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听觉神经病谱系障碍(ANSD)患儿的管理。

Management of children with auditory neuropathy spectrum disorder (ANSD).

作者信息

Gökdoğan Çağıl, Altınyay Şenay, Gündüz Bülent, Kemaloğlu Yusuf Kemal, Bayazıt Yıldırım, Uygur Kemal

机构信息

Gazi University Hospital, Department of Audiology, Ankara, Turkey.

Gazi University Hospital, Department of Audiology, Ankara, Turkey.

出版信息

Braz J Otorhinolaryngol. 2016 Sep-Oct;82(5):493-9. doi: 10.1016/j.bjorl.2015.08.022. Epub 2015 Dec 18.

Abstract

INTRODUCTION

ANSD is a challenging problem.

OBJECTIVE

To present our experience on management of the children with ANSD with respect to clinical data.

METHODS

This retrospective study included all children younger than 16 years of age who applied to the department between 2005 and 2013 (with the exception of newborn hearing screening NHS referrals). The data were derived from pure tone, OAEs and ABR tests, and further medical risk factors of the subjects were evaluated.

RESULTS

ANSD was recognized in 74 ears of 40 children (B/U: 34/6) among 1952 children with SNHL (2.04%) detected among 9520 applicants to the department (0.42%). The clinical tests revealed that hearing loss greater than 15dB was present in both ears of 38 cases. The degree of hearing loss was profound in 48% children, severe in 12% children, moderate in 28% children, mild in 10% children and normal in 5% children. ABRs were absent/abnormal in 37/3 ears and CMs were detected in all. Acoustic reflexes were absent in all ears. Rehabilitation was managed by CI and hearing aids in 15 and 23 cases, respectively. FM system was given to two cases displaying normal hearing but poor speech discrimination in noisy environments.

CONCLUSION

ANSD is a relatively challenging problem for the audiology departments because of its various clinical features and difficulties in management. Our patients with ANSD most commonly displayed profound hearing loss. The number of overlooked cases may be minimized by performing ABR and OAE in every case referred with the suspicion of hearing loss.

摘要

引言

听神经病谱系障碍(ANSD)是一个具有挑战性的问题。

目的

介绍我们在处理患有ANSD儿童的临床数据方面的经验。

方法

这项回顾性研究纳入了2005年至2013年间到该科室就诊的所有16岁以下儿童(新生儿听力筛查转诊病例除外)。数据来源于纯音、耳声发射(OAE)和听性脑干反应(ABR)测试,并对受试者的其他医学风险因素进行了评估。

结果

在该科室9520名申请者中检测出1952例感音神经性听力损失(SNHL)儿童(占0.42%),其中40名儿童的74只耳朵被诊断为ANSD(双侧/单侧:34/6)(占2.04%)。临床测试显示,38例双耳听力损失大于15dB。听力损失程度为极重度的儿童占48%,重度占12%,中度占28%,轻度占10%,正常占5%。37只/3只耳朵的ABR缺失/异常,所有耳朵均检测到耳蜗微音电位(CM)。所有耳朵的声反射均缺失。分别有15例和23例通过人工耳蜗(CI)和助听器进行康复治疗。给2例听力正常但在嘈杂环境中言语辨别能力差的儿童配备了调频(FM)系统。

结论

由于ANSD具有多种临床特征且管理困难,对听力学科室来说是一个相对具有挑战性的问题。我们的ANSD患者最常见的表现是极重度听力损失。对于每一例疑似听力损失的转诊病例,通过进行ABR和OAE检查,可将漏诊病例数降至最低。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/297c/9444594/0a055d1c2de8/gr1.jpg

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