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.
D000200:Action Potentials; D000328:Adult; D001933:Brain Stem; D002648:Child; D005072:Evoked Potentials, Auditory; D005260:Female; D005621:Friedreich Ataxia / Q000150:complications* / Q000235:genetics; D006319:Hearing Loss, Sensorineural / Q000209:etiology*; D006801:Humans; D008297:Male; D008875:Middle Aged; D010375:Pedigree; D000159:Vestibulocochlear Nerve