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Association between superior semicircular canal dehiscence and other dehiscences in temporal bone

Whyte, J.; Fraile, J.; Garcia-Barrios, A.; Lahoz, M.; Crovetto, R.; Cisneros, A.I.; Whyte, A.

Abstract

Background: The study of the association between superior semicircular canal and other dehiscences in the temporal bone. Materials and methods: We have studied computed tomography of radiologically diagnosed people with superior or posterior semicircular canal dehiscences, in four health centres. In addition, we have studied one isolated human temporal bone, one skull and one cadaver head belonging to the collection of the Department of Human Anatomy and Histology of the University of Zaragoza that had dehiscence in the superior semicircular canal. Results: The most frequent association that we observed was between superior semicircular canal dehiscence and tegmen tympani dehiscence (37.33%). Three cases (two clinical cases and one isolated temporal bone) showed multiple associated dehiscences (tegmen tympani, mastoid antrum, posterior semicircular canal, internal auditory canal, glenoid cavity, tympanum bone and geniculate ganglion) associated with superior semicircular canal dehiscence Conclusions: When the superior semicircular canal dehiscence is associated to other in the petrous bone (tegmen tympani, mastoid antrum, posterior semicircular canal, internal auditory canal) could be grouped into the same syndrome called "otic capsule syndrome", since they have the same origin and common aetiology (otic capsule). Whyte, J.; Cisneros, A.I.; Garcia-Barrios, A.; Fraile, J.; Whyte, A.; Crovetto, R.; Lahoz, M.

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Folia Mo phol. Vol. 79, No. 4, pp. 823–828 DOI: 10.5603/FM.a2019.0138 Copy igh © 2020 Via Medica ISSN 0015–5659 jou nals. iamedica.pl O R I G I N A L A R T I C L E 823 Add ess o co espondence: D . A.I. Cisne os, Ana omy and His ology Depa men , School o Medicine, Za agoza Uni e si y, Spain, e-mail: aicisne @uniza .es Associa ion be ween supe io semici cula canal dehiscence and o he dehiscences in empo al bone J. Why e1, A.I. Cisne os1, A. Ga cia-Ba ios1, J. F aile2, A. Why e3, R. C o e o4, M. Lahoz1 1Ana omy and His ology Depa men , School o Medicine, Za agoza Uni e si y, Spain 2Pa hology Depa men , Miguel Se e Hospi al, Spain 3Pa hology Depa men , School o Ve e ina y, Za agoza Uni e si y, Spain 4Bilbao Clinic, Spain [Recei ed: 14 Oc obe 2019; Accep ed: 3 Decembe 2019] Backg ound: The s udy o he associa ion be ween supe io semici cula canal and o he dehiscences in he empo al bone. Ma e ials and me hods: We ha e s udied compu ed omog aphy o adiologically diagnosed people wi h supe io o pos e io semici cula canal dehiscences, in ou heal h cen es. In addi ion, we ha e s udied one isola ed human empo al bone, one skull and one cada e head belonging o he collec ion o he Depa men o Human Ana omy and His ology o he Uni e si y o Za agoza ha had dehiscence in he supe io semici cula canal. Resul s: The mos equen associa ion ha we obse ed was be ween supe io semici cula canal dehiscence and egmen ympani dehiscence (37.33%). Th ee cases ( wo clinical cases and one isola ed empo al bone) showed mul iple associ- a ed dehiscences ( egmen ympani, mas oid an um, pos e io semici cula canal, in e nal audi o y canal, glenoid ca i y, ympanum bone and genicula e ganglion) associa ed wi h supe io semici cula canal dehiscence Conclusions: When he supe io semici cula canal dehiscence is associa ed o o he in he pe ous bone ( egmen ympani, mas oid an um, pos e io semici cula canal, in e nal audi o y canal) could be g ouped in o he same synd ome called “o ic capsule synd ome”, since hey ha e he same o igin and common ae iology (o ic capsule). (Folia Mo phol 2020; 79, 4: 823–828) Key wo ds: bony laby in h, o ic capsule, bony de ec s g oup, hi d window INTRODUCTION A miles one in he s udy o supe io semici cu- la canal (SSC) pa hology was he manusc ip by Mino [22] in 1998, who i s desc ibed he p es- ence o dehiscence in he canal. This dehiscence is a a e and uncommon pe iphe al es ibulopa hy, which is cha ac e ised by a lack o bone co e age o he SSC in he a ea closes o he du a o he middle ce eb al ossa. Since ha da e, s udies ha e been conduc ed o in es iga e i s p e alence, pa hophysiology, clinical a iables, diagnosis and su gical p ocedu es. The e a e cu en ly wo heo ies abou he gen- esis o dehiscence: congeni al (s op o delay in he ossi ica ion o he canal du ing i s de elopmen ) and acqui ed (bone laye up u e co e ing he canal as 824 Folia Mo phol., 2020, Vol. 79, No. 4 a esul o ano he e en , as inc eased in ac anial p essu e, auma, e en a e childbi h o coi us). The associa ion o SSC dehiscence wi h o he ea diseases [4, 9, 27] and o he dehiscences p oduced in he bone laby in h is cu en ly being in es iga ed. In his line, associa ions be ween dehiscence o he SSC and dehiscence o he pos e io semici cu- la canal ha e been desc ibed [6, 15, 18, 20, 21]. Bo h dehiscences show a simila , nonspeci ic clini- cal p esen a ion, some imes he audi o y symp oms (hypoacusis, inni us) and some imes he es ibula ones (dizziness, nys agmus, Tullio phenomenon) p e- domina e; hey may no e en p esen any. The e may also be cases whe e he e a e dehis- cences in o he s uc u es such as he egmen ym- pani [1, 5, 24], genicula e ganglion [10, 13, 17] and in e nal audi o y canal [19]. The objec i e o his s udy is o de e mine adio- logically on he one hand, he possible associa ions be ween dehiscence in he supe io and pos e io semici cula canal, and on he o he , bo h dehiscences wi h o he mo phological al e a ions in he empo al bone (glenoid ca i y and in e nal audi o y canal). MATERIALS AND METHODS We ha e s udied compu ed omog aphy (CT) o adiologically diagnosed people wi h supe io o pos- e io semici cula canal dehiscences, in ou heal h cen es: Basu o and C uces Hospi al, Gene al Hos- pi al o he De ence and Miguel Se e Hospi al in Za agoza. An in o med consen o m was signed by e e y pa icipa ing pa ien . All he pa ien s ac- knowledged ha hey canno be iden i ied ia he pape ; he esea che s ully anonymised hem. The e hics commi ee o each cen e app o ed his s udy in acco dance wi h he guidelines o he Helsinki Decla a ion o 1983. The s udies ha e been pe o med wi h mul i-splice helical CT equipmen (Philips B illiance 6), ob aining he images on he axial plane and wi h he pa ien ’s neck in hype ex ension o a oid di ec adia ion dam- age o c ys alline. La e , co onal econs uc ions ha e been ca ied ou in all he cases, in he plane o he SSC o each ea (Pöschl plane) and in axial planes o he pos e io semici cula canals. The oo o he glenoid ca i y has been s udied wi h sagi al plane econs uc ions. The “ aw da a” ha e been econs uc ed using a bone algo i hm. The ollowing adiological p o ocols o he acqui- si ion and o ma ing o he images ha e been used: 2 × 0.6 mm collima ion, 0.65 mm splice hickness, 0.32 mm splice inc ease, 0.75 s o a ion ime, 0.38 pi ch, 120 CV, 300 mAs, 1024 × 1024 ma ix, 180 mm ield o ision, 0.5 mm econs uc ion hickness and 0.5 mm econs uc ion inc ease. The CT scans we e upda ed by 3 adiologis s wi h mo e han 10 yea s o expe ience in neu o adiology and e iewed by he signa o ies o he manusc ip , all o hem wi h ex ensi e expe ience in dehiscence o semici cula canals. RESULTS We ha e pe o med a adiological s udy on a o al o 61 pa ien s (20 men, 41 women) wi h an a e age age o 55.98 yea s ( anging 2–89), in e ms o he possible associa ions be ween (supe io and pos e i- o ) e ical semici cula canal dehiscence, and o he dehiscences loca ed in he empo al bone. Two o he pa ien s, who p esen ed unila e al SSC dehiscence, we e sis e s. Se en y one cases o SSC dehiscence and 4 o pos- e io semici cula canal dehiscence we e de ec ed. Thi y-one dehiscences we e de ec ed on he igh side in he SSC, 20 on he le side, and bila e al dehiscences (28.16%) we e obse ed in 10 pa ien s (20 cases) (Table 1). Two dehiscences we e de ec ed on he igh side in he pos e io semici cula canal, and ano he wo on he le side, and he e was one bila e al case. The mos equen associa ion ha we obse ed was be ween SSC dehiscence and egmen ympa- ni dehiscence, which was e i ied in a o al o 28 (39.44%) cases. Two cases p esen ed mul iple associa ed dehis- cences (Fig. 1). Thus, he i s case p esen ed bila e al supe io semici cula dehiscences (Fig. 1A), bila e al egmen ympani dehiscences, and dehiscence in he glenoid ca i y o he empo omandibula join on he Table 1. Dis ibu ion o supe io semici cula canal (SSC) dehiscence and pos e io semici cula canal (PSC) dehiscence Righ side Le side Bila e al SSC 31/71 (43.67%) 20/71 (28.17%) 20/71 (28.16%) PSC 1/4 (25%) 1/4 (25%) 2/4 (50%) 825 J. Why e e al., Associa ion be ween SSC dehiscence and o he s igh side (Fig. 1B). The o he cases showed bila e al supe io and pos e io semici cula canal dehiscences (Fig. 1D), bila e al egmen ympani dehiscences, and dehiscence o he oo o he in e nal audi o y canal (Fig. 1C) on he igh side (Table 2). DISCUSSION Supe io semici cula canal dehiscence, as a s u- c u al cause o dizziness and hypoacusis, is only 20 yea s old [22], so he e a e s ill knowledge gaps ela ed o his pa hology. Fi e yea s la e i would be K ombach e al. [14] who desc ibed pos e io semi- ci cula canal dehiscence. The i s au ho s who desc ibed associa ions be- ween de ec s o he bony co e age o he SSC and o he pa s o he bony laby in h was Mino [23] who obse ed an associa ion be ween egmen dehiscence and he SSC. One yea la e , Gianoli [10] unde lined he high incidence o pa ien s who also p esen ed a- diog aphic supe io canal dehiscence wi h genicula e ganglion dehiscence. La e , Manza i and Modugno [18] and Chen e al. [3] desc ibed isola ed cases o SSC dehiscences associa ed wi h pos e io canal dehis- cences, he o me in adul s, and he la e in child en. In la ge se ies o pe sons wi h SSC dehiscence, he a ec a ion was ound o be bila e al, in pe cen ages ha a ied be ween 16% and 37%. These da a a e e y simila o hose obse ed by us, as we ha e eg- is e ed an incidence o 34.48%. Figu e 1. Di e en associa ions be ween supe io semici cula canal dehiscence wi h o he s in he empo al bone; A. Supe io semici cula canal dehiscence (black a ow); B. Tegmen ympani (black a ow), glenoid ca i y (whi e a ow); C. Tegmen ympani (black a ow), in e nal audi o y canal (IAC; whi e a ow); D. Pos e io semici cula canal dehiscence (PSCD; whi e a ow). Table 2. Associa ion o empo al bone dehiscences TTD IACD GFD SSCD 28/71 (39.44%) 1/71 (1.41%) 1/71 (1.41%) PSCD 2/4 (50%) 1/4 (25%) 0/4 (0%) SSCD — supe io semici cula canal dehiscence; PSCD — pos e io semici cula canal dehiscence; TTD — egmen ympani dehiscence; IACD — in e nal audi o y canal dehi- scence; GFD — glenoid ca i y dehiscence A B C D 826 Folia Mo phol., 2020, Vol. 79, No. 4 The p esence o bila e al pos e io semici cula ca- nal dehiscences is no e y equen , and hey a e lim- i ed o isola ed cases such as hose desc ibed by Saxby e al. [30] and Bea e al. [2]. We ha e also obse ed one single isola ed case o bila e al dehiscence, bu we mus conside ha pos e io semici cula dehis- cence is much less equen han supe io semici cula dehiscence, being 3.6% and 0.6%, espec i ely [5]. The associa ion be ween supe io and pos e io ca- nal dehiscence has been desc ibed by K ombach e al. [14], who obse ed 8 pa ien s wi h pos e io and SSC dehiscences. Chen e al. [3] also ob ained 1 case wi h bo h dehiscences. Gopen e al. [11] obse ed ha 2 o he 12 pa ien s wi h pos e io semici cula canal dehiscences also p esen ed SSC dehiscences. Russo e al. [29] desc ibed ha in 3 o he 5 cases wi h pos- e io semici cula canal dehiscence we e associa ed o dehiscences in he supe io canal. Saxby e al. [30] communica ed 1 case o a child wi h bila e al supe io unila e al and pos e io canal dehiscence. Kundaga i e al. [15] also p esen ed a case o a pa ien wi h bila - e al pos e io semici cula canal dehiscence, oge he wi h igh supe io canal dehiscence. We ound only 1 case in which he 2 dehiscences we e associa ed, among he 61 pa ien s s udied, which deno es he low equency o his associa ion since hey we e selec ed pa ien s. The mos equen associa ion ound, which has clinical epe cussion, was wi h he egmen ympani. C o e o e al. [5] ound a 36.4% incidence o dehis- cen egmens associa ed wi h SSC dehiscence, and Nada aja e al. [24] ound 76%. In he e e se s udy, El Hadi e al. [7] ound a 56.5% incidence o dehis- cen semici cula canals in pa ien s who p esen ed e i ied dehiscence o he egmen ympani. We ha e obse ed his associa ion in 28 o he cases s udies (37.33%) and we hink ha he explana ion would be, as p oposed by F aile Rod igo e al. [8], ha bo h s uc u es ha e a common o igin, he o ic capsule, and ha he p ima y cen es o he ex e nal o la e al SSCs, which, when hey g ow, ex end o he base o he egmen al p olonga ion, collabo a e in he ossi ica ion o he egmen ympani. In addi ion o his, he ex e nal pe ios ium laye , which sepa a es he egmen om he middle c anial ossa, is a con inua ion o he laye ha co e s he SSC. We ha e no ound in he li e a u e any manusc ip ha ela es he pos e io semici cula canal dehiscence wi h he absence o egmen ympani, while we ha e ound i in in 2 o he 4 cases o pos e io semici cula canal dehiscence (50%). The associa ion o SSC dehiscences wi h in e nal audi o y canal dehiscences has only been desc ibed in li e a u e by Manza i and Scagnelli [19]. We ha e obse ed 1 case in which SSC dehiscences we e as- socia ed wi h in e nal audi o y canal and pos e io canal dehiscence. In he li e a u e consul ed, we ha e no obse ed any associa ion be ween SSC dehiscence and glenoid ca i y dehiscence o he empo omandibula join o o he ympanic bone, and only Ku e al. [16] associa es SSC dehiscence wi h symp oms in he empo omandibula join , desc ibing he la ening o he condyle, i egu- la i ies on he su ace and o ma ion o os eophy es, as adiological indings. We ha e seen hese associa ions in a adiological s udy which leads us o conside ha one same cause could ac du ing de elopmen , bo h in he squamous po ion o he empo al bone and in he o ic capsule, al hough he o igin is no ye clea . The e o e, we ha e obse ed ha he cases o SSC dehiscence associa ed wi h o he empo al bone dehiscences a e ew and a be ween, ei he loca ed in he pe ous apophysis, in he squamous po ion o he empo al bone o in he ympanic bone. To unde s and he ae iology o mul iple dehiscenc- es, an emb yological cause ha ac s upon he o ic cap- sule mus be sough . Thus, Isaacson and V abec [13] and Pa k e al. [26] obse ed ha he bony o ic cap- sule was signi ican ly hinne in pa ien s wi h associ- a ed SSC dehiscence. G acia-Tello e al. [12] concludes ha he exis ence o supe io semici cula dehiscence is associa ed wi h he hinning o he bone o he ca- nal on he opposi e side, and Manza i and Modugno [18] hink ha he associa ion be ween wo bone s uc u es ha p esen dehiscences, and de i e om he o ic capsule, is p esen ed as suppo o he idea ha i is a congeni al ype diso de . Nies en e al. [25] in o m o h ee amilies in which he i s -deg ee ela i es show SSC dehiscences, and El Hadi e al. [7] egis e ed wo wins in he se ies ha hey s udied, wi h bo h SSC dehiscence and egmen ympani dehiscence. These e en s sugges gene ic ae iology. In ou s udy, we ha e ound SSC dehiscence in 2 sis e s, which sugges s, in ha case, a gene ic componen . Howe e , in ou s udies abou COACH synd ome and in he s udies conduc ed by Roknic e al. [28], demons a ing a cons an gene ic diso de associa ed wi h hese dehiscences has no been possible. Wackym e al. [31] coins a new e m, “o ic capsule synd ome”, o de ine he exis ence o associa ed de- 827 J. Why e e al., Associa ion be ween SSC dehiscence and o he s hiscences be ween he di e en semici cula canals, and he bases his on he ac ha hese pa hologies ha e e y simila symp oms, whe he implan ed in (supe io and pos e io ) e ical canals o in he (la e - al) ho izon al canal. We would use his e m o when in he CT scans ind mul iple dehiscences o s uc u es de i ed om he o ic capsule ( egmen ympani, mas- oid an um, pos e io semici cula conduc , in e nal audi o y canal). We ha e no ound clinical ea u es o dis inguish pa ien s wi h SSC dehiscence om he pos e io , and bo h wi h o he empo al bone dehiscences. CONCLUSIONS The e o e, and in conclusion, we hink ha SSC dehiscence may ha e a congeni al cause and be as- socia ed wi h o he dehiscences, and in ou clinical p ac ice, we mus seek hese associa ions whene e we encoun e a diso de in he bony co e age o he empo al bone, as al eady ecommended when a egmen ympani o SSC dehiscence is diagnosed. 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