34例婴幼儿听神经病临床特征及康复效果分析OA

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.