伴发神经系统疾病的听神经病36例临床分析

A Clinical Analysis of 36 Nervous System Disease Patients Acompanying Auditory Neuropathy

王锦玲;王剑;石力;薛飞;吴保仁;高磊;谢娟;韩丽萍;

1:第四军医大学西京医院耳鼻咽喉头颈外科

2:南京军区总医院耳鼻咽喉科

3:第四军医大学西京医院神经内科

摘要
目的分析一组神经系统疾病伴听神经病(auditory neuropathy,AN)患者的临床特点及两者间的关系。方法回顾性分析36例神经系统疾病伴AN患者的临床资料,并报告10例典型病例。结果 36例伴发AN患者的神经系统疾病包括:弗雷德赖希(Friedreich)共济失调5例,腓骨肌萎缩症(charcot-marie-tooth,CMT病)4例,植烷酸储积病(Refsum病)1例,视神经萎缩9例,下肢周围神经病9例,多发性硬化2例,慢性炎症性脱髓鞘性多发性神经病(CIDP)1例,脊髓亚急性联合变性2例,运动神经元病1例,缺血缺氧性脑病1例,红斑性肢痛症1例,其中23例同时伴发其他1种或2种神经系统疾病。本组患者中14例(38.89%)首先出现双下肢无力、走路不稳、行走困难或视力减退,其余22例(61.11%)为首先出现听力减退,辨不清说话;有行走困难及耳聋家族史5例,视力减退家族史2例,新生儿黄疸史1例。神经系统检查:14例视诱发电位出现视觉径路传导障碍,16例胫神经运动传导速度减慢(12~30 m/s)和/或潜伏期延长(4.1~5.8 ms),6例腓肠神经感觉传导速度减慢(0~35 m/s)及/或5例波幅下降(0~0.85μV);颞骨HRCT及MRI多未见异常;冷热试验26例中半规管麻痹14例;前庭诱发肌源性电位(VEMP)检测13例中9例未引出,引出的4例均幅值降低,2例潜伏期延长;纯音听力图呈低频上升型37耳(54.41%,37/68);听力损失呈中重、重及极重度共47耳(69.12%,47/68);言语识别率不成比例差于纯音听阈;ABR自波Ⅰ起均未引出;DPOAE除个别频率外,全部可引出。结论 AN可伴发于多种神经系统疾病,多为周围性、遗传性神经病,多表现为下肢无力、行走困难、听话不清或视力减退一组综合征,听力障碍在病程中可先后出现,听力检测呈现AN的听力学特征,影响原发神经系统疾病的病理过程也可能影响听神经。
关键词
听神经病;神经系统疾病;周围神经性;遗传性
基金项目(Foundation):
作者
王锦玲;王剑;石力;薛飞;吴保仁;高磊;谢娟;韩丽萍;
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