Semin Neurol 2020; 40(01): 151-159
DOI: 10.1055/s-0039-3402738
Review Article
Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.

Superior Semicircular Canal Dehiscence Syndrome

Kristen K. Steenerson
1   Department of Otolaryngology–Head and Neck Surgery, Department of Neurology, Stanford University School of Medicine, Stanford, California
,
Benjamin T. Crane
2   Department of Otolaryngology, Bioengineering, and Neuroscience, University of Rochester, Rochester, New York
,
Lloyd B. Minor
3   Department of Otolaryngology, Department of Bioengineering, and Department of Neuroscience, Stanford University School of Medicine, Stanford, California
› Author Affiliations
Further Information

Publication History

Publication Date:
27 January 2020 (online)

Abstract

Superior canal dehiscence syndrome (SCDS) is a vestibular disorder caused by a pathologic third window into the labyrinth that can present with autophony, sound- or pressure-induced vertigo, and chronic disequilibrium among other vestibulocochlear symptoms. Careful history taking and examination in conjunction with appropriate diagnostic testing can accurately diagnose the syndrome. Key examination techniques include fixation-suppressed ocular motor examination investigating for sound- or pressure-induced eye movements in the plane of the semicircular canal. Audiometry, vestibular evoked myogenic potentials, and computed tomography confirm the diagnosis. Corrective surgical techniques can be curative, but many patients find their symptoms are not severe enough to undergo surgery. Although a primarily peripheral vestibular disorder, as first-line consultants for most dizziness complaints, neurologists will serve their patients well by understanding SCDS and its role in the differential diagnosis of vestibular disorders.

 
  • References

  • 1 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 (03) 249-258
  • 2 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 (12) 1833-1841
  • 3 Minor LB. Superior canal dehiscence syndrome. Am J Otol 2000; 21 (01) 9-19
  • 4 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
  • 5 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 (02) 137-147
  • 6 Ward BK, Carey JP, Minor LB. Superior canal dehiscence syndrome: lessons from the first 20 years. Front Neurol 2017; 8: 177
  • 7 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 (03) 323-332
  • 8 Tullio P. Sulla Funzione Delle Varie Parti Dell'orecchio Interno. Bologna: Cappelli; 1926
  • 9 Mayer O, Fraser J. Pathological changes in the ear in late congenital syphilis. J Laryngol Otol 1936; 51: 683-714
  • 10 Hennebert C. A new syndrome in hereditary syphilis of the labyrinth. Presse Med Belg Brux 1911; 63: 467-470
  • 11 Fox EJ, Balkany TJ, Arenberg IK. The Tullio phenomenon and perilymph fistula. Otolaryngol Head Neck Surg 1988; 98 (01) 88-89
  • 12 Kacker SK, Hinchcliffe R. Unusual Tullio phenomena. J Laryngol Otol 1970; 84 (02) 155-166
  • 13 Ishizaki H, Pyykkö I, Aalto H, Starck J. Tullio phenomenon and postural stability: experimental study in normal subjects and patients with vertigo. Ann Otol Rhinol Laryngol 1991; 100 (12) 976-983
  • 14 Cawthorne T. Otosclerosis. J Laryngol Otol 1955; 69 (07) 437-456
  • 15 Grieser BJ, Kleiser L, Obrist D. Identifying mechanisms behind the Tullio phenomenon: a computational study based on first principles. J Assoc Res Otolaryngol 2016; 17 (02) 103-118
  • 16 Kaski D, Davies R, Luxon L, Bronstein AM, Rudge P. The Tullio phenomenon: a neurologically neglected presentation. J Neurol 2012; 259 (01) 4-21
  • 17 Hirvonen TP, Weg N, Zinreich SJ, Minor LB. High-resolution CT findings suggest a developmental abnormality underlying superior canal dehiscence syndrome. Acta Otolaryngol 2003; 123 (04) 477-481
  • 18 Schutt CA, Neubauer P, Samy RN. , et al. The correlation between obesity, obstructive sleep apnea, and superior semicircular canal dehiscence: a new explanation for an increasingly common problem. Otol Neurotol 2015; 36 (03) 551-554
  • 19 Jan TA, Cheng YS, Landegger LD. , et al. Relationship between surgically treated superior canal dehiscence syndrome and body mass index. Otolaryngol Head Neck Surg 2017; 156 (04) 722-727
  • 20 Kuo P, Bagwell KA, Mongelluzzo G. , et al. Semicircular canal dehiscence among idiopathic intracranial hypertension patients. Laryngoscope 2018; 128 (05) 1196-1199
  • 21 Minor LB. Clinical manifestations of superior semicircular canal dehiscence. Laryngoscope 2005; 115 (10) 1717-1727
  • 22 Merchant SN, Rosowski JJ, McKenna MJ. Superior semicircular canal dehiscence mimicking otosclerotic hearing loss. Adv Otorhinolaryngol 2007; 65: 137-145
  • 23 Niesten ME, McKenna MJ, Grolman W, Lee DJ. Clinical factors associated with prolonged recovery after superior canal dehiscence surgery. Otol Neurotol 2012; 33 (05) 824-831
  • 24 Naert L, Van de Berg R, Van de Heyning P. , et al. Aggregating the symptoms of superior semicircular canal dehiscence syndrome. Laryngoscope 2018; 128 (08) 1932-1938
  • 25 Jung DH, Lookabaugh SA, Owoc MS, McKenna MJ, Lee DJ. Dizziness is more prevalent than autophony among patients who have undergone repair of superior canal dehiscence. Otol Neurotol 2015; 36 (01) 126-132
  • 26 Crane BT, Lin FR, Minor LB, Carey JP. Improvement in autophony symptoms after superior canal dehiscence repair. Otol Neurotol 2010; 31 (01) 140-146
  • 27 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 (06) 1444-1450
  • 28 Sheehy JL, Brackmann DE. Cholesteatoma surgery: management of the labyrinthine fistula--a report of 97 cases. Laryngoscope 1979; 89 (01) 78-87
  • 29 Brantberg K, Verrecchia L, Westin M. Enhanced auditory sensitivity to body vibrations in superior canal dehiscence syndrome. Audiol Neurotol 2016; 21 (06) 365-371
  • 30 Welgampola MS, Rosengren SM, Halmagyi GM, Colebatch JG. Vestibular activation by bone conducted sound. J Neurol Neurosurg Psychiatry 2003; 74 (06) 771-778
  • 31 Browaeys P, Larson TL, Wong ML, Patel U. Can MRI replace CT in evaluating semicircular canal dehiscence?. AJNR Am J Neuroradiol 2013; 34 (07) 1421-1427
  • 32 Williamson RA, Vrabec JT, Coker NJ, Sandlin M. Coronal computed tomography prevalence of superior semicircular canal dehiscence. Otolaryngol Head Neck Surg 2003; 129 (05) 481-489
  • 33 Curtin HD. Superior semicircular canal dehiscence syndrome and multi-detector row CT. Radiology 2003; 226 (02) 312-314
  • 34 Curthoys IS, Manzari L. Evidence missed: ocular vestibular-evoked myogenic potential and cervical vestibular-evoked myogenic potential differentiate utricular from saccular function. Otolaryngol Head Neck Surg 2011; 144 (05) 751-752
  • 35 Janky KL, Nguyen KD, Welgampola M, Zuniga MG, Carey JP. Air-conducted oVEMPs provide the best separation between intact and superior canal dehiscent labyrinths. Otol Neurotol 2013; 34 (01) 127-134
  • 36 Zuniga MG, Janky KL, Nguyen KD, Welgampola MS, Carey JP. Ocular versus cervical VEMPs in the diagnosis of superior semicircular canal dehiscence syndrome. Otol Neurotol 2013; 34 (01) 121-126
  • 37 Fife TD, Colebatch JG, Kerber KA. , et al. Practice guideline: cervical and ocular vestibular evoked myogenic potential testing: report of the Guideline Development, Dissemination, and Implementation Subcommittee of the American Academy of Neurology. Neurology 2017; 89 (22) 2288-2296
  • 38 Welgampola MS, Akdal G, Halmagyi GM. Erratum to: Neuro-otology-some recent clinical advances. J Neurol 2017; 264 (01) 204
  • 39 Zhou G, Gopen Q, Poe DS. Clinical and diagnostic characterization of canal dehiscence syndrome: a great otologic mimicker. Otol Neurotol 2007; 28: 920-926
  • 40 Gopen Q, Zhou G, Poe D, Kenna M, Jones D. Posterior semicircular canal dehiscence: first reported case series. Otol Neurotol 2010; 31 (02) 339-344
  • 41 Gubbels SP, Zhang Q, Lenkowski PW, Hansen MR. Repair of posterior semicircular canal dehiscence from a high jugular bulb. Ann Otol Rhinol Laryngol 2013; 122 (04) 269-272
  • 42 Ward BK, Wenzel A, Ritzl EK. , et al. Near-dehiscence: clinical findings in patients with thin bone over the superior semicircular canal. Otol Neurotol 2013; 34 (08) 1421-1428
  • 43 Patel NS, Hunter JB, O'Connell BP, Bertrand NM, Wanna GB, Carlson ML. Risk of progressive hearing loss in untreated superior semicircular canal dehiscence. Laryngoscope 2017; 127 (05) 1181-1186
  • 44 Beyea JA, Agrawal SK, Parnes LS. Transmastoid semicircular canal occlusion: a safe and highly effective treatment for benign paroxysmal positional vertigo and superior canal dehiscence. Laryngoscope 2012; 122 (08) 1862-1866
  • 45 Agrawal SK, Parnes LS. Transmastoid superior semicircular canal occlusion. Otol Neurotol 2008; 29 (03) 363-367
  • 46 Carey JP, Migliaccio AA, Minor LB. Semicircular canal function before and after surgery for superior canal dehiscence. Otol Neurotol 2007; 28 (03) 356-364
  • 47 Shaia WT, Diaz RC. Evolution in surgical management of superior canal dehiscence syndrome. Curr Opin Otolaryngol Head Neck Surg 2013; 21 (05) 497-502
  • 48 Rodgers B, Lin J, Staecker H. Transmastoid resurfacing versus middle fossa plugging for repair of superior canal dehiscence: Comparison of techniques from a retrospective cohort. World J Otorhinolaryngol Head Neck Surg 2016; 2 (03) 161-167
  • 49 Crovetto de la Torre MA, Whyte Orozco J, Cisneros Gimeno AI, Basurko Aboitz JM, Oleaga Zufiria L, Sarrat Torreguitart R. Superior semicircular canal dehiscence syndrome. Embryological and surgical consideration [in Spanish]. Acta Otorrinolaringol Esp 2005; 56 (01) 6-11
  • 50 Succar EF, Manickam PV, Wing S, Walter J, Greene JS, Azeredo WJ. Round window plugging in the treatment of superior semicircular canal dehiscence. Laryngoscope 2018; 128 (06) 1445-1452
  • 51 Alkhafaji MS, Varma S, Pross SE. , et al. Long-term patient-reported outcomes after surgery for superior canal dehiscence syndrome. Otol Neurotol 2017; 38 (09) 1319-1326
  • 52 Crane BT, Minor LB, Carey JP. Superior canal dehiscence plugging reduces dizziness handicap. Laryngoscope 2008; 118 (10) 1809-1813
  • 53 Remenschneider AK, Owoc M, Kozin ED, McKenna MJ, Lee DJ, Jung DH. Health utility improves after surgery for superior canal dehiscence syndrome. Otol Neurotol 2015; 36 (10) 1695-1701
  • 54 Adams ME, Levine SC. The first new otologic disorder in a century: superior canal dehiscence syndrome. Minn Med 2011; 94 (11) 29-32
  • 55 Welgampola MS. Evoked potential testing in neuro-otology. Curr Opin Neurol 2008; 21 (01) 29-35
  • 56 Welgampola MS, Myrie OA, Minor LB, Carey JP. Vestibular-evoked myogenic potential thresholds normalize on plugging superior canal dehiscence. Neurology 2008; 70 (06) 464-472
  • 57 Ward BK, Agrawal Y, Nguyen E. , et al. Hearing outcomes after surgical plugging of the superior semicircular canal by a middle cranial fossa approach. Otol Neurotol 2012; 33 (08) 1386-1391
  • 58 Barber SR, Cheng YS, Owoc M. , et al. Benign paroxysmal positional vertigo commonly occurs following repair of superior canal dehiscence. Laryngoscope 2016; 126 (09) 2092-2097
  • 59 Staab JP, Ruckenstein MJ. Which comes first? Psychogenic dizziness versus otogenic anxiety. Laryngoscope 2003; 113 (10) 1714-1718
  • 60 McCaslin DL, Jacobson GP, Burrows HL, Littlefield P, Haynes DS. Transforming superior canal dehiscence to chronic subjective dizziness: from SCD to CSD. J Am Acad Audiol 2010; 21 (05) 293-300