The neuro-otology of Susac syndrome.

Journal of Neurology
Todd A HardyG Michael Halmagyi

Abstract

We characterised the clinical and neuro-otological characteristics of patients with Susac syndrome. The medical records of 30 patients with Susac syndrome were reviewed for details of their clinical presentation and course, neuro-otological symptoms, investigation results including audiology and vestibular function tests, treatment and outcomes. Our findings demonstrate that 29 of our 30 patients with Susac syndrome developed neuro-otological symptoms such as hearing loss, disequilibrium, tinnitus or vertigo during their disease course. Hearing loss was the most common neuro-otological symptom occurring in 93% of patients. A rising configuration of low-frequency greater than the high-frequency sensorineural hearing loss was the most characteristic finding on audiological testing (37% of reviewed audiograms). Disproportionately poor speech discrimination was identified in 20% of cases, and one case demonstrated a retrocochlear pattern on electrophysiological testing. Four patients required hearing aids and a further two patients required a cochlear implant due to severe hearing loss. Two out of two treated patients had improvements in hearing after the prompt administration of corticosteroids, indicating the potential for recove...Continue Reading

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Citations

Jan 12, 2021·Case Reports in Neurological Medicine·Yazan Al-HasanSaha Kamala
Jun 14, 2021·La Revue de médecine interne·C DavidT Papo

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