中华耳科学杂志2026，Vol.24Issue(7)：630-634,5.DOI:10.3969/j.issn.1672-2922.2026.07.005
34例婴幼儿听神经病临床特征及康复效果分析
Clinical characteristics and rehabilitation outcomes in 34 infants with auditory neuropathy
摘要
Abstract
Objective To report clinical characteristics and rehabilitation outcomes of auditory neuropathy(AN)infants,for improving diagnosis and treatment strategies.Methods This study included 34 young children with AN(20 males,14 females;32 bilateral,2 unilateral)diagnosed at Guangzhou Women and Children's Medical Center between 2015 and 2021.The age at diagnosis ranged from 0.25 to 2.67 years(mean=0.68±0.40 years).Etiology and audiological features were analyzed,as well as rehabilitation methods.Audimetric and speech outcomes were evaluated 2 years post-diagnosis using the Category Auditory Performance and Speech Intelligibility Rate scales.Results Associated conditions included hyperbilirubinemia(n=20,58.9%)(8 with perinatal hypoxia,7 with preterm/low birth weight),bilirubin encephalopathy(n=16),perinatal hypoxia without hyperbilirubinemia(n=4)and meningitis(n=2),while there was no significant disease history in 8 cases,with 2 of them carrying OTOF and 1 carrying TIMM8A gene variants.Auditory brainstem responses were either severely abnormal or absent,despite detectable otoacoustic emissions in 29 cases(85.3%)and cochlear microphonic in all cases.Hearing aids were fitted in 11 children,with 2(18.2%)showing improved auditory-speech abilities and 9 exhibiting no significant improvement.Of the 18 children receiving cochlear implant,13(72.2%)demonstrated improvement and 5(including 3 with postsynaptic pathology)showed no notable benefits.In the 5 cases receiving no intervention,2 with unilateral AN developed normal auditory-speech abilities,while 3 with bilateral AN showed no improvement.Conclusions Hyperbilirubinemia is the primary etiological factor in this cohort of infants with AN.Patients who developed kernicterus exhibited poor rehabilitation outcomes.关键词
听神经病/胆红素脑病/助听器/人工耳蜗Key words
auditory neuropathy/hyperbilirubinemia/hearing aid/cochlear implant引用本文复制引用
黄宇童,侯超,吴秀娟,高胜利..34例婴幼儿听神经病临床特征及康复效果分析[J].中华耳科学杂志,2026,24(7):630-634,5.基金项目
广州市科技计划项目(2023A03J0909,2023A04J1240) （2023A03J0909,2023A04J1240）
广州医科大学附属妇女儿童医疗中心临床博士启动科研基金项目(2021BS027) （2021BS027）
