Brainstem auditory-evoked responses and clinical picture in a one year follow-up of 18 patients with Friedreich ataxia.

Brainstem auditory-evoked responses (BAER) and behavior audiometry were evaluated on 18 patients with Friedreich ataxia. Behavior audiometry showed a normal pure tone test, normal performance intensity and phonetically balanced functions in all patients. BAER were abnormal in all patients. The data obtained show a good correlation between BAER and clinical picture and suggest that BAER could be useful in monitoring the clinical condition of FA patients.