一家系遗传性进行性感音神经性聋耳声发射测试分析

Measuring and analyzing of otoacoustic emission tests of a family with genetic progressive sensorineural hearing loss

目的 :探讨耳声发射 ( OAE)测试对遗传性进行性感音神经性聋的诊断意义。方法 :对一家系 6代中 5 4例 ( 10 8耳 )和 5 0例正常人进行了纯音测听、瞬态诱发耳声发射 ( TEOAE)和畸变产物耳声发射 ( DPOAE)测试 ,了解其耳蜗功能。结果 :本家系纯音听阈 >2 0 d B HL组中 ,84%出现 TEOAE反应幅度下降或消失 ,82 %出现DPOAE振幅下降或消失 ;纯音听阈正常组中 ,75 %出现 TEOAE反应幅度下降 ,6 4%出现 DPOAE某个频率的振幅下降或缺失 ;对照组 TEOAE和 DPOAE的检出率均为 10 0 %。结论

Objetive:To study the diagnostic significance of otoacoustic emission in patients with genetic progressive sensorineural earing loss.Method:To analyse the cochlear function pure tone andiometry Transied Evoked Otoacoustic Emission (TEOAE) and distortion Products Otoacoustic Emission (DPOAE) were measured in 54 members of a six generation family with genetic progresive sensorinearal hearing loss (108 cases) and 50 normal people.Result:The family was autosomal dominant heritage.In the group with pure tone thresholds more than 20 dB HL,the amplitudes of TEOAE descended or disappeared in 84% cases and the amplitudes of DPOAE declined or vanished in 82% cases.In the group with normal pure tone thresholds,the amplitudes of TEOAE descended in 75% cases and the amplitudes at one of the frequencies of DPOAE dropped or disappeared in 64% cases.But the incidence of TEOAE and DPOAE in normal people was 100%.Conclusion: OAE had important significance in the diagnosis of genetic hearing loss at early stage.