大前庭水管综合征患儿听力学检测特点分析

刘佳星; 黄丽辉; 傅新星; 刘辉; 杨亚利; 程晓华; 倪婷婷

doi:10.13201/j.issn.1001-1781.2016.21.009

大前庭水管综合征患儿听力学检测特点分析

- 首都医科大学附属北京同仁医院北京市耳鼻咽喉科研究所(北京, 100005)
基金项目:
卫生公益性行业科研专项基金(No:201202005)

首都医学发展科研基金项目(No:2009-1049)

首都临床特色应用研究专项基金(No:Z131107002213123)

详细信息

通讯作者: 黄丽辉,E-mail:huangpub@126.com

中图分类号: R764.3

收稿日期: 2016-08-21

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

- Beijing Institute of Otolaryngology Head and Neck Surgery, Beijing Tongren Hospital, Capital Medical University, Beijing, 100005, China

More Information

Corresponding author: HUANG Lihui, E-mail: huangpub@126.com

Received Date: 21 August 2016

摘要

摘要: 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:①248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);③ABR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。
- 大前庭水管综合征 /
- 听力损失 /
- 听力曲线 /
- 骨导、气导ABR反应阈值
Abstract: 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 hearing loss curve, acoustically evoked short latency negative response in ABR and ABR bone-air threshold difference, that will remind clinicians of LVAS.
- large vestibular aqueduct syndrome /
- hearing loss /
- audiometric configurations /
- ABR air conduction-bone conduction threshold

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