An integrative approach for pediatric auditory neuropathy spectrum disorders: revisiting etiologies and exploring the prognostic utility of auditory steady-state response

被引：12

作者：

Lin, Pei-Hsuan ^{[1
,2
,3
]}

Hsu, Chuan-Jen ^{[3
,4
]}

Lin, Yin-Hung ^{[3
]}

Lin, Yi-Hsin ^{[3
,5
]}

Yang, Shu-Yu ^{[3
]}

Yang, Ting-Hua ^{[3
]}

Chen, Pei-Lung ^{[1
,5
,6
]}

Wu, Chen-Chi ^{[3
,6
]}

Liu, Tien-Chen ^{[3
]}

机构：

[1] Natl Taiwan Univ, Coll Med, Grad Inst Clin Med, Taipei, Taiwan

[2] Natl Taiwan Univ Hosp, Yunlin Branch, Dept Otolaryngol, Touliu, Yunlin, Taiwan

[3] Natl Taiwan Univ Hosp, Dept Otolaryngol, Taipei, Taiwan

[4] Taichung Tzu Chi Hosp, Dept Otolaryngol, Taichung, Taiwan

[5] Natl Taiwan Univ, Coll Med, Grad Inst Med Genom & Prote, Taipei, Taiwan

[6] Natl Taiwan Univ Hosp, Dept Med Genet, Taipei, Taiwan

来源：

SCIENTIFIC REPORTS | 2020年 / 10卷 / 01期

关键词：

SENSORINEURAL HEARING-LOSS; INNER-EAR; COCHLEAR IMPLANTATION; OTOF MUTATIONS; AUDIOLOGICAL CHARACTERISTICS; RISK-FACTORS; BRAIN-STEM; WFS1; GENE; CHILDREN; PREVALENCE;

D O I：

10.1038/s41598-020-66877-y

中图分类号：

O [数理科学和化学]; P [天文学、地球科学]; Q [生物科学]; N [自然科学总论];

学科分类号：

07 ; 0710 ; 09 ;

摘要：

Auditory neuropathy is an important entity in childhood sensorineural hearing loss. Due to diverse etiologies and clinical features, the management is often challenging. This study used an integrative patient-history, audiologic, genetic, and imaging-based approach to investigate the etiologies and audiologic features of 101 children with auditory neuropathy. Etiologically, 48 (47.5%), 16 (15.8%), 11 (10.9%), and 26 (25.7%) children were categorized as having acquired, genetic, cochlear nerve deficiency-related, and indefinite auditory neuropathy, respectively. The most common causes of acquired and genetic auditory neuropathy were prematurity and OTOF mutations, respectively. Patients with acquired auditory neuropathy presented hearing loss earlier (odds ratio, 10.2; 95% confidence interval, 2.2-47.4), whereas patients with genetic auditory neuropathy had higher presence rate of distortion product otoacoustic emissions (odds ratio, 10.7; 95% confidence interval, 1.3-85.4). In patients with different etiologies or pathological sites, moderate to strong correlations (Pearson's r=0.51-0.83) were observed between behavioral thresholds and auditory steady-state response thresholds. In conclusion, comprehensive assessments can provide etiological clues in similar to 75% of the children with auditory neuropathy. Different etiologies are associated with different audiologic features, and auditory steady-state responses might serve as an objective measure for estimating behavioral thresholds.

引用

页数：11

共 64 条

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