Overnight on set of acute pe riphe ral facial c ha n ne ls-vessel wall s o f varying size
n erve paralysis • Capillary typ e: Small vascu lar cha n ne ls
• Imaging: T l C+ MR shows prominent en h an ceme nt o f • Cavernous typ e: Lar ge vasc ular cha n ne ls
en tire in t rate m por al e N? • Ossifying typ e: Tum o r p roduces spic ules o f lam ellar
• Com men t: No foca l ma ~ ~ ; ho ne CT n ormal ho ne
o Wh en seen , called OSSIFY ING IIEMANGIOMA
• All :{ histologic typ es can be see n in sa me tum o r
• Small avidl y e n ha nc ing lAC tumor wit h hoth SNHL I<
ICLI N ICA L ISSUES I'N pa ralysis is susplclous for FNH
Presentation
• Most co m mo n signs /sy mpto ms
o lntratem poral I'NI I produces pe riphe ral facial ne rve ISELECTED REFERENCES
par alysis ea rly in its natural hi st o ry I. Pal acio s E et a l: Facial n erve h em an giom a. Ear Nose Throat
J. H21I I ):HJ 6· 7, 21Xn
2 • Occu rs ea rly because o f in ti mat e re lat io ns h ip
between facial nerve & FNH 2. Friedma n 0 ct al: Te mpo ral bone h emangiom a.. involving
• Ca uses e N? injury by invasio n, n o t co m p ressio n the facial n erve . O to l Ncu ro to l. 23 (5):760·6, 2lXI2
1'10 :I. Alohid I ct al: Cavernous haemangioma o f t he in ternal
• O nset of eN? paralysis usuall y acu te hut may be a ud itory ca n a l. Act a O to lar yn gol . 122 (5):501 · ] , 2002
slowly p rogressive O f in ter mit ten t 4. Ach illi V et al: Facia l n er ve h em an gio m a. O to l Neurotol.
• May be de scr ibed as "a typ ical Bell palsy; beware! B(6): lIX).1· 4,2002
o lAC I'NII s. Sa lih RJ c t a l: The cruc ia l ro le of im aging in det ecti on of
• Sensorineural h earing loss (SNIIl.) ma y be m ore faci al nerve hncm an gtcm as. J Laryngo l ( HoI. 115(6):5 10-3,
p rom in ent sy m p to m 200 1
• Cli n ical pr ofile 6. Petit -Lacour Me e t al: Hem a n gio m a o f the po rus acusticus.
o Intrat emporall'NII : Adu lt witb rel ati vel y rap id onset Im pa ct o f imagi n g studies: caw repo rts. Neumradiol ogy,
HI 12 ): 1102· 7, zoo t
of peri p heral I'N paralysis (o ver wee ks)
7. Gj ur ic ~( et al: Ca ve rnous h ema ng ioma of th e in te rnal
o lAC I'NII : Ad u lt wit h relatively rapid o ns e t o f I'N auditory ca nal arisi ng fro m m e in ferior vest ibula r ne rve:
pa ralysis with co ncom ita n t SNIIL (o ver wee ks) G J')l' report a nd revi ew o f th eliterat ure . Am J Otol.
• O the r sign s/sym p to ms 2 1(1): 11O·4,2IX)0
o Hemifacial spas m Illa y prog res!l to facial nerve H. Sha ida 1\ \ 1 et al: Ca ve rno us h aemangkuu a o f the internal
pa ralysis aud itory ca nal . J Laryn go l O tol. 11-t(6 ):-t53-5, 2000
o When in fund us o f lAC. ma y present with 9. Asuok a K et a l: Hem ifacial spasm ca use d by a h eman gioma
senso rin eural h eari ng lo ss at t he gcn fcu latc ga ng lio n: ca se repor t. Neu ros u rgery,
41 (5 ): 1195- 7, 199 7
D em o graphi cs 10. Escad a P et al : Cnvc m o us haema n gioma o f t he facial nerve.
j Laryn go l Otol. 11 1(9 ):KSK-6 I, 199 7
• Agl': Wid e range but usu all y ad u lts
11. Omojola MF ct ;11: CT and ~m 1 feat ur es o f G IVCCIlOUS
Natu ral Hi story & Pro gn o sis hncm angtom a o f int ernal audi to ry cana l. Br J Radlul.
70 IRJ9 ): 1I H4· 7, 19 9 7
• FNIi is a slowly-g rowing, benign tumor
12. Pulec JL: Faci a l ner ve a ng io ma. Ear Nose Th roat J.
• Presents wh en small as a resu lt of locati on ad jace nt to 7S(4 ):22S·:lH, 1996
facial n erve U. Du four JJ et a l: In t rat c m poral vascu lar m al form ations
• Relat ed to size o f tumor at di agnosis, seve rity &: (an gio m as): particula r cli n ica l fea tu res. J Otolaryngo l.
dura tio n of p re-ope rative fN pa ralysis 2:l(4) :2S0·3 , 19 94
• Afte r su rgery, fu ll facial nerve fun ctio n is gene rally no t 14. [by 11. et .11: Faci a l n er ve m an agemen t in tem po ral bon e
regained h em angio mas. Am J o rot, 13(3) :223-32, 1992
15 . vt an tn : e t al: Hacman g lum a o f th e pct rous ho ne: ~I R I.
Treatment Ne u ro radi ology. :\4 (5 ):420 -2 , 1992
• Sma ll FNH are ext rane u ra l 16. Sh elto n C vt a l: ln tra tem po ra l facia l nerve h ema n giom as.
Otala ryngol Hea d Nl'Ck Su rg. 1O-tO ): 116· 2 1, 1991
o May be resect ed with p reser vation o f facial nerve
17. Gavila n J ct al: O ') s ify in ~ he m an gio m a o f t h e tem poral
function bone. Arch O tul ar vngo l Head Neck Su rg. 116tK):965-7,
• Large r FNH invad e facia l n e rve 19 90
o Seg mental facial 1H.' f VC' resection co m p leted I H. Lo WW ct al: Facial n erv e hemangioma . Ann Otol Rhinol
o Fo llowed by pr imary or ca ble repa ir of facia l n e rve I.ary n go l. 9H(2 ): 160 -1, 19H9
o W hen n ecessa ry. yields poorer o u tco me 19 . Lo W W ct a l: tn trat cm po ra! vascul ar tumo rs: detec tio n
with C f a nd Mit imagin g. Rad iology. 171(2 ):-t-t5-H, 1989
20 . Cur t in HI> ct a l: · O ssify in g" h em a ngio mas o f th e tem poral
IDIAGNOSTIC CHECKLIST bo n e: eva lua tio n wit h CT. Rad iolo gy. 16-t(3 ):RU -S, 1987
Consider
• Early d etection while st ill ext ra ne u ral ma y save facial
nerve at surg ery
• Remembe r that sma ll FNII may he d ifficu lt to see o n
TI C+ MR
o Usc T..bo ne c r libera lly in n egative o r eq u ivocal MR
• Max imi ze YOUT cr « MR imagi ng technique s
Image Interpretati on Pearl s
• Poo rly circu m scr ibed , e n h a nci ng mass in genicu lat e
ganglio n in pa tie n t with facial ne rve para lysis most
likely FNII
• Me t iculo us scru t in y req u ired by rad io log ist to ide nti fy
ea rly, sma ll in t ra te m po ral FNII
IIM AG E GALLERY
Typical
(/.1/1 ) A l ial lefe eer T'!K>Ilt'
CT revcills a typical
medium-sized f~K;.l1 nerve
h~m.mgjoma ( a ffOW S)
2
emanating (10m gt'nic u/dlC'
(OSSJ .)found stw sn tenor 19 1
cocbtcsr surface. Note
re/alive 5pdring of otic
C.lp5u le. (Highl ) Axial T 1 C t
M R sholVS diifu!i(,
enh.mct>mt'nt of J
nlf.'<!ium -s;zp</ EN
hem.lng/oma (.u rows). AIR ;5
no t fal -5.ltur.lt('(/ m.lbng if
difficult 10 di!i!ingui!>h
potro u s ape'\: (.my m arrow
(open imow) from tumor.
Typical
(l.eft) A xial l-bo ne CT
(('\'f' als .l very smafl facia l
nerve hemangiom a as d
sing h. dOl of ossific meuix
(arrow) witbtns mildly
{·nl.ugl'd gellicul.llt· (0::.5.1.
Rddio/og ist lwwere as rhis
would he easily mi.55M!
( Ri1:1I1) A xial TI C+ I\I R
H·l/l'.l/s .1 tiny (·nh.lOt";rlg
f.lci.lf n (,H'f ' hf'm,lng;om.l
(,)(ro w) in till:' I()(,:al;o n of the
geniculate ganglion in th i5
p.lti('nt with olc utt' ()n.~f:'t
pe rip heral facial nerve
pdralysis.
Variant
(l-Lft) A~ia"efl car T·iJo ne
CT sho\\'s ,I highly unu~ual
f,K;,ll rJ('n 'C' hC'm.lrJgioma
n 'n tl'H'Cfon the porus
dl 'U.\tic us of thl:' internal
auc/itOfY canoll (.lfIO\ !I_~ 1.
,\ IosI IAC FNff occur in the
fundus . (Ri/:lrI) A';oll T1 C+
AIR f('V(>,l15C'nhancing
;nrl'rn.l l auditor y c andl facial
nl:'rvl:' hl'rnJ ng;QfJ),l (<1" o \\,s).
Tht·lf·sinn Sh.l(H.> J. modist
loca tion ,UI:' both highly
un usual varian t fe.l tuft's of
FN II , O pen srrow: L( K"'t' .I.
PERINEURAL PAROTID MALIGNANCY, T-BONE
2
192
Sagittal graphic ckpiclS parotid cancer (arrow ) Coronal T1 C+ MR shmvs parotid adenoid cy.;tic
spreadingalong eN7, through the stylomastoid kxemen carcinoma (arro'!'v) spreeding along proximal
(open arrow). No te it travels Oil the mastoid segment to cMracraniaJ e N7 through stylomastoid foramen (up en
pos(('rior W'nu (CLIn ro arrow ). arrow), tbon up ma$toid .~ egmen t (cur ved arrow ).
ITE RM INO l O GY • Size

o Cross-sectio n size: 1-4 mil lim eters
Abbreviations a nd Syno nyms o l.en gth : May be ma n y cen time te rs in length
• Per ineural tum or (PNT) o n intrat emporal facia l nerve • Mor phology: Tubular en largeme nt of intratem poral
(FN) CN? most co m mon
• Facial perin eural metastat ic d isease; neurotrop ic spread CT Findings
o n in tratem po ral facia l nerve
• NECT
Definitions o T-bo ne CT sho ws asy m metric widen ing of SMF &
• " NT o n FN in T-hon e: Form of metastat ic d isease mastoid FN canal
where ma lignant tumor extends along cou rse of o Adjacent mastoid a ir cells often show tumor
in tratem po ral CN? invasio n
• CECT
o CECT h eip ful in defini ng in t ra pa ro tid malignancy
o CECT n ot helpful in defi ning in trat em por al CN?
IIMAGING FINDINGS " NT
General Features MR Findings
• Best dia gnosti c clue: Poorl y circumscribed , en hancing,
• '1'1 W I: Loss of fat in SMF o pening best seen on axial Tl
invasive ma ss arising wit h in parotid gland exte nd ing
MR images
th rou gh sty lo ma stoid fora men (SMF) to in vo lve
mastoid FN segme nt • T2W I
o High -resolution thin -secti o n T2 images define lAC
• Locati on PNT if presen t
o Malig nan t so urce of PNT most co m mo n ly arises in
• Thicken ed CN? in lAC fun dus co n nects to
pa rotid
en larged lab yrin thine seg me nt CN?
o Facial nerve inv o lved fro m di stal mastoid segmen t
to proxima l • T1 C+
o Axial im ages delineat e tympa n ic, ge niculate
o PNT can ex te nd as far along eN? as roo t ex it zone in
gang lio n & labyrinth ine FN " NT best
cereb ellopontine an gle
DDx: Enha ncing Facial Nerve l esion
Bell Palsy FN I lemangioma FN SChWcl fl fl oma TM Schws nr oms

Key Facts
Terminolo gy • Transmod io lar (fM) schwannoma
• Perine u ral t u mor (PNT) o n intrate mporal facial nerve Path ology
(FN)
• Any malignancy may demo nstrate PNT
• 1'1'.'1' o n FN in T-bone : Form o f met astatic di sease • Adeno id cystic carci noma (ACCa) i s most co mmon
whe re malign ant tum or ex te nds along co u rse o f
intra te m poral CN?
pa ro tid m align an cy to show 1'1'.'1' alo ng CN? 2
Diagnosti c Checklist
Ima gin g Findings • Becau se PNT is co n tiguous ex te ns io n from primary
193
• Best d iagnostic clue: Poorl y circumscribed, parotid ma ligna ncy, it must he removed with primary
en ha ncing , in vasive mas s arising with in parotid tumor during 1st surgery
gland ext ending through sty lo m asto id foram en (SMF) • Im aging fin di ngs o f I'NT may be subtle
to in volve ma stoid FN seg me n t • If th e radiolog ist does not think to search fo r 1'1'.'1'
Top Differential Diagn oses when suspected parotid malignancy is seen, th e
• Bell palsy dia gn osis o f I'NT will probably be m issed
• Facial nerve (FN) heman gioma
• Facial n er ve (FN) schwa n noma
o Co ron al images th rough T-bo ne shows I'NT

exte n d ing through SMF in to ma stoid segme n t of FN
IPATHOLOGY
best General Featu res
o Axial images wit h fat-saturation revea l in filtrating • General pa t h co mments
parotid ma lignancy o Any malign ancy may d emonstra te PNT
Imaging Re commendation s o PNT o n in tratempor al FN 1110st co mmo n fro m
• Co n t rasted M il of 'l- bo ne with fat- saturati o n is best for intrap arot id malign an cy
eva luati ng ex te n t of pNr alo ng CN? th rou gh • FN is "peri ne ura l hi ghway" followed by any
stylo mastoi d for am en pa rotid space ma ligna ncy if give n eno ugh tim e
• No nc on trastc d, bo ne algorithm CT bes t to eva lua te o PNT can extend retrograde or an teg rade
osseo us SMF as we ll as FN segmen t involve men t a PNT can al so oc cur from direct parotid invasion by
skin malignancy
• Etiology: PNl ' is o ne of "pat hs o f least resistan ce" as
IDIFFERENTIAL DIAGNOSIS malignant tUl110 r en larges
• Epidemiology
Bell palsy o Aden oid cysti c carcino ma (AeCa) is mo st co m mon
• Clin ical: Abrupt o nset perip heral FN paralysis (ofte n parotid malignancy to show PNT along CN?
overn igh t) o Squamous ce ll carcinoma (SCCa) is most co m mo n
• Imaging: Enti re in t rat emp oral FN conspicuously 11&1'.' malignancy showin g I'N r sprea d
en ha nces o n Tl C+ MR o Others malignancy with PNT from parotid
• Mucoe pide rmoid carcino ma (MECa)
Facial nerve (FN) heman gioma • Prim ar y or seco n dary (fro m skin) SCCa
• Clin ical: Facia l ner ve pa ralys is early in disease process • No n-Hodgkln lym ph o ma
• Imagin g: Infi ltrating fo cal en ha nci ng FN les ion in o 3.71}6 inci de nce o f PNT wit h II&: N m ali gn an cies
gen icu late fossa o n Tl C+ MIt • Associ ated abnorma lities : In vasive m alignant tu mor in
o T-hone C I': SOIMI wi th "h o neyco m b bo ne" pa ttern parot id space
Facial nerve (FN) sc hwa n noma Gross Path ologic & Surgica l Fea tures
• Clin ical: Presen t most co m mon ly with heari ng lo ss, • PNT patterns with H&N ca ncers ca n occu r ea rly in
less co m mo n ly FN pa ralysis d isease process
• Imaging: Tubular en ha n cin g mass along course o f • PNT allow s sp read over grea t di stance without local
intratemporal facial nerve o n T1 C+ MR invasion o f ad jace n t structures or sign ifican t
o T-bone (.1: Fusiform en la rge me n t o f in t ratem pora l lymphad en opath y
FN ca n al; m ost commonl y at gen icu late gang lio n
Microscopic Featu res
Transmodiolar (TM) schwa n noma • Exte ns io n o f neoplast ic p rocess alon g neural
• Clin ical: Slowly progressive senso rineu ral hear ing loss; pathways-endoneural spaces
FN paralysis n ot pre sen t
• Imaging: Dumbbell -shaped enhancing mass exte n d in g Stag ing, Grad ing o r Classification Criteria
from coc h lea throu gh coc h lear a per tu re in to lAC • Stag ing criteria : Salivary glan d tumor with PNT o n
fundus o n T l C+ MR CN?
o T4: Tumor inva des eN?
o Stage IV: 1'4, 1'.' 1,1.10
• If stylo masto id fora men fat is invaded , dedi cated bone
IClI N ICA L ISSUES CT & enha nced MR of 'l-bo ne in dicat ed to assess
Presentati on exte nt of PNT alo ng e N?
• Most co m mo n signs/ sym pto ms • Remember that there may be radi o log ic "skip areas"
o Asymptom atic (60% ) along CN? ; Visua lly in terrog ate enti re CN? into CPA
• FN fibers th em selves are resistant to neoplastic cistern
2 in vasion wh ile ad jacen t struc t ures m ay be
194
dest royed
o Perip heral facial nerve paresis or pa ra lysis ISELECTED REFERENCES
o Palpabl e parotid mass I. Ga rd a-Serra A ct al: Management o f neu rotropic low-grade
o Othe r signs/ sym ptoms B-ccll lymph o ma : repor t o f two cases . Head Neck,
• Burni ng or stinging facial or ear pain 25 ( II ):9 72-6, 21Mn
2. Kaylic n~1 e t al: Preoper ati ve fac ial muscle im agin g
• Fo rm icatio n (sen sation of an ts craw ling)
pr edict s fin al facial fu nct ion after facia l nerve graft ing.
• Clin ical profil e: Adult with parotid m ass & ipsilat eral AJ NR Am ) Ne ur o radl ol. 24(3) :]26-30 , 21X):J
facial nerve para lysis :I. Scles n ick Sli ct al: Regiona l sp read o f non neu rogen ic
tum or s to th e skull hase via t he faci a l nerve. 0101 Neurotol.
Dem ograph ics 24 (2):326 ·33 , 201n
• Age: 40-60 yea r o lds 4. Sch m alfuss 1M ct al : Per in eural tumor spread alo n g the
au ricu lot empo ral nerve . AjNR Am J Ne u rn radiol.
Natural Hi story & Progn osis 23 (2):303 · 11, 2002
• Carcino mas wit h PNT usual ly have relentless 5. Fisch bei n NJ et al: ~1R imag in g in two cases of su bacute
prog ressio n dcncrvan o n c ha n ge in till' m uscles o f recta! ex pression.
• FN in vasion can lead to devastating physical defor mity AjNR Am J Nc u ro radi o l. 22(5 ):880 -4, 2001
I< dysfu nct io n 6. Will iams L~ ct a l: Pcn neur nl spread o f cutaneous sq uamous
• H&N neoplasms can exist with in nerves for years a nd ba sal ce ll ca rcino m a : CT a nd M R d etecti on and its
impact o n patient m an agem en t a nd prog nosis. Int J Radiat
without sym pto m s
O nco l Rio l Ph ys. 49 (4 ) : 106 1 ~9 , 200 1
o Especia lly true in low grade ad en o id cys tic 7. j u uge h uel sin g ~ 1 l' t al: l.tm ita t ions o f m agn eti c reso nance
ca rcino ma (ACCa) im agin g in t he evaluat io n of perin eura l tu mo r sp read
• Diagnosis is frequ en tly delayed & o utco me is poor ca using facial nerve pa ra lysis. Arch O to la ryng ol Head Neck
once clini cal ma ni festat ions arise Su rg. 126 (4):506-10 , 200 0
• 5 year o ve ra ll su rviva l: 251M , 8. Ca ld cm vyer KSvt al : Im agin g fea tu res a nd cli n ical
• Paro tid ACCa is spec ia l case signi fica nce o f perin eural sp read or e xte ns io n o f head and
o Recurrence & su rvival rates dep end o n specific nec k tum or s. Rad io gra p h ies. 18 (1):9 7-110 ; q uiz 147, 1998
9. McNab AA et a l: Perineur a l sp read o f cuta neo us squa mous
tum o r grad e
ce ll carci no ma via th e or bit. C lin ica l featu res and o utcome
o 65 1}h o ve rall 10 year surviva l rat e in 2 1 cases . Oph th alm o logy. 104(9 ):1457-62, 199 7
o Lo ng term (5-10 yea r) ima ging fo llow-up is 10. G insberg LE ct a l: G rea te r su pe rficial pet rosal nerve:
reco mmended given tendency of ACCa to recur late an a to m y an d MR find in gs in peri n eural tum or spread.
AJNR Am J Ncur o radiol . 1 7 ( 2 ) : :~ 8 9 - 9 3 , 1996
Treatm ent 11. Catala no PJ et a l: Cran ia l n eu ro pat h y seconda ry to
• Treatm en t N. progn osis alt ered by PNT per in eur al sp read o f cu ta neo us m alignancies. Am J Otol.
• FN inva sio n = primary ind icatio ns fo r parotidectom y 16 (6):772 · 7, 199 5
• Surgery co mbined wit h post-opera tive rad iation 12. Sch ifte r M et a l: Perineur a l sp read of sq ua mo us cell
therapy carcino m a Involvin g tri ge m in a l a nd fac ia l nerv es. Oral
• Ad juva n t chemo the rapy & rad iatio n th erapy may Sur g Or a l Mcd O ral Path o l. 75 (5):58 7-90 , 199 3
13. Fran kcnt h ule r RA c t al: Progn ostic var iables in parotid
improve disease-Iree surviva l
gland ca ncer . Arch O tol ary ngol Head Nec k Su rg.
11 7(11 ):1251 · 6,1 991
1-1 . Parker GD et al : C lin ica l-rad iologic issues in perin eural
I DIAGNOSTIC CHECKLIST tu mor sp read o f m a lignan t d isea ses o f th e e xt racranial
he ad an d neck. Radiographies. 11(3):31H -99, 1991
Co nsid er IS. C lo usto n PI) et a l: t'cnn cu ra! sp rea d o f cu taneo us head and
• Becau se PNT is co ntiguo us ex tensio n from primary nec k Cam-No Its o rbital and cen tral neu rol ogic
parotid malig nancy, it must be removed wit h prima ry co m p lica tio ns. Arch Nc u rol . .f 7 ( 1 ) : 7 :~ - 7 , 1990
tumor d uring Ist surgery 16. Nels o n BRet al: Faci al n erve pa lsy ali a result o r sq uamous
cel l carci nom a o f til l' skin . J Dcmmt o l Surg O ncol.
• Imagin g findi ngs of PNT m ay be su btle 1.\ (5):510-:1,1 989
o If the radiol ogist does no t th ink to search fo r PNT 17. Vrielinck LJ et a l: Th e sign ificance o f perin eural spread in
wh en suspect ed pa rot id malignancy is seen, the ad enoid cyst ic carci nom a o f t he ma jor a nd m inor salivary
dia gn osis of PNT will probably be m issed g la nds. In t J Ora l Maxillofac Su rg. 17(3):190 -3, 1988
18. Co ttel WI: Per ineu ral invasio n by sq uam o us-cell
Im age Interpret ati on Pearls ca rctnoma. ] Dermato l Surg O ncu l. 8(7):5 89· 600, 1982
• If the radio logist sees invasive lesion in pa ro tid space, 19. Cu m m in gs CW: Ade noidcystic ca rci no ma (cy lind roma) of
a sea rch for PNT sho uld begin by evaluating th e parotid g la nd . An n O to l Rh ino l Laryn gol. 86(3 Pt
stylo masto id fo ram en fat I ) :280~92 , 1977
IIM AG E GALLERY
(/ LflJ Ih ial CCCI ~ hO\V5

infiltrating PJ rolid adenoid
cys tic ce rcino ma (.lrfOW)
with deep lo b(' ;nv,h in n
2
(open arrow ). /'t-' ;nt'UfJ !
sp rC'.l d S('('n .H mund IlOSiOll 195
(curved arrow ) jw.t helow
5l)' lom,H l()icJ ft"dm('n.
(High" A, i,11 Ci.Ct
dt>mons tfJl eS facia l n('( v{'
p f"fineUfdl tumor (,lfIO'oV) in
the right sty lom.uloid
(of,lml'n in t h ;~ p .ltient with
parotid spece (I(/PtJoid cystic
carcinoma.
(I.f![ l ) " x;dl 11 WI AIR R'Ved/)

wbtk- {pft f.lci.1J nerve
('nlargem f'nf (arrow) in fall y
"hf·/I" of Ill(' slylomdS(oicJ
(J r.lI1ll'n. Psuont ha r!
/" ' rilleuf,l' HfU<lllJow. n 4!
Ct!f( ; 1l0Il J.l Jt biop sy. U p ('/l
.lffO W: No rm al righ t eN7.
(Higl,,, Axi.ll lxJflf· C T ~ hnlV~
subtle t>nl, lfJ.:eTnenl of
m.Hto id spgmt'nl of eN7
(arro w) imm pa rotid
m ucoep idermoid cmcinoma.
Tumor also in v.lC lt·~ mu!ti"lt·
_~ urrounding 0JMcifjf-'d air
C('lJs (open ,,,,o w) .
Variant
(LLf " A \ial TI C+ M R
((' \"(' .11" p...fi m·uf .11tu mor on
righl r,K;al ner ve lh.lt h.1S
sprmd '10m J>.lm tid 10
im l'rn,J/.w rli trlry C.1IJd l
tetrowt. Noll' tbo l ymp.m ;c
wgm l'n l ;s m uch !om"IIt'r bu t
is involved (o~n d lroW }.
(Hight ) A lo;i,JI TlWI M R \Vilh
thin-section h igh-rem lu tion
tec hnique sho lVs pt-'rineuf al
fM ro tid nJ.1 /ign,'fl(·y h d S
Sp f" ,U{ n·ntrally along eN7
int o lh l:' ;ufl(/u ~ 0; fill'
int elll,lI .wditor y cerul
( d tlo\\') .
TEMPORAL BONE FRACTURES
2
19 6
Axial bO/Je' CT (('W'aI5 a longitudinal fracture (arrow s) Axial bone a shows (raflS\I('rse fracture (aff ows)
('x/ending from mastoid /0 petrous <1p<.' x. Malleoincudal extC'f1ding through fundus of in tern JI auditory canal
disruption (UfX'1l arrow) is notm. Middle ear debris associated w ith acute complete hearing loss du e to
(hemolymp anum ) presmt. ccxhteer nerve transection.
o Majo rity of fractu res a re actually o bliq ue!

ITE RM INO l O GY
CT Findin gs
Abbreviati ons and Syno nyms • NECT
• Long itu dinal, transverse &: ob liq ue fracture of 'l-bon e o Long itud ina l 'l-bo ne fractures
Definitions • Parall el to long axi s of 'I-bo ne
• Fract u re throu gh petrous T-bo ne; includes trau m atic • Typica lly ex tralabyrin th ine
ossicular d isruption • TM rupt ure , hem ot ympan um & ossicular
disruptio n arc co m mon
• f acial nerve cana l inv o lvem ent is co m mo n but
IIM AGING FINDINGS less so th an wit h transverse fract u re
• Anterior subtype: In volves sq ua mo us "l-bo ne,
Ge nera l Features tegm en , glenoid fossa & facial nerv e
• Best diagn ostic cl ue • Posterior subtype : Involves masto id, ossicular
o Acute: Fracture lin e in pati ent with co mpatible cha in & facia l n erve
cli ni cal hi sto ry o Tran sverse T-bon e fractu res
• Middl e ea r flui d (hemo tyrn pa nu rn) • Perp endicul ar to lo ng axis of T-bone
o Ch ro nic: Fracture line o r ossicular d isru pti on in • Ofte n involv es l AC or o tic capsu le
patien t with co m pa tible clin ica l hi stor y • Middle ear & EAC involved less co m mon ly than
• Often n o midd le ea r flui d o r o th er debris lo ngitudin al
• I.ocation : Pet rou s tem poral bone • Facial nerve canal in vol vem en t is co m mo n
• Size: May be d iastatic (m illimeters) o r subtle • Medi al subty pe: Posteri or petrou s surface through
• Mo rp hology fund us of lAC to 1st ge nu of facial nerve;
o Longitudinal fract u re: Parallel to long ax is o f T-bone permanent co m plete hear in g loss co m mo n
• Most co m mo n (estima ted as h igh as 8 6 (}(,) • Lat eral subty pe : Posteri or petro us sur face through
o Transve rse fractures: Per pen d icul ar to lo ng ax is labyrinth , co m mo n ly with associated
'l-bo ne perilymphatic fistu la
o Fractures actually are often "obliq ue" o r "m ixed" o All fract ure lines best identified on axial images
DDx: Temporal Bone Pseudofr actures
Mastoid Canaliculus In!' Tympan. Canal Petromastoid Canal Singular Canal

Key Facts
Imagin g Find ings • 2.S')£. of T..bo ne fractu res involve ot ic caps ule
• All fracture lin es best identified o n axia l ima ges • ]<YMl associated with 7t h nerve injury; 85'M. in
• All vari eti es: Facia l nerve most co m mo n ly in ju red in ge n iculate ga ng lion
ge n icu late fossa Clinical Issues
• Im po rtan t to delineate ma rgin s facing in tr acran ial
co mpart men t du e to CSF leak potential
• Associat ed CSF leak co m mon (15% )
• Associat ed 7t h nerv e injury (10 %): Mo st reso lve
2
• Pneumolabyrinth o r peril ympha tic fistula may be spon ta neous ly 19 7
associated if th ere is con tiguity of labyrin t h with
middle ear or mastoid Diagn ostic Check list
• l\1RA: Petrous ca rotid oc cl usion &. pseudoaneurysm s • In "aty p ica l" fracture, co ns ider pseudofra ctures
a Te definite co nce rn in co mplicated cases • In "atypica l" ossicu lar in jury, co nside r oss icu la r
ch a nges seco ndary to in cide ntal chroni c oti tis
Pathol ogy • Crucia l no t to m isdiagnose a pseudof racture
• Most co m mo n fractures of sku ll base
• 20% of pat ien ts with skull fracture have T-bo ne
fractures
o 1\11 vari eties: Facial nerve most common ly injured in • Direct co ron al ima ging rarel y possible with severe
geniculate fossa tra uma
o Im po rta n t to delineate margins facing in tracranial o Co rona l reconstructions is alternative
co mpart me n t du e to C..5 I; leak po te n tia l o Mu lt idetecto r cr ma king reco nstru ctions m ore
o Pneumolabyrin th or pe rilym ph a tic fistula may be useful
associated if th ere is con tigui ty of labyrinth wit h • MR useful on ly if int racrani a l co m plica tions are
middl e car o r ma stoid suspected
o Ossicular d isrupti on , m ost co m mo n types o Trau mat ic cepha loce le, h em or rh agic intracrani al
• ln cud ostap ed ial jo int (IS)) di sruption: Most co m plica tio ns
common
• Malleoincudal dis ruption
• Co m plete in cus d isloca tion IDI FFERENTIAL D IAGNOSIS
• Stapediovestibular d isruption
• Malleus di slocation: Rare Pse ud ofract ure
• Ext rin sic sutu res-fissu res
MR Find ings o Occipito mast oid, petrooccipi ta l, tem po rop a rietal
• TlWI • Intrin sic fissu res
o Fractu res may be visua lized o Pet rotympan ic &. petrosqu am osal fissu re
o Hem ot ympanum may he appreciated o Tympan osqua m ous & tympan om astoid fissures
• Acute fracture: Low signa l fluid • Int rin sic cha n n els
• Subacu te frac tu re: lligh signa l fluid o Mastoid ca na licul us
• 1'ZWI: Midd le ear & masto id debris appears • Bet ween jugu la r foramen &. m asto id segment of
h yperin ten se facial nerv e ca nal
• 1'Z· GRE: Usefu l for d etecti on of seco nda ry cerebral • Co n ta ins n erve of Arnold (l Ot h nerv e branch )
injury o Infe rio r ty mpan ic ca na licu lus
• Tl C+ • O riented vertically between caro tid ca na l &.
o Enhancem ent along fracture line ha s been jugu la r foramen
demonstra ted bu t cr far m ore valuable • Co n ta in s Jaco bsen nerve (9t h n erve branch) &:
o Most va luab le fo r suspect ed in tra cra nia l in fe rior tym pani c a rtery (ECA bra nch )
co m plica t ions o Pet rom astoid cana l
o Facia l ner ve ofte n en ha nce s wh en inv o lved in • Petrou s a pex
fract ur e • Suba rcua te bra nc h of ante rio r in fcr lor ce rebellar
• MRA: Pctrous caro tid occl usion & pseudoan cu rysm s ar ter y
are defi ni te concern in complica ted cases o Coch lear aqueduct
• MRV: Sigmoid sinu s thrombosis = rare complication • Parall el &. inf erior to I,\ C
Imaging Re co mmend ations • Peril ymphati c cha n ne l
o Vestibular aqueduct
• Axial & co rona l thin-secti on bone-only cr is exam of
• Po steri o r petrous su rface
ch o ice
• Endol ymp ha tic du ct & sac
o e rA o r MRA if fract ure line extends to ca rot id ca n al o Singular ca na l
o Overlap ping images th rough oval wind ow usefu l if
• Parallel to lAC
stapes fract u re or peril ymphat ic fistula is suspected
• Posterio r a m pulla ry ner ve (to posteri or su perio r
semicircular ca na l)
IPATHOLOGY Treatm ent
" • Managemen t of severe head in ju ry is pri ority
" General Features o Ossicu lar recon struction for pos t-trau m atic CHL
• Etiology: Blun t head t rau ma; 1,875 lbs req uired to a fte r pa tien t recovers
fractu re cad aver T-bo ne • An t ihiot ics a re used if CSF lea k is demonstrated
• Epidemiolog y
"
t-
2 o Most com mon frac t u res o f skull base
o 20(M> of pat ien ts wit h skull fractu re have T-bo ne IDI AGN O STIC C HECKLIST
l: fractu res
198
,-~
o 2 .59,6 of Tvbonc fract ures in vo lve otic capsule Co nside r
~ , o 10 1M) associa ted with 7th ner ve injury; 85 1M, in • In "aty pica l" frac tu re, co nsi de r pseu d o fractures
.1 gen icula te gang lio n • In "aty pical" oss icula r in jur y, co ns ide r ossicu lar
Gross Path ol ogic & Surgical Features c h an ges seco nda ry to inci de n ta l ch ro nic otit is
• Vario us form s of ossicular injury may he identified at Im age Interpretat ion Pearls
su rgery • Crucia l n ot to m isdiag nose a p seudo fract ure
• Surgery need ed fo r diagn osis of perilymp hat ic fistula
ICLIN ICA L ISSU ES 1. Gross Met al: Cochlear involvement in a temporal bone
Presentati on fracture. Oto l Ncurotol . 2 4 ( 6): 95 X~ 9, 200 3
2. gergem alm 1'0 : Progressive hearing I() ~ s after closed head
• Most co mmo n sign s/sym pto ms injury: a p redict able outcome? Acta Otolaryngol.
o Lo ngit ud in al fract u re 123(7):836-45, 2003
• Tcm pora l-pa rlcta l trau ma Sudhoff H et al: Temporal bone fracture und Iatent
• Typically spa res otic caps u le, sensorine u ral meningitis: temporal bone hi stopat h ol ogy study of the
h ea ri n g lo ss (SN II I.) u nu sua l mont h. Otol Ncurotol. 2 4 ( 3 ): S 2 1~ 2, 20 tH
• High incidence of conduc tive h earin g loss (CHI,) 4. Exadakt ylos AK ct al: The cli ni cal correlation of temporal
seco nd a ry to o ssic ula r in jury bone fract ures and spiral computed tomographic scan: a
prospective and consecut ive study at a level I trauma
o Tran sve rse frac t ures
center. J Trauma. 55 (4 ):70 4-6, 20 03
• Fro n ta l-occipita l traum a s. Gross M ct al : I'ncum o labyrinth : all u nu su al finding in a
• Often involves in n er ea rl SNI IL co m mon temporal hon e fractur e. Int J I'ed iatr Otor hinolaryngol.
• Lowe r incidence of CHL 67(5):553-5, 20llJ
a In pa tien t s wit h acu te severe head inju ry 6. l.in T Io" ct al: Isol ated tr an sverse t rnnscochlca r temporal
, , • Fin d in gs relate d to tem po ral bone a re of bone fracture. Otol Ncu ro tol . 23 (4):615 -6, 200 2
,
seco nd a ry importa n ce 7. Singh S et ul: Traumatic fracture of the stapes
I '
• Exception is when CSF leak, ICA d isruption o r su p rast ruc ture followi ng minor h ead in jury. J Laryngol
"",
, 01 0 1. 11 6(6):457-9. 2002
sigm o id sin us com pro m ise is sus pected
II 8. Kromhach GA ct af The petroma stoid ca n a l on co m puted
• Mo st co m m o n sign o r ac ute inju ry: tomography. Eur Ra dio!. ] 2(l l) :2 770-S, 2002
I,; Hcm otym pa n u m 9. Swartzj l): Temporal bone trauma . Scnu n in US, C f MR
:",
II"
o Ch ro n ic p rese n tat io n 22:219-28,200 1
• C HI. suggests ossicu lar disruption 10. Kim SH ct al: Traumatic perilymphatic fistulas in children:
'"
t': • May a lso p resen t w it h facia l weak ness or etiolog y, di agn osi s lind man agem en t. Int ] Pcdl utr
.
~a
II
p er il ymph atic fis t u la
• Clin ica l profile
o Acute in jury
It.
Oto rhin olaryngol. 20;60(2):147-53, 200 1
Veillon F et al: Imaging of the win do ws of th e temporal
bo ne. Semin Ultrasound CT MR. 22(3):27 1-HO, 2001
12. Dar rou zet V er al: Ma na gemen t of facial par al ysis resu lting
• Fractu re usu a lly in cidenta lly d iscovered from temporal bo ne fractures: Our experience in I IS cases.
a Hea ring loss n oted after recovery fro m acu te in jur ies O lo la ryn go l Head Neck Surg. 1 25( 1 ) : 7 7 ~8 4 , 200 1
• O ccasionally h istory o f trau m a is re m o te 13. Ja ger L ct a l: CT an d i\1R imaging of the normal and
pathologtc conditions of the facial nerve. Eur J Radiol.
Demographics 40(2):133-46,200 1
• Age: All ag es 14. JD Swartz & HR Harn sbcrgcr: Imaging of t he Tem por al
• Ge nder: M > F Bone, .Ird Edition, Th ieme, Inc. e ll. 6, 199H
15. Brod y H A ct al: Management of complications from 820
Natural Hi story & Prognosis tempora l bone fractures. Am ] 0 101. ]8 : 188-9 7, 199 7
• Re la ted to in t racr an ial co mplica tio ns 16 . Alvi A et aI: Trauma lo the temporal bone: diagnosis and
• Associated CSF lea k co m mo n (15 %) ma nagement of co mplications. J Craniomax illofac Trau ma.
o Vast m a jor ity reso lve spo ntaneo us ly wit hi n 7 days 2(3):36-48. 1996
o Pe rsist ing CSF lea ks b eyo nd 7 days requ ire su rgery
a 10 l Yb or < develop meningit is
• Assoc iated 7t h n er ve in ju ry (10%) : Most reso lve
spontaneously
IIM AG E GALLERY
(/1 ft ) Coronel bone CT

shows transvt'r.~( ·
ffacture
through fundus of lAC
(arro w) with itwotv enwm of
2
bibi/.If tu rn of cocbteo (opt'n
.1f((JW). Absence of Illidd/t· 199
ear fluid sugg<'.\rs ctmsnic
f1.1lUre of (faU II't'. (lligh t )
A x;al bone C1 reveals
fon gitLJdin.J1 frac tu re
(an tt>rior suhI Y/)(_') li mited Co
mas/oid (arr o w s) but
(,'/ilending medially .H felr .H
tho "ditu.~ dd antrum (ope n
arr ow ) .
(lLlt) A X;JI bone C T ((''0'(',115

lr,105'o'( '(5(' irs cuno (/.1ft >fal
sulltyp(' ) involving f,Jhyrinth
(''''len ding from pos tonor
p d m ll\ \ Urf.1Ct' (arro w) 10
\'l 'sri/) ult-' (op pn .u ro w ),
M iddle l 'df fluid from
p('(ilymphatic fi!itula. ( Hig h l )
, h i,ll hone C1 c/emomtr.JIi'.'
comp lete incus dis/oc.llion
with incus ;'agml·nt Itxfg('(!
within extern.lf ,w (/ilory
ca nal (arrow). Tympanic
nJf'mbr.lne rupture! W.H
.1~sc)Ci,ltf'd.
(/ .RflJ Axi,l / bone C I fl 'Vl'< lfo,;

abnormal widen ing of
fll.lllf'fJincu dJI Jrl ;cu /.l liofl
c()mi_~ terJt wir1l1 r,W m.ltic
di~ /oCd liofJ (arr o w) . P"ti('(l r
wa s im.JgC'd I}( 'c.lu.~e u f
per sistent co nduc tive hm ring
lo ss a fter head trauma .
(His:I!' ) A " i,)1 shows trau mat ic
di~/oc ation o f incus (.m o w)
rota ted clockwise to impinge
upon p'm.imalcymp.lnic
wgmpnt o f facia l nerve ca nal
(o/X'n .mow). O IL .':. eN7
p.Jr.llys;s were presenting
symp to m s.
CSF LEAK, T-BONE
2
200
Coronal T2WI MR shows legmen defeel (arrow ) with Axial T-bone C T show s mas coick'Ctomy with iatrogen ic
protrusion o f cerebrospinal fluid & brain tissue (ope n defect of anterior wall of attic (arrow ).
Long history of chronic
arro w ) into masto id. otitis mf:'dia MeningoetlCfphdfoct!/e protrudes into cavity (open
but no trauma or surgery! arrow). CSf /('\ '('/ no ted (curved Jrrow).
ITE RMI N O LO GY CT Findings

Abbreviations and Synonyms • NECT
o Congen ital-developmental CSF leak
• Cerebrospinal fluid (CSF) o torh inorrhea, CSF fistula • Inn er ear anoma ly with large coc hlear apert ure ±
Definitions absen t modiolu s (exam ple = Xvlin ked deformity)
• CSF leak into pn eumatized portion of temporal bone • Arach noid gran ulatio n in tegmen area
d ue to di srupt ion of bo th cortica l bo ne I< d ura o Post-trau m ati c CSF leak
o Co ngen ital-develo pmental CSF leak • Transve rse fract ure m ore likely culprit
• Arach no id gran ulations are im plicated • Fracture usually thr ou gh tegm en tympani
• Trans laby rin th ine or perilabyri nt hine • Fractur ets) through cor tex leadi ng to aerated
o Acq ui red CSF leak 'l-bone
• Post-traumatic CSF otorrhea: Disruption of • Opaci fication of m iddle ea r I< mastoid
tym pan ic mem brane (TM) • Tegmen tym pani dehi scence
• Post-trau mat ic CSF rh inorrhea: Int act TM; CSF MR Find ings
egress via eus tach ian tube • TJ WI: Tegm en defect wit h fluid signa l in attic ±
• Iat rogen ic CSF leak: Followi ng ret rom astoid protrud in g tempor al lobe
craniecto my o r tym panom asto id su rgery
• T2W I
o May show other fluid in m iddle ca r-ma stoid
o Congen ita l-developme n tal CSF leak
IIM AG IN G FINDING S • Inner ear anom aly
Ge ne ral Features o Post-traumat ic CSF leak with tegmen dehiscence
• MR defines co n tents of cepha locele
• Best diagnostic clue: For post-traumati c CSF leak: Fluid
in m iddle ea r persists following T-hone trauma Imaging Reco mm en d at ions
• Location: Any intracrani al surface of tem poral bon e • Axial I< coronal f -bone CT
• Size: Varies from subtle to ob vio us o Reformations m ay be necessary as coronals
importan t
DDx: Ca uses of Midd le Ea r Fluid
X-Linked D eformity O to m astoid itis Stapes Fx with PLF Later alizerl Pro sth esis
CSF LEAK, T-BONE
Key Facts
Termino logy Pathology
• Arach no id granu latio ns a re implicat ed • End ocho nd ral bon e heals by fibro us u nion; r
• Post-traumat ic CSF otorrhea: Disruption of tympani c inciden ce o f CSF leak
membrane ('I'M)
Clinica l Issu es
• Post-t raum ati c CSF rh inorrhea: Intact TM; CSF egress
via eustac h ia n tube • Most CSF leaks resolve spo n ta neously 2
• Iat rogen ic CSF leak: Followin g ret romastoid Diagn ostic Checklist 20 1
cran iecto my o r tympa nomastoid su rgery • Tegmen thi nning co m mo n nor ma l varian t
• Axial & coro na l en ha nced MR

o Used if suspect ceph alocele from cr Treatment
o Used wh en meningit is suspec ted • Prophylactic an tibiotics not recommended
• Surg ical repair
o In trath ecal fluo rescein used for detecti o n
IDI FFERENTIAL DIAGNOSIS
Perilym ph a tic gu sher IDIAGNOSTIC CHECKLIST
• Profuse CSF flow after sta pedec to my
Co nside r
Perilymphati c fistula (Pl F) • T-bo ne as sou rce o f leak eve n if CSF rhi n orrh ea
• Abnormal inner ea r to m idd le ear co m m un ication
o Con gen ita l: Stapes malfo rmati ons Image Interpretatio n Pea rls
o Tra umatic: Tran sla byrin thi ne fract u res • Tegme n thi n n in g com mo n normal var ian t
o Spo n tane ous: Without know n cause
o Iat rogen ic: Malfu nct ion ing stapes p rost hesis
I. Cha n DT ct al: Ho w useful is glucose detect ion in
IPATHOLOGY d iagnosing cere brospina l fluid leak'! The rationa l use of cr
311t1 Bet a-Z transferrin assay in detection of ce rebrospi nal
Ge nera l Features Fl uid fistula. Asian J Su rg. 2 7( 1):39- 42, 2004
• Gene ral pa th co m me n ts 2. Rupa V et al: Adult onset spon taneo us CSf o torrhea with
o Ana tomy : Barr ier between 'l- bonc Sr brain ova l window fist ula and recurrent men ingit is: MRI
find ings. Oto laryngo l Head Neck Su rg. I 24 (3)::i 44-6 , 2(XI1
• Teg me n tym pan i forms floor of midd le crania l 3. Patel RB ct al: Spo nta neo us cere brospinal fluid leakage and
fossa m idd le ear enceph alocele in seven patien ts. Ear Nose
• Sigmo id sin us pla te for ms an terior wall o f Th roat }. 79 (5):372 .3, 3 76·H, 200 0
poster ior cran ial fossa 4. Ston e)A et al: Evalu at io n of CSF lea ks: high-reso lution CT
• Dura faces brain, m ucosa faces middle ea r co mpared with co n trast-e nhanced CT an d radionuclldc
• Etio logy cisterno graphy. A)N R Am J Neuro radio l. 20(4) :706--12,
o Post-trau mat ic CSF oto rrhea 19 99
• Endoch o nd ral bon e heals by fibro us union ; I
in cidence o f CSF leak
• Tegm en defect most co m mo n IIM AG E GALLERY
ICLIN ICA L ISSUES

Presentation
o Fluid leakin g from EAC: High index of suspicion
o Othe r signs /sy m pto ms
• Recurrent men ing itis
• Co nd uctive hea ring loss
• Laborator y test for CSF: B2·t ran sferrin = prot ein foun d
in CSF, perilym ph I< aqueous
Nat ural History & Prognos is (1£11) Coronal T,oolJ (> C T revC'als wide tegm t>n de (C"ct (arrows) in
• Most CSF leaks resolve spon taneously patient following radical m astoidecto my with abnormal tissue
• Surgical repai r is exce ption, not ru le protruding through dehiscence (open arrow). MR shol\'oo
cephalocele. (Righ' ) Axial I-bone C T shows trscusrc through emonor
waif o( m iddle car (arrow). A/mormal fluid throughout ma.5loid .
Associated ossicular disruption (open arrow) also present .
FIBROUS DYSPLASIA, T-BONE
2
202
Caron.ll bone CT !ohO\V5 exp..l flsi/t:> "ground R'ass" Axial lxme CT revt>il/~ "p,]gt't()id~ (K/erotic ll\ ~ tic)
fibrous drll',Hia ~ ;()fJ (arro w) involving pm l eri()( variety of [i/)(()u~ cfyspl.Hia. Elip.l mil(' aVx'Ct of thi.~
superior tn.Jstoid <fIu1 p t>lTOUS apl'x (O/x 'fJ arrow). lesion m croaches on the ppilymp.1IJum (arrow ) and
C Uf\fl ! emxv: Ma.\ toid (.lciJ/lll""-' canal. mastoid JnfrUl l J (open arrow ).
o FD co n form s to gene ral sha pe of affected hon e

ITERM INO LO GY o Af fected bon e en larges ( ' volume)
Abb reviatio ns a nd Syno nyms CT Findings
• Fibro us dysplasia (I'D) • N ECT
• Mono stot ic fibro us dysp lasia o f tem po ral bo n e o Appea ran ce relat es to relative co n te n t of fi brous
Defi nit ion s versus osseous tissue
• FD: Bo n e diso rder cha racte rtzed by progressive • Pageto id = hot h fibrous and osseous com ponents
rep lace ment of no rmal bo n e ma rrow by mi xture o f • Sclero tic = more osseous
fihrous tissue & di sorga n ized bo n y t rabecu lae • Cystic = mo re fib rou s
• Albri gh t synd rome: Tri ad of fi brous dysplasia, o Pagetoid I'D (50%)
cafe-au-lair spo ts & e nd ocr ine dysfunction wit h • Mixed o sseous &: fib rou s co m pone n ts
pr eco cio us p ubert y • Bo n e cr shows eit he r classic "gro und glass" or
m ixed scle ro tic-cystic appea ran ce
o Sclero ti c I'D (25%)
IIM AGIN G FINDINGS • Predomi na n tly os seo us co m po ne n t
• Bone Cf shows d en sit y a pp roac h es co rtical bone
Ge ne ral Features o Cyst ic FD (25%)
• Best d iagn o st ic clue: Bo n e cr sh ow s increased bone • Predo mi na n tly fib rou s co m po ne n t
vo lume wit h "grou nd glass" a ppea ra nce • Bone CT sh ows hypod cnsc lesio n
• Locati on • Cystic FO can m im ic o th er hone tumo rs
o May affect a n y bone ill bod y o Disease activ ity may relat e to CT a p pea ra nce
• Skull, skull base & facial bones in vo lved 2S% wi t h • Cystic, pag eto id & scle rotic FO ma y represent
monostoti c I'D I< 50% wit h poly ostoti c I'D most active to lea st ac tive
• Size o All va rieties of FD c ha racte rized by in creased bone
o Locali zed or d iffuse vo lu me
o Lesio ns may reach man y em in size • CECT
• Morphology o Co n t rast not need ed o r reco mmen d ed
o If used , FD will ln hom ogcn eously en hance
DDx: Other Otod yst ropies and Mimics
Oeteopeuosis Pagel Di5eJ5e Otosclerosis (O W) Lab. Os sllice ns

Key Facts
Te rm inology • Increased rad ionuclide accum ulati on seen o n
• FD: Bone d isorder c ha rac te rized by p rogressive perfusion and delayed hone phase
repl acem ent of n orma l bon e m arro w by m ixture of Pathol ogy
fibro us tissue &. di sorgani zed bony tr abecu lae • Monostot ic FD is ox m or e co m mo n th an po lyo stot ic
Imaging Findings •
•
M on ostotic form (75%): Skul l & face 25% o f tim e
Polyostoti c form (25%): Skull & face 50% o f tim e
2
• Best diagn ost ic clue: Bone c r shows inc reased bo ne
vo lu m e w it h "gro u nd glass" ap peara nce • Pagetoi d (SOI)h): Mixed sclerotic &. fib rou s zo.
• Cystic, pagetoid &. scle rotic FD ma y repr esen t most • Sclerotic (2S'MI) : Pred omlnnnrly sclero tic
ac tive to least ac tive • Cys tic (25%): Predominantly fib rou s
• All va rieties of FD ch a racte rized by in creased hone Clinica l Issu es
volume • Gender: M :r = 1:3
• Co n t rast not n eed ed o r recommended • Most spo n ta neous ly "bu rn o ut" o r cease to g row by
• Ma y show a reas o f avid en ha ncemen t wh ich age 20-25
co rrela tes with in creased ac tivity
• C lin ica l set ting of 'l-bo ne in fecti o n ver y d iffe ren t fro m
MR Findi ngs fibrous d ysp lasia
• T1 WI
o Expa ns ile lesio n with low signal o n bot h T l &. T2 Oss ifying fib ro ma
images • Cys tic form o f FD m im ics
o Foci of T l h ypersign a l do occ ur • li as a ty p ica l th ic k, bo n y rim N. lowe r den sit y cen ter
• Hi gh signa l foci may be fro m tra pped fa tt y
mar ro w o r hemorrhage Gia nI ce ll tum or
• T2 W I • T B lesion no t ra re
o Low signa l is pred o m in ant findin g • G ia n t ce ll tum or may be indisting u ishable from cysuc,
o Pat ch y h igh signa l withi n FD lesio n poss ible monostotic FD
• Co rrelates wit h in crea sed ac tiv ity
• FLAI R: No ns pec ific d imi ni shed signa l
• '1' 1 C+ IPATHOLOGY
o May show a reas o f a vid en ha nce me n t w h ich
co rrela tes with increa sed actlvit y
Ge nera l Features
o In active pha se, hete rogen eo us en ha nce me n t of te n • Ge ne ral pa th co m me n ts: Il ene d iso rde r chara ctcrrzcd
by progressiv e rep lace me nt o f no rm al bo ne ma rro w by
present
mi xture o f fibrou s tis su e N. d iso rgan ized bon y
Nu cle ar Me di cine Fi nd ings t rabeculae
• Bone Sca n • Ge netics: Sporadic gene m utat ion
o Increased radio nucl lde acc u m u latio n see n o n • Etiology: All cells d escended from t h is mutat ed cell
pe rfu sio n a nd d ela yed bo n e p has e ca n man ifest feat ures o f mo no stotic o r polyosto tic FD
o No ns pecif ic find ing • Epidem io logy
o Sen sit ive to exte n t o f ske leta l lesion s in pol yostotic o Monosto t ic FD is 6x m o re co m mon t han polyost o tic
rn o Monostot ic fo rm (75 IMl): Skull N face 25 1Ml o f tim e
o Pol yost otic form (25%) : Sku ll N. Iacc 50% of ti me
Imaging Re com mendati ons • Asso cia te d ah nor ma lit ies: Albrigh t syndro rne » 1"0
• T hi n- sectio n CT in axia l &. co ro na l planes wit h ca fe-a u- lai t spots , endocr ine d ysfu nct io n w ith
• MR shou ld be reser ved fo r unu sua l cases precocious pu be rty
o W he n process is un usu ally aggressive o r associat ed
wit h u nexp lai ned SN HL Gross Pat ho logic & Su rgica l Features
• Tan -yell ow to w hi te lesio n
• Vari abl e co nsis te ncy from so ft- ru bbe ry to g rit ty -firm
IDIFFERENTIAL DIAGNOSIS depending on fib rous ve rsu s osseo us co n te n t
Page t disease Microscopic Feature s

• Paget o id g ro u nd-g lass FD m im ics Paget d iseas e • FD lesion co n ta ins fibrous tiss ue wit h in t ra m ura l bone
• In vo lves T-ho ne &. ca lva rium, no t cra nio fac ia l a rea trabecu lae
• "Cotto n-wo o l" CT a ppea ra nce &. o ld age suggest Paget • Fibro us stro ma is myxofibrous ti ssu e o f mix ed
• Hearin g lo ss much m or e seve re in Paget vascu lar ity
• OSSl'O US metapl asia c rea tes ho n e t rab ecu lae mad e up o f
Osteo mye litis of T-bone imm ature, wo ven bone
• Cys tic FD may mi m ic o See n as pecu liar shapes floatin g in fibrou s stro ma
o May cause ma lign an t t ransfo rma tion
Stag ing, Gra d ing or Classification Crite ria
• Pagetoid (5(~*,): Mixed sclerotic I:< fibrous
• Sclerotic (25%) : Predo minantly sclerotic IDIAGNOSTIC CHECKLIST
• Cystic (25%): Predominantly fibro us
• All va rieti es FD characteri zed by in creased bone
2 ICLINICAL ISSUES vo lu me
204 Presentation
• Most co mmo n signs/sy mpto ms ISELECTED REFERENCES
o Bulging of tempo ral area
o Stenos is o f EAC with recu rren t otit is I: Gup ta A ct 31: Large cyst ic fibrou s dys plasia o f the tempo ral
bo ne : case report a nti review of Hteraturc. ) Clin Neurosci.
o Hearin g lo ss: Co nd uctive, senso rin eural o r mi xed 10(3):364-7, 2003
• Clinica l p ro file 2. Fakh ri Set a l: Fibrous dyspla sia of the tem por al bc ne. j
o M:F = 1:3 Otolaryngol. 32(2):132-5, 2003
o You ng affec ted « 30 yea rs o ld) 3. Ozbck C et al: Fibrous d ysp lasia o f th e te mporal bo ne. Ann
• 3 presen ta tions: Monostotic, pol yo sto tic & Olol Rhinoll.aryngol. 112(7):654-6, 2003
McCune-Alb righ t syndrome -I. C hee GH et 31: Fibrous d yspl asia of the tem po ral bo ne.
o Monostotic FD Otol Neu ro tol . 23(3):405-6, 2002
• 70% of all FD cases; single osseous site is affec ted 5. Lusti g lR e t a l: Fibrous d yspla sia involving th e skull base
a nd temporal bone. Arch Ot olaryngol Head Neck Surg.
• Older child ren &: young adu lts (75% p resent 127(10):1239-41, 2001
befo re age 30) 6. Papadakis CE e t al : Fibrous d yspl asia of the tem pora l bone:
• Skull base I:< face inv olved in 25%; maxilla report of a case a nd a revi ew of its cha rac te ristics. Ear Nose
(especially zygoma tic process) I:< mand ible (molar T'hroat ] . 79(1):52-7,2000
area) > > fro nt al bo ne > ethmoid I:< sp he noid 7, Mag llulo G et al: External cholesteato ma and fibrous
bo nes > 'l-bone d ysp lasia of temporal bon e. An O torrtnolar ingo llbcro Am.
• May be asy m p to matic, inciden ta l im aging 27(-1):315-22,2000
find in gs 8. Falclo ni M ct al: Hbrou, d ysp lasia of the tem por al bone .
AmJ Otol. 21(6):887-8, 2000
• Other sym pto ms nonspcciftc: Pai n, fo cal swelling 9, Degutne C et a l: Fibrous d yspl asia o f t he tem po ral hone.
&. tenderness Ea r Nose Throat J. 79(11):834, 2000
o Po lyo sto tic FD 10. Xencl lls ] ct al: Mo nostotic fibro us dyspl asia of th e
• 25% of all FD cases; involves ", 2 separate osseous temporal bone. J Laryngol Otol. 113(8):772-4, 1999
sites I I.Palacios E ct al: Fibrou s d ysplasia of th e temporal bon e. Ear
• Skull base &: face involved in 50 % No sc Throat ] . 78(6):414-6, 1999
• Younger patien t gro up, mean age at diagnosis o f 8 12. Ch inski A et a l: Fibrous d ysplasia of th e temporal bone. lnt
yea rs J Pediatr Olorhi nolaryngol. 15;47(3):275·81, 1999
13. Morrissey DO e t al: Fibrou s d ysp lasia of th e temporal bone:
• 2/3 have symptoms by age 10 incl uding reversal of senso rine ural hear ing loss aft er decompression
craniofacial asy m me try of the internal aud itory canal. Laryngoscope.
o McCu ne -Albright syndrome 107(10):1:1:16--10, 1997
• Subt yp e of polyostotic FD de fined by clinical t riad 1-1. l ee WH et at: Flbrouv d yspl asia: :VIR imaging ch aracteristics
of polyos toti c FD (usua lly u nila te ral), endocrine with radi opathologic co rrelation. A]R. 167:1523· 7, 1996
dysfun cti on I< cut aneous hyp er pigrnen tatio n 15. Megerta n CA et al: Fibrous d yspla sia of th e tem por al bone:
(cafe-a u-lait spots) ten new cases demonstrati ng t he spectrum of o to logic
seq uelae. AmJ Otol. 16(-1 ):408-19, 1995
• S% o f FD cases
t 6. Cassel ma n jw et al: M RI in cra niofacia l fibrous d ysplasia.
• Appears ea rlier I< affects mo re bo nes more seve rely Neu rorad io logy. 35 :23 4· 7, 199 3
De mog rap hics 17. Kessler Act al: Fibrous d ysplasia of t he tempor al ho ne
presenti ng as a n os teo ma o f t he ex ternal aud itory canal.
• Age : Mo st act ive in yo ung patien t, typ ically q uiescen t Ear Nose Th roat j . 69( 3):197·9, 1990
afte r pu berty 18. Talm i VI' et al : Rad io logica l case of th e mo nth . Mo nostotic
• Gende r: M:F = 1:3 fibrou s dysplasia of th e te mpora l bone. Am ] Dis Ch ild.
1-I3(1t ):13S1 -2,1 989
Natural History & Prognosis 19. Pouwcls All et .11: Fibrous dysplasia o f the temporal bone. ]
• Mo nostotic cra niofacial FD has an exc ellen t prog nosis la ryngol 01 01. 102(2):171-2. 1988
• Most spo n ta ne o usly "bu rn o ut - o r cease to grow by age 20. Smouha EE et al: Fibrous d ysplasia in volving t he temporal
20-25 bone: report of three new cases. AmJ 01 01. 8(2):103-7,
• Polyostotic FD rarely life threaten ing but has poorer 1987
prognosis
Treatm ent
• Aggressive surgical man agem ent not reco m mended in
most cases
• No n-dis abling surgical interven tio n is u tilized when
safe
• No rad iati on th erap y!
IIM AG E GALLE RY
Typical
tu ft ) Axial bone CT reveals
sclero tic varie ty of fibr ous
d ysplas ia (arro w) . I f' s;(m
e.\p,msion ceases [ A e
2
s lenosi.~ (OIX'fl arr ow).
Cur ved arrow: Site of 2 05
pre\'i ou s exci~i()nJ I bi opsy:
(Right) Coro,,,, 1 hmw Ct
shows sclero tic fD with high
grade stenosis of external
au dito ry cenet (arrow)
,esulting in se veve
conductive nesting deficit
Typical
(l~fl) AX;<11shows sctennic
vJr;f..·t y of FD w ith in ante rior
portion of pet ro us be ne
en croclc hing on the m ic/rIlL'
('.If and ossicular chain
(,l rrow) resulting in
con duc tive hearing deficit.
(Rig" l ) Axial show s {'ys/ie
v,l r;ety of FD involving both
petroas (arro w) & squamous
(ope n JrrolV) t -bonc. This
l)'lX ' of fibrous dysp'.JSi.l
ma squerades as moft'
aggtess;ve proc es s likl·
m teom yeJitis.
Variant
(lLf l) A xial bon e CT reveals
an aggressive-appca ring
anterior I(>ft lempo ral bo ne
foci of Nc ystic" iibrous
dysp lasia (arrow s). rhis
lesion W.105 initially biopsied
in search of m.llignant tu mor
his!op. Jtholog y. ( H;I: " I) I h i.11
bone CT shows polyostotic
fibrous d ysplasia at7ffling
right frontiJl bone and
temporiJl bone (.l ffOW).
Multiple other foc; oro
appiJrent including the left
OCcipital bone (ope n .m ow) .
PAGET DISEASE, T-BONE
2
206
Axial lefr. ear l-bone CT shows ngrollnd gless" Coronal T1 Ct- /'viR reveals difTuw Paw,tic c.l/v,lrial
appeclf.1Ilc(' of maslaid (arrows) and pcuoo s c1fX'X lhickefJiflJ.: wi th ifllefJ5e 1}('I f'roge/ leotl .~ p<llllo/(>gic
(open arrow ). O tic capsule (/eminCfaliZJ(io/l (curved enhancemen t of diploic span '. t'1'1rOU5 apex
arrow) a/so HOlt'll. pnhancem(,1l1 (c1rfO\Vs) is alsoapprC'Cialoo.
o Sku ll se ries
ITE RM INO l OGY • Early: Osteo porosis circu msc ripta
Abb rev iatio ns and Syno nyms • Lat e: "Co tto n -wo o l" pa tter n sec o n dary to
• Paget d isease (PO) coexiste nce o f os teolys is & scle ro sis
• Osteitis deforma n s CT Findings
Definitio ns • N ECf
• PD: Bone d ysp lasia c ha racterized by excessive o Sku ll base & calva riu m
remodeling of bone result ing from alternati ng waves • Diffu se h omo gen eou s thi c kenin g of cra nia l base I<
of os teo clastic &: osteoblastic activity calva riu m
• Prog ressive osteo dyst ro phy with mo nostoti c & • Paget oid c ha n ges avo id nose, sinuses &. mand ible
polyostotic va riet ies • Early phase: Dem in e ralization of petrous apex
• In termed iat e phase: "Gro u nd glass" a ppea rance
• Lat e pha se: Ext re me bo ne t h ickening with diploic
IIM AGING FINDINGS hete roge neity
o In n er ea r-otic ca ps u le
Gene ra l Features • Otic ca psu le deminer ali zation (pe riph eral to
• Best diagn ostic clue ce n tral) in vo lves all 3 laye rs (pe rioste um,
o Calva riu m &: cra nia l ba se: Diffu se thi ckening wit h endoc hond ral & e ndoste um )
"cotton-wool" ap pea rance • In vol vement o f peri o steum initiall y
o T-bo ne CT: "Grou n d glass" bo n y chan ges wit h • f o llo wed by en docho n d ra l & fin all y end osteal
th in ning of otic capsule layer
• Location • Bilatera l &: asymm et ric (com pa red w it h cochlear
o Ca lvariu m &. cran ia l bas e o toscle ros is, bilat eral & sym me t ric)
o Invol vem en t of spine, pelvis &: long bones o Ex te rna l au ditory ca nal (EAC) & m iddl e ea r
co m mo n ly associa ted • EAC tortuosit y & ste nosis
• Mi dd le ca r ca vity co n st rictio n
Radiograph ic Find ings • Page to id oss icu la r in vo lvement
• Rad iog ra p hy
DDx: Bony l esions o f th e T-bone

, "" , ,
~tj'."~r~
Otosyphilis Osteo radion ecro sis Cochlear O tosclerosis
C
Fibrous Dysplasia
Key Facts
Terminology • Osteo gen esis Impcrfecta
• PD: Bon e dysplasia cha racterized by excessive • Fibrou s dysplasia
remo d eling o f bone resultin g from alter na ting wav es Pathol ogy
of oste oclastic & osteoblastic activi ty • Marrow- co n ta in ing struc tu res a re in vo lved first
Imaging Findi ngs • Pet rou s a pex undergoes in itial ch a nges
• De mineralizati on of o tic ca ps ule & enc roac h me n t
2
• Earl y pha se: Dem inera lizatio n of pet rou s apex
• Inter med iat e phase: "Gro u nd glass" a ppea rance upon mid d le ea r occ u r late 207
• Late ph ase: Ext reme bon e thi cken in g wit h dipl oic Clinical Issues
h ete rogen eity • Age: Onse t un commo n befor e age 40
• Otic capsule dem ineralizatio n (peripheral to central) • Ge n de r: M:F = 4:I
involves all 3 layers (perioste u m , e ndocho n dra l &
en dos teum) Diagnost ic Check list
• Earliest CT findin g is "groun d glass" d emineralizati on
To p Diffe rential Diagnoses o f pctrous ap ex
• Otosyph tlis
• Coc h lea r o toscl ero sis
o In vol vem e n t o f o tic capsu le usua lly bilat eral,

MR Findings sym me t ric
• T1WI o Ad jacen t sku ll base & calvariu m no rmal
o Dimin ished TI signal • Fe nestra l o toscl ero sis prese nt wit h coch lea r
• Marrow repl acem en t by fibro us tissue o toscle ros is
o Heterogeneo us pat chy '1"1 hyperint ense signal or
hi gh slgna) O steogenesis imp erfecta
• Areas o f h em o rrhage & slow flow in vascu lar • C lin ical: History o fte n da ting to ch ild hood ; bl ue sclera
cha n ne ls . o Recu rrin g fractu res a nd skeletal deformity
• T2W I: High -reso luti o n T2: Shows ab no rmal h igh • CT: Findi ngs sim ilar to seve re coc h lea r oto scle ro sis
sign al o r h yp er intense signa l; nonspecific
Fi brou s dysplasia
• T1 C+
o Hete rogen eou s e n h a nce me n t wit h in thicke ned • C lin ical: You nger patient
calva riu m, sku ll base ± T-IJOne possib le • CT: In creased bon e vo lume
• Seco n da ry to h ypcrvascu lar n atu re o Com mon ly in vo lves facial bones
o Men in geal en h anceme n t has also been rep o rted
• May reflect incr eased met aboli sm &. blood flow of
PD lesions IPATHOLOGY
Imaging Recommendati ons Gen er al Features
• Temporal bon e C1' o n ly st udy 1H..-eded to ma ke • Ge ne ral path co m me n ts
di agn o sis o Mar row-co nt ain in g st ructures a re in vo lved first
o Axial images mo st im po rtan t • Pet ro us a pex u nd ergoes initial ch ange s
o Co ro nal im ages often di fficult to o bta ine d in t hese o Dem in eralizati on o f o tic ca psu le &. enc roac h me n t
elder ly pa tien ts with sku ll ba se d eformity u pon m idd le ear occur lat e
o Maligna n t degener at io n - 1% o f cases
• Osteoge n ic sa rco ma most co m mo n
IDIF FERENTIAL DIAGNOSIS • Usually occurs in po lyosto tic disea se
o Degree o f senso rine u ral h earin g lo ss co rrelates wit h
O to syphilis d emi n era lizati on o f o tic ca psu le
• Clin ical: Syste m ic illn ess o Dysp lastic hone serves to da mp e n motion o f
• C l: Diffuse d emin eralization o f otic ca psu le appear s mech an ica l clements of midd le ea r
"mot h -eate n" • Genetics
• MR: Labyrin t h in e en h an ce men t at T l c+ o Mostly spo rad ic
o 15')(, inci dence o f a utoso mal do mina n t inhe ritan ce
Os teo radio necros is pa tt ern
• C lin ical: Rad iation hist ory key • Defects in ch romosome 6 & 18q im plicated
• C'l: De min era lizatio n o f cra n ial base simil ar to PO • Etio logy : Nuclear viral inclusio ns suggest viral etio logy
o Otic ca psu le in volvem en t more permeative • Epide m io logy
Co chlea r oto sclerosis o Affects 3% o f populati on > 40 yea rs of age
o Affect s l OlJ{, o f po pu lation > 80 yea rs of age
• Clin ical: Much yo u nger pat ien t co m pa red wit h PD
• Cf: Oe mi n era liza tion o f o tic ca psu le typ ica lly • Assoc iated abno rma lities : Cha racte ristic invo lvemen t
of ve rtebral', pelv is & lo ng ho nes
p laq ue-like
M icro scop ic Features IDIAGNOSTIC CHECKLIST
• Th ree ph ases observed
o I: Osteoclastic de structi on o f haver sian syste ms
Co nside r
o II : Repeat ed mi xed osteoclastic-osteobl astic activity • Osteorad io nec ros is if clin ica l hi stor y is appropriate
• Newly formed bo ne less den se & st ruct u rally • Fibrous d yspl asia if in creased bon e vo lu me
substa n da rd • O tosyp h ilis in eld erly pat ie n t
2 o Ill: Sclero t ic phase Image Interpretation Pearls
• Earliest CT find in g is "gro u nd g lass" demin eralization
200 of pet rou s a pex
IC LIN ICA l l SSUES • Ca refully stud y thi ckness o f o tic ca psu le
Presen tati on o Ot ic ca psu le demineral ization closely co rrelates with
d egree of h ea rin g loss
• Mo st com mon sign s/symp to ms
o Mixed hearing loss
• Ofte n advan ces mo re q uic kly t h a n presby cusis
o Senso rine u ral loss etio logic facto rs are multiple
• Hair cell dep o pulatio n 1. Do n at h ] et a l: Effect o f b isp hosphon at e treat ment in
• Arteriovenous vascular sh unts pa t ien ts wi t h Paget's diseuse o f th e sku ll. Rhe uma tology
(O xford ). ~ :J ( 1):89- 94, 200 ~
• O tic ca psu le m icro fracturcs
2. Sakai 0 et Oi l: Otosclero sis a n d Dysplasia of t h e Temporal
• lAC narrowing Hon e In Scm I' an d Cur ti n II, Head a nd Neck Imaging, -lth
• Aco ustico facia l bu nd le elo nga tio n ed it ion. Mo sby. In c. 12 77- 1360, 20 0] 2(XH
• Microneu romas :1. Hul lur T E e t al: Paget' s d isease and fibrous dy splasia.
o Co nd uctive Joss et io log ic fac tors a rc m ulti ple Otola ryn go l Cli n Nor t h Am . ]6(-1):707 -32 , 2003
• EAC ste nosis 4. Uppa l li S ct ill: Osseo- In tegratt o n in Paget's dis ease: the
• Tym pan ic mem b ra ne (TM) fibros is a nd ho n e-anch or ed h ear in g aid in th e rehabilit at ion of Pagcttc
ossification d eafness, J I.aryn go l O tol. 115 (11 ):903-6, 200 I
.I. Nabil i V et ill: Rad iol ogy quiz cas e. I.ahyrint h ine
• Ossicular fixa tio n and impingem ent
o blileration du e to Paget di seuse. Arch O to lary ngo l Head
o Other signs /sympto ms Neck Surg. 12 7(9 ):1 U 7-9, 2{)() 1
• Tin n itus (intraossco us a rterioven o us sh un ts) 6. Va nde Be rg BCo et al: Magn etic reso n ance ap pea rance of
• Vertigo (20%) u n com p licate d Paget 's di sea se of bo ne . Scmi n
• He mi facial spas m Mu sculoskele t Radi o l. 5( 1):69 -77. 200 1
• Trigem ina l ne uralgia 7. Teh ran zad eh ] e t a l: Com p u ted to mog ra phy of Paget
• Clin ical profil e: Progressive b ilat eral mi xed h earing d isease o f th e sku ll ve rsu s fibrous dysp lasia. Skeletal Radiol.
loss in elde rly pat ien t 27 : 6 6 ~ -72, 1998
• Lab or at o ry abno rm alit ies 8. Swanz ju and Harn sherger II R: Im ag ing th e Tempor al
Bo n e, Cha p te r 5, Th ieme, Inc, 1998
o Eleva t ion o f se ru m al kali n e p h osphat ase
9. Mo n sell EM et al: Hear in g loss in Page t's disease o f bo ne:
o Eleva t io n of ur in ary hyd ro xpro lin e Ev iden ce o f auditory ne rve int egrit y. Am] Otol. 16:27-33,
199 5
D em ographics 10. lu g EB et al: Hem ifacial spasm and o stei tis defor mans. Am]
• Age: Onset u ncommon before age 40 Ophthal mo l. 119(:1):3 76-7, 1995
• Gender: M:F = 4:1 11. Mo n 'iC'11 EM ct al : Heari n g loss in Page t's di sease of bon e:
till' relations hip be twe e n pure-tone t h resh o lds and mineral
Natural Hi story & Prognosis density o f th e coc h lear capsule. " car Res. S3( 1-2): 11-4-20,
• Diso rd er usual ly prog ressive desp ite th erapy 19 95
• Progressive hlla tera l m ixed h ea ring loss ofte n leads 10 12 . Chole RA: Differential os teocl ast act ivation in
total deafn ess en doc h o n d ral a nd in t ra membra n o us bone. An n Otol
Rhi n o! l.aryn gol . 102(8 Pt 1):61 6-9 , 199 :i
Treatm ent 13. Ram say t-1 A et ill: Coc h lear hi stopath ol ogy in Paget's
• Calciton in ina ctivat es osteoclasts, ma y stab ilize di sea se. Ain J Oto lar yn gol. 14(1):60· 1, 1993
hea rin g 14. Schukne ch t HI': Myt h s in n curo to lo gv. Am ) Ot ol.
1 3 ( 2 ) : 1 2 ~ - 6 , 1992
o Enzymes such as alkaline phosph a tase typ icall y
IS. Kh ctar pal U ct a l: In sea rch o f pat h o logic correla tes for
ret urn to m or e normal levels follo wing th erapy h ea rin g los s am i ver tigo in Page t's disease. A clin ical and
• Sodi u m ctld ro na tc ,1110 othe r d lphosphonat cs h isto pa th ol ogic stu dy o f 26 tem po ra l bones . An n Otol
o In h ihit calciu m de position Rhin o l l.aryngo l Su ppl. 1-1 5 : 1-16, 1990
• Calcito n in a nd sod iu m et id ron a te co m bin at io n 16 . Milroy eM ct al: Pat h ol ogy o f t he o t ic caps ulc . ] La ryngol
o May sta bi lize o r eve n rever se hear in g loss 0 101. 104 (2 ):83-90 , 1990
• Mithram ycin inhibits RNA syn t hes is 17. d'A rcha mbcau 0 ct ill: Cf d iagn o sis a nd different ial
o Throm bo cytope n ia a n d hepatot oxicity limi t use dia gn o sis o f otod ystroph ic It'sions of th e temporal bo ne.
Em ] Radiol. 11(1 ):22-]0, 1990
• Sur gery of limi ted value
o l'ro sthet ic sta ped ec to my ge ne rally di sappointi ng
o Surgery fo r lAC ste n osis is ha zardous
IIMAGE GALLERY
Typical
(l~JI) 11,,;.11 h.'f, <'<1( T-hOf1f'
,e\l('J /~ f',lI ly c/emineraliLi ng
ph .me> of P,I/WI diwsse 2
involving lhf:' l X'110US dpeJi
( .J I ({J ",!» ,JOti p(-riplwI.)1
mastoid (ope n ,m ow ). No 209

booe thick cning Of ("od Jlt',jf
inVO/Yl'menl 5('(-'0. (High' )
Axial t -bone C I shO\\ls
pagetoid d t>mio{,fafiz.lli,)fJ of
the s"'ul/ bsse. ('spt'CiJlly the
fX'lrOU S ape_ (,1110\\,5 ) . ttwre
is etso de m;fIt 'f JlizJl ion of
the orrc cdp~ u/t' dt the
("'oc hl(>.1( dpt·)f, (op t·o .lffOW ) .
(I .I.'f') A\;a! right('.J( t-bonc
C I shows leter phaH' flagel

<IiSf.'JR' willl elitluw ly
thid.ent·d bone.
[) pmil ll' r" /i7.lI iofJ of petrous
a/lc-'x (.w u w) (~coehled
(o /)(-'f) ,11m",) . fl w m.Jlll'tlS is
.1/<; 0 invo/W'c / (curve-d
drmw). (/li~ /") I \ Xitll T f C+
MR sho ws hemp thickening
with p.l th()/()~ ic in/pmt'
c'nhaf1cemenl o f (J(>trou.~
.ll wX (.)/Ttr.v ) .~ otic C<lp~u/t·
of corh le.) (op{'n ,1rrow si
1)'Pic.,/ of ,.,,tR fin d inM!j o f
Jc!v.1 nu'(/ T-hom' P.)gf.'l
disease .
Typical
tlLf l' A xi.,' TlWI MR (t>w J/s
",.fR li nd ing!j of Jc1Vdnc('d
P<Jgel di.~t>dW . Notin' bony
enlargt>l)J{' nl (.l rrow !j) dnd
cam p /cIt' oblitt'f.Jtion of th e
internal auditory can,l l (open
,m o w ). tlli1:lrt) Co rcmst right
e<Jr I-bone C T shows PJgel
diwsse . 1.5 -gro und gl.HS·
sppcsrence of mmr of
mide//{> S inner { 'Jf bon es.
Diffuse d emineriJliution
(thinning ' o f otic CiI/N lle
(a rrO'lvs) also prest·nt.
OSTEOPETROSIS, T-BONE
2
2 10
l ill ff.l ! f.ldios raphy shol.vs di{fu<;(' thickening 01 Coronal k>ir esr 1-bone asha.vs dense sclercM &
cstvmium .md Hanial beN" char.1ctf·';.l.tk~ of the fTJ()f(' Ihickf.'fJing of f Jf. 'ffOU5 apex & X/UdillOUS fB. St.lpf.~
.w\,...,.e chHdhcKxl dutosonl.ll (t'C l.'SsiV(' form of iJPfJt'tlfS thickC'rK'd (.l rraov). Note t/l(' sm.111, (J,](("C/
rnlt>o(>t·rrosis. int{'m.1/.1LKlitory canal (open arrow).
• Gene ralized I dens ity of en tire skull base

!TE RM INO LO GY including T-bo ne
Abbreviat io ns and Synonyms • Sclerot ic otic capsule beyo nd normal bony
• Syno ny ms: Marble bone disease; Albers-Schonberg labyrint h margins
d isease • Enlarg ed suba rcuate fossa, resu lting in fetal or
in fant appea rance
Definitions • Intern al aud itory cana l (lAC) shor t & trumpet
• Ra re heritable metabolic hon e disease with defectiv e sha ped
ho ne remodeling o AROI'
• Autoso mal Recessive Ost eope trosis (AROI'): Ch ild hood • Type 1: Den se sclerosis of sku ll base & calvarium
form; severe osteopetrosis • Type 2: Den se sclerosis of skull base, spares
• Autoso ma l Dominan t Osteopetrosis (ADOI'): Adult ca lvariu m
form; less severe osteopetrosis • CECr: Contrast not needed o r reco m mended
MR Findings
IIM AGING FINDINGS • T1W I
o AROI'
General Features • Severe calvarial th ickening
• Best d iagn ostic clue: Dense, scle rotic ho nes ("cha lk • Th ickened mastoid co m plex. underpneum atized
bo nes") • T2WI: No specific add itio nal features
• Locat ion : T-ho ne, calvariu m &. en tire skull base • '1' 1 C+: En ha ncing extra-cerebra l spaces »
Extram edu llary hem atopoiesis (AROI')
Radi ographic Find ings • MRA: Petrou s ICA com promise in AROI'
• Radiography: Dense bo ne is usually well appreciated in • MRV: Dur al ven ous sin us ste nos is in AROP
AROI'
Imaging Recomm endations
CT Find ings • Th in-section c r is best imagi ng tool
• NliCl' • MR useful in defin ing associa ted lesion s in AROP
o ADOI'
DD x: Other Otodystrop hies
Paget Disease Fibrous Dysplasia Cochlear O tosclerosis Os teog coes t«

OSTEOPETROSIS, T-BONE
Key Facts
Termino logy Path ology
• Aut oso mal Recessive Os teopetrosis (ARO P): • AROP is less common than ADOI'
Ch ild hood form; severe osteopetros is
• Autoso ma l Dominant Osteopet ros is (ADOP): Adult Clinical Issues
• Ch ild re n with AROP rarel y surv ive ch ild hood
form; less severe os teo pe tros is
Diagn ostic Checklist
2
Imaging Findings
• MRA: Pri mus leA co m pro mise in AROP • Flared lACs « lar ge suba rcuate fossae a re classic 2 11
ID IFFE RENTIA L DI AG N O SIS Treatment

• Tym pa nosto my tubes for midd le ea r effus io ns
Paget disease
• Clinica l: Elde rly pati ents
• Usua lly see n as a d iffuse, "cotto n-woo)" a ppeara nce
• Dem inera lized o tic ca psu le cor relates with SNIIL
IDIAGNOSTIC CH ECKLIST
Fibro us dysp lasia Co nsider
• Sku ll bas e findings ide n tlcal to o steope tros is for
• Relative spa ring o f o tic capsu le
fo llow ing syndro mes
• Lyti c, sclerotic or mi xed
o Progressive d iaphyseal d yspla sia (Engelma n n)
• Increased bo n e vo lu me is ch arac te ristic
o Generalized co rtical hyperostosis (va n Buch cm)
o Cra ruo meta ph yseal dy splasia
IPATH O LO GY Image Inte rpretation Pea rls
• Fla red lACs & la rge subarcuat e fossae a rc classic
Ge neral Featu res
• Ge ne ral path co m me n ts
o Overprod uction o f im mature hone ISELECTED REFEREN C ES
• Osteoclast fu nction is defective
I. Sakai 0 et al: Otosclerosis a nd Dysplasia... of th e Te m po ral
• Ge ne tics
Bone, in Som PM and Curti n HD Head and Neck Imaging,
o Au toso ma l recessive O ( dominant
Four th Ed itio n, 1275· 1360, 2003
• AROP is less co m mo n th an ADOI' 2. C u re J K e t .11: Cran ial t\IR im agin g o f o st eopet ro sis. AJS R.
• Etio logy: Hereditary disord er 2 1: 1110-5. 2()()()
• Epidemi ol ogy: Rar e o todys trop ny 3. Cu rl' J K ct .11: Petr o us carotid ca na l steno...ts in mattgnant
ostcopctrosts: <"1 docu mentati o n wit h MR angiogruphic
Gross Path ologic & Surgical Features correlatio n. Radiology, 199:-l1 S·4 21, 1 9~ 6
• Dense, sclerotic bo nes
• Tlilcke ned co rtex , n a rrowed medullary cav it y
Micro scopi c Features IIM AG E GALLERY
• Persisten t pr imar y hon y spongiosa
IC LIN ICA L ISSUES

Presentation
• Most co m mo n signs /sy m ptoms
o AROP
• Ma rrow rep laced: Ane m ia & neu tropeni a
• Frag ile bo ne s: Fractu res wit h m in or traum a
o ADOI'
• May be asym pto ma tic
• Con d uctive h earing loss more co m mo n t ha n
OLlt) Axialli'ft ear T-bone CT sholVs densf! sclerosis of cranial bs w
SNIIL with highly comp romised middfe fO":U r avuy ,1m/ {'ocro.lchment on
Demographics ossicular ma ss (arrow s). (R iglr l) Axial sJ.. ull b,JW bone CT shows
diffuse sclerosis of entke cranial h,lW. 1here is narrowing of bo th
• Age: ,\ ROP is a ppa ren t in infancy
middle t>.lrs (arrows). Note a/50 compromis e o f each lAC topon
Natu ral Histo ry & Prognosis ,mows).
• Ch ild re n with AROP ra rely su rvive ch ild hood

• Progressive bilutcra l h ea ring loss in ADOr
POSTIRRADIATED T-BONE
2
2 12
A xial/eft ear T,!x)rl(' CT (ollow ing radiotherapy !>how!> Axial T-I)()II(' CT ..flf)'lv" (/('!Jr;s in f1Ids/oid Cor/fCdl bOlly
middll' ear-mastoid debris. Noto S('W/f' elitiiJ,w m.:ugins of IXh l('rior ( 'x/pm,11 .w(/i /ory canal (arrow ) 8
~ rnolh -ea tenn c!cminNa!izatioll ofl)f'trolJ_~ <1!>t>x ("rrow) ;1I1j{J1.1r (o{'Jmm (open arrow) indicafe XR l-ind uced
•1\ n>lJtral_~k.[JII IH,>{· (oJX'{l a llO w). confluent otoH1<1.'itoiditis.
o Radiati on-induced o to mas toid itis. co m plicated

ITE RM IN O l O GY • Coalesce n t masto id it is identica l to tha t caused by
Abbrev iat ions and Syno nyms infecti on alone (coalesce nt o to mastoid itisj
• Osteorad io necrosis (O RN): En d stage on ly • Erosio n & t hinnin g o f ma sto id sep tat lo ns
• Rad iation-indu ced inju ry o f T-h OI1 l' • Disru p tio n of ex te rnal o r in ternal mastoid co rtex
a Focal rad iati on-induced in jury
Defi nit ions • EAC e rosio n with bon y seq ue st ratio n appears
• Injury seconda ry to irrad iatio n (XRT) whe n T..bon e is sim ila r to "maligna n t" exte rna l o ti tis
wit h in t reatment po rtals • l.ocalizcd tympan ic hone o steo radionecrosis
o OS'1'EOI{ADIONECI{OSIS
• End-stage diffuse rad iati on-induced in ju ry
IIM AGIN G FINDINGS • Severe, perm eative, mo th- eat en ap pe ara nce of
bon y l ab yri n th I< usu all y enti re skull base
Ge ne ra l f ea tur es • CECT
• Best d iag nosti c clue: Bone c r sho ws "m ot h-ea te n" o SOIll <.'. en ha nc emen t ma y occur
dem ineralization o f bony labyrinth , tympa nic bone &. • Co n t rast no t need ed o r reco mmend ed
exte rnal aud itory ca na l
• Locat io n: T..hone &. ad jacen t skull base MR fi nd ings
• Size: Foca l o r d iffuse • T1\VI: No ns pec ific low signal in m idd le ear-mas toid
(ef fusi on)
CT find ings • '1'2WI
• NEC'!' o No ns pec ific high signa l w ith in m idd le ea r-mastoid
o Radiation- ind uced o to ma stoiditis, u nco m plicated o High signal w ithi n brain = foci of ischemic necrosis
• Mo st co m mo n form of d iffuse rad iation inj ury to • Tl C+
T-bon e o In flam mat or y granu lauo n will en ha nce
• Diffuse mu co sal thi ckening in external aud ito ry (no nspecific)
can al (EAC), m idd le ear (M E) I< ma stoid o Dem inerali zed a reas in late stages w ill also enha nce
• Mid d le car-m asto id effus io n • MRV: Usef ul in searc h of veno us sin us thromhosis
• No underl yin g bone ab normalities wh en otomas to id itis is co m plicate d
DD x: Radiation Change Mimics
Ac ute External Otitis Coalescent Mastoidi tis Olosyphilis Paget Disease

Key Facts
Term inology • Avascular necrosis result s from bloo d vessel exposure
• Osteora d ionec ros is (O RN): End stage o n ly to h igh dose XRT
• Radiation- i n duced i n ju ry of T-bon e • Oto mas toi ditis: Muco sal hypert ro ph y, loss of ciliary
• In jury seco nda ry to irradiat ion (XRT) whe n 'f-bo ne is function & eustach ian tube obstruc tion
within treat ment portals Clinica l Issues
• Loca lized XRT in jury ave rage lat ency pe riod = 12
2
Imaging Findings
• Best diagn ostic clue: Bone CT sho ws "mo t h-eate n" yea rs
d em ineraliza tion o f bo ny lab yrin t h , tympani c bone • Diffuse XRT in jury ave rage laten cy period = 8 yea rs
&. exte rna l aud ito ry canal • Rad iati o n-in d uced t u mors are also d elayed
• Radia tio n-ind uced o to masto id itis. un complicat ed complication
• Rad iatio n -i n duced oto ru asto id it ts, co m pl icated
• Focal rad iation-ind uced inj u ry
• OSTEORA DIONECROSIS
Path ology
• T-bo nc at hig her risk than most tissues
• Etiolog y
Imaging Recom men dati on s o Avascular necrosis result s fro m blood vessel
• Th in-sec tion axial & corona l bo ne c r ex pos ure to high dose XRT
• Mit useful for co m plications of o to masto id ltis o Degen erati ve vessel wall changes o f smoot h mu scle
o Po sterior fo ssa men in git is or abscess & co llage n ens ue
o Dural sin us th rom bosis o Ob literative enda rte ritis leads to ischemia
o Otomastoidi tis: Mucosa l h ypert ro p h y, lo ss of ciliary
fu nc tio n &. eustac h ian tu be obst ruc tion
I DIFFERENTIAL DIAGNOSI S • Epid em iology: Increasingly rare X RT co m plicatio n
Malignan t exte rnal otitis Gross Path ologic & Surgica l Fea tures
• Clinica l: l m rn u n oco m p ro m ised ho st (d iabet ic) • O RN: Dead bone with seq uestratio ns
• Imagin g: Erosion of co rtex o f EAC • Fibrosis o f su rro u nd ing tissues incl uding TM]
o May m im ic XRT changes
Microscopic Fea tures
Coalescent masto iditis • Oblite rative endar te riti s & periarteritis
• Clin ical: Acute or ch ro nic o to mastoid ilis o Os teocy te d eat h N osteolysis
• Imaging: Disrup tion o f mas toid scpta tlons o I New bone fo rm at io n w ith loss of healt h y ho ne
o Retr oauricu lar abscess, subd u ral em pye ma, veno us marrow
sin us t h ro mbo sis = co m plicat io ns
Staging, Grad ing or Classificatio n Crite ria
O tos yp hilis • Ea rly. mild radi ati on -induced in ju ry
• Clin ical: Syp h ilis; no XRT o Co nd uctive hearing lo ss (CI Il.) secondary to
• Imaging: Ostei tis ph ase permeative T..bo ne cha nge in flam mato ry cha nges o f EAC, m idd le ear &. ma stoi d
• Co m p licated . diffuse rad iati o n- ind uced in ju ry
Aggressive cho lesteato ma o Rad iat ion-indu ced otomastoidit is
• Clin ical: Cho lesteato ma seen at otosco py; no X HT • Diffuse m uco sal th ickening of EAC, m idd le ea r &
• Imaging: O tic ca psule invasio n late fin din g mastoid
Page t d isease • Mo st (om man findi ng fol lowi ng X RT to area
• Clin ical: Bilat eral senso rine ura l hearing loss • Mny progress to coa lesce n t d isease & su b ject to
• Imagin g: En ti re crania l base usua lly in vo lved numero us regio nal co m plicatto ns
o O tic capsule involvement is d iffuse t hin nin g rath er • Focal, rad iati o n-ind uced injury
th an "mo th-eaten" o EAC eros io n with bony seq ucs t rntio n
• Late, mo re severe radi ati on-in duced in jur y
o Atro p hy o f spiral ligam en t &. degen eration o f org an
IPATH O LO GY of Corti
o Sensorine ural heari ng loss resu lts (SNI IL)
Ge ne ral Feat ures • End stage. d iffuse radia tio n.. ind uced in jury
• Gene ra l pat h co m me n ts o ORN: Demi nerali zati o n of skull base incl udi ng otic
o T-bone at higher risk t han most tissues ca psule
o Risk results from su pe rficial lo cat io n, lack of soft
tissu e pr o tectio n. poor blood su pply & ex pos u re to
respirato ry t ract pa th ogens
IC LIN ICA L ISSU ES Image Inte rpretation Pe arls
• Permeat ive dem in erali zati o n o f sk u ll base incl ud ing
Prese n tatio n tempo ra l ho ne in pati ent wit h hi sto ry o f regio nal XRT
• Most common signs/sy mpto ms • Fin d in gs identical to nec ro t izing ex te rn a l o tit is or
o Post-X RT o ro mas to ld tt ts
co a lesce n t m ast o iditis in patient w it h h isto ry of
• Hea rin g Lo ss (C HL, SN HL) regio nal X I ~T
2 • Drain ing car
• Clin ica l pro file
2 14
o Hear in g lo ss ± drainin g ea r in pat ien t wit h h isto ry of
regional irrad iat io n
ISELECTED REFEREN C ES
• Mo st co m m on XRT targe ts = bra in , I. Lin skey J\. IE ct al: Rad ia ti o n tolera n ce o f n orm al tem po ral
bon e structu res: implicati ons for gam m a kn ife stereotact ic
na sopharyn gea l or parotid neo plasm s
radi osurger y. In tl Radi a t OIlCO] Rio l Ph ys. 1;5 7( 1):196-200,
• Other signs /sy m pto ms 2lXU
o Seve re ota lgia 2. Ca ud clljl vt .11: Rad iothe ra py in t h e ma nagem ent of giant
o Ei\C inflammati on cell tumo r o f ho n e. lnt J Radiat O ncol Bio i I' h ys.
o Profuse ot orrhea 1;570): l 5 H·6 5, 21X):J
o CN? para lysis :I. l.invkcy ~ IE e t <II : Rad ia ti o n ex p0 'iu re o f n or m al tem poral
• In tr acrani al co m p li cat io n s due to dest ruc tio n of d urn bon e , trucl un'\ d uri ng ster eota ct icall y guided ga m ma kn ife
& hone 'iur gl'ry for vest ibula r sch wa nno mas . J Neu rosurg.
9H(-II:HOO-6,2(KB
o M enin giti s
4. 'silrin gl' r \V et ill: l'ar a giingli OlIl<l\ o f th e te m pora l bo ne:
o Brain ab scess re sul ts of differe n t trcn uucnt m odalit ics ill 53 pa tien ts.
o Sin us thrombosis ,\cIa Ncuroc hi r (Wil'Il ). 14-1 ( 12): 1 2 5~ ·6-1, 2()02
o Cerebrospin a l flu id (CSF) o to rrhea 5. Ferreira MA 1'1 a l: Endolym phatlc ' ill" tu m or : u ni q ue
featu res o f two cas es and review o f ti ll' liter atu re. Acta
De mograp hics Ne ur och ir (W iell ). I-I-I ( II ) : 1()-I7. 5:{ , 2()02
• Age: Any age 6. j oh annescn T B ct <I I: Lan- rndia t fon d fl'ch on hearing .
ve stib ula r Iunctto n, lind ta ste in b rain tu mor pat ie n ts. lnt ]
Nat ural Histo ry & Prognosis Rad ial O ncol Bio i l' h ys. I ; ~ 3 { I ):K6-90, 200 2
• Local ized X RT i n ju ry average latency period = 12 years 7. Fe igen berg S.I ct al: Radiosurgery for pa rag a ng lio m a of the
• Diffuse XRT injury av era ge la ten cy pe riod = 8 yea rs tempora l bon e . Head Nl'd.:.. 24(4) :;{H-I.9, 200 2
• Com m on rad iation i n jury ± a RN H. Vudln ta bola S c t a l: Hy per ba ric OX yg l'11 i ll till' th erapeutic
o SN IIL resu lts fro m da ma ge to h air cells &. cochlear managemen t o f o stcor udio nccros!v 0 1 ti ll' facial bones. Int J
O ra l Maxillo fac Surg. 29(6) :4:{ 5-H, 2000
n eu rons
9. Armour A et at: Late rad ia tio n sidc-ctfcctv in th ree patien ts
o Dose dependent, > 70 Gy und ergo ing par ot id Irrad lat lo n for hl' n ig n di sca w . Cl in
• Radia tion-induced tu mors are a lso del ayed O ncol (R Coli Radiol). IZ(6l:403-X, ZOllO
co m p lica t io n 10. O ndrey FG et a l: Rad iation d ose to o to logic st ruct u res
o 2n d neo pla sm a rises in radia ted field d u rin g hea d an d I1lTk GIIK t'r radia tion th erapy,
o Late ncy u suall y > 3 years Laryngo scope. 110(2 I't 1):21 7·2 1, zoco
o Pos t-XRT mali gnan cy has excep t io nal ly poo r I I, T~a n g \VS et al: Ostcoradt oncc rostv o f th e te m poral bo ne in
progno sis na so pha ryn gea l ca rcinoma a fter radiot he rapy: a case
repo rt. Ear Nose Th roat J. 79(2 ):9 -1-5, 200 0
Treatment 12. l'a thak l e t <I I: Temporal bone n ecrosi s: D iag n O\is,
• Depe n ds of ex te n t o f XRT in jur y clasviflcutio u , and m an agem ent. Ot o lary ngol l l >.: Surg.
123 :25 2-7, 2IXXI
o w hen in ju ry is m ild
U. Hac SP et ill: Syste ma tic manage m ent o f o steo radion ecrosis
• Aura l to ilet &: otic d rops in t he head and ne ck . La ryngo sco pe . 109 (8 ): 1324-7, 1999
o Locali zed injury 14. Co il YH c t al: Temp oral bon e tu mours in patient s
• Hype rba ric oxygen & a n t ih iotic s may suf fice irra d ia ted for na so pha ryngea l neo plasm . J Laryngol Otol.
o Diffu se, seve re inju ry 1 U <:I ):222-H, 19 99
• Su rgica l deb ridement req uired IS. l'fre u n d uer L e t at : Ca rc in o ma of tilt.' ex terna l auditor y
• Rcv ascul ar izat ion with su rgica l flaps can al a nd middle car. lnt ] Ra dlat O n co l Hiol Ph ys.
1;-I-l(-l ):777-KH, 1999
16. l.ustig LR et al : Radiat ion-induced tumor s of the te m po ral
IDIAGNOSTIC C H ECKLIST bo ne. Am J Ot o l. I H:230 -S, 1997

17. l.co n et ti]! ' ct 'II: Intra crania l complicati on s of temporal
hu nt' O~ll'Ofild iOl l l' lTO \ i ~ . Am ] O to l. IH : 2 2 3 ~ 9, 1997
Co ns id e r I H. Sm o uha EE vt .11: No n-osteit ic complications of th erapeutic
• O t her ca uses of 'I-bo n e and oti c ca psule radiation to ti ll' te m por a l bon e. Am J Ot ol . 16( l) :H3-7,
dc min cral lzatl o n o r d estructi on 1995
o In fect io us: Necrotiz ing ex te rna l o tit is; co nfluent 19. Fo n g RS ct al : l'cdia t ric sen so rineu ra l h r a ring lo ss after
o to m as to id it is; o tosyp hi lis te m po ra l bone radia tio n . Am .I 0 101. 16(6):79] -6, 11.)95
o In flamm a to ry: Aggr t'ssive acqui red cho lestea tom a 20. Nish im u ra R et 'II: M R evalu ati on o f rad iat io n
o to m a stol di t is. In t J Radia t On col Bio i l'h ys.
o O to dy st ro p h ies: Pagl't dis ease; co ch lea r o toscl erosis
I ;]9( I ): I SS· flO, I tJtJ 7
IIM AG E GALLERY
(l~JI) Axial lett. t>.1f T-lxJn t,

CT shows XRT-indu foo
injur y as (Iel>ris through ou t
midd/(' eJf·n", ,~toid with
2
(,fO~i(Jn of !it'gmt'nt of
p o Mer ior pot ro us sUrf,lCP 2 15
(.u ro l'l) S e;d('In.ll m.ntoicJ
corrcx (op e/J ,}(f o w) . (Hig ht)
Alli.11 h·it Pel f 7-ho ne CT in
radiated p tl tit'nl fl'ym/s XR1
injury as disrup tion of norm al
,1ir cell.~eptc1 tiot1S (arrow)
along wilh dehisc£!nn' in
la l('r<11 ma stoid cortex (open
Jtrow) .
(U / I) Axial Tbono C T
(e vea/s radiation Ch<lOge as
stvbris in m iddle ear-mastoid
with .~ om e mest oid
lr a!JI'ClJlaf breakdow n
(,mu w). Nol l' mOIIll'd bon y
ChaHBl' in o tic C'lfH Ull' .'\.
l )f'tmu s apex (opt'n ,,,,ow) .
(Rigll " /lxiallert e,Jf t -txn w
CT 51lO\V.s XRT-inducro
injury d .S cOale 5Cf'n! (fi5('J ~{'
involving mdstoid air cells
(arrow) . D issolution of
~igmoid sinus plJtt, ,1/,,0
present (open J ((O....'],
OLf t) A x;al T 1 C+ AIR with

iu-setu retion sho w.\
gra lJu /ation tis ~!lP in middle
f!ar-mastoid thaI entvsnces
in t(' fJ5c,ly w it h contrJst
(a ((()IV.~). Coch/('ar
IdlJyrinlht'rll' l'nhanct'mf..·nt is
a /~ () no/cel (op en arrow).
(Right) Coronal T-bone Cl
S/ lO\ V5 mtror,lC/iofl ffros;s as
c/em;neraf;zdfion of pctrcus
.1fX'x (.l ((OW) with (rank
flt''C.'f()!iis of hone superior to
cochleJ (open o1((OW) just
a bo ve genicula re fossa
(curved Jrrmv) .
~ ~ SECTION 3: Skull Base 1
Introduction and O verview

Skull Base Ana tomy and Imaging Issu es 1-3-2
Clivus
x ten tngl oma, Clivus 1-3-6
Giani Pituitary Ma croaden oma , Clivus 1-3-8
C hord o m a, C livus 1-3- 10
Jugular Foramen
Jugula r Bulb l'seu dol csto n 1-:1-14
Deh isce nt Jugu lar Bulb I-:l-l ll
Ju gu la r Bulb Diver ticu lu m 1-:1-22
Glo mus jugutare Parag<l llgl iom<l 1·3·26
Jugular Foramen Schwa n noma 1-3-30
Hyp oglo ssal Schwannoma 1-3-3 4
Jugular Foramen Men ingioma 1·3-36
Dural Sinuses
Arachnoid G ra n ula tio ns, Skull Base I-:H O
Dural Sinus Th rombo sis, Skull Base 1-3-42
Dura l A-V Fistula , Sku ll Base 1-3-46
Diffus e Skull Base Disease

Gia n t Ce ll Tumor, Skull Base 1·3·50
Plas macyto ma , Sku ll Hase 1-3·54
Ch o nd rosa rco m a , Skull Base 1-3-58
Langer ha ns Htstocyt osis, Sku ll Base 1-3-6 2
Fibrous Dys plasia . Sku ll Bave 1-3·66
SKULL BASE ANATOMY AND IMAGING ISSUES
3
2
Graphic of superior skull base shc1tving con uibu ting Graphic of superior skull IJdSe show ing major foramina
1)()(l(>S on the left &. major lx>ny landmarks on the rinht. on the k fr l': ('),iring uanial ner \,-~ Of) the right.
• Basisph eno id = sella turcica & dorsum sella &

IIM AGING ANATOMY co ntains sphe noi d sin us
Anato m ic Rel ationships o Greate r wing sphe noid (GWS)
• S bo nes make up skull base (Sllj: Eth mo id, sphe noi d, • Pair ed, form ing an te rior aspect of m iddl e cranial
occi pita l, paired fro n tal N: paired temporal bo nes fossa & posterolat eral o rbit
• Occipita l bone • Separate d fro m LWS hy su perio r o rbital fissure &.
o Forms floo r of pos terior fossa m ax illa by inferior o rbita l fissure
o Ma jor SB co m po nents o Lesser wing sphe noid (I.WS)
• Basiocci pital po rtion = anterior ma rgin of fora men • Paired, fo rm ing me dial por tio n of orbita l apex &.
mag num & lo wer clivus posterio r floor of anterior cra nial fossa
• Exo-o ccip ita l portion (paired ) = lat eral margin of • Co n tains o ptic canal &. is separated frum GWS by
for am en magnu m , oc cipita l co nd yles &: jugular su pe rio r orbita l fissure
tubercles o Pterygo id process
• Squamous por tion = posterior ma rgin o f foram en • Media l & lateral pterygoid processes are paired
mag n um in ferior extensio ns o f spheno id hone at jun ction
o Apertures & cana ls of body & GWS
• For amen ma gnu m o Apertures & cana ls
• Hyp oglossal canal • Forame n ova le
• Co nd ylar foram en • Foram en spinosum
• Ju gu lar fora me n - pos te rior margin • For am en rotu nd um
• Tem poral bone • O ptic canal
o Pct rou s & mastoid portions of tem por al bo ne • Superio r o rbital fissure
co n tribute to SIl • Ca rotid ca na l - precavern ous segment
o Trian gu lar pe trous portion sep arates posterior N: • Forame n lacerum
middle cra n ial fossae • Frontal bone
o Apertures & ca na ls o Forms latera l floor & anterolat eral wall s of anterior
• Ju gu lar forame n - an te rio r margin cranial fossa
• In tern al auditor y can al o Ape rtures &. canals: Foram en cec um
• Facial nerve cana l • Ethm oid bo ne
• Eustac h ian tube o Hoof of ethm o id bone forms me d ial floo r of anterior
• Caro tid cana l - petr o us segme nt cranial fossa
• Sph en oid hone o Bordered antero lat erally by frontal bo ne &
o Forms central sku ll base &: a nterior wall of midd le posteriorly by sph enoid ho ne
cran ial fossa o Majo r SIl co m po ne nts
o Ma jo r SB co m po ne nts are bo dy, grea ter wing • Cribriform plate = roof of nasal cavity which
spheno id (GWS). lesser wing spheno id (LWS) & co ntains m ultiple per fo rat ions th rough which the
pt erygoid process o lfactory nerves (e N ! ) pass
o Bod y co ns ists of prespheno id &: basisph en oid • Fovea etluuo ldalls = roof of ethm o id lab yrin th ,
• Presphen oid = plan um sphenoida le forming 5-10 mm h igh er th an cribrifor m plat e, formed by
posterior, cen tral floor of anterio r cran ial fossa med ial aspect of o rbita l plate of fro nt al bones
DIFFERENTIAL DIAGNOSIS
Cranial ner ves & fora mi na • Glosso pha ryngeal n erve (CN9): Jug u lar foram en (pars
• Olfacto ry nerve (CN I): Cribrifo rm plate nervosa)
• Optic nerve (CNZ): Optic ca na l • Vagus nerve (CN 10): Jugu lar forame n (par s vascu laris)
• Oc ulomotor n erve (CN3) : Superior orbital fissure • Accesso ry ne rve (CN11): Ju gu lar foramen (pa rs
• Trochl ea r nerve (CN4): Sup erior o rbita l fissure vascu laris)
• Trigemi na l n er ve (eNS) • Hyp oglossal nerve (CN 12): Hypoglos sal ca nal
• Oph t ha lmic div isio n (VI ): Sup erior orbital fissure
• Max illa ry d ivis io n (V2) : Foramen ro t undum
• Mandibular division (V3): Fora me n ova le
• Abduce n t nerve (CN6): Superior orbi ta l fissure
• Facial nerve (eN?) : lAC, t hen into facia l nerve ca na l;
exits via sty lo masto id foramen
• Vest ibu lococ h lea r ne rve (CNS): lAC
3
• Vest ibula r ner ve: Macu la crib rosa, in to vestibu le 3
• Cochlea nerve: Coc h lea apert ure into modiol us
• Cr ista ga lli = midline, ve rtica l int racran ial • Tran smit s: Mandihular div ision o f CNS &.
ex te ns ion of per pen dic u lar plate o f et h mo id bo ne accessor y meni n geal branch o f in ternal max illary
o Apertu res & canals artery
• Fora men cecu m o Fora me n spinosu m
• Majo r 5B su tures &. fissures • Locat io n : Postero late ral to fora me n ova le --
o Petro-occi pit al fissu re = betwee n baslocciput &. apex masticat or space
o f petr ou s te m po ral bon e • Transm its: Middle men in geal a rter y &. recurre n t
o Sphe no-occipital syn cho nd rosis = between branch of mandibul ar nerve
bas ioccipu t &. basisp h en oid o Foramen laceru m
o Sp he nope trosa l fissure = between basisphe noid &. • Location: Un dersu rface of SB at medi al end of
a pex of pet rous te mpor al ho ne petr ou s carot id ca nal
o Occi p itomasto id sut ure = between masto id tempor al • Trans m its: Meningeal branches of ascend ing
bon e &: exo-occlpita l portio n of occ ipita l bon e ph ar yn geal a rtery
«
• Ma jor SB ape rtu res co n te nts o Vidian ca na l
o Fo ram en cec u m • Location: Or iented in AP dir ection wit h in
• Locatio n: Mid line , a n te rior to crista galli, betwee n sph en oid bone -- jo in s foram e n laceru m to PPF
fro n ta l &. et hmoid ho nes • Tran smits: Vidi a n a rte ry &: ner ve (for med from
• Em bryo log ic rem nant of pre nasal space betw een greater su perficial pet rosal &: deep petrosal nerves)
nasal ho nes &. ca rt ilagino us na sal ca psule wh ich o In tern al audit o ry can al (lAC)
in volut es &: is filled with fibrou s tissue in ad ult • Locati on : Within pctro us tempor al bone
o Cribrifo rm plate • Porus acustic us = in t racran ial o pen ing of lAC
• Roof of nasal cavity in med ial floor o f ante rior alo ng midportion of posteri o r pct rou s T-bo ne
cra n ial fo ssa • Transmit s: Facial nerv e (eN?). vest ibulococh lea r
• Transm its: Olfacto ry nerves (CN !) & et h moid ner ve (CN!!) & labyrin th in e ar tery
ar ter ies o Ca rot id can al
o Opt ic ca na l • Loca tio n: Wit hi n pe trou s te m poral bon e
• Locati on: Medial LWS - o rbita l ap ex • Verti cal segmen t en ters SB anterio r to jugu lar
• Tra ns m its: Ophtha lmic artery, optic ne rve (CNZ) fo ram en
& shea th (duro, arach noid & suba rach noid fluid ) • Ascends sho rt di sta nce befo re turning 90 0 to head
o Su perior o rbita l fissu re (SOF) a n teromedi ally as ho rizo nt al segme n t
• Lo cat io n: Between LWS &. GWS - o rbita l a pex • At foram en lacerurn IC A turn s 90 0 supe riorly to
• Trans m its: Cran ial n erves 3, 4, 6, ophthalmic e n te r caverno us sin us
d ivision of eNS & su perior oph t ha lm ic vein • Tran smits: Int ernal carotid arte ry & sym pa the tic
o Foramen rot undum n erve plexu s
• Location: Midd le crani al fossa floor, in ferior to o J ugular forame n
SOF - pterygop alatin e fossa (1'I'r ) • Locati on: Floor of po sterior cra n ial fossa betw een
• Tra ns m its: Maxilla ry division of e NS, arte ry o f pet rou s te m po ral ho ne ant ero laterally &. occ ipita l
fo ram en rotund um &. e m issary veins from bon e po stero me di ally
cave rnous sin us to pterygoid plexu s • Divided in to smaller a nterome d ial com po ne n t
o Forame n ovale (pars n ervo sa) & la rger pos te ro lateral co mpo nen t
• Locati on : Floo r o f m idd le cran ial fossa lateral to (pa rs vascularis), separated by jugu lar sp ine o f
sella - masticat or space petrous bone
3
4
CraplJic or inFerior skull bast.' showing contrib uting Graphic of inferior skull base showing aftclchment of
bores on the right S major Foramina on the leff. deep cervical faKia (yellow,= superficiaf fayer, p urple '=
middle layerlplJaryng()ba.\ ilar f~Hcia, blue = deep layer)
&. relatiumhip of d('('p spaces of suprahyoid rJpck to 58
• Pars nervo sa transm its: Glosso pha ryngea l nerve • Du ral d iverticu lum ex te n ds from intracrani al
(CN9), Jaco bsen n erve (branch CN9) & inferior space to prenasa l space & bri efly contac ts skin at
petrosal sin us bridge of n ose
• Pars vascu laris transmits: Vagus nerve (eN I O), • Dura l di verti cu lum retract s & qu ickly invo lutes
sp in al accesso ry nerve (CN I I ), Arnold nerve • Prenasa l space reduced to a sm all cana l an te rior to
(b ranc h CN IO) & sigmoi d sin us - interna l jugul ar crista ga lli - forame n cec u m
ve in • Ave. newb orn foramen cec u m diamet er =4 mm
o Eustach ian tube o Birth : Cen tral, anteri or SB is co m posed en tirely of
• Locati on : Bon y cana l bet ween sq uamo us & carti lage which p rogressively ossifies
pet rous portio ns of tempor al ho ne, exte nd ing • Ossification of crista ga lli & crib riform plate
from hyp o tympanum - nasophar yn x begi ns at about 2 months o f age & is nearly
• l.at eral to hor izon ta l por tion pct rou s carotid co m plete by 14 m on th s
ca na l, in ferior to ten sor tympani mu scle • Crista galli co n ta ins fat at a bo u t 12 months age
o Styloma sto id forame n • Area surrou n ding foram en cec u m ossifies last
• l.ocatio n : Un de rsu rface o f SB between stylo id reaching ad u lt co nfigu ration by 2 years
process & mastoid -. parotid space • Cent ral SB d evel opment
• Trans m its : f acial nerve o Sp heno id bo ne ossifies fro m ± 13 o ssificatio n centers
o Hyp oglossal ca na l o Major segmen ts o f sp h en o id hone
• Locati on: Infero lat era l ( OU TSe betw een jugu lar • Presphenoid - planum sp hc noidale
tubercl e supe rio rly Sr occi pita l co n dy le in feriorl y • Basisphen oid - sella turcica &. dor sum sella &
- carot id space co n tai ns sp hen o id sin us
• Trans m its: Hypoglossal nerve (CN 12) • Alisphen oid - greate r wing
o For am en magnum • O rbitosp henoid - lesser wing
• Locati on : Ce n ter o f posterior cran ial fossa floor o Spheno-occ ipita l sync hon d rosis
• Trans m its: Med ulla & men in ges, spina l segmen t • Between hasiocciput &. ba sisphen oid
o f acces sory nerve (CN I I ), verteb ral arteries & • Site o f ma jority of SII growth fusing by III yrs
veins, & an terio r & po sterior spina l arter ies o Cran io ph aryn geal ca nal
• Remnant o f Rath ke pouc h be twee n presph en oid &
bas isphenoi d
IEMBRYOLOGY • Exte n ds from floor of sella tu rcica co n nec ting th e
pit u itary fossa with nasophar yn x
Emb ryo log ic Events • Rarely ma y persis t as ver tical midline defect that
• Anterior SB d evelopment usu all y measu res < 1.S mm in diam et er
o Ponticulus frontalis o Mar row signa l of cen t ral SB
• Naso fro n tal fon tane lle whic h temporaril y • Pred ominant ly red hem opoiet ic marrow in
separates developing na sal & fronta l bon es ch ild hood (hypoi n te nse on Tl WI)
• Nasa l & frontal bon es fuse ea rly oblite rati ng • Trans forms to fatty ma rrow d u ring lat e
fonti culus fro n talis - nasofrontal sut ure ch ild hood/e ar ly ad u lthood (h yperin tense o n TI )
o Pren asa l space
• Trans ient region separating nasal bones &
cartilag ino us Basal ca psu le
A ....
IIM AG E GALLERY
Normal
(/.£fl) Midline sagiu.ll N ECT
shows foramen cecum
(curved <1frmv) ,m lt-rior 10
crisla galli (arro w).
SphPIJo-oc cipit.ll
!lynch(m d"lHi.~ topon .1fro w)
~f;'IJ<lfi1 1{;'S cli vus into dnrerior
l,.l ~ i.~ pht'noid ~~ b asio ccip ut.
(Hit.:ht) Coronet N ECT
11lro ugh an/ prior 58 .\ hm vs
cnste galli, cribriform p latt'
(curved arrOlv ) & fovea 3
ethmoirldlis (ope n arrow).
Note bony canals for 5
ethmoid arteries (arrows)
bil.ltNally.
Normal
(/~fl) Central 58 NECT
shows vidia n canal (black
arrow) ex/end ing (rom r.
tocerum (op C'n whito arrow)
to pt('rygop 'l/" tine fo_~ _~.J
(curved wh ite arrow ). t.
oval<' S spinosum (open ~I\
("uf\'('CI bl,lCk arrows) <Jr('
shown. (HiKIII) CefllrJ /58
5upt'rio r to provious iigurC'
shows f. rotundum (bI.l rk.
arrow) entering PPf, bon y
eustachian tube lateral to
c<l rotid canal (curved ~t
open arrows ) ~'\
..ph( 'no-occipit.ll
synchondrosis ( whitt' imow) .
Normal
(l A,'ft) CO(()fMI N ECf through
("m lral SIJ .~I)(}ws f.
rolulJ(/um & vidisn canal
(or en g curved arrows )
withi n sp henoid bone.
Intracranial olw ning of op tic
canal (b lac tc arrow) & medial
SOF (white arrow) arc seen.
( HiKht J Jugul ar spine (w hite
arrow) separales pars
nervusa & par s va5Cularis
{curved & open arrows) of
jugular foramen . Inferior
petrosal sinus runs along
peuo-occipitsl fissure (black
arrow) to pars ne rvosa
MENINGIOMA, CLIVUS
3
(,
Sagitr.11 T1 C+ MR .~ "()W5 en plaque meningiom.r .J/OilS Ax i.l l ereT fl'\1.'<1/s eXlemiw..· tlll'n ingiotn d imolving
t-m ire d ivot surf..lCl' (arrow ) with tranSOSS<'O(/~ tumo r pr('pon/int. ciM('(n (,lrrO\ V). IImb/ying divtJ.~. ~pht>noid
.lfJfX1aring in IUllIeTl of !>ph('fJoid sinus (oP«" ) ," TOW). sinus (oP(' " ,mow) .Ii. middk' Cfdn;,ll ((Y.,.'\<l (curvt"'C1
CurV('(/ arrow : 1'/<1fwm component. arm....,).
ITE RM INO LOGY MR Find ings

• TZWI
Abbreviatio ns and Synonyms o Ext ra-axial m ass with brain stem co m pression when
• Basisph enoid -bas ioccl put men in gioma la rge
De finitions o Z5% atypi cal appea rance (necrosis. cysts.
hemo rrh age)
• Be nign , u nen ca psul at ed neop lasm a rising from
a rach no id cap cells o f c1ival dura o e SF-vascu la r cleft in la rge lesions
• Peritu m ora l brain ede m a corre lates with pial
b lood supply
IIMAGING FINDINGS • Bewar e! Difficult rem oval & early recurren ce
ahead
Ge nera l Featu res • TZ" GRE: If sign ifica n tly calcified. m ay "bloo m"
• Best di agn ostic clu e: TI c+MR: En ha nci ng d ura l-based • T1 C+
mass with d ural "ta ils" o '1' 1 C+ MR: Semil unar o r e n plaque, en ha ncing,
• Size: Cliva l m en in gioma usua lly a m ajo r co m po n en t du ral-based mass wit h dural "tails" ce n tered a long
of large lesio n cliva l co rtex
• Mo rph ology: Sessile > globose o 9SlJ-i1 e n h an ce strong ly
• Du ral th icken in g "tail" in 60'M'1
CT Findings o If lar ge, e SF-vascula r "cleft" be twee n mass « hrain
• NEe I'
o 751Ml t um o r mass h yp erd en slty o n NEeI' Angio gra phi c Fi nd ings
o 251M) in tra mura l ca lcificatio n (sa nd-like or d ense • Dural vessels su pply ce n te r, p ial vesse ls supply th e rim
ch unks ) • "Su n bu rst" patte rn o f en la rged d ural feede rs com mon
o Bone Cf: Permeative, perm eative-sclerot ic o r • Prolon ged vascular "sta in " in to veno us phase
h yp erostotic bo ny ch an ges
• C ECr: 90% stro ng, u nifo rm en hance men t; HYM)
in ho moge neo us
DD x: Cliva l Le sions
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r':
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.,
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Sarcoidosis Pla smacytoma Chordoma Clival Metastasis
MENINGIOMA, CLIVUS
Key Facts
Imaging Find ings • Plasmacyto m a
• BOlle c r: Pe rm eati ve, permeatt vc-sclero tic Of • Ch ordo m a
h ype rostot ic bon y cha nges • Met astasis & lympho m a
• Tl C+ MR: Semilu na r o r en plaq ue, en han ci ng, Clinica l Issu es
du ral -based mass wi th dural "tails" ce n tered along • Most co mmo n signs/sy mpto ms: Inciden tal find ing
clival co rte x • \Vhen large, invo lving basisp heno id &. basiocciput,
Top Diffe re ntial Diagn o ses "cure ma y be wor se t han di sease-
• Ncu rosa rco tdos is
ID IFFERENTIA L DIAGNOSIS Natu ral History & Prog nosis 3

• Pe rltu mor a l bra in ed ema presages high surgica l 7
Ne urosa rco idosis co m plica tion s &. recurrence rat e
• l.oo k for infu ndibular sta lk en h a nceme n t &
en largemen t Treatment
• MR: Mu lrifocal , dura l-based en ha nc ing foc i • Su rgica l remova l if med icall y sa fe
• Radio therap y prim ar y o r adj unc tive
Plasma cyto ma • \Vhcn lar ge, invol vin g basisphen oid &. basiocc iput ,
• Cf: Destru ctive mass o f clivus "cure ma y be worse th an di sease"
• MI{: '1"2 sig na l usua lly int ermed iat e to low
Cho rdo ma
• CT: Mid lin e dest ructive mass o f cl ivu s with intramural ID IAG N O ST IC CHECKLIST
bo ne fragm ent s Co ns ide r
• Mit '1'2 sig na l hi gh
• Always search fo r 2nd meningioma
Metastasis & lymphoma o Mul t iple m eningiomas in IOIYcI of spo radic cases
• CT: Destru ctive cllva l mass
• M){: Multlfoca l m culngeal n odular e n ha nce me n t
possible ISELECTED REF EREN C ES
I. Men d en h all WM ct al: Radi ot herap y alone or a fte r subto tal
resect ion for benign skull ba..c men i ngioma... C UKcr.
IPATHOLOGY 2.
9K(7):H 73-K2, 200:l
Buet ow ~ (l'I al: Typ ica l, atypical and mi sll·;ldillg fea tu res
Genera l Features i n mentngtoma . RadioGraphics. II : 1087· 1106, 199 1
:I. Gol d sh er D et a l: Dural "tail " associ a ted with meni ngiomas
• Genet ics: Long-arm deletion s of ch romo so me 22
o n Gd -D'l'l'Acen ha nccd MR im ages: Ch aracteristics,
co m mon
d iffer en tial di agno stic va lue and po\\ihll' Im plic atio n s for
• Eti o logy: Arises h om a rachno id "ca p" ce lls, no t dura treat m en t. Rad iol ogy. 1 76 :-t-t 7- ~O. 1990
• Epidemiology
o 2nd most co m mon primary in t racranial t u mo r
o 15-251)( 1 of primar y int racra n ia l tum ors
o HY)(I occu r in posteri o r fossa
(IM AG E GALLERY
o ) ()l )() mul ti ple (NF2; m ultiple mc ningio matosis)
• Associated abnormali ties: Meningioma + schwan no ma
= N f2
Gross Pathologic & Surg ical Featu res
• "Sem il unar" or "en plaque" > round or globose
• Sha rply circu mscr ibed, un en ca psulated
• Ad jacent d u ral thicke n ing ("ta il")
o Usually reactive du ral proc ess, no t neoplasti c
IC LIN ICA L ISSUES

Presen tati on (/..../ 1) Sagitt.JI T1 C+ AIR shows g/ol>ost' ctiv el meningioma . Ayid
cnhanct>mt>nt in combination with obvious du ral · tails- (.]( (()I\'s) arc
• M ost com mon sign s/ sym pto ms: In cid ental find in g
hlj.;h/y .w ggt>stive of meningioma diagnosis. (Highl) l \x i,l! Tl WI MR.
Demograph ics dt'molH tril les meningiom a .lri5ing from clivet dur,l (oml'fl'ssing
,l dj,K I'fl t pom. Notice the prom;m'nt csr -vdscul,lf c!t·;t found
• Age : Midd le-aged , elderly patients (peak age = 60 )
b d Wt 'f.'fl the tumo r and the po ns (arrows).
• Gender: M :F = 1:3
GIANT PITUITARY MACROADENOMA, CLIVUS
SaMillal graphic c/('piClS /:i,1fl1 piluildry lll.Jao.1C/l'I1oma. Sagill d! T1 WI MR ,~ ho\V,~ giimt piw il,lIy m acro.lCJt'fIOllU
Notice' normal pituitary gl<lIld is nol 51.1..'/1. The lesio/l ('fl w ldting from ':if'1I,1 (,1rI'OW ) cln lt>riorly info
( ·xt( ·nrl~ an/c'roin[c'riorl y imo ba<;;,'"pll('lloid " bd ~Lr;ph{,lJ()id (open arrow) S por;/eroinft'riorly in to
/x I\/p(();n/f.r;(Jd}, into ha,;eXl "iptlt. lJasi(x'cipul (curved arrow ) S 1l, Ir;o,,!w YIl X.
ITE RM INO LO GY MR Findin gs

Abb re viatio ns a nd Synonyms • TlWI
o Sellar-inf rasellar mass, lso ln tcnsc to gray matter
• In vasive pituita ry macroadcno ma • Ca n no t separate sellar & Infraset lar d isease
Definit ions o Mass ma y extend into cavernous sin us
• Pitu itary ma cr oad enom a w it h i n ferior ex tension to • Cavernous ICA encasem ent > 66 lYcl
basisp he noid & basioccip ut • TZWI: Best delineat es cystic necrosis when present
• T I C+
o Early, in tense, but heterogeneous en hancemen t
IIM AG ING FINDING S • Dural "tail" may be seen, mimic meningioma
• Asym met ric te ntoria l en ha nceme n t suggests
Gene ra l Features cavernous sin us compression/invasion
• Best diagn osti c clue: Ca n 't find a pitu ita ry gla n d, Imaging Re commendat ions
sepa rate from invasive mass
• Enhanced bra in MR focu sed to sellar-parasellar region
• Size: > 5 em
d efines ext ent of soft t issue mass
• Morp hology: Multilobular
• Axial & coro nal skull base CT
CT Findin gs
• NECT
o Hemorrh age HNf" calcificat io n i n 291, IDIFFERENTIAL DIAGNOSIS
o Bone c r
• Ex pa n sio n o f sella wit h in vasion o f su rro u nd in g
Clival cho rdo ma
subjace n t anat omic land scape • Displaced pituitary gland visible
• Bone CT shows ben ign bony margin s typ ical • Midline clivus; lacks coarse chond ro id calcifications
• W he n bo ne co m p letely de hi sced, lesions appe a rs • High '1'2 signal suggestive of cho rdoma
inva sive, mimi c ma lig na ncy
• CECT: Moderate, in homogeneous enhancement
DDx : Basisphenoid-Basiocciput Tumors
Clival Chordoma Chondrosa rcoma , po r Plasm acytom a Metastasis

GIANT PITUITARY MACROADENOMA, CLIVUS
Key Facts
Termin ology • When bone co m pletely dehisced, lesions appea rs
• Pituita ry macroad cn oma with inferio r ex tensio n to in vasive, mimic mal ign an cy
basisp he no id & ba siocciput Top Differ en t ial Diagnoses
Imaging Fi nd ings • Clival chordo ma
• Best diagn ostic clue: Can' t find a pituitary gland , • Cho nd rosarco m a, petr o occipit al fissur e
separate fro m invas ive mass • Plasm acytoma
• Bon e cr sh ows beni gn bon y ma rgin s typ ical • Met astatic tum or, cent ral skull base
Cho ndrosa rco ma, petrooccipital fissure Natu ral Histo ry & Prognosis 3
• Cen tered alo ng lat e ral ma rgin o f clivus in • Benign, slow gro win g 9
petrooccipital fissur e • Som e aden o ma s hehave in m ore agg ressive m an n er
• Displays cho nd ro id calcificatin ns (50%) wit h hi gh recu rre n ce rat e
• 15(M, recurren ce a t 8 yea rs, 3 591, a t 2 0 yea rs
Plasmacytoma
• Cen tered anywh ere in skull base ma rro w Treatm ent
• T2 sign al is lo w to in termediate • Resect ion
• > 50% have co ncu rre n t multip le m yeloma • Med ica l, stereota x ic radi osurgery, co n ve n tio na l X RT
Metastati c tum or, ce ntra l skull base
• Destructive mass th at ca n be an yw he re in sku ll base
• Ofte n m u ltiple w it h kn own pr im ar y tu m or
Co nsider
• Ch ec k h o rm o ne lev els before su rge ry w h e n la rge,
IPATHOLOGY in vasive cen t ra l sku ll ba se m a ss as it m ay be
ma croad en oma!
Ge neral Featu res
• Gen eral pat h co m me n ts
o Pitui ta ry rnacroad cn o ma usual gr owt h pattern = ISELECTED REFERENCES
bu lges upward in to su prase lla r ciste rn
o Alt ernate in va sive is basisphenoid &: basiocciput I. Yokoyama S et al: Are nonfunctioning pituitary adeno mas
exte nding int o the cavernous sinus aggressive and/or
• Ge net ics in vasive? Neuro surg. 49(4) :8 5 7· 62, 200 1
o Allelic loss o f chromoso me l l q in MEN region 2. Nakasu Y et al: Ten tor ial enhancement o n MR images is a
o MENl gen e invo lved in ad e noma fo rm ati on sign of cave rnous sin us invo lvem en t in patien ts wit h sella
• Etiology tu mo rs. AJNR. 22 :1528·:n . 200 1
o Hypothesis for pi t u itar y tu m or formati on 3. Chanso n P et al: Nor mal pitu itary hypertrophy as a
• Il ypoph ysiotro phi c ho rm one excess, su p p ressive frequen t cause of pituitary incldentaloma: A follow-up
hormone in suffi cien cy o r gro wth facto r e xces s study.] Clin Endocrino l Metal>. 86:3009 -15, 200 1
lead s to h yperpl asia
• 1 Proliferati on predi sp oses to ge netic in stability
• Adeno m a fo rm s IIM AG E GAllERY
o Relea sin g hormo n es prom o te growt h o f genetica lly
tra nsfor med ce lls
• Epide m iology
o Pitu itary ade no ma : 15% of intracrani al tu m or s
o Pro lacti n secre t ing = 40% of sym pto m a tic ad e no m as
• Monot onous sh eets o f uniform cells
• Ce ll type iden ti fied wit h immu n ohisto ch e m ical sta ins

Presen tati o n (I.ef t) Axial bom' CT of giJm pituitary me croedenome $hows
• Mo st com mon signs /sy m p to ms remodeling of the central portion of the skull base. Note the medial
o Pitu ita ry h ormonal a ct ivity aspects of bo th horizo nl<11 potrous ICA cenn ts .1((' C'ro</ed (.l rrow.~ ) .
• 75% e ndocr ino logi ca lly act ive (Rig"') Sagiltc1/ TI C+ MR demonstretes large pillJitary
• 25 tMl vi sua l field defect o r ot h e r cra n ial nerve palsy m acroadenoma invading pmteroinferiorly into besioc clput (arrow).
No tice the cyst ic changt' at the infprior ma rgin of the tum or (open
arrow).
CHORDOMA, CLIVUS
3
III
Sclgilt,l l gr.J/)hic .~ / J( rws an ('.'(p..m~ ik', d(I.~truClh'(' 171J$.~ Sagill<ll T1 C+ AIR ff'~'(·.1 1.~ /l( ' /('f{)J.:PlJoou, /)' f'nhmcing.
origifMling from cli vu..;, "1Ilt/mhing" pons (arro w) 8 c!cslrucliVl' (/iv, 11 C!JoH/cHna iljv<Jdin~ botlJ hJsisphL'floid
('/ev,ll ing {he pituitary gl,me! (open arrow ). Note bone (arrow) '-~ h..bj-<xd/Jut (OPf '/l arrow ). Mass also
(ragmenl5 t7oa lin~ in chor e/o m,1. "thumbs" tho pons (curw d arrow).
• Morph o logy: Expa n sllc mu lt ilobulated well

ITERM INO l O GY ci rcu m scribed m ass
Abbreviation s a nd Syno nyms • Expa nding tum or invad es o r di sp laces
• Abb rcvlatl o n : Cliva l ch o rdo m a (Cell ) o Cave rn o us sin us N. sell" su pe rio rly
• Syn o nym: Basicran ial ch o rdo m a o Jug u lar fo ra me n &. pet rou s apex late ra lly
o Basila r a rter y &. br a in stem poste rio rly
Definitions o Basisp he no id , sp he no id &. poste rio r et h m o id sin uses
• ceil: Ra re m ali g na n t tu m o r o f clivus a rising fro m a nt e rio rly
remnants of cra nial end of primitive not ocho rd o Na so ph ary n x a n tcroi nfe rio rly
o Jugu lar fo ram en & fo ram e n m agn um
poste ro in fer ior ly
IIM AG IN G FINDINGS CT Find ings
Ge ne ra l Features • Nl.C f
• Best d iag n ost ic cl ue: Destruct ive m id lin e m ass o Cliva l ma ss w it h associa ted bo n e destructi o n (95 I KJ)
ce n te red in c livus w it h hi gh T2 sig n al in ten sit y & h igh a tt e n ua tion foci w it h in tu mo r matrix (Sm!(l)
• Locatio n • High a tt enua tion foci rep resen t ossi fic frag ment s
o :1S(MI a rise in sku ll ba se aro u n d sp h eno-oc cipi ta l of de stroyed cl lva l bo ne floa ti ng in chordo ma
syncho nd rosis m atr ix
o Loca lly in vasive tumor co m mo n ly fo und in m id line o Ce n t ra lly loca ted, well c ircu mscribed. cx pa ns ile soft
from sella to coccyx ti ssue m ass w it h lyti c bo ne dest ru cti on
• Ca n occu r a nyw h e re a lo ng pa t h o f pr imi tive o Bul k of tUl11 0r is h yp erden sc re lati ve to th e ad jacent
not ocho rd neu ra l axis
o O th e r rare loca tio n s • CECr
• Sella r regio n , sp he n oi d sin us, naso phar yn x, o Mixed d en sity' wit h area s of cyst ic necro sis m ixed
m a xilla , pa ran asa l sin uses &. in t rad ura l w it h en h an ci n g so ft ti ssu e
• Size: Usua lly 2-5 cm a t pr ese n tati o n o May co n ta in lo w atte n ua t io n area s th at represents
m yxoi d ma te ria l at gro ss exa m ina t io n
DDx: Clival Masses
tovestve Adenoma Chondrosarco ma Plasmacytoma C1ival Metastasis

CHORDOMA, CLIVUS
Key Facts
Terminology Top Differential Diagnoses
• Abbreviation : C lival chordo ma (CC h) • Gian t invasive pituitar y ma croade n oma
• Ceh: Rare m ali gnan t tum or o f cli vu s arisin g from • Ch o n d rosa rco ma
remn ants of cranial e nd of primitive no toch ord • Plasmacyt oma
• Cliva l m et ast asis
Imaging Findings
• Best dia gn osti c clue: Destructive midlin e mass Path ology
cen te red in clivu s with high T2 signa l intensit y • Typi cal chordo ma hi stol ogy: Cor d s o f physaliphorous
• 35% a rise in sku ll ba se a rou n d sp h en o-occipital ce lls wit h a reas o f n ecrosis, hem o rrh age & e n t rap ped
sync h on d ro sis bon e
• Sagitta l im ages sh ow tu m or "thu mb" inden tin g • PHYSALlI'HO RO US C ELL co n firms d iagn o sis
a n te rio r pons
• Calcification , h em orrh age & mu coid a reas sh ow as Clinical Issu es 3
het erogeneo us h ypo in te nsity • Clin ical p ro file: Classic pati en t p rofil e: 30-50 yea r o ld
wit h grad ua l o nset o f o ph th almop legia I'< h ead ach e 11
• "I loneycom b" enha nce men t pattern seco ndary to
intratu moral ar eas of low signa l inte nsit y • Recurrence free 5 yea r su rvival 60- 70% wit h
co m b ined su rge ry & radiat io n
MR Findings IDIFFERENTIAL DIAGNOSIS

• TlW I Giant invasive pituitary ma croadenoma
o In te rme d iate to low sig na l intensit y
o Sma ll foci o f h ypc rin ten sity rep resen ts h em orrh age • Ema na tio n from sella abov e d iffere ntiates th is lesio n
o r mu co id material
from cho rdom a
o Sagitta l images show tumo r "th um b" inden ting Cho nd rosa rco ma
an te rior pons • Arises along lat eral ma rgin o f clivus in pctro-occip ital
• T2WI fissu re, n ot in mid lin e clivu s
o Classically high T2 signa l in tensity • Simi la r T l &. '1'2 ch a racte ristics to Ceh
o t '1'2 signa l seco n dary to h igh flu id co n te n t o f • C ho n d ro id ca lcifica tio n s (> SO'MI)
vacuo lated cellular co m pone nts
o Calcificatio n, hem orrhage &. mu coid areas show as Plasm acytom a
het eroge neo us hyp o intensit y • Can b e m idline destructi ve mass o f clivus
• 'f 2* GRE: Foci o f hem o rrh age low signa l • T2 signa l usually in te rmedi ate to lo w (co m pa red wit h
• Tl C+ chordo ma h igh T2 signa l)
o Mos t so mod e rat e to mark ed en ha nce men t
o "Hon eycomb" en h a nce me n t patte rn seco nd a ry to Clival metastasis
int rat um o ral areas o f low signa l in ten sity • Destru cti ve cliva l lesio n wit h exte ns ive so ft tissue
o Sub tle o r n o en han ce ment may reflect n ecrosis ± co m po nen t
la rge amo u nt of mucino us material • Prim ar y neoplasm ofte n known at ti me of
• t\fRA : Tu mo ral e nc asemen t &. d isp lacem e n t of vessels presentation
seen ~ 80 !)")
Angiographic Findings IPATHOLOGY
• Avascu lar mass typ ical
o Propensit y to d ispla ce &. e n case inter n al carotid General Features
a rte ries a nd ve rteb robasi lar system • Ge ne ral path co m men ts
• Better dem onstrat es de gree of lu m in al n a rro win g & o Two h istopat ho logic su bty pes
co llate ral vessel s • Typica l (classic) ch ord o ma I'< chondro id
• Significa nt ar te ria l n arr ow in g rare as cho rdo mas arc ch ord o ma
so ft & n on -a n giotnvasiv e • Ge netics: Fami lial cho rdoma ra rely reported
• Etiol ogy: Rar e mali gnant t u mo r o f bone a risin g fro m
Imaging Recommendati on s rem nan ts o f cra n ial e nd of pr imitive n o to ch ord
• f ocused en ha nc ed MR of sku ll base • Epide m iology
o In clude magnetic resonan ce a ngiog raph y (MRA) & o 3SCYh of all cho rdo ma in skull base
magn etic reson an ce venogra p hy (MRV) o SOlJ.{, a rc sacrococcygea l
• Axial &. co ro na l bone-only un enhan ced CT o f sku ll o 159·'<1 ari se from verteb ral bod y
base
• Ang iog rap hy co m pleted wh en MRA shows sign ifican t Gross Pathologic & Surgica l Features
displacem ent o r e ncas eme nt of vessels • Gross appe a rance is semi -tra n slu cent . mu ltllobu lat ed ,
o Balloo n test occl usio n used to eva luate ris k o f gela tino us (myxoid matri x), gray mass
neuro logi c impairm ent wit h vessel occlusion
CHORDOMA, CLIVUS
Micro scopi c Features IDIAGNOSTIC CHECKLI ST
• Typ ical chordoma hi stology: Co rd s o f physali phorous
cells with areas o f necrosis, hem orrhage &. en trap ped
Con sid er
bo ne • T1 C+ MR, MRA &: MRV best demonstrat e t u mo r
• PHYSALlI'H O ROUS CELL co nfirms d iagn osis ch aracte ristics &. exten t
o Large cell co n tain ing mu cin &. glycogen vac uo les • Bone CT can bett er cha racterize th e bony d est ruction
wit h "bubb ly" a ppea ra nce to t he cyto plasm Im age In terpret ati on Pearl s
• Chond roid chordo ma histology: Stro ma resem bles • Destructive midline mass o rigina ting fro m clivu s
h yalin e cartilage with neopl astic cells in lacunae hyperinten se o n T2\VI is most co m mo n presentation
o Ter m "ch ond ro id - in cho nd ro id cho rdo ma is • Loo k for enc ase me n t o f ICA I< verteb ro basilar system
m i sn om er; refers to histologic mimic
o Lesio n docs not co n tain tru e cartilage o r cells o f
cartilage o rigin ISELECTED REFERENCES
3 Erd em E et al: Co mp rehe ns ive review o f in tr acranial
1.
11 chordo ma . Radiog raphies. 2 :~ ( 4 ) :99 5 - 1(X)9 . 20(U
ICLIN ICA L ISSUES 2. Asauc S et al: In trad ural spina l seeding o f a cltva l
chord oma. Acta Nc urochi r (wlc n ). 1.t5(7):599-603;
Presentati on
discussio n 603 , 2003
• Most co mmo n signs/sympto ms: Oph thalmoplegia & :l . Fisch bein NJ ct al: Recurrence o f cliva l ch o rdoma alon g the
o rbit of ron ta l headach es most co mmo n su rgical pat hw ay. AJ Nn Am J Neu ro radiol. 2 1(3):578-83,
• Clin ical p rofile: Classic pat ien t profile: 30-50 year o ld 2(XIO
with grad ual o nset of op h tha lmoplegia &.: headache 4. Uggowttzer MM et al: Dro p m etastases in a pati ent with J
• Other signs/sy mpto ms cho ndroid ch or d o ma of th e clivus. Neu ro radiology.
o Opht halmo plegia results from tum or proximity to 41(7):504-7,1 999
cran ial ner ves 5. Do uce t V et 'II: MIU o f intracrani al ch o rdomas. Extent of
tum o u r an d co n trast en h ance me n t: crite ria for di fferential
• Cran ial nerves 3, 4 & 6 in caverno us sin us diagn o sis. Neu ro radi o logy. 39 (8):5 71-6, 19 9 7
• CN6 in Do rell o ca na l 6. Harad a T et nl: Clival ch o rd o ma presen tin g as an ile
o Large cho rdo ma may reach jugu lar fo ramen eso tro pia d ue to bilate ral abducen s pal sy.
in ferolat erall y affecti ng CN9-12 Oph thalmo logica. 211(2):109-11, 1997
o Visual disturban ce signals o ptic nerve inj ur y 7. Malta G ct al: Sur gica l treat ment of cllva l ch or do mas: the
o Lateral grow th can in ju re CN7 o r H in CPA-lAC tr an ssphen oid al ap p roa ch rev isited . J Neu ros urg.
o Headach e likely related 10 st retc hi ng of d ura 85(5):784·92, 1996
8. Web er AL et al: Ca rt ilag inous tu mor s an d ch ordo mas of
D em ographics th e cran ial base. Oto laryngol Clin No rt h Am . 28(3):453-71,
• Age: Ca n occu r at an y age but most co m mo n ly occu rs 1995
bet ween age 30 -50 years 9. Tash iro T et al : In tradural chordoma: case report an d
review o f th e literature. N euro radiology . 36(4):3 13-5, 1994
• Gender: 2: I male predil ection 10. w eb er AL e t al: Chordomas o f th e sku ll vase . Rad iologic
• Ethni cit y: Caucasians mor e co m monly affecte d than an d cl in ical eva lu atio n . Neuroim agin g Clin N Am.
African-Ame ricans 4(3):515-27, 1994
11. Pro bst EN et al: Co ngen ita l clivus ch or doma . AJNR Am J
Natural History & Progn o sis Ncuroradiol. 14(3):537-9, 1993
• Recurren ce free 5 yea r su rvival 60-701){, wit h co m bine d 12. Sch utusch ula RG ct a l: Clival ch or d o mas. Australas Radiol.
su rge ry &. rad iation 37(3):259-64, 1993
• Poorer prognosts in wo me n, necroti c tumor s by 13. l.eprou x F et al: MRI of cra n ial chord o m as: the valu e of
imagin g &: volume > 70 ml gadolini um . Ncu ro radiology , 3S(7):5.t3-S. 1993
1·1. Meyer s SP ct al: Ch or dom as of t he sku ll base: MR features.
• Cho nd roid chordo ma associated with a bet ter
Aj NR. 13:1617-36, 1992
progn osis than typ ical cho rdo ma I S. Wozn lca J et al: Value of C I"an d NMR imaging in
• l.ocal recu rren ce is co mmon despite co m bine d th erap y d iagn o sing o f ch o rdo mas. An n Uni v Ma rtae Cur ie
• Tum o r recurren ce a long su rgical pa t h way can occ u r Sklodowska IMcd). 45:t 81-6, 1990
rarely (5%) 16. Szc G ct <11: Ch o rd omas: Mn im ag ing. Rad io logy.
• Distant met astasis rare; describ ed to hone, lun g, liver 166:187-9 1,1 988
I< lymp h n od es 17. Oot RF ct al: ThL' ro le of ~ 1R a nd (;1· in evaluating cllval
• "Dro p" met astasis wit h su barach no id seed ing ca n cho rdo mas a nd ch ond ros arco mas. AJR Am J Roen tgencl.
occ u r rarely 151(:1):567-75,1 988
Treatment
• Co m plete su rgica l resection is t reatme n t of cho ice
• Co mbined with fractio nated p roto n beam rad iatio n
th erap y
CHORDOMA, CLIVUS
IIM AG E GALLE RY
Typica l
([.KI t) Sagittal hu n!' C 1
rec onstructio n showl'
df '~lfllcti()n of rhp ctivu c with
entrap/wc/ b on e Ir..WIlt 'n l"
(,m o w5) in th is p ,ltil'n t with
dival chordom a. (Nighl )
1\\;<11bO(1(' C I rf:'Vl'''/.~
L'"l('mi~'(' bcnv c1l,~trtl cti()n of
tho clivus by <I "](1-:<-
chordoma. N Olt' that the
m('dial wall o f the righ t
potrous carotid c.lO.11 is 3
('(oded by tumor ( .1" 0 \\,).
u
Varia nt
(I.el' ) Sagittal f2 WI MR
shows diva' tip chordoma as
d hv pcr tenw ma ss arising
fro m ;1l{N ;or J Spffl of c1i\'u5
with -uurrior c xumsion into
1lt1!>ophll yn x (.lffOW) ,.;.
po.<;/f'riOrly into h.H.l1 cisu-m
topon ,,,(my). (HiRll t) Sc1~i(f <l/
11 C+ AIR of ctivet tip
dm,dom,) f(·\'(·ah.
he/c·'Of.:I>I'PO U'O
enh.lfln'menl. Notice rht·
sUf x'rio r margin 0; the
oc/ontoid has been cJP..troy('cl
(a/fOw). The medull.J is ,lIsa
intt·nclt'<.l (opon .1ffUl \ ') .
(I .eJ I) Axial graph ic i/lu strJt {'s

farge· diva! ch orclom ,}
pu.~hing p ostc' riorly 10 inc/{'n(
th e low p o ns l? b.t~ildr dr /er y
ts rro w), BeHi'_~ph elloid
invs sion (ope n .1frow) ,1/~o
SC't'11 lifting pituirary gland in
the sella. (Hix ht) Ih i,ll c rc r
~h()ws an ull/ 'nh,lIl cint: diva l
(-hordnm a with .~ i8nit7can t
mp.oid co ntent (' ngu/fing the
right intern<)1c.lmrid J rt(' ry
«U fOW). botw frdgmenb
(open affolVs) in <Interior
tumor margin l)'pir .1J.
JUGULAR BULB PSEUDOLESION
3
14
I h i.111"'\1 ,'vIR 5fK,..vs .:J mun<1ed .Ifed of soft li5SlJ(1 M ial T2WI MR ww.l/s inae.l.'i{>(] sign.ll inlt.,,~ity .It tht·
irJl('mily in jugu/.1f t(Jf,mH'fJ (.mow) 5U5p idoU5 frx right jUgUI.lf fi.}(J/lICIl (Jrrow ) ~u~pi(iou~ fix If
f)JlhokJ1{Y. OthPr ,'vIR ~'(/lJ('fJn's tr\'P.ll it to be jUgUl,lf sc..hw<lnnoma. Bone a sha.VPd rxvmsl, <1,wmnwlric.J1ly
bulb P<,('tKk>lf ~ion. largejugul.1r bulb.
• No fillin g d efect to sugg est thrombo sis

ITERM IN O LO GY • e TA: Asymmetr ic JR dem o nstrates sim ilar
Abb revi ation s a nd Syno nyms en hanc eme n t cha racte ristics as sigmo id sin us &.
• Jugu la r bulb UB) pseudomass, "lea ve-me-alo ne" lesio n in terna l jugular vein
o f jugu lar fora men UF) MR Fi ndings
Definitions • Tl WI: Variab le signal: May have so ft tissue int ensity
• JB pseudol cslo n occu rs w hen an asymm etric, large o r het ero gen eous signa l
jugular hulb flow ph eno men o n sim ulates • T2W l
sch wan noma o r thr o mbosis o n m ost MR seq uences o Het erogeneous signal in ten sit y
• Usuall y co n spicuous wh en iso / h igh in ten sity
• n~ IR : Heterogen eo us signal int ensity, o ften
h yper int e n se
IIM AG ING FINDINGS
• T1 C+
Ge nera l Features o Avid en ha ncemen t of jugu lar bu lb
• Best diagnostic cl ue: Co m plex MR sign a l intensity in o Id ent ical en hanc eme n t cha racte ristics as adja cen t
)R with n ormal) F co rtex , jogular spine in te rnal jugular vei n &. sigmoid sin us
• Locat io n • MIN
o Jugula r forame n-bulb o Jugular bulb d em o nstrates asymmet ric en largement
o Promine n t JB more co m mo n ly right-sld ed without evide nce of t h ro mbosis
• Size: Typica l jugula r bulh measu res 1.0 -1.5 em o Phase co n trast ~Ht V : Sho ws normal flow in sigmoid
• Mo rph o logy: Rou nd ed area o f heterogen eo us sig nal sin us 1i< ) 11
in tensit y ce n tered o n jugular foramen • Dyn a m ic Mil
o No rma l ) 1I sho ws decrease in dy na mi c cur ve a t - 30
CT Find ings sec fo llo wing co n tras t ad m inis tration
• NEC I': Bo n e C I: Asymmet ric ) B, with in ta ct co rtica l
ma rgin s &.: jugular spi ne Angiographic Findin gs
• C EC I' • Ca th e ter venograph y findin gs
o Nor ma l enhan cing sigmoid sin us &; jugular b ulb
DD x: Jugular Foramen l esions

' .. .,
J
j :"' ,
""
\'~
\' -"'.l.,...
'
\
', -': ~.
I • - 'I
:~.
..,
.
55-/8 Thrombosis C/ Paraganglioma IF Schwenno ms

Key Facts
Termin ol ogy • Sigm oid sin us-jug ular hu lb (55-jll) thrombosis
• jugula r bulb 011) pscudomass, "leave-me-alo ne" lesion • Glom us jugul a re (GJ) par agan glioma
o f jugul ar foramen OF) • jugular forame n OF) schwa n noma
• 1B pseudol esio n occu rs when an asymm etri c, la rge • Ju gular forame n menin gioma
jugular bulh flow phenomen on sim u lates Clini cal Issu es
schwa n noma o r th rom bosis 0 11 most MR seq uen ces • Patient un dergoes MR of brain for u n relat ed
Imagin g Findings sym pto ms
• Best d iagnostic clu e: Co m plex MR sign al in ten sity in • j B pseud o lesion re por ted on MR lntc rp rctatfon as
JB wit h no rmal j F co rtex, jugu lar sp ine possible J B th rom bosis or JF sch wa n n o ma
• NEer: Bone CT: Asym metri c JB, with in tact cortical • T'bonc CT reveals no rmal , asym met ric j B wit h intact
ma rgins & jugu lar spine co rtical ma rgins & spine
• Furth er MR work-up shows evidence of flowi ng bl ood
3
Top Differential Diagn oses on o ne or more seque nces with norma l MRV
• High -riding jugu la r bulb • Patient retu rns to th eir regul ar life. "cu red" by 15
• jugular bulb diverticulum follow-up imag ing
• Deh iscen t jugular bulb
o No rma l, asy m me trically large sigmoi d sin us N jl\ fill • MR: Depends o n stage o f clo t
with co n tras t o Will be b right in th e subac ute ph ase o n TI MR
o Usua lly righ t-side d seco nda ry to me themogl obin
o jugular bulb often h igh-r id in g
Glo mu s jugul are (GJ) pa ragan gliom a
Imagin g Re commendation s • Bone CT: Perm eati ve bo ny c ha nges alo ng j fi
• 'l-bo ne CT helpful to show in tact surrounding jIl su perola te ral ma rgins
cor tica l margi ns & spine • Tl C~ l\.1R: j ugular foramen mass wit h h igh veloci ty
• T1 C+ MR, MRV & phase cont rast MRV all add to flow voids ("pepper")
certa in ty of di agn osis • Vector o f spread: 5uperolate ral fro m jll to floor midd le
ca r hyp otympan um
IDIFFERENTIAL DIAGNOSIS ju gul ar fo ra men OF) sc hwa nno m a

• Hone CT: Smoothly sca llo ped en larged jug ular
High - rid ing jug ular b ulb foramen
• Defined by most ceph alad po rtio n exte n d ing superio r • T1 C+ MR: Dumbbell -sh aped e n ha nci ng mass in
to floor of lAC ± basal turn o f coc h lea jug ular foramen
• Bone Cf: jB co rtical mar gin s intact ; does not enter • Vecto r o f spread: Exte nd supero me d ial a long expected
m iddl e ca r cou rse of cra n ial ner ves 9- 11
• May provides o ne context for jugular bulb
pseudol eslon
jugu lar fora me n meningioma
o In creased sign al from co m p lex flow may be p resen t • Bon e Cf: Pcrm ca tive-sclero tlc or h yp ero stotic bony
on so me MR seq uen ces c ha nges alongjB margin s
• Tl C- MR: No high velo cit y flow vo ids
jugul ar bulb diverti culum • T l C+ MR: Oural "ta ils" alo ng margins of avid ly
• Focal pol ypo id mass ex te nd ing from cep ha lad JB into e n ha nci ng mass
middle ea r • Vector o f spread: Ce n trifugal alo ng dura l su rfaces
• Sigmoid pla te is intact
• Bone Cf: Smo o th ho ne margin s
De hisce nt jugul ar b ulb
IPATHOLOGY
• Usua lly present wit h vascular "mass" beh ind intact TM Ge neral Feat ures
• Bone Cl: Sigmo id plat e d ehi scen ce • Ge ne ral path co m ments
• May provides o ne co n text for jug ular bul b o Emb ryo logy-ana to my
pseudol esio n • jugular bulb formation is pos t nata l event
o Increased sign al from co m p lex flow ma y be p resent • Begin s wh en infant changes fro m "feta l" to
o n some MR seq ue n ces "pos t na ta l" circulatory type (fro m lyin g d own to
erect posture)
Sigmo id sinus-jugula r b ulb (SS-jB) • jugula r bulb s l st see n o n a nglog rams of chi ldren
throm b o sis z 2 yea rs of age
• CEC r: Loo k for in tralumina l thrombus • jB thoug ht to result from "pou n d ing effects " of
o Vasa vaso ru m of vei n wa ll Illay e n ha nce as a thi n ascen d ing n egative pu lse waves origi na ti ng in
wh ite rim right at riu m
• Bo n e C l: Nor ma l bon e in jugul ar foramen
• Hammering effects of negati ve pu lse waves hi tti ng • Do n ot m istak e j b p seudolesio n for jugul ar foram en
roo f of "j ugular sinus" en larges surrounding osseus sch wa n noma o r ve no us sin us thromb osis
st ruc tu re
• Direct lin e from right atr iu m to rig ht "jug u lar Image Interpret ati on Pearl s
sin us" co m pared to ind irect co u rse o n left via left • If jugul a r bulb pseudo lesio n is obse rved wh ile pati en t
bra chiocepha lic ve in ex p lai ns why right jll is is in im agin g cen te r, a dd M IN to protocol to cla rify
lar ger th a n left • If st ill uncertain as to etio logy o f jugula r for a me n
• Large, asymm etric jug ula r bu lb provide s sett ing lesio n , fo llow -up wit h T-b one CT
w he re MR sig nal from slow, com p lex flow m ay o Use 'l-bone CT to eva lua te bo n y m a rgin s o f j l'
mim ic pathology • Jugu la r bu lb pseudole sio n is d iagn osed w h en MR &; c r
• Et iology: Co ngenita l no rmal vari atio n shows t h e fo llowin g
• Epide m io logy : Most co m mon "lesio n" o f jugu lar o MR: Large jB m ixed-hi gh signa l d oes n o t ho ld up on
fo ramen fo und o n M R im agin g a ll MR seq ue nces
o T..bone CT: Shows n o rm a l bon y m a rgin s to a n
3 Gross Pat ho logic & Surgica l Feature s asy m me t rica lly la rge ± h igh jugu la r b ul b
• No rma l va ria n t, n o t a surg ica l lesio n
16
ICLINICAL ISSU ES I. Bllgcn C ct ul: j ugu lar bu lb di verticul a: clinica l a nd
rad iolog ic aspect s. Otola ryngol Head Neck Su rg.
Presentatio n 128(3):382-6, 2003
• Most com mon signs/sy mpto ms 2. Kobanawa Se t ill : j ugu lar b ulb divertic ul um associa ted
o Asymptomatic with lower cra n ial ne rve palsy a nd mu ltip le aneurysms.
o Found in ci dentall y on brain MR durin g work-up for Surg Neurol. 53(6):559·62, 2000
un related sym pto ms 3. Tsunoda A: Sensorin eura l hea ring loss caused by it high
• Clinical profil e jugular hulb. j La ryngol 0101. t 14(t 1):H67-9. 2000
4. Palacios E ct al: Jug ular bulb appearing as a m ass. Ea r Nose
o Following sequence of events com mon ly associa ted
Tbroat .l. 78(8):536, 1999
w ith jugu lar bu lh pscudo lcston 5. Sa ito T e t al: High jugular hul h ad he ring to IIll' ea rd rum.
• Patie n t undergoes MR of brai n for unrelat ed Ann Otol Rhinol Laryngol. 108(6):620-2, 1999
sy m p to ms 6. Caldem eyer KS ct al: The jugu lar fo ram en : a review of
• JH pseudo lesion reported on Mll int erp retati o n as ana to my, m asses, an d im agin g ch aracter ist ics.
possible JB thrombo sis o r JF schwa n no ma Radiograph ies. 17(5):1123-39, 1997
• Patie n t is referred to n eu ro-otol ogist for therapy 7. Atilla S et al: Co m pute d to mographic evaluatio n of
• 'l -bone CT d one to clarify natu re a nd ex te n t of surgica lly sign ifica n t vascular var iatio ns related with the
temporal bon e. Eur .I Radiol. 20(1):52-6, 1995
lesion
8. Tomura N et al: Nor m al variatio ns o f the tem poral hon e on
• T-bo ne CT revea ls n orm al, asym metric JB wit h h igh-reso luti o n (.1 : t heir incide nce and clinica l
intact co rtica l ma rgi ns &: spine sign ificance. Clin Radi ol . 50 (3 ):144-8, 1995
• Furth er MR work-u p sh o ws evid en ce o f flowing 9. Tsu noda A ct a l: Laten t h igh jugu lar bulb: case report and
b lood on one or m o re sequen ces with no rm al sign ifica nce of neck co m pressio n test. ORLj
M RV Olorh ino!aryngol Relat Spec. 57(1):44-7, 1995
• Pati ent return s to th eir regul ar life, "cu red " by 10. Dietz RRet al : MR imaging and MR a ngiograp hy in th e
fol low-up im aging eva luati o n o f pu lsatile tin n itu s. AjNH ,\ 11I J Neuro radlol.
15(5):879-89, 1994
D em ographi cs I I. Okudera T et al: Develo pmen t or pos terior fossa dural
sin uses, em issa ry veins, a m i jugula r bulb: Mor phological
• Age: May be discovered at any age
and radi ol ogic stud y. Aj NR. 15:1R71 -fB , 1994
Natu ral Hi story & Progno sis 12. Pap pas DG j r et 31: Pctro us jugular m alpo sition
(diver ticul um) , Otolaryngo! Head Nec k Surg.
• Asym met rica lly la rge JB does n o t e n large with time
109(5):847-52, 1993
• No rm al a n atomic st ructure 13. Shouo n JC et a l: Te m pora l bo ne ven ou s ano ma ly of
• Su rgica l ex p lora tio n m ust be avo ided by rad io lo gist surgi cal sign ifica nc e. j La ryn go l Oto l. 103(1 ):10 1-6, 1989
m a kin g co rrect d iag n os is 14. Smit h B ct al : Dehiscen t jug ula r hulb. Ann Otol Rhinol
I.aryngol. 96(2 1'1 1):2] 2-3, 1987
Treatm ent 15 . Wad in K et a l: The jugu lar bu lb dl ver tlcu lum. /\
• No tr eat m e nt o r fo llo w-up req uir ed rad ioan ato m ic inve stiga tio n. Acta Rad lo l Dlagn (Stockh).
• Im po rta n t rad iol ogi c di ag no sis to prevent su rgica l 27(4):395-40 1, 1986
ex p lo ra tion 16. Sekha r LN et al: Tra nste m po ral approach 10 the skull base:
an anato mic al st udy. Ne uro surgery. 19 (5):799-808, 1986
17. Jinki ns JR et al: The abe rra n t jugu lar bulb. Co m put Radiol.
9(6):395·7, 1985
IDIAGNOSTIC CHECKLIST 18 . Ongre A: The jugu lar bulb o nce m ore. AJR Am J
Rocntgeno l. 135(5):1117, 1980
Co nside r 19 . Stern J et al: j ugular b ulb diverticula in medi al pctro us
• JR psc ud o leslon is m ost com mo n JB "lesion" bon e. A.lRAm ] Rocntgc nol. 134(5):959-61, 1980
• So, o nce an ab no rma lity is see n in JF on MR, 1st
q uest io n th at m ust be as ked is, "a m 1 looking at a
jugul a r bu lb pseu do lesion?"

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IMAG!:N POR RESON.

IRM 940324 FL5

Richard H. Wiggins III, MD Andre J. Macdonald, MBChB

Patricia A. Hudgins, MD Christine M. Glastonbury, MBBS

Michelle A. Michel, MD Joel K. Cure, MD

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Drawing s - Copy righ t ,\ m ir\ ys irK 20tH

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l'r tnt ed hy rrit....e n v, ,\ IIOIM. M 'lIl ilo oa. Ca nada

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To To ny Man cu so , w ho turn ed m e o n to H I'< N im a g i n g . To j eff Hall er I'<

Below are li sted the intrepid group o f ph ysician s wh o took the ti me

Thanks to yo u all. No hook of th is na t ure could have been don e

II. Ric Harn sbe rger, MD

An il T. Ah u ja, MO; H on g Kong , Ch ina

Will iam P. Dillon, i\.1 D

II. Hie Ha m sberger, Mil

Art Direction and Design

Medical Text Editing

Co nge nita l External Auditory Cana l

ka m say lIu nt Syndrom e 1- 1-20 EAC Exo stoses 1-2-22

Sarco idosis, C PA-lAC 1-1-24 F.AC Os teo ma . -2-24

Acousti c Schwa n noma 1- 1-26

Metastases, C PA-lAC 1- 1-36 Aberrant In tern a l Caro tid Arte ry 1-2-38

Ane ury sm , C PA-lAC 1- 1-40

CO M With Tym panosclerosls 1-2-56 Inner Ear, Miscell an eou s

In trat emporal Faci al N erve, Tum ors

Clivu s Co nge nita l

Cho rdom a, Clivus 1-3- 10 Dermoid and Epidermoid , O rbit 11 -1- 12

j ugula r Bulb Divert icul um 1-3-22

Hypog lossa l Sch wa n no ma 1-3-3 4 Optic Neurit is 11 -1-34

Du ral Sinuses Neoplasm, Beni gn Tum ors

Miscell an e ous Osteoma, Sin us 11-2-74

In fecti on & In fl amm ati on

Kim ura Disease 111-2 -16

Introdu cti on and Overview Ra n ula 111-4-26

SCCa, O ra l Tongu e 111 -4-3 4 Mo to r Dener vati on eNS 111-6 -8

SCC a, Mand ibul ar Alveo la r Rid ge 111 -4-38

Su blingu la r Gla nd Ca rcin o ma 111-4-42

Sarco ma , O ther, Ma stica to r Spa ce 111-6-24

Introdu cti on and Ove rview

Neop lasm Infection & In fl ammatio n

Miscellaneou s Neoplasm, Benign Tum ors

Metastatic Disease, Noda l, Pa rot id 111-7-30

Skin Mali g nan cy In Paro tid Nodes 111 -7-3 6

Normal Variants & Pseudolesion s Normal Variants & Pseudolesions

Vascular Infection & Inflammation

Ca rotid Arte ry Dissect ion, Nec k III -H- t 2

Glom us Vagale Paraganglio ma 111·8- 16

Posterior Cervical Space (PCS) Ne oplasm, Maligant Tum ors

SCCa , Spina l Accessory Node 11I -12-8 Lipoma IV-2-6

11I -12- 10 Liposarco m a IV-2- 10

Introdu ction and O verview

Infection & Inflammation

M alignant Tum ors

• Leaves spiral ga nglio n o f coc h lea as aud itory

CPA-lAC ANATOMY AND IMAGING ISSUES

o If unilat eral SNII L present, injury oc cu rred be twee n

IEMBRYOLOGY I.?EJECTED RE,FERENCES "

IIM AGE GALlERY

o Spread vecto r is cephalad int o medi a l m idd le cran ial

DDx: CPA Cyst ic Mass