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The neuro-otology of Susac syndrome.

Abstract：

OBJECTIVE:We characterised the clinical and neuro-otological characteristics of patients with Susac syndrome. METHODS: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. RESULTS: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 recoverable hearing loss if relapses are treated early. Effects on vestibular function were variable in ten patients who were tested, with most showing preservation of function despite significant hearing loss. CONCLUSIONS:Neuro-otological symptoms in Susac syndrome are almost universal. In the correct clinical context, a rising configuration of low to high-frequency sensorineural hearing loss should prompt consideration of Susac syndrome. Treatment of inner ear symptoms in Susac syndrome requires further research as early immunotherapy may be beneficial.

journal_name

J Neurol

journal_title

Journal of neurology

authors

Hardy TA,Taylor RL,Qiu J,O'Brien B,Gopinath S,Trewin B,Spring PJ,Shaffi M,Bolitho SJ,Garsia RJ,Roxburgh R,Mason DF,Ip J,Chan F,Chen L,Wilson I,Beadnall HN,Barnett MH,Parratt JDE,Watson JDG,Welgampola MS,Reddel S

doi

10.1007/s00415-020-10086-0

subject

Has Abstract

pub_date

2020-12-01 00:00:00

pages

3711-3722

issue

eissn

0340-5354

issn

1432-1459

pii

10.1007/s00415-020-10086-0

journal_volume

267

pub_type

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The neuro-otology of Susac syndrome.