Cerebellar ataxia with neuropathy and bilateral vestibular areflexia syndrome
Patients usually present in the fifth or sixth decade of life, although the onset of symptoms is variable and may take over a decade to develop the classic triad of bilateral vestibulopathy, cerebellar ataxia, and sensory neuropathy. Most patients will have a slowly progressive course. Hearing is typically preserved.
For more information see OMIM.
G11.2 – Late-onset cerebellar ataxia
G11.9 – Hereditary ataxia, unspecified
193165008 – Neuropathy in association with hereditary ataxia
95814001 – Peripheral vestibular disease
- Friedreich ataxia
- Spinocerebellar ataxia type 1, 2, 3, 6, or 7
- Multiple system atrophy (cerebellar type)
- Kearns-Sayre syndrome
- Wernicke encephalopathy
- Autosomal dominant sensory ataxia
- Gerstmann-Straussler-Scheinker disease
- Superficial siderosis of the central nervous system
- Paraneoplastic syndrome