Muratsuka Y, Nishihira O, Inoue H, Komune S
Department of Otolaryngology, Hamanomachi Hospital, Fukuoka.
Nihon Jibiinkoka Gakkai Kaiho. 1991 Jan;94(1):22-7. doi: 10.3950/jibiinkoka.94.22.
Two siblings of Laurence-Moon-Biedl (L-M-B) syndrome with nystagmus and/or sensorineural hearing loss were reported. One sibling had a slight hearing impairment as well as gaze and rebound nystagmus. The auditory brainstem responses (ABR) showed increased latencies of wave V on both ears. The optokinetic nystagmus (OKN) was limited toward the both horizontal sides. Nystagmus was superimposed on the eye tracking movement. Ocular hypermetria was observed, with bilateral weakness of caloric responses and even a failure of visual fixation suppression. Another sibling had a left-beating spontaneous nystagmus. The latencies of wave V of ABR were prolonged in both ears. The leftward OKN was limited and bilateral saccadic pursuits observed on eye tracking test. These results suggest that cerebellar of brain-stem lesions may be involved in L-M-B syndrome.
报告了两例患有劳伦斯-穆恩-比德尔（L-M-B）综合征且伴有眼球震颤和/或感音神经性听力损失的兄弟姐妹。其中一名兄弟姐妹有轻微听力障碍以及凝视性和视动性眼球震颤。听觉脑干反应（ABR）显示双耳V波潜伏期延长。视动性眼球震颤（OKN）向两侧水平方向均受限。眼球震颤叠加在眼球跟踪运动上。观察到眼球过强症，伴有双侧冷热反应减弱，甚至视觉注视抑制失败。另一名兄弟姐妹有向左跳动的自发性眼球震颤。ABR的V波潜伏期双耳均延长。向左的OKN受限，在眼球跟踪测试中观察到双侧扫视性追踪。这些结果表明，脑干小脑病变可能与L-M-B综合征有关。
