Auditory neuropathy spectrum disorder (ANSD) in referrals from neonatal hearing screening at a well-baby clinic
Auditory neuropathy spectrum disorder (ANSD) in referrals from neonatal hearing screening at a well-baby clinic
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DOI：
10.1007/s00431-016-2735-5
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发表时间：
2016-07-01
影响因子：
3.6
通讯作者：
van de Heyning, Paul
中科院分区：
文献类型：
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作者：
Boudewyns, A.;Declau, Frank;van de Heyning, Paul
听觉神经病谱系障碍（ANSD）是一种特殊的听力障碍，其特征是外毛细胞功能正常，而听觉脑干反应异常或缺失。关于这种疾病在健康新生儿中的患病率，几乎没有数据。我们对一家健康婴儿诊所的791例新生儿听力筛查（UNHS）转诊病例进行了回顾性病历分析，以调查ANSD的患病率。听力筛查通过自动听觉脑干反应（ABR）测试进行。ANSD的诊断建立时，ABR描记不存在耳声发射和/或耳蜗颤噪。在201名确诊为先天性听力损失的婴儿中，13名婴儿被诊断为ANSD。6名婴儿为单侧，7名婴儿为双侧。在三个婴儿中可以确定听力损失的危险因素。结论：在通过UNHS确诊的先天性听力损失的健康婴儿中，ANSD的患病率为6.5%。在我们的健康婴儿诊所的新生儿人群中，ANSD的估计发病率为0.09/1000活产。磁共振显示约一半的患者存在潜在的解剖异常。
Auditory neuropathy spectrum disorder (ANSD) is a particular kind of hearing disorder characterised by normal outer hair cell function and abnormal or absent auditory brain stem responses. Little data are available regarding the prevalence of this condition in healthy newborns. We performed a retrospective medical records review of 791 referrals from universal neonatal hearing screening (UNHS) at a well-baby clinic to investigate the prevalence of ANSD. Hearing screening was performed by automated auditory brain stem response (ABR) testing. A diagnosis of ANSD was established when ABR tracings were absent in the presence of otoacoustic emissions and/or a cochlear microphonic. Amongst 201 infants with confirmed congenital hearing loss, 13 infants were diagnosed with ANSD. The condition was unilateral in six and bilateral in seven infants. A risk factor for hearing loss could be identified in three infants. Abnormalities on magnetic resonance imaging were found in six infants; five of them had cochlear nerve deficiency.Conclusion: The prevalence of ANSD was 6.5 % amongst well babies with confirmed congenital hearing loss identified through UNHS. The estimated incidence of ANSD in our population of newborns at the well-baby clinic was 0.09/1000 live births. Magnetic resonance revealed an underlying anatomical abnormality in about half of the patients.