Auris, nasus, larynx
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Auris, nasus, larynx · Jan 1987
Case ReportsNeuro-otological findings in a case of Marinesco-Sjögren syndrome with nystagmus.
Marinesco-Sjögren syndrome is a very rare disease that develops in childhood with main symptoms of cataract, ataxia, mental deficiency and microsomia, and is regarded as a type of spinocerebellar degeneration. The author performed the neuro-otological tests on a case of suspected Marinesco-Sjögren syndrome. ⋯ Marked atrophy of the cerebellar hemisphere in CT scans supported the neurootological findings. The author agrees to the opinion of Skre and Löcken who included Marinesco-Sjögren syndrome in the cerebellar form of spinocerebellar degeneration.