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J Neurol Surg B Skull Base 2012; 73(06): 365-370
DOI: 10.1055/s-0032-1324397
Review Article
Georg Thieme Verlag KG Stuttgart · New York

Characteristics and Management of Superior Semicircular Canal Dehiscence

Andrew Yew
1   Department of Neurosurgery, University of California Los Angeles, Los Angeles, California, United States
,
Golmah Zarinkhou
1   Department of Neurosurgery, University of California Los Angeles, Los Angeles, California, United States
,
Marko Spasic
1   Department of Neurosurgery, University of California Los Angeles, Los Angeles, California, United States
,
Andy Trang
1   Department of Neurosurgery, University of California Los Angeles, Los Angeles, California, United States
,
Quinton Gopen
3   Department of Head and Neck Surgery, University of California Los Angeles, Los Angeles, California, United States
,
Isaac Yang
1   Department of Neurosurgery, University of California Los Angeles, Los Angeles, California, United States
2   Jonsson Comprehensive Cancer Center, University of California Los Angeles, Los Angeles, California, United States
› Author Affiliations
Further Information

Publication History

03 March 2012

08 June 2012

Publication Date:
08 August 2012 (online)

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Abstract

Objectives To review the characteristic symptoms of superior semicircular canal dehiscence, testing and imaging of the disease, and the current treatment and surgical options.

Results and Conclusions Symptoms of superior semicircular canal dehiscence (SSCD) include autophony, inner ear conductive hearing loss, Hennebert sign, and sound-induced episodic vertigo and disequilibrium (Tullio phenomenon), among others. Potential etiologies noted for canal dehiscence include possible developmental abnormalities, congenital defects, chronic otitis media with cholesteatoma, fibrous dysplasia, and high-riding jugular bulb. Computed tomography (CT), vestibular evoked myogenic potentials, Valsalva maneuvers, and certain auditory testing may prove useful in the detection and evaluation of dehiscence syndrome. Multislice temporal bone CT examinations are normally performed with fine-cut (0.5- to 0.6-mm) collimation reformatted to the plane of the superior canal such that images are parallel and orthogonal to the plane. For the successful alleviation of auditory and vestibular symptoms, a bony dehiscence can be surgically resurfaced, plugged, or capped through a middle fossa craniotomy or the transmastoid approach. SSCD should only be surgically treated in patients who exhibit clinical manifestations.

Keywords

superior semicircular canal dehiscence syndrome - vertigo - hearing loss - autophony - Tullio phenomenon
 

  • References

  • 1 Chien WW, Carey JP, Minor LB. Canal dehiscence. Curr Opin Neurol 2011; 24: 25-31
    CrossrefPubMedGoogle Scholar
  • 2 Amoodi HA, Makki FM, McNeil M, Bance M. Transmastoid resurfacing of superior semicircular canal dehiscence. Laryngoscope 2011; 121: 1117-1123
    CrossrefPubMedGoogle Scholar
  • 3 Minor LB. Clinical manifestations of superior semicircular canal dehiscence. Laryngoscope 2005; 115: 1717-1727
    CrossrefPubMedGoogle Scholar
  • 4 Friedland DR, Michel MA. Cranial thickness in superior canal dehiscence syndrome: implications for canal resurfacing surgery. Otol Neurotol 2006; 27: 346-354
    CrossrefPubMedGoogle Scholar
  • 5 Minor LB, Carey JP, Cremer PD, Lustig LR, Streubel SO, Ruckenstein MJ. Dehiscence of bone overlying the superior canal as a cause of apparent conductive hearing loss. Otol Neurotol 2003; 24: 270-278
    CrossrefPubMedGoogle Scholar
  • 6 Minor LB, Solomon D, Zinreich JS, Zee DS. Sound- and/or pressure-induced vertigo due to bone dehiscence of the superior semicircular canal. Arch Otolaryngol Head Neck Surg 1998; 124: 249-258
    CrossrefPubMedGoogle Scholar
  • 7 Mikulec AA, McKenna MJ, Ramsey MJ , et al. Superior semicircular canal dehiscence presenting as conductive hearing loss without vertigo. Otol Neurotol 2004; 25: 121-129
    CrossrefPubMedGoogle Scholar
  • 8 Hirvonen TP, Carey JP, Liang CJ, Minor LB. Superior canal dehiscence: mechanisms of pressure sensitivity in a chinchilla model. Arch Otolaryngol Head Neck Surg 2001; 127: 1331-1336
    CrossrefPubMedGoogle Scholar
  • 9 Stimmer H, Hamann KF, Zeiter S, Naumann A, Rummeny EJ. Semicircular canal dehiscence in HR multislice computed tomography: distribution, frequency, and clinical relevance. Eur Arch Otorhinolaryngol 2012; 269: 475-480
    CrossrefPubMedGoogle Scholar
  • 10 Brantberg K, Ishiyama A, Baloh RW. Drop attacks secondary to superior canal dehiscence syndrome. Neurology 2005; 64: 2126-2128
    CrossrefPubMedGoogle Scholar
  • 11 Songer JE, Rosowski JJ. The effect of superior canal dehiscence on cochlear potential in response to air-conducted stimuli in chinchilla. Hear Res 2005; 210: 53-62
    CrossrefPubMedGoogle Scholar
  • 12 Schmuziger N, Allum J, Buitrago-Téllez C, Probst R. Incapacitating hypersensitivity to one's own body sounds due to a dehiscence of bone overlying the superior semicircular canal. A case report. Eur Arch Otorhinolaryngol 2006; 263: 69-74
    CrossrefPubMedGoogle Scholar
  • 13 Krombach GA, DiMartino E, Schmitz-Rode T , et al. Posterior semicircular canal dehiscence: a morphologic cause of vertigo similar to superior semicircular canal dehiscence. Eur Radiol 2003; 13: 1444-1450
    CrossrefPubMedGoogle Scholar
  • 14 Carey JP, Minor LB, Nager GT. Dehiscence or thinning of bone overlying the superior semicircular canal in a temporal bone survey. Arch Otolaryngol Head Neck Surg 2000; 126: 137-147
    CrossrefPubMedGoogle Scholar
  • 15 Strupp M, Eggert T, Straube A, Jäger L, Querner V, Brandt T. [“Inner perilymph fistula” of the anterior semicircular canal. A new disease picture with recurrent attacks of vertigo]. Nervenarzt 2000; 71: 138-142
    CrossrefPubMedGoogle Scholar
  • 16 Zhou G, Gopen Q, Poe DS. Clinical and diagnostic characterization of canal dehiscence syndrome: a great otologic mimicker. Otol Neurotol 2007; 28: 920-926
    CrossrefPubMedGoogle Scholar
  • 17 Lee GS, Zhou G, Poe D , et al. Clinical experience in diagnosis and management of superior semicircular canal dehiscence in children. Laryngoscope 2011; 121: 2256-2261
    CrossrefPubMedGoogle Scholar
  • 18 Rosowski JJ, Songer JE, Nakajima HH, Brinsko KM, Merchant SN. Clinical, experimental, and theoretical investigations of the effect of superior semicircular canal dehiscence on hearing mechanisms. Otol Neurotol 2004; 25: 323-332
    CrossrefPubMedGoogle Scholar
  • 19 Pfammatter A, Darrouzet V, Gärtner M , et al. A superior semicircular canal dehiscence syndrome multicenter study: is there an association between size and symptoms?. Otol Neurotol 2010; 31: 447-454
    CrossrefPubMedGoogle Scholar
  • 20 Crane BT, Carey JP, McMenomey S, Minor LB. Meningioma causing superior canal dehiscence syndrome. Otol Neurotol 2010; 31: 1009-1010
    CrossrefPubMedGoogle Scholar
  • 21 da Cunha Ferreira S, de Melo Tavares de Lima MA. Superior Canal Dehiscence Syndrome. Braz J Otorhinolaryngol 2006; 72: 414-418
    CrossrefPubMedGoogle Scholar
  • 22 Minor LB, Cremer PD, Carey JP, Della Santina CC, Streubel SO, Weg N. Symptoms and signs in superior canal dehiscence syndrome. Ann N Y Acad Sci 2001; 942: 259-273
    PubMedGoogle Scholar
  • 23 Minor LB. Superior canal dehiscence syndrome. Am J Otol 2000; 21: 9-19
    CrossrefPubMedGoogle Scholar
  • 24 Brantberg K, Bergenius J, Tribukait A. Vestibular-evoked myogenic potentials in patients with dehiscence of the superior semicircular canal. Acta Otolaryngol 1999; 119: 633-640
    CrossrefPubMedGoogle Scholar
  • 25 Cremer PD, Minor LB, Carey JP, Della Santina CC. Eye movements in patients with superior canal dehiscence syndrome align with the abnormal canal. Neurology 2000; 55: 1833-1841
    CrossrefPubMedGoogle Scholar
  • 26 Nadgir RN, Ozonoff A, Devaiah AK, Halderman AA, Sakai O. Superior semicircular canal dehiscence: congenital or acquired condition?. AJNR Am J Neuroradiol 2011; 32: 947-949
    CrossrefPubMedGoogle Scholar
  • 27 Sequeira SM, Whiting BR, Shimony JS, Vo KD, Hullar TE. Accuracy of computed tomography detection of superior canal dehiscence. Otol Neurotol 2011; 32: 1500-1505
    CrossrefPubMedGoogle Scholar
  • 28 Belden CJ, Weg N, Minor LB, Zinreich SJ. CT evaluation of bone dehiscence of the superior semicircular canal as a cause of sound- and/or pressure-induced vertigo. Radiology 2003; 226: 337-343
    CrossrefPubMedGoogle Scholar
  • 29 Williamson RA, Vrabec JT, Coker NJ, Sandlin M. Coronal computed tomography prevalence of superior semicircular canal dehiscence. Otolaryngol Head Neck Surg 2003; 129: 481-489
    CrossrefPubMedGoogle Scholar
  • 30 Piton J, Négrevergne M, Portmann D. [Dehiscence of the superior semicircular canal: approach and CT scan classifications]. Rev Laryngol Otol Rhinol (Bord) 2008; 129: 17-26
    PubMedGoogle Scholar
  • 31 Chen EY, Paladin A, Phillips G , et al. Semicircular canal dehiscence in the pediatric population. Int J Pediatr Otorhinolaryngol 2009; 73: 321-327
    CrossrefPubMedGoogle Scholar
  • 32 Crovetto M, Whyte J, Rodriguez OM, Lecumberri I, Martinez C, Eléxpuru J. Anatomo-radiological study of the Superior Semicircular Canal Dehiscence Radiological considerations of Superior and Posterior Semicircular Canals. Eur J Radiol 2010; 76: 167-172
    CrossrefPubMedGoogle Scholar
  • 33 Calli C, Pinar E, Oncel S, Erdoğan N, Sadullahoğlu K. [Evaluation of superior semicircular canal dehiscence in patients with vertigo and tinnitus]. Kulak Burun Bogaz Ihtis Derg 2009; 19: 77-81
    PubMedGoogle Scholar
  • 34 Cloutier JF, Bélair M, Saliba I. Superior semicircular canal dehiscence: positive predictive value of high-resolution CT scanning. Eur Arch Otorhinolaryngol 2008; 265: 1455-1460
    CrossrefPubMedGoogle Scholar
  • 35 Curtin HD. Superior semicircular canal dehiscence syndrome and multi-detector row CT. Radiology 2003; 226: 312-314
    CrossrefPubMedGoogle Scholar
  • 36 Watson SR, Halmagyi GM, Colebatch JG. Vestibular hypersensitivity to sound (Tullio phenomenon): structural and functional assessment. Neurology 2000; 54: 722-728
    CrossrefPubMedGoogle Scholar
  • 37 Streubel SO, Cremer PD, Carey JP, Weg N, Minor LB. Vestibular-evoked myogenic potentials in the diagnosis of superior canal dehiscence syndrome. Acta Otolaryngol Suppl 2001; 545: 41-49
    PubMedGoogle Scholar
  • 38 Welgampola MS, Myrie OA, Minor LB, Carey JP. Vestibular-evoked myogenic potential thresholds normalize on plugging superior canal dehiscence. Neurology 2008; 70: 464-472
    CrossrefPubMedGoogle Scholar
  • 39 Aw ST, Todd MJ, Aw GE, Magnussen JS, Curthoys IS, Halmagyi GM. Click-evoked vestibulo-ocular reflex: stimulus-response properties in superior canal dehiscence. Neurology 2006; 66: 1079-1087
    CrossrefPubMedGoogle Scholar
  • 40 Crane BT, Minor LB, Carey JP. Three-dimensional computed tomography of superior canal dehiscence syndrome. Otol Neurotol 2008; 29: 699-705
    CrossrefPubMedGoogle Scholar
  • 41 Colebatch JG, Halmagyi GM, Skuse NF. Myogenic potentials generated by a click-evoked vestibulocollic reflex. J Neurol Neurosurg Psychiatry 1994; 57: 190-197
    CrossrefPubMedGoogle Scholar
  • 42 Ferber-Viart C, Dubreuil C, Duclaux R. Vestibular evoked myogenic potentials in humans: a review. Acta Otolaryngol 1999; 119: 6-15
    PubMedGoogle Scholar
  • 43 Colebatch JG, Halmagyi GM. Vestibular evoked potentials in human neck muscles before and after unilateral vestibular deafferentation. Neurology 1992; 42: 1635-1636
    CrossrefPubMedGoogle Scholar
  • 44 Ozeki H, Matsuzaki M, Murofushi T. Vestibular evoked myogenic potentials in patients with bilateral profound hearing loss. ORL J Otorhinolaryngol Relat Spec 1999; 61: 80-83
    CrossrefPubMedGoogle Scholar
  • 45 Murofushi T, Curthoys IS, Gilchrist DP. Response of guinea pig vestibular nucleus neurons to clicks. Exp Brain Res 1996; 111: 149-152
    PubMedGoogle Scholar
  • 46 Didier A, Cazals Y. Acoustic responses recorded from the saccular bundle on the eighth nerve of the guinea pig. Hear Res 1989; 37: 123-127
    CrossrefPubMedGoogle Scholar
  • 47 Murofushi T, Halmagyi GM, Yavor RA, Colebatch JG. Absent vestibular evoked myogenic potentials in vestibular neurolabyrinthitis. An indicator of inferior vestibular nerve involvement?. Arch Otolaryngol Head Neck Surg 1996; 122: 845-848
    CrossrefPubMedGoogle Scholar
  • 48 Gopen Q, Zhou G, Poe D, Kenna M, Jones D. Posterior semicircular canal dehiscence: first reported case series. Otol Neurotol 2010; 31: 339-344
    CrossrefPubMedGoogle Scholar
  • 49 Mikulec AA, Poe DS. Operative management of a posterior semicircular canal dehiscence. Laryngoscope 2006; 116: 375-378
    CrossrefPubMedGoogle Scholar
  • 50 Crane BT, Lin FR, Minor LB, Carey JP. Improvement in autophony symptoms after superior canal dehiscence repair. Otol Neurotol 2010; 31: 140-146
    CrossrefPubMedGoogle Scholar
  • 51 Brantberg K, Bergenius J, Mendel L, Witt H, Tribukait A, Ygge J. Symptoms, findings and treatment in patients with dehiscence of the superior semicircular canal. Acta Otolaryngol 2001; 121: 68-75
    CrossrefPubMedGoogle Scholar
  • 52 Hillman TA, Kertesz TR, Hadley K, Shelton C. Reversible peripheral vestibulopathy: the treatment of superior canal dehiscence. Otolaryngol Head Neck Surg 2006; 134: 431-436
    CrossrefPubMedGoogle Scholar
  • 53 Pletcher SD, Oghalai JS, Reeck JB, Cheung SW. Management of superior canal dehiscence syndrome with extensive skull-base deficiency. ORL J Otorhinolaryngol Relat Spec 2005; 67: 192-195
    CrossrefPubMedGoogle Scholar
  • 54 Limb CJ, Carey JP, Srireddy S, Minor LB. Auditory function in patients with surgically treated superior semicircular canal dehiscence. Otol Neurotol 2006; 27: 969-980
    CrossrefPubMedGoogle Scholar