Analysis of clinical audiological characteristics in children with Williams syndrome in China

This study revealed that children with WS frequently exhibit middle and inner ear dysfunction, often accompanied by HL or subclinical cochlear impairment, which can emerge before age 3. Prolonged ABR latency indicates delayed auditory nerve myelination, while shortened interpeak latency III-V may serve as an electrophysiological marker in this population. Long-term, regular hearing follow-up is recommended to enable early HL detection and timely treatment of contributing conditions.

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Publication Details

Journal
Orphanet Journal of Rare Diseases
Published
2025-05-20
DOI
https://doi.org/10.1186/s13023-025-03650-2
Primary Topic
Williams Syndrome Research
Type
article
Field-Weighted Citation Impact
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article

Analysis of clinical audiological characteristics in children with Williams syndrome in China

Junxia Guo, Bin Xu, Jiyang Shen, Fangfang Li et al.
Orphanet Journal of Rare Diseases
Williams Syndrome Research
article

Analysis of clinical audiological characteristics in children with Williams syndrome in China

Junxia Guo, Bin Xu, Jiyang Shen, Fangfang Li, Dan Yao, Jie Shao, Weijun Chen, Chai Ji
article en

Abstract

This study revealed that children with WS frequently exhibit middle and inner ear dysfunction, often accompanied by HL or subclinical cochlear impairment, which can emerge before age 3. Prolonged ABR latency indicates delayed auditory nerve myelination, while shortened interpeak latency III-V may serve as an electrophysiological marker in this population. Long-term, regular hearing follow-up is recommended to enable early HL detection and timely treatment of contributing conditions.

Orphanet Journal of Rare DiseasesVol. 20(1)
Children's Hospital of Zhejiang University (CN)
Good health and well-being
Openalex Percentile: Top 87%
Williams Syndrome Research
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Analysis of clinical audiological characteristics in children with Williams syndrome in China — Junxia Guo, Bin Xu, et al. · Orphanet Journal of Rare Diseases (2025) | TGRS Research Map | TGRS