Surgical Treatment of Superior Semicircular Canal Dehiscence Syndrome

  • Francis X CreightonEmail author
  • John P. Carey


Superior semicircular canal dehiscence syndrome (SCDS) is a clinical entity resulting in a myriad of audiological and vestibular symptoms. Pressure and/or sound-induced vertigo/nystagmus, autophony, conductive hearing loss, and conductive hyperacusis are commonly seen in patients with SCDS. The physiologic mechanism of this syndrome is thought to be due to the dehiscence creating a low-impedance outlet for fluid waves in the labyrinth, commonly referred to as a third window. This shunts flow from the cochlea to the labyrinth, which both activates the vestibular system and decreases pressure driving the traveling fluid wave in the cochlea. Diagnosis of SCDS can be difficult and requires both radiographic evidence of a dehiscence and clinical evidence supporting SCDS as the etiology for a patient’s symptoms. Testing for SCDS includes high-resolution CT imaging, audiogram, cervical and ocular VEMP testing, head impulse testing, and visualization of sound- or pressure-induced eye movements in the plane of the affected superior canal. Traditional surgical repair of SCDS is via the middle fossa approach. This approach allows for the dehiscence to be seen directly and for it to be both plugged and resurfaced. This approach is preferred in the vast majority of patients, but in certain situations a transmastoid approach can offer benefits over the middle fossa approach. Surgical outcomes for repair of SCDS are quite favorable, with patients having improvement of autophony, imbalance, and vertigo. Patients with predominately audiological symptoms have the highest likelihood of postoperative improvement. The majority of patients report an improvement in their quality of life postoperatively. Complications are rare but do occur in both the middle fossa and transmastoid approaches to SCDS repair. The most common complication is hearing loss, which is typically mild but can be profound in a low percentage of patients. The importance of proper patient selection and preoperative counseling on the risks of surgery cannot be overstated to ensure good surgical outcomes in SCDS repair.


Superior semicircular canal dehiscence Vertigo Conductive hearing loss Superior semicircular canal dehiscence syndrome SSCD SCDS Middle fossa craniotomy 


  1. 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;124:249–58.CrossRefGoogle Scholar
  2. 2.
    Tullio P. Das ohr und die entstehung de sprache und schrift. Berlin: Urban Scharzenberg; 1929.Google Scholar
  3. 3.
    Mayer O, Fraser JS. Pathological changes in the ear in late congenital syphilis. J Laryngol Otol. 1936;51:683–714.CrossRefGoogle Scholar
  4. 4.
    Fox EJ, Balkany TJ, Arenberg IK. The Tullio phenomenon and perilymph fistula. Otolaryngol Head Neck Surg. 1988;98:88–9.CrossRefGoogle Scholar
  5. 5.
    Kacker SK, Hinchcliffe R. Unusual Tullio phenomena. J Laryngol Otol. 1970;84:155–66.CrossRefGoogle Scholar
  6. 6.
    Ishizaki H, Pyykko I, Aalto H, Starck J. Tullio phenomenon and postural stability: experimental study in normal subjects and patients with vertigo. Ann Otol Rhinol Laryngol. 1991;100:976–83.CrossRefGoogle Scholar
  7. 7.
    Pisano DV, Niesten MEF, Merchant SN, Nakajima HH. The effect of superior semicircular canal dehiscence on intracochlear sound pressures. Audiol Neurootol. 2012;17(5):338–48. Scholar
  8. 8.
    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(3):323–32.CrossRefGoogle Scholar
  9. 9.
    Chien W, Ravicz ME, Rosowski JJ, Merchant SN. Measurements of human middle- and inner-ear mechanics with dehiscence of the superior semicircular canal. Otol Neurotol. 2007;28(2):250–7. Scholar
  10. 10.
    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;126:137–47.CrossRefGoogle Scholar
  11. 11.
    Re M, Gioacchini FM, Salvolini U, et al. Multislice computed tomography overestimates superior semicircular canal dehiscence syndrome. Ann Otol Rhinol Laryngol. 2013;122:625–31.PubMedGoogle Scholar
  12. 12.
    Pfammatter A, Darrouzet V, Gartner M, et al. A superior semicircular canal dehiscence syndrome multicenter study: is there an association between size and symptoms? Otol Neurotol. 2010;31:447–54.CrossRefGoogle Scholar
  13. 13.
    Chien WW, Janky K, Minor LB, Carey JP. Superior canal dehiscence size: multivariate assessment of clinical impact. Otol Neurotol. 2012;33:810–5.CrossRefGoogle Scholar
  14. 14.
    Hunter JB, O’Connell BP, Wang J, et al. Correlation of superior canal dehiscence surface area with vestibular evoked myogenic potentials, audiometric thresholds, and dizziness handicap. Otol Neurotol. 2016;37(8):1104–10. Scholar
  15. 15.
    Niesten MEF, Stieger C, Lee DJ, et al. Assessment of the effects of superior canal dehiscence location and size on intracochlear sound pressures. Audiol Neurootol. 2015;20(1):62–71. Scholar
  16. 16.
    Niesten MEF, Hamberg LM, Silverman JB, et al. Superior canal dehiscence length and location influences clinical presentation and audiometric and cervical vestibular-evoked myogenic potential testing. Audiol Neurootol. 2014;19(2):97–105. Scholar
  17. 17.
    Minor LB. Clinical manifestations of superior semicircular canal dehiscence. Laryngoscope. 2005;115:1717–27.CrossRefGoogle Scholar
  18. 18.
    Watson SR, Halmagyi GM, Colebatch JG. Vestibular hypersensitivity to sound (Tullio phenomenon): structural and functional assessment. Neurology. 2000;54:722–8.CrossRefGoogle Scholar
  19. 19.
    Halmagyi GM, Aw ST, McGarvie LA, et al. Superior semicircular canal dehiscence simulating otosclerosis. J Laryngol Otol. 2003;117:553–7.CrossRefGoogle Scholar
  20. 20.
    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;55:1833–41.CrossRefGoogle Scholar
  21. 21.
    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;942:259–73.CrossRefGoogle Scholar
  22. 22.
    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;13:1444–50.PubMedGoogle Scholar
  23. 23.
    Sheehy JL, Brackmann DE. Cholesteatoma surgery: management of the labyrinthine fistula—a report of 97 cases. Laryngoscope. 1979;89:78–87.CrossRefGoogle Scholar
  24. 24.
    Merchant SN, Rosowski JJ. Conductive hearing loss caused by third-window lesions of the inner ear. Otol Neurotol. 2008;29:282–9.CrossRefGoogle Scholar
  25. 25.
    Mikulec AA, McKenna MJ, Ramsey MJ, et al. Superior semicircular canal dehiscence presenting as conductive hearing loss without vertigo. Otol Neurotol. 2004;25:121–9.CrossRefGoogle Scholar
  26. 26.
    Adams ME, Kileny PR, Telian SA, et al. Electrocochleography as a diagnostic and intraoperative adjunct in superior semicircular canal dehiscence syndrome. Otol Neurotol. 2011;32:1506–12.CrossRefGoogle Scholar
  27. 27.
    Wenzel A, Ward BK, Ritzl EK, et al. Intraoperative neuromonitoring for superior semicircular canal dehiscence and hearing outcomes. Otol Neurotol. 2015;36(1):139–45. Scholar
  28. 28.
    Welgampola MS, Colebatch JG. Characteristics and clinical applications of vestibular-evoked myogenic potentials. Neurology. 2005;64:1682–8.CrossRefGoogle Scholar
  29. 29.
    Welgampola MS, Myrie OA, Minor LB, Carey JP. Vestibular-evoked myogenic potential thresholds normalize on plugging superior canal dehiscence. Neurology. 2008;70:464–72.CrossRefGoogle Scholar
  30. 30.
    Zhou G, Gopen Q, Poe DS. Clinical and diagnostic characterization of canal dehiscence syndrome: a great otologic mimicker. Otol Neurotol. 2007;28:920–6.CrossRefGoogle Scholar
  31. 31.
    Crane BT, Minor LB, Carey JP. Three-dimensional computed tomography of superior canal dehiscence syndrome. Otol Neurotol. 2008;29:699–705.CrossRefGoogle Scholar
  32. 32.
    Sheykholeslami K, Schmerber S, Habiby Kermany M, Kaga K. Vestibular-evoked myogenic potentials in three patients with large vestibular aqueduct. Hear Res. 2004;190:161–8.CrossRefGoogle Scholar
  33. 33.
    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;34:121–6.CrossRefGoogle Scholar
  34. 34.
    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;34:127–34.CrossRefGoogle Scholar
  35. 35.
    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;226:337–43.CrossRefGoogle Scholar
  36. 36.
    Williamson RA, Vrabec JT, Coker NJ, Sandlin M. Coronal computed tomography prevalence of superior semicircular canal dehiscence. Otolaryngol Head Neck Surg. 2003;129:481–9.CrossRefGoogle Scholar
  37. 37.
    Minor LB. Meniere’s disease and migraine. Arch Otolaryngol Head Neck Surg. 2005;131:460.CrossRefGoogle Scholar
  38. 38.
    Muchnik C, Hildesheimer M, Rubinstein M, Arenberg IK. Low frequency air-bone gap in Meniere's disease without middle ear pathology. A preliminary report. Am J Otol. 1989;10:1–4.CrossRefGoogle Scholar
  39. 39.
    Poe DS. Diagnosis and management of the patulous eustachian tube. Otol Neurotol. 2007;28:668–77.PubMedGoogle Scholar
  40. 40.
    Minor LB. Labyrinthine fistulae: pathobiology and management. Curr Opin Otolaryngol Head Neck Surg. 2003;11:340–6.CrossRefGoogle Scholar
  41. 41.
    Hakuba N, Hato N, Shinomori Y, Sato H, Gyo K. Labyrinthine fistula as a late complication of middle ear surgery using the canal wall down technique. Otol Neurotol. 2002;23:832–5.CrossRefGoogle Scholar
  42. 42.
    Friedland DR, Wackym PA. A critical appraisal of spontaneous perilymphatic fistulas of the inner ear. Am J Otol. 1999;20:261–76. discussion 276-269.PubMedGoogle Scholar
  43. 43.
    Eggers SD. Migraine-related vertigo: diagnosis and treatment. Curr Pain Headache Rep. 2007;11:217–26.CrossRefGoogle Scholar
  44. 44.
    Tepper SJ. A pivotal moment in 50 years of headache history: the first American migraine study. Headache. 2008;48:730–1. discussion 732.CrossRefGoogle Scholar
  45. 45.
    Kayan A, Hood JD. Neuro-otological manifestations of migraine. Brain. 1984;107(Pt 4):1123–42.CrossRefGoogle Scholar
  46. 46.
    Jacobson GP, Newman CW. The development of the dizziness handicap inventory. Arch Otolaryngol Head Neck Surg. 1990;116:424–7.CrossRefGoogle Scholar
  47. 47.
    Shaia WT, Zappia JJ, Bojrab DI, LaRouere ML, Sargent EW, Diaz RC. Success of posterior semicircular canal occlusion and application of the dizziness handicap inventory. Otolaryngol Head Neck Surg. 2006;134:424–30.CrossRefGoogle Scholar
  48. 48.
    Tufarelli D, Meli A, Labini FS, et al. Balance impairment after acoustic neuroma surgery. Otol Neurotol. 2007;28:814–21.CrossRefGoogle Scholar
  49. 49.
    Humphriss RL, Baguley DM, Moffat DA. Change in dizziness handicap after vestibular schwannoma excision. Otol Neurotol. 2003;24:661–5.CrossRefGoogle Scholar
  50. 50.
    Badke MB, Pyle GM, Shea T, Miedaner J. Outcomes in vestibular ablative procedures. Otol Neurotol. 2002;23:504–9.CrossRefGoogle Scholar
  51. 51.
    Crane BT, Minor LB, Carey JP. Superior canal dehiscence plugging reduces dizziness handicap. Laryngoscope. 2008;118:1809–13.CrossRefGoogle Scholar
  52. 52.
    O'Reilly RC, Elford B, Slater R. Effectiveness of the particle repositioning maneuver in subtypes of benign paroxysmal positional vertigo. Laryngoscope. 2000;110:1385–8.CrossRefGoogle Scholar
  53. 53.
    Perez N, Martin E, Garcia-Tapia R. Dizziness: relating the severity of vertigo to the degree of handicap by measuring vestibular impairment. Otolaryngol Head Neck Surg. 2003;128:372–81.CrossRefGoogle Scholar
  54. 54.
    Limb CJ, Carey JP, Srireddy S, Minor LB. Auditory function in patients with surgically treated superior semicircular canal dehiscence. Otol Neurotol. 2006;27:969–80.CrossRefGoogle Scholar
  55. 55.
    Wilkinson EP, Liu GC, Friedman RA. Correction of progressive hearing loss in superior canal dehiscence syndrome. Laryngoscope. 2008;118:10–3.CrossRefGoogle Scholar
  56. 56.
    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;33:1386–91.CrossRefGoogle Scholar
  57. 57.
    Xie Y, Sharon JD, Pross SE, et al. Surgical complications from superior canal dehiscence syndrome repair: two decades of experience. Otolaryngol Head Neck Surg. 2017;157(2):273–80. Scholar
  58. 58.
    Mantokoudis G, Saber Tehrani AS, Wong AL, Agrawal Y, Wenzel A, Carey JP. Adaptation and compensation of vestibular responses following superior canal dehiscence surgery. Otol Neurotol. 2016;37(9):1399–405. Scholar
  59. 59.
    Barber SR, Cheng YS, Owoc M, et al. Benign paroxysmal positional vertigo commonly occurs following repair of superior canal dehiscence. Laryngoscope. 2016;126(9):2092–7. Scholar
  60. 60.
    Sanna M, Taibah A, Russo A, Falcioni M, Agarwal M. Perioperative complications in acoustic neuroma (vestibular schwannoma) surgery. Otol Neurotol. 2004;25:379–86.CrossRefGoogle Scholar
  61. 61.
    Oghalai JS, Buxbaum JL, Pitts LH, Jackler RK. The effect of age on acoustic neuroma surgery outcomes. Otol Neurotol. 2003;24:473–7.CrossRefGoogle Scholar
  62. 62.
    Alkhafaji MS, Varma S, Pross SE, et al. Long-term patient-reported outcomes after surgery for superior canal dehiscence syndrome. Otol Neurotol. 2017;38(9):1319–26. Scholar
  63. 63.
    Sharon JD, Pross SE, Ward BK, Carey JP. Revision surgery for superior canal dehiscence syndrome. Otol Neurotol. 2016;37(8):1096–103. Scholar
  64. 64.
    Shaia WT, Diaz RC. Evolution in surgical management of superior canal dehiscence syndrome. Curr Opin Otolaryngol Head Neck Surg. 2013;21:497–502.CrossRefGoogle Scholar
  65. 65.
    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;122:1862–6.CrossRefGoogle Scholar
  66. 66.
    Deschenes GR, Hsu DP, Megerian CA. Outpatient repair of superior semicircular canal dehiscence via the transmastoid approach. Laryngoscope. 2009;119:1765–9.CrossRefGoogle Scholar
  67. 67.
    Carter MS, Lookabaugh S, Lee DJ. Endoscopic-assisted repair of superior canal dehiscence syndrome. Laryngoscope. 2014;124:1464–8.CrossRefGoogle Scholar
  68. 68.
    Agrawal SK, Parnes LS. Transmastoid superior semicircular canal occlusion. Otol Neurotol. 2008;29:363–7.CrossRefGoogle Scholar
  69. 69.
    Crovetto M, Areitio E, Elexpuru J, Aguayo F. Transmastoid approach for resurfacing of superior semicircular canal dehiscence. Auris Nasus Larynx. 2008;35:247–9.CrossRefGoogle Scholar
  70. 70.
    Carey JP, Migliaccio AA, Minor LB. Semicircular canal function before and after surgery for superior canal dehiscence. Otol Neurotol. 2007;28:356–64.CrossRefGoogle Scholar
  71. 71.
    Cheng YS, Kozin ED, Lee DJ. Endoscopic-assisted repair of superior canal dehiscence. Otolaryngol Clin N Am. 2016;49(5):1189–204. Review. PubMed PMID: 27565386.CrossRefGoogle Scholar
  72. 72.
    Driscoll CL, Jackler RK, Pitts LH, Banthia V. Extradural temporal lobe retraction in the middle fossa approach to the internal auditory canal: biomechanical analysis. Am J Otol. 1999;20:373–80.PubMedGoogle Scholar
  73. 73.
    Agrawal Y, Migliaccio AA, Minor LB, Carey JP. Vestibular hypofunction in the initial postoperative period after surgical treatment of superior semicircular canal dehiscence. Otol Neurotol. 2009;30:502–6.CrossRefGoogle Scholar
  74. 74.
    Janky KL, Zuniga MG, Carey JP, Schubert M. Balance dysfunction and recovery after surgery for superior canal dehiscence syndrome. Arch Otolaryngol Head Neck Surg. 2012;138:723–30.CrossRefGoogle Scholar

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© Springer Nature Switzerland AG 2019

Authors and Affiliations

  1. 1.Johns Hopkins Outpatient CenterBaltimoreUSA

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