The cases of three brothers, all males and aged 16, 8 and 6 years with heredo-degenerative ataxia associated, in two of them, with hypoacusia are reported. There are references of a similar disease in a grand-father and a great-uncle of the patients. The differential diagnosis with Pièrre Marie's disease, Charcot-Marie-Tooth's disease, Refsum's syndrome and hypertrophic interstitial neuritis is discussed, the similarity between the cases studied and Friedreich's disease being stressed. Comments are made on the association of Friedreich's disease with auditory disturbances.