Sano Masaki, Kaga Kimitaka, Takeuchi Naonobu, Kitazumi Eiji, Kodama Kazuo
Department of Otorhinolaryngology and Head and Neck Surgery, Graduate School of Medicine and Faculty of Medicine, University of Tokyo, 7-3-1 Hongo, Bunkyo-ku, Tokyo 113-8655, Japan.
Int J Pediatr Otorhinolaryngol. 2005 Nov;69(11):1583-6. doi: 10.1016/j.ijporl.2005.04.016. Epub 2005 May 31.
Mobius syndrome is characterized by congenital bilateral facial palsy and abducens nerve paralysis, but reports of radiological and electrophysiological findings are scarce. A 4-year-old boy presented with mask-like facies noted at birth after a 34-week pregnancy. Examination revealed bilateral facial and abducens nerve paralysis with no other neurological abnormalities. Computed tomography revealed bilateral absence of facial nerve canal in the middle ear. Brain magnetic resonance imaging indicated a narrow than expected nerve in the internal auditory canal (IAC). Evoked electromyography and blink reflex testing to evaluate facial nerve function yielded no responses bilaterally. Facial palsy thus appears to be caused by facial nerve dysplasia or aplasia.
莫比乌斯综合征的特征是先天性双侧面瘫和展神经麻痹,但关于放射学和电生理学检查结果的报道较少。一名4岁男孩在孕34周出生后即被发现有面具样面容。检查发现双侧面部和展神经麻痹,无其他神经学异常。计算机断层扫描显示中耳双侧面神经管缺如。脑部磁共振成像显示内耳道(IAC)内神经比预期狭窄。用于评估面神经功能的诱发肌电图和眨眼反射测试双侧均无反应。因此,面瘫似乎是由面神经发育异常或发育不全引起的。