大前庭水管综合征患儿听力学相关检查结果及其特点

摘要

Objective To study on the audiological characteristics of large vestibular aqueduct syndrome(LVAS)in young children.Methods 80 ears diagnosed as LVAS by Department of ENT in our hospital from May 2014 to May 2017 were selected as the observation group.while 80 ears diagnosed as non-LVAS were selected as the control group.Tympanometry,Otoacoustic emission,ABR and ASSR were performed on them.Results 69 ears were diagnosed as severe and extremely severe deafness,accounting for 89.61％.77 ears were all type A tympanogram.DPOAE of 77 ears were not elicited.The hearing curves of 34 ears(75.56％,34/45)showed there was an obvious difference between air and bone conduction thresholds at low frequency (0.25 kHz and 0.5 kHz),while high frequency decreased.The control group showed a full frequency hearing loss.ASNR of 41 ears (53.25％,41/77)were elicited by high intensity (≥90 dB nHL),with the mean latency of (2.97±0.31) ms.ASNR was not recorded in the control group.The reaction rate between 2 groups at 0.5 kHz frequency was significantly different (P＜0.05).While there was no significant difference at 1 kHz,2 kHz and 4 kHz (P＜0.05).Conclusion The young children with LVAS mostly have the characteristics of the high frequency hearing loss curve with significant air-bone gaps at low frequencies.ASNR negative phase waves are recorded in ABR tests.The audiological characteristics provide an important reference for initial diagnosis of LVAS.%目的 分析大前庭水管综合征(LVAS)患儿的听力学检查结果,探讨其听力学特点.方法 选择我院耳鼻喉科确诊的大前庭水管综合征(large vestibular aqueduct syndrome,LVAS)患儿40例(77耳),作为观察组;将同期就诊的非LVAS患儿40例(77耳)作为对照组,分别行纯音听阈测试、声导抗测试、耳声发射测试、ABR测试及ASSR测试,并对结果进行统计分析.结果 诊断为重度或极重度听力损失的患儿69耳(89.61％,69/77);鼓室导抗图均为A型曲线,77耳DPOAE均未引出;34耳(75.56％,34/45)听力曲线在低频(0.25和0.5 kHz)存在明显气骨导差,呈高频下降型,而对照组呈全频听力下降;41耳(53.25％,41/77)在高强度刺激(≥90 dB nHL)下引出声诱发短潜伏期负反应(acoustically short latencynegative response,ASNR),潜伏期均值为2.97±0.31 ms,而对照组均未记录到ASNR;两组在0.5 kHz低频引出的电位反应率对比差异明显(P＜0.05),而在1、2、4 kHz高频时无明显差异(P＜0.05).结论 LVAS患儿听力曲线表现为明显的低频气骨导差,出现高频下降型听力损失;ABR检测可记录到ASNR负相波;在0.5 kHz低频时的ASSR电位反应率明显升高.听力学特征为LVAS的早期初步诊断提供了重要的参考依据.