周围神经遗传病合并听神经病4例并文献复习

目的 探讨听神经病与其他神经系统疾病之间的关系。方法 初步调查4例神经系统遗传病合并听神经病患者的临床表现、神经系统体征及听力学特征等。结果 4例患者的临床诊断分别为：进行性腓骨肌萎缩症1例、Friedreich共济失调2例和Refsum病1例，听力学检测结果表明他们均同时合并听神经病。结论 听神经病可单独发病，也可并发于多种神经系统疾病，听神经病在神经系统疾病的病程中可先后发病，是其他神经系统疾病的症状之一。

Nervous system hereditary disease patients combined with auditory neuropathy: a literature review of 4 cases

Objective To analyze the relationship between nervous system diseases and auditory neuropathy (AN). Methods By a retrospective study, we summarized the clinical signs and results of audiometry and other electrophysiological examinations. ResultsOf the four patients, one was diagnosed as Charcot- Marie-Tooth disease, two as Friedreich′s ataxia and one as Refsum′s disease. Audiometry of them indicated that they all had auditory neuropathy. Conclusion Some nervous system diseases can combine with auditory neuropathy. An episode of AN can be early or late during the course of nervous system diseases. AN may be one symptom of other nervous system diseases.