遗传性无综合征耳聋一家系畸变产物耳声发射测试  

Examinations of distortion product otoacoustic emission in hereditary progressive non syndromic hearing loss

作　　者：柯肖枚[1] 于红[2] 刘玉和[1] 顾之平[1] 路远 李蔷 

机构地区：[1]北京医科大学第一医院耳鼻咽喉科,100034 [2]北京医科大学人民医院耳鼻咽喉科

出　　处：《中华耳鼻咽喉科杂志》2000年第2期102-104,共3页Chinese Journal of Otorhinolaryngology

摘　　要：目的　探索遗传性进行性无综合征耳聋患者的听功能改变特点。方法　一个常染色体显性遗传进行性无综合征耳聋家系 5 2名成员及听力正常组 15名 ,进行了纯音测试及畸变产物耳声发射 (distortionproductotoacousticemissions,DPOAE)测试。结果　无综合征耳聋家系中 34名成员纯音测试为感音神经性聋 ,其中纯音听阈均值 (puretoneaverage ,PTA)≥ 40dB的 15例 (2 9耳 )DPOAE反应缺失 ,PTA≤ 35dB伴高频下降的 12例 (2 3耳 )DPOAE高频或高、中频振幅下降或缺失 ;2 1名 (42耳 )纯音听阈正常成员中 ,有 12例 (15耳 )DPOAE高频或高、中频振幅下降或缺失。结论　DPOAE能鉴别出耳蜗亚临床的病理改变 ,对该病的早期诊断 ,遗传咨询有指导意义。Objective To evaluate the hearing function in patients with hereditary progressive non syndromic hearing loss Methods Distortion product otoacoustic emissions (DPOAE) and pure tone audiometry were carried out in 52 individuals from a family with non syndromic hearing loss and 15 persons with normal hearing Results ① Sensorineural hearing loss (SNHL) was found in 34 individuals of the family Among these individuals, DPOAE was totally absent in 15 cases (29 ears) with pure tone average ≥40 dB and low amplitude or absent middle to high frequencies in 12 cases (23 ears) with high frequency hearing loss but pure tone average ≤35 dB ② Among 21 individuals (42 ears) with normal audiograms, DPOAE presented lower amplitude or absent high and middle frequencies in 12 individuals Conclusion DPOAE can be used in identification of subclinical pathologic alterations in the cochlea This would be of particular value in early diagnosis and genetic consultation

关 键 词：无综合征耳聋 遗传性 耳声发射测试 DPOAE 

分 类 号：R764[医药卫生—耳鼻咽喉科]