• 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.
(Arch Otolaryngol 1981;107:254-256)
Shanon E, Himelfarb MZ, Gold S. Auditory Function in Friedreich's AtaxiaElectrophysiologic Study of a Family. Arch Otolaryngol. 1981;107(4):254–256. doi:10.1001/archotol.1981.00790400056014