Abstract
Seven male infants showed wave I only or waves I and II on auditory brain stem response, congenital horizontal pendular nystagmus and hypotonia of the head and limbs in the early infantile period, and later developed paresis. The age of these patients ranged from three months to six years at the first examination, and they could attain neither head control, sitting, nor walking. The follow-up study of auditory brain stem responses revealed neither improvement nor deterioration of wave configuration. These infants responded well to voices and other sounds. The congenital nystagmus of frequency of 2-3Hz in each case was demonstrated by electronystagmography. The correlation between clinical signs and ABR was uncertain. However, these findings strongly indicated the nonprogressive inborn abnormalities in the lower brain stem. Patients with these characteristics have never been reported in the literature.
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