大前庭水管综合征患儿听力学检测特点分析

The audiological characteristics of large vestibular aqueduct syndrome in infants and young children

目的:回顾性分析大前庭水管综合征患儿的临床听力学检测结果,探讨其听力学检测"异常征兆",提示检测人员及临床医生及时排查大前庭水管综合征,为早期诊断提供帮助。方法:124例大前庭水管综合征患儿,通过颞骨CT或内耳MRI及声导抗测试排除中耳异常,入组248耳,对患儿行听性脑干反应、小儿行为测听和(或)听性稳态反应测试,分析其听力曲线类型、听力损失程度、声诱发短潜伏期负反应和ABR骨导与气导反应阈差值。结果:(1)248耳听力曲线类型分布:高频下降型44.8%(111/248),平坦型19.0%(47/248),上升型13.7%(34/248),U型3.6%(9/248)和无法判别19.0%(47/248);(2)听力损失程度分布:极重度73.4%(182/248),重度16.9%(42/248),中度6.9%(17/248)和轻度2.8%(7/248);(3)ABR测试中出现声诱发短潜伏期负反应占27.4%(68/248);(4)出现ABR骨导与气导反应阈差值占24.6%(61/248),平均差值为(19.3±14.2)dB nHL。结论:听力学检测中出现高频下降型听力损失、ABR测试中声诱发短潜伏期负反应和骨导及气导反应阈差值等"异常征兆"时,检测人员与临床医生应及时排查大前庭水管综合征。

Objective： To investigate the audiological characteristics of large vestibular aqueduct syndrome （LVAS） in infants and young children, and to provide suggestion for the early diagnosis and early intervention. Method： One hundred and twenty-four cases diagnosed as LVAS were enrolled in our study. Acoustic immittance, pediatric audiometry and（or） auditory steady-state responses and auditory brainstem response test were tested to analyze the degree and configuration of hearing loss, and air-bone threshold difference and short latency negative response in auditory brainstem response. Result：The configuration of the hearing loss, includes 44.8 % （111/248） of high frequency loss, 19.0%（47/248） of flat, 13.7%（34/248） of rising, 3.6%（9/248） of U type, and 19.0% （47/248） of the configuration which cannot be distinguished. The distribution of the degree of the hearing loss in total 124 cases （248 ears） includes 73.4%（182/248） of profound hearing loss, 16.9%（42/248） of severe hearing loss, 6.9%（17/248） of moderate hearing loss, and 2.8% （7/248）of mild hearing loss. The acoustically evoked short latency negative response in ABR accounted for 27.4% （68/248）. The emergence of ABR air-bone threshold difference accounted for 24.6% （61/248）, and the mean difference was （19.3 ± 14.2） dB nHL. Conclusion： Infants and young children with large vestibular aqueduct syndrome mostly has the characteristics of high frequency hear- ing loss curve, acoustically evoked short latency negative response in ABR and ABR bone-air threshold difference, that will remind clinicians of LVAS.