Superior Semicircular Canal Dehiscence Syndrome

Superior Semicircular Canal Dehiscence Syndrome

1. Ade Wijaya , MD – May 2020 Superior Semicircular Canal Dehiscence Syndrome

2. Outline Introduction Pathophysiology Clinical presentation Diagnostic Criteria Differential Diagnosis Treatment Summary

3. Introduction Chronic disequilibrium and sound- or pressure-induced vertigo and nystagmus in the plane of the superior semicircular canal. Introduced in the last 2 decades. 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

4. Pathophysiology

5. Clinical Presentation Dizziness Vertigo Sound- and/or pressure-induced vertigo ( Tullio Phenomenon / Hennerbert Sign) Autophony Hearing loss Aural pressure Nonpulsatile tinnitus Pulsatile tinnitus Hyperacusis Oscillopsia Positional vertigo Drop attacks Motion sickness Imbalance 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 Minor LB. Clinical manifestations of superior semicircular canal dehiscence . Laryngoscope 2005;115(10):1717–1727 Merchant SN, Rosowski JJ,McKenna MJ. Superior semicircular canal dehiscence mimicking otosclerotic hearing loss. Adv Otorhinolaryngol 2007;65:137–145

6. Diagnostic Criteria Ward BK, Carey JP, Minor LB. Superior canal dehiscence syndrome: lessons from the first 20 years. Front Neurol 2017;8:177

7. Differential Diagnosis Posterior and horizontal canal dehiscence Perilymphatic fistula Near dehiscence Vestibular migraine Steenerson KK, Crane BT, Minor LB. Superior Semicircular Canal Dehiscence Syndrome. InSeminars in Neurology 2020 Jan 27. Thieme Medical Publishers.

8. Treatment Avoidance of the environmental factors that can produce symptoms (such as loud noises ) Surgery

9. Summary Uncommon but treatable cause of vestibulocochlear symptoms. Differential diagnosis of all patients presenting with either episodic or chronic dizziness , particularly in those with auditory symptoms as well. A mobile “third window” Multidisciplinary approach

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