Translated Titles

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


王锦玲(Jin-Ling Wang);王剑(Jian Wang);石力(Li Shi);薛飞(Fei Xue);吴保仁(Bao-Ren Wu);高磊(Lei Gao);谢娟(Juan Xie);韩丽萍(Li-Pin Han)

Key Words

听神经病 ; 神经系统疾病 ; 周围神经性 ; 遗传性 ; Auditory neuropathy ; Nervous system disease ; Peripheral neuropathy ; Hereditory



Volume or Term/Year and Month of Publication

19卷3期(2011 / 05 / 15)

Page #

197 - 202

Content Language


Chinese Abstract

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。

English Abstract

Objective To analyze the clinical features of nervous system disease patients with auditory neuropathy (AN) and the relations between them. Methods The clinical data of 36 syndromic AN patients were retrospectively examined and ten typical patients were described in this study. Results Thirty-six nervous system disease patients consisted of Friedreich ataxia (5 patients), Charcot-Marie-Tooth disease (4 patients), Refsum disease (1 patient), optic atrophy (9 patients), pelvic limb peripheral neuropathy (9 patients), multiple sclerosis (2 patients), chronic inflammatory demyelinating polyneuropathy (CIDP, 1 patient), subacute combined degeneration of the spinal cord (2 patients), motor neuron disease (1 patient), hypoxic ischemic encephalopathy (1 patient), and erythromelalgia (1 patient). Twenty-three out of 36 patients developed more than one type of nervous system diseases. Twenty-two (61.11%) patients appeared to have hearing loss and poor speech recognition abilities as the first symptoms while the other patients (39%) showed the features of peripheral neuropathy firstly, including the numbness of lower limb, difficulty or weakness in walking, and visual impairment. Seven patients had family hereditary history. According to etiological factors, neurological examinations showed various findings as presented in this paper. Fourteen out of 26 patients who received vestibular caloric tests showed canal paresis, and vestibular evoked myogenic potentials (VEMP) wave could not be observed in 9 out of 13 patients. All the audiological diagnostic results were within the AN diagnostic criteria. Conclusion AN may be accompanied with nervous system diseases in that most are peripheral and hereditary neuropathy. Most patients appeared to have hearing loss, difficulty in walking or visual impairment. The audiological tests showed that they all had the characteristics of auditory neuropathy. The hearing disorder may be before or late during the course of nervous system disease. AN symptoms may indicate that the primary neurological disorders have affected the auditory nerve.

Topic Category 醫藥衛生 > 外科