We assessed the usefulness of brainstem auditory evoked potentials (BAEPs) for differentiating types of degenerative diseases in children. The findings of BAEPs were abnormal in one out of two with polioencephalopathies and in seven of the eight with leukoencephalopathies, showing prolonged interpeak latencies of waves I-III, I-V or disappearance of wave components. On the other hand, normal responses were observed in four with corencephalopathies and one with spinocerebellopathy. The serial BAEPs in eight patients had deteriorated with progression of the clinical symptoms. These data on 16 children suggest that BAEPs are indeed a useful tool for monitoring pathophysiologic processes of neurodegenerative diseases.

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