母系遗传性聋家系的纯音测听及畸变产物耳声发射测试的相关分析  

Audiological Features of the Matrilineal Non-syndromic Deafness and Distortion product Otoacoustic Emissions

作　　者：张楠[1] 王力红[1] 乔晓明[1] 梁传余[1] 

机构地区：[1]四川大学华西医院耳鼻咽喉科,成都610041

出　　处：《听力学及言语疾病杂志》2002年第1期6-7,共2页Journal of Audiology and Speech Pathology

摘　　要：目的 探讨母系遗传非综合征性聋的听力学特征及畸变产物耳声发射测试对其听力改变早期诊断的优越性。方法 对六个母系遗传家系成员共１０２人进行纯音测听、ＤＰＯＡＥ评估其听力，比较纯音测听与ＤＰＯＡＥ检测耳蜗早期病变的灵敏性。结果 纯音测听为感音神经性聋的 ３８例中，ＰＴＡ≥４５ ｄＢ ＨＬ的 １８例，ＤＰＯＡＥ反应缺失；ＰＴＡ＜４５ｄＢ ＨＬ的２０例，ＤＰＯＡＥ高频或高、中频振幅下降或缺失；纯音测听正常者中４例ＤＰＯＡＥ显示高频或高中频振幅下降。结论 母系遗传非综合征性聋的听力损害为双侧、对称性、进行性感音神经性聋，早期表现高频损害，ＤＰＯＡＥ可在纯音听阈改变之前，发现早期耳蜗轻微的病理变化，对早期诊断及遗传咨询有较大指导意义。Objective To explore audiological features of the pedigrees with matrilineal non-syndromic deafness and the value of distortion product otoacoustic emissions(DPOAE) in early diagnosis of hearing loss. Methods Pure tone audiometry DPOAE were earned out in 102 members of six pedigrees. Compared the value of DPOAE and pure tone audiometry for indicating early cochlear dysfunction was compared. Result Sensorineural hearing loss(SNHL) was found in 38 individuals. Among them, DPOAE was totally absent in 18 cases with pure tone average(VFA) ≥45dB and low amplitude or absent middle to high frequencies in 20 cases with PTA < 45 dB. Conclusion Hearing impairments in these families are bilateral symmetrical progressive SNHL. High frequency impairment was found in their early stage. DPOAE could identify the mild pathologic alterations in cochlea. It has particular value in early diagnosis and genetic consultation.

关 键 词：遗传性聋 听力测试 畸变产物耳声发射 听阈 相关分析 

分 类 号：R764.43[医药卫生—耳鼻咽喉科]