Brainstem auditory evoked response in adrenoleukodystrophy

Abstract

Clinical, laboratory, and electrophysiological data, including brainstem auditory evoked responses, are reported in a case of adrenoleukodystrophy. A striking asymmetry was noted in wave VI of the brainstem auditory evoked potential, followed by absence of any recognizable wave on the abnormal side. The presumed site of origin of wave VI is the medial geniculate body, a structure severely involved in adrenoleukodystrophy. It is suggested that the brainstem auditory evoked response may promise noninvasive diagnostic aid in this disorder and that absence of wave VI may emerge as a clinically useful finding in diseases of the central nervous system.