JOURNAL OF SHANDONG UNIVERSITY (OTOLARYNGOLOGY AND OPHTHALMOLOGY) ›› 2009, Vol. 23 ›› Issue (3): 25-27.

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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. 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

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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

CLC Number: 

  • R764.4
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