Audiological characteristics in infants and young chindren with cochlear nerve deficiency
10.16066/j.1672-7002.2025.04.004
- VernacularTitle:婴幼儿单侧听神经发育不良患者的听力学特征分析
- Author:
Yongbo ZHANG
1
;
Wendi SHI
;
Qing ZHANG
;
Zhuo WU
;
Yan ZHAO
Author Information
1. 宁波大学附属妇女儿童医院,浙江 宁波 315012
- Keywords:
Deafness;
Audiometry;
Evoked Potentials,Auditory,Brain Stem;
cochlear nerve deficiency;
unilateral deafness;
behavioral audiometry;
auditory steady-state response
- From:
Chinese Archives of Otolaryngology-Head and Neck Surgery
2025;32(4):221-224
- CountryChina
- Language:Chinese
-
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.
