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Serial studies of auditory brainstem evoked responses (ABR) were performed on two patients with subacute sclerosing panencephalitis (SSPE), one with Tay-Sachs disease (TSD), and two with adrenoleukodys-trophy (ALD). The principal pathophysiologic findings were found in interwave intervals (I-V), and amplitude ratios (V/I), and elimination of specific components was also observed.<BR>1) SSPE: The ABR was normal at the onset but showed an abnormal pattern in stage N. There was a diminution of V/I ratio or lengthening of I-V intervals.<BR>2) TSD: The ABR was abnormal at the early stage in the left ear. There was lengthening of wave I and wave V latencies, but I-V intervals were normal. There was also a progressive diminution of V/I ratio bilaterally.<BR>3) ALD: The ABR was normal at the onset, but became abnormal subsequently. There were lengthening of I-V intervals, diminution of V/I ratio, and disappearance of later components. It was concluded that the ABR was a promising new tool for the investigation of pediatric degenerative disease.
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