Adult-onset hereditary cerebellar ataxia and neurosensory deafness

A family has been studied in which four sibs were affected with an adult-onset form of cerebellar ataxia and deafness. The ataxia and hearing loss have been slowly progressive over a 10 year follow-up period, and no other neurological signs have developed in the interim. This variety of hereditary ataxia, which appears to be a distinct autosomal recessive entity, is compared and contrasted with other genetic types, compiled in tabular form. It is emphasized that closer attention to other symptoms, either in the central nervous system or elsewhere, might have considerable use in the differential diagnosis of these conditions and thus provide for more effective genetic counselling.