Alport综合征畸变产物耳声发射测试

Distortion product otoacoustic emission test in Alport syndrome

目的 分析遗传性血尿肾病耳聋综合征(Alport syndrome,As)病人的听功能改变特点,畸变产物耳声发射(DPOAEs)测试的意义。方法 对10例As病人进行了纯音测听、声导抗、DPOAEs和听性脑干反应(ABR)测试。结果 As病人全部DPOAEs反应缺失或高频、中频振幅下降,声导抗测试10例病人鼓室压图均为A型曲线,除2例重度感音性耳聋病人声反射阈与听阈之差<60dB,提示Metz重振试验阳性外,其余病人各波潜伏期和波间期均在正常范围。结论 10例As病人DPOAEs均不正常,声导抗、ABR均能引出,验证了As病人耳部病变定位为耳蜗基底膜,与肾脏基底膜病变为共同靶器官,DPOAEs能反映耳蜗外毛细胞的功能微病理改变,对As的早期诊断、鉴别诊断及对遗传咨询有指导意义。

Objective To analyze the altered features of auditory functions of hereditary nephritis-nerve deafness syndrome (Alport syndrome, As) patients and the significance of distortion product otoacoustic emissions (DPOAEs) test. Methods All the As patients underwent the pure tone test, the acoustic immitance test, the DPOAEs test and ABR test. Results All the As patients were absent of DPOAEs response, but had a descendent high or intermediate frequency amplitude. The tympanogram of 10 patients were A-type curves in the acoustic immitance test. Two patients with severe sensory deafness had a margin less than 60 dB between the sound-reflection threshold and auditory threshold, indicating that the results of Metz loudness recruitment test were positive. Conclusion As patients have an abnormal result of DPOAEs test, whereas the results of acoustic immitance test and ABR test are normal; the location of pathological changes of As patients is the cochlea basilar membrane, which is one of the same target organs as the kidney basilar membrane. DPOAEs may reflect the micropathological alterations in the function of outer hair cells of cochlea, and may instruct the inchoate diagnosis, differential diagnosis and genetic counselling of As.