中国耳鼻咽喉头颈外科2025，Vol.32Issue(4)：221-224,4.DOI:10.16066/j.1672-7002.2025.04.004
婴幼儿单侧听神经发育不良患者的听力学特征分析
Audiological characteristics in infants and young chindren with cochlear nerve deficiency
摘要
Abstract
OBJECTIVE To investigate the auditory characteristics of patients with congenital unilateral cochlear nerve deficiency(CND).METHODS This study enrolled 21 patients(21 ears)with unilateral auditory nerve dysplasia confirmed by inner ear MRI,including 9 males and 12 females,aged 10 months to 7 years,(1.91±1.38)years.A retrospective analysis was performed on the correlation between behavioral audiometry and auditory steady-state response(ASSR)thresholds,chirp-evoked auditory brainstem response(chirp-ABR),otoacoustic emission(OAE),and other results to analyze the audiological characteristics of unilateral auditory nerve dysplasia.RESULTS In all 21 patients(21 ears)with auditory nerve dysplasia,behavioral audiometry,chirp-ABR,and ASSR results all indicated severe to profound hearing loss in the affected ears.There was a high correlation between behavioral audiometry thresholds and ASSR thresholds,with small differences observed between ASSR and behavioral audiometry results.CONCLUSION The difference between ASSR and behavioral audiometry is small in patients with CND.A-ABR can't be elicited.OAE has a certain false negative rate.The ABR often predominantly shows wave III,with prolonged latency,and a threshold indicative of profound hearing loss.These audiological characteristics can improve the early detection and diagnosis of CND and enhance the effect of intervention.关键词
耳聋/测听法/诱发电位,听觉,脑干/听神经发育不良/单侧聋/行为测听/听性稳态反应Key words
Deafness/Audiometry/Evoked Potentials,Auditory,Brain Stem/cochlear nerve deficiency/unilateral deafness/behavioral audiometry/auditory steady-state response引用本文复制引用
张永波,史文迪,张青,吴卓,赵燕..婴幼儿单侧听神经发育不良患者的听力学特征分析[J].中国耳鼻咽喉头颈外科,2025,32(4):221-224,4.基金项目
国家自然科学基金项目(82471160、82171137) （82471160、82171137）
上海市医企融合创新成果转化专项(SHDC2022CRD013) （SHDC2022CRD013）
中国高校产学研创新基金(2023IT059) （2023IT059）
