Kaga K, Yokochi K, Kitazumi E, Kodama K, Marsh R
Ann Otol Rhinol Laryngol. 1986 Mar-Apr;95(2 Pt 1):203-6. doi: 10.1177/000348948609500220.
Five male infants, all of whom showed findings of wave I or waves I and II only of the auditory brain stem response (ABR), congenital horizontal pendular nystagmus, and hypotonia of head and limbs in the early infantile period with later paresis, are discussed. Their ages ranged from 3 to 13 months at the first examination, at which time neither head control, sitting, nor walking had been attained. Follow-up studies of ABRs revealed persistent abnormalities with neither remission nor progression. In spite of these abnormalities, these infants responded well to voices and other sounds. The congenital nystagmus in each case was demonstrated by electronystagmography to have a frequency of 2 to 3 Hz. The correlation between clinical signs and ABR is uncertain as yet. However, our findings are strongly indicative of nonprogressive inborn abnormalities in the lower brain stem.
本文讨论了5名男婴，他们在婴儿早期均表现出听觉脑干反应（ABR）仅为I波或I波和II波、先天性水平摆动性眼球震颤以及头和四肢肌张力低下，后期出现轻瘫。首次检查时他们的年龄在3至13个月之间，此时他们尚未获得头部控制、坐立或行走能力。ABR的随访研究显示存在持续异常，既无缓解也无进展。尽管有这些异常，但这些婴儿对声音和其他声响反应良好。通过眼震电图检查发现，每个病例的先天性眼球震颤频率为2至3Hz。临床体征与ABR之间的相关性目前尚不确定。然而，我们的研究结果强烈提示脑干下部存在非进行性先天性异常。
