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Hearing loss trajectory and prediction model for children with enlarged vestibular aqueduct

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收录情况： ◇ SCIE

作者：
Deng Lin[1,2,3] * ; Yang Xiaozhe[1,2,3] ; Cheng Xiaohua[1,2,3] ; Wen Cheng[1,2,3] ; Yu Yiding[1,2,3] ; Li Yue[1,2,3] ; Gao Shan[1,2,31] ; Liu Hui[1,2,3] ; Liu Dongxin[1,2,3] ; Ruan Yu[1,2,3] ; Xie Jinge[1,2,3] ; En Hui[1,2,3] ; Xian Junfang[4] ; Huang Lihui[1,2,3,*1,*2,*3] ;
机构： [1]Otolaryngology-Head and Neck Surgery, Beijing Tongren Hospital, Capital Medical University, Beijing, China [2]Beijing Institute of Otolaryngology, Beijing, China [3]Key Laboratory of Otolaryngology Head and Neck Surgery, Ministry of Education, Beijing, China [4]Department of Radiology, Beijing Tongren Hospital, Capital Medical University, Beijing, China
出处：
DOI： 10.1016/j.amjoto.2024.104573
ISSN：

关键词： Enlarged vestibular aqueduct Children Progressive hearing loss Linear mixed-effects model Prediction model

摘要：
To explore how hearing changes over time and the characteristics associated with progressive hearing loss in children with enlarged vestibular aqueduct (EVA), and develop a prediction model for anticipation of hearing progression probability.A retrospective analysis was conducted on 48 children (92 ears) diagnosed with EVA. A total of 314 audiograms were included in the analysis of hearing loss trajectories using linear mixed-effects model. Progressive hearing loss was defined based on the difference between the initial and final hearing threshold. All participants had underwent one or two gene detection methods, including deafness gene screening and SLC26A4 whole coding exon sequencing.The pure-tone thresholds (PTTs) at frequencies of 500 Hz, 1000 Hz, 2000 Hz, 4000 Hz and the average are expected to increase by 0.34, 0.49, 0.54, 0.57, and 0.55 dB HL per each additional month. Age and genotypes have an interactive effect on PTT at frequencies of 500 Hz, 1000 Hz, and the average. The hazard ratio for the genotype without SLC26A4 c.919-2 A > G mutation was 4.91 (95 % confidence interval 1.76-13.7, P < 0.01). This prediction model fitted using age, initial average PTT, midpoint size of vestibular aqueduct, incomplete partition type II, and genotypes of SLC26A4 showed strong consistency and differentiation.These findings reveal that the PTT would deteriorate over time in patients with EVA. The hearing threshold at high frequency and genotype without c.919-2 A > G heterozygous mutation deteriorated relatively fast. Genotype is an important predictive factor and the nomogram helps to predict progressive hearing loss.Copyright © 2024 The Authors. Published by Elsevier Inc. All rights reserved.

基金：
This work was supported by the Capital's Funds for Health Improvement and Research under Grant (CFH 2022-2-1092); and the Natural Science Foundation of China under Grant (82071064).
语种：
外文
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中科院(CAS)分区：
出版当年[2025]版：
大类 | 3 区 医学
小类 | 3 区 耳鼻喉科学
最新[2025]版：
大类 | 3 区 医学
小类 | 3 区 耳鼻喉科学
JCR分区：
出版当年[2023]版：
Q2 OTORHINOLARYNGOLOGY
最新[2023]版：
Q2 OTORHINOLARYNGOLOGY

影响因子： 最新[2023版] 最新五年平均 出版当年[2023版] 出版当年五年平均 出版前一年[2022版]

第一作者：
第一作者机构： [1]Otolaryngology-Head and Neck Surgery, Beijing Tongren Hospital, Capital Medical University, Beijing, China [2]Beijing Institute of Otolaryngology, Beijing, China [3]Key Laboratory of Otolaryngology Head and Neck Surgery, Ministry of Education, Beijing, China
通讯作者：
通讯机构： [1]Otolaryngology-Head and Neck Surgery, Beijing Tongren Hospital, Capital Medical University, Beijing, China [2]Beijing Institute of Otolaryngology, Beijing, China [3]Key Laboratory of Otolaryngology Head and Neck Surgery, Ministry of Education, Beijing, China [*1]Otolaryngology-Head and Neck Surgery, Beijing Tongren Hospital, Capital Medical University, Beijing 100730, China. [*2]Beijing Institute of Otolaryngology, Beijing 100005, China [*3]Key Laboratory of Otolaryngology Head and Neck Surgery, Ministry of Education, Beijing 100005, China.
推荐引用方式(GB/T 7714)：
Deng Lin,Yang Xiaozhe,Cheng Xiaohua,et al.Hearing loss trajectory and prediction model for children with enlarged vestibular aqueduct[J].American Journal Of Otolaryngology.2025,46(1):104573.doi:10.1016/j.amjoto.2024.104573.
APA：
Deng Lin,Yang Xiaozhe,Cheng Xiaohua,Wen Cheng,Yu Yiding...&Huang Lihui.(2025).Hearing loss trajectory and prediction model for children with enlarged vestibular aqueduct.American Journal Of Otolaryngology,46,(1)
MLA：
Deng Lin,et al."Hearing loss trajectory and prediction model for children with enlarged vestibular aqueduct".American Journal Of Otolaryngology 46..1(2025):104573

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