Auditory function of Friedreich's ataxia. Electrophysiologic study of a family

Hearing function and auditory brainstem potentials were evaluated in nine members of three generations of a family affected with Friedreich's ataxia. The characteristic findings were high-frequency sensorineural hearing impairment of varying severity and diminished or absent N1, N2, and N3 responses. These electrophysiologic aberrations correlate topographically with previously reported pathological observations.