Brainstem auditory evoked responses in spinocerebellar degeneration and Wilson disease.
Brainstem auditory evoked responses (BAERs) were studied in 30 healthy subjects, 20 patients with spinocerebellar degeneration (SCD), and 6 patients with Wilson's disease. In addition to the standard 8 click per second stimulation rate, increased stimulation frequency was applied which identified wave V for its stability under different stimulation rates. No prolongation of wave latencies was noted in SCD patients, irrespective of clinical features, except in 1 patient who had a latency slightly above the normal range. Wilson disease with neurological symptoms produced prolongation of wave latencies in every case, whereas the patients without neurological involvement showed a normal BAER pattern.
D000161:Acoustic Stimulation / Q000379:methods; D000293:Adolescent; D000328:Adult; D001933:Brain Stem / Q000503:physiopathology*; D002526:Cerebellar Diseases / Q000175:diagnosis* / Q000503:physiopathology; D005072:Evoked Potentials, Auditory; D005260:Female; D006527:Hepatolenticular Degeneration / Q000175:diagnosis* / Q000503:physiopathology; D006801:Humans; D008297:Male; D008875:Middle Aged; D011930:Reaction Time / Q000502:physiology; D013118:Spinal Cord Diseases / Q000175:diagnosis* / Q000503:physiopathology; D013577:Syndrome