Heredo-ataxia and cochleo-vestibular disorders.

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.