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\NCIA
MAGNtTICA, SA DE C.V.
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
111
AM IRSYS·
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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.
v
CONTRIBUTORS
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.
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!
XII I
ACKNOWLEDGMENTS
Illustrations
L11ll' R. Ben nion. ~1 S
Richard <:00 1111>:-;, '''is
ja mes A. Cooper, MD
Image/Text Editing
An gie D, Masrarcnaz
Kacrl l M ain
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
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
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)
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
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)
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
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
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
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
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>
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
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
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)
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
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>
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
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
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
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
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).
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
CPA-lAC ANATOMY AND IMAGING ISSUES
..
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) .
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 ).
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
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.
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
ARACHNOID CYST, CPA-lAC
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
ARACHNOID CYST, CPA-lAC
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.
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
IDIAGNOSTIC CHECKLIST
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
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 ) .
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
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).
•
8e" Palsy Sarcoid, CPA Met.15t<lSis, lAC
RAMSAY HUNT SYNDROME
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
RAMSAY HUNT SYNDROME
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
ISELECTED REFERENCES
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
RAMSAY HUNT SYNDROME
IIMAGE GAL LERY 1
23
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
f
o Systemic sarcoidos is: Pulmon ary sympto ms .::f! ~
.. . .-'
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.
DD x: CPA Ma ss
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)
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) .
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
• 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
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.
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)
/ 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}.
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
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.
(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' .
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
ISELECTED REFERENCES
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
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 ).
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 .
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
VASCULAR LOOP COMPRESSION, CPA-lAC
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)
Microscopi c Features
• 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
VASCULAR LOOP COMPRESSION, CPA-lAC
IIM AGE GALLERY 1
49
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 .
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 ),
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
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
IIM AG E GALLERY
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).
DD x: EAC Le sion s
EAC Chole steatoma Med ial Can al Fibrosis Kera tos is O b turans EAC SCCa
NECROTIZING EXTERNAL OTITIS
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
• 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
NECROTIZING EXTERNAL OTITIS
• Im munologic abn orm alities (i.e., neutropenia),
neoplasm, dermatitis, medicati ons, iatrogenic
IDIAGNOSTIC CHECKLIST
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
NECROTIZING EXTERNAL OTITIS
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 ).
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) ,
IDIFFERENTIAL DIAGNOSIS
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
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/.
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
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~ .
DD x: EAC l e sion
'
,
("""""'-
,...!"f .., J
4ft
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
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
EAC MEDIAL CANAL FIBROSIS
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
IDIAGNOSTIC CHECKLIST
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
EAC MEDIAL CANAL FIBROSIS
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 .
2
22
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
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.
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
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}.
DD x: EAC Lesion
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
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.
I\cq . Cho /('Slealom a (flO/esterol Granulom a Glom us Tvmpen icum F.trial Sch wannom a
CONGENITAL CHOLESTEATOMA, MIDDLE EAR
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)
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
CONGENITAL CHOLESTEATOMA, MIDDLE EAR
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).
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)
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.
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
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
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).
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\ \' ).
DDx: Lesions Invo lving Horizo nta l Segme nt Petro us Facia l Ne rve
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
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.
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
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}.
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) .
ff:"
.'
-~
-'-'. ' . .•
, 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
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).
2 Parallel Lines Incus Lon g Pro cess Ice Cream COile Malleus H eed / Neck
COM WITH OSSICULAR EROSIONS
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
COM WITH OSSICULAR EROSIONS
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
COM WITH OSSICULAR EROSIONS
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~.~ .
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
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
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
• 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
ACQUIRED CHOLESTEATOMA, PARS FLACCIDA
• 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.
.
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:
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
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
Image Interpretati on Pearls
• Coro nal C I' necessary to assess lateral semicircular
ca nal integ rity &: int act tegm en
ACQUIRED CHOLESTEATOMA, PARS TENSA
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.
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
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
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
ISELECTED REFERENCES
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
Microscopi c Features
• 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.
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
CHOLESTEROL GRANULOMA, MIDDLE EAR
• 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
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
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.
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
ID I FFERENTIA L DIAGNOSIS
IDIAGNOSTIC CHECKLIST
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
:(~;-~~~,:_<.~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) .
Dehisceru luguler Bulb Aberranl /CA Glomus Tym panicum Glom us Jugulare
MIDDLE EAR MENINGIOMA
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
MIDDLE EAR MENINGIOMA
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
IDIAGNOSTIC CHECKLIST
• 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"
(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.
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
GLOMUS TYMPANICUM PARAGANGLIOMA
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
GLOMUS TYMPANICUM PARAGANGLIOMA
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
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.
- 01 f I
', ~
~ J.[ -" ~
.' ;': ~, 'lLl1
, ,
I~ ,~ , i . ".
I- ". I
I
'.
,:' )1"
~ \ -.; .... . "
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
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 ).
~ -
, ~. ,
•
"
~ ~
'.,. '~
~
J
~'' ,-.
•
......---'
" • ,..JI.,
c • •
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
!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
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
OSSICULAR PROSTHESIS
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
Image Interpretation Pearl s
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
ISELECTED REFERENCES
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
OSSICULAR PROSTHESIS
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.
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 ) .
IPATHOLOGY
ISELECTED REFERENCES
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
Micro scopic Features
• Inner car st ructu res not presen t
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.
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
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.
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
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).
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 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
LARGE ENDOLYMPHATIC SAC ANOMALY
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
LARGE ENDOLYMPHATIC SAC ANOMALY
IIM AG E GALLERY
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).
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
• •
(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
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)
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 ).
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
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
• Most co m mo n signs/sym pto ms
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
INTRALABYRINTHINE SCHWANNOMA
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).
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 ).
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
IIM AG E GALLERY
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).
• 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
Natural History & Progn osis
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
IDIAGNOSTIC CHECKLIST
• 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
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.
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
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).
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
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.
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
FENESTRAL OTOSCLEROSIS
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
FENESTRAL OTOSCLEROSIS
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).
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.
Osteogen. tmperiects PJgel D isease Fib rous Dysplasia Ost eoradion ecro sis
COCHLEAR OTOSCLEROSIS
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
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
COCHLEAR OTOSCLEROSIS
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).
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
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
IPATHOLOGY
ISELECTED REFERENCES
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
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}.
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
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"'}.
. --..
"1
. f:c" .-
/~ _.~
, \ ',:
'\ .
Trapped Fluid Apical Petrositis Cholesterol Granuloma PA !\m.' urysm
CHOLESTEATOMA, PETROUS APEX
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
CHOLESTEATOMA, PETROUS APEX
• 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
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}.
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
ICLINICAL ISSUES
ISELECTED REFERENCES
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
IDIAGNOSTIC CHECKLIST
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
PETROUS APEX CEPHALOCELE
IIM AGE GALLERY
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 /~.
IDIAGNOSTIC CHECKLIST
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
2
162
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
TRAPPED FLUID, PETROUS APEX
• 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
TRAPPED FLUID, PETROUS APEX
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
~
' , Oil tht' right (arr o w ) . Nolin'
), ." , . th e db w /l ce of t'fl hafK em ent
..,
.
.,,'~\
Varia nt
' \ .' ~.'
\. ,. . "~.: . - t j
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.
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
IDIAGNOSTIC CHECKLIST
Co nside r
• Co nside r CG-PA in any non-aggressive lesion of PA
with h igh T1 & T2 signal
CHOLESTEROL GRANULOMA, PETROUS APEX
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.
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
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
leA ANEURYSM, PETROUS APEX
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 } .
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).
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
Natural History & Progn osis
• 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
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
Microscopic Features
• Makes di agn osis of speci fic tumor typ e
,6""
I
. '".; '"
.~
(t ' ...
'
~.
t~,,:
.\
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.
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
Micro scopic Features
• 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
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.
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
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) .
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)
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
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.
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
..
·
Microscopic Features
• 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
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).
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
FACIAL NERVE HEMANGIOMA, T-BONE
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 ).
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
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
PERINEURAL PAROTID MALIGNANCY, T-BONE
IIM AG E GALLERY
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.
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)
TEMPORAL BONE FRACTURES
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
ISELECTED REFERENCES
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
TEMPORAL BONE FRACTURES
IIM AG E GALLERY
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).
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
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 ).
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
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!
FIBROUS DYSPLASIA, T-BONE
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
~tj'."~r~
Otosyphilis Osteo radion ecro sis Cochlear O tosclerosis
C
Fibrous Dysplasia
PAGET DISEASE, T-BONE
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
• 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
PAGET DISEASE, T-BONE
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
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).
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
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.
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
POSTIRRADIATED T-BONE
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
(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....'],
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
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.
• 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
SKULL BASE ANATOMY AND IMAGING ISSUES
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....,).
DD x: Cliva l Le sions
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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
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.
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
IDIAGNOSTIC CHECKLIST
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
Microscopi c Features
• 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
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).
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
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 \ ') .
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.
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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