Brainstem auditory evoked responses in spinocerebellar engeneration 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.