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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。
Objective To investigate the relationship between auditory neuropathy and other neurological diseases. Methods The clinical manifestations, neurological signs and audiological features of 4 patients with neurological genetic diseases complicated with auditory neuropathy were investigated. Results The clinical diagnosis of four patients were as follows: 1 case of progressive charring dysplasia, 2 cases of Friedreich’s ataxia and 1 case of Refsum disease. The results of audiometry showed that they all had auditory neuropathy. Conclusions Auditory neuropathy can occur independently or in a variety of neurological diseases. It can be one of the symptoms of other neurological diseases in the course of the nervous system diseases.