title: Heredo-ataxia and cochleo-vestibular disorders.
abstract: Cochleo-vestibular dysfunction of patients suffering from a heredo-ataxia, have been frequently described. A family and a single case are presented, which show the association of a heredo-ataxia, more specifically the Roussy-Lévy syndrome, progressive sensorineural loss, nystagmus and vestibular a- or hyporeflexia. A review of the literature is included and the standpoints concerning the nosological entities are discussed.
mesh_terms: D000293:Adolescent; D000328:Adult; D003638:Deafness / Q000235:genetics*; D005260:Female; D006801:Humans; D007759:Labyrinth Diseases / Q000235:genetics; D008297:Male; D009069:Movement Disorders / Q000235:genetics*; D009759:Nystagmus, Pathologic / Q000235:genetics; D010375:Pedigree; D012021:Reflex, Abnormal; D013577:Syndrome; D014722:Vestibule, Labyrinth