周围神经遗传病合并听神经病4例并文献复习

山东大学耳鼻喉眼学报 ›› 2009, Vol. 23 ›› Issue (3): 25-27.

周围神经遗传病合并听神经病4例并文献复习

薛飞，江满杰，程友，王锦玲，周玫，王秋萍，李泽卿

1. 南京军区南京总医院耳鼻咽喉-头颈外科，南京 210002；2. 第四军医大学西京医院耳鼻咽喉-头颈外科，西安 710032

收稿日期:2009-02-16 修回日期:2009-05-06 出版日期:2009-06-16 发布日期:2009-06-16
通讯作者: 李泽卿，男，医学博士，副主任医师。
作者简介:薛飞（1978- ），男，医学硕士，主要从事听神经病的基础与临床研究。 Email: flyxue123@163.com

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

XUE Fei，JIANG Man-jie，CHENG You，WANG Jin-ling，ZHOU Mei，WANG Qiu-ping，LI Ze-qing

1. Department of Otorhinolaryngology & Head and Neck Surgery, Nanjing General Hospital of Nanjing Military Command, PLA, Nanjing 210002, China; 2. Department of Otorhinolaryngology & Head and Neck Surgery, Xijing Hospital, Fourth Military Medical University, Xi′an 710032, China

Received:2009-02-16 Revised:2009-05-06 Online:2009-06-16 Published:2009-06-16

摘要/Abstract

摘要：

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。

关键词: 听神经病；神经系统疾病；临床特征

Abstract:

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.

Key words: Auditory neuropathy; Nervous system disease; Clinical characteristics

中图分类号:

R764.4

薛飞，江满杰，程友，王锦玲，周玫，王秋萍，李泽卿. 周围神经遗传病合并听神经病4例并文献复习[J]. 山东大学耳鼻喉眼学报, 2009, 23(3): 25-27.

XUE Fei，JIANG Man-jie，CHENG You，WANG Jin-ling，ZHOU Mei，WANG Qiu-ping，LI Ze-qing. Nervous system hereditary disease patients combined with auditory neuropathy: a literature review of 4 cases[J]. JOURNAL OF SHANDONG UNIVERSITY (OTOLARYNGOLOGY AND OPHTHALMOLOGY), 2009, 23(3): 25-27.

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