Auditory brain-stem responses in adrenomyeloneuropathy.
We studied three patients with adrenomyeloneuropathy. Complete audiologic assessment was obtained: two patients showed unimpaired peripheral hearing and one showed a mild high-frequency hearing loss. Auditory brain-stem responses were abnormal in both ears of all subjects, with one subject showing no response above wave I, and the other two having significant wave I to III and wave III to V interval prolongations. We concluded that auditory brain-stem response testing provides a simple, valid, reliable method for demonstrating neurologic abnormality in adrenomyeloneuropathy even prior to evidence of clinical signs.
D000293:Adolescent; D000326:Adrenoleukodystrophy / Q000175:diagnosis / Q000503:physiopathology*; D000328:Adult; D001933:Brain Stem / Q000503:physiopathology*; D002549:Diffuse Cerebral Sclerosis of Schilder / Q000503:physiopathology*; D004569:Electroencephalography; D005072:Evoked Potentials, Auditory; D006801:Humans; D008297:Male