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一名患有感音神经性耳聋儿童的脑桥发育不全。

Pontine hypoplasia in a child with sensorineural deafness.

作者信息

Maeoka Y, Yamamoto T, Ohtani K, Takeshita K

机构信息

Division of Child Neurology, Institute of Neurological Sciences, Faculty of Medicine, Tottori University, Yonago, Japan.

出版信息

Brain Dev. 1997 Sep;19(6):436-9. doi: 10.1016/s0387-7604(97)00056-9.

DOI:10.1016/s0387-7604(97)00056-9
PMID:9339875
Abstract

A 2-year-old girl with bilateral sensorineural deafness showed pontine hypoplasia as well as a bulging contour of the pontine tegmentum on magnetic resonance imaging (MRI). There were no bilateral responses of brainstem auditory-evoked potentials (BAEPs). The absent late components of blink reflex (BR) indicated brainstem dysfunction. Chromosomal abnormalities and neurodegenerative or neurometabolic disorders, which might have been the cause of the pontine hypoplasia, were ruled out. The authors describe a rare case with pontine hypoplasia combined with sensorineural deafness and absent blink reflex and suggest that the brainstem in this child may become involved in the early gestation period.

摘要

一名患有双侧感音神经性耳聋的2岁女童在磁共振成像(MRI)上显示脑桥发育不全以及脑桥被盖部轮廓膨出。脑干听觉诱发电位(BAEP)无双侧反应。瞬目反射(BR)晚期成分缺失表明脑干功能障碍。排除了可能是脑桥发育不全原因的染色体异常以及神经退行性或神经代谢性疾病。作者描述了一例罕见的脑桥发育不全合并感音神经性耳聋和瞬目反射缺失的病例,并提示该患儿的脑干可能在妊娠早期就受到了影响。

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