Central vestibular involvement in peroneal muscle atrophy: a preliminary report

A family is presented in which 6 out of 8 members were found to have peroneal muscle atrophy. Neurophysiological and histopathological evidence for the hypertrophic form of this disease was obtained in 3 patients. Three patients with peroneal muscle atrophy and 1 unaffected family member had abnormal differential caloric tests. This is the first report of an apparently hereditary dysfunction of the central vestibular system associated with a hereditary neuropathy.