威廉综合征儿童畸变产物耳声发射特征：单中心76例患儿回顾性分析

doi:10.12372/jcp.2024.24e0652

临床儿科杂志 ›› 2024, Vol. 42 ›› Issue (12): 1021-1024.doi: 10.12372/jcp.2024.24e0652

威廉综合征儿童畸变产物耳声发射特征：单中心76例患儿回顾性分析

胡可心, 李芳芳, 曾艳, 陈维军, 季钗()

浙江大学医学院附属儿童医院儿童保健科国家儿童健康与疾病临床医学研究中心（浙江杭州 310003）

收稿日期:2024-06-28 出版日期:2024-12-15 发布日期:2024-12-02
通讯作者: 季钗电子信箱：6198011@zju.edu.cn
基金资助:
浙江省基础公益研究计划项目(LTGY24H260003)

Williams syndrome children's distorted product otoacoustic emission characteristics: a retrospective analysis of 76 patients at a single center

HU Kexin, LI Fangfang, ZENG Yan, CHEN Weijun, JI Chai()

Department of Pediatric Health Care, The Children's Hospital, Zhejiang University School of Medicine, National Clinical Medical Research Center for Child Health and Disease, Zhejiang 310003, Hangzhou, China

Received:2024-06-28 Published:2024-12-15 Online:2024-12-02

摘要/Abstract

摘要：

目的分析威廉综合征（WS）患儿的畸变产物耳声发射（DPOAE）特征，为WS患儿的听力随访提供临床依据。方法选择2019年6月—2023年6月于儿童保健科确诊并随访的WS患儿作为研究对象；以同期来医院体检的健康儿童作为对照组。比较WS组和对照组间DPOAE差异及不同年龄段间WS患儿的DPOAE差异。结果 WS患儿76例，男42例、女34例，中位年龄4.7（2.6~6.7）岁；对照组51例，男32例、女19例，中位年龄3.9（2.9~5.2）岁；WS组不同年龄段之间耳声发射通过率差异无统计学意义（P>0.05）。WS组患儿耳声发射通过率为78.8%，低于对照组（97.0%），差异有统计学意义（P<0.001）。3~5岁组，WS组耳声发射通过率为76.6%，低于对照组（96.7%）；≥6岁组，WS组耳声发射通过率为69.6%，低于对照组（100%），差异均有统计学意义（P<0.05）。WS组患儿不同年龄段之间2 000、4 000Hz信噪比差异均有统计学意义（P<0.05）；3~5岁组2 000Hz的信噪比低于<3岁组，≥6岁组在2 000Hz、4 000Hz的信噪比均低于<3岁组，差异均有统计学意义（P<0.05）。WS组各年龄段的耳声发射2 000、3 000、4 000、5 000Hz 4个频率信噪比均小于对照组，差异均有统计学意义（P<0.05）。结论听力筛查异常在WS患儿中常见，并有亚临床听力损失的表现；建议对该群体进行长期有规律的听力随访，以便早期发现听力损失并及时采取相应的干预措施。

关键词: 威廉综合征, 畸变产物耳声发射, 听力损失

Abstract:

Objective To analyze the characteristics of distorted product otoacoustic emissions (DPOAE) in children with Williams syndrome (WS) and provide clinical basis for the follow-up of the target population in the future. Methods Children with WS who were diagnosed and followed up in the Department of Pediatric Health Care from June 2019 to June 2023 were selected as the study objects. Children who came to the hospital for routine physical examination during the same period were taken as the control group. The difference of DPOAE between WS group and control group and the difference of DPOAE among WS children of different ages were compared. Results A total of 76 children with WS (42 boys and 34 girls) were included, with a median age of 4.7 (2.6-6.7) years. The control group consisted of 51 patients (32 boys and 19 girls) with a median age of 3.9 (2.9-5.2) years. There was no significant difference in DPOAE passing rate among different age groups in WS group (P>0.05). The DPOAE passing rate of WS group was 78.8%, lower than that of control group (97.0%), and the difference was statistically significant (P<0.001). In the 3-5 year-old group, the passing rate of DPOAE in the WS group was 76.6%, lower than that of the control group (96.7%). In the group of 6 years or older, the passing rate of DPOAE in the WS group was 69.6%, lower than that in the control group (100%), and the differences were statistically significant (P<0.05). There were statistically significant differences in the signal-to-noise ratio (SNR) of 2000Hz and 4000Hz between different age groups in WS (P<0.05). The SNR of 2000Hz in the 3 to 5 years old group was lower than that of the < 3 years old group, and the SNR of 2000Hz and 4000Hz in the ≥6 years old group was lower than that of the < 3 years old group, with statistical significance (P<0.05). The SNR of DPOAE at 2000, 3000, 4000 and 5000Hz at all ages in WS group was lower than that in the control group, with statistical significance (P<0.05). Conclusions Hearing screening abnormality is very common in children with WS, and there are manifestations of subclinical hearing loss. It is recommended to carry out long-term regular hearing follow-up for this group in order to detect hearing loss early and take corresponding intervention measures in time.

Key words: Williams syndrome, distortion product otoacoustic emission, hearing loss

胡可心, 李芳芳, 曾艳, 陈维军, 季钗. 威廉综合征儿童畸变产物耳声发射特征：单中心76例患儿回顾性分析[J]. 临床儿科杂志, 2024, 42(12): 1021-1024.

HU Kexin, LI Fangfang, ZENG Yan, CHEN Weijun, JI Chai. Williams syndrome children's distorted product otoacoustic emission characteristics: a retrospective analysis of 76 patients at a single center[J]. Journal of Clinical Pediatrics, 2024, 42(12): 1021-1024.

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参考文献 18

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组别	年龄段	耳数	正常耳
WS组	<3岁	58	51（87.9）
	3~5岁	47	36（76.6）
	≥6岁	46	32（69.6）
χ²值			5.38
P			0.068
对照组	<3岁	27	26（96.3）
	3~5岁	60	58（96.7）
	≥6岁	14	14（100）
P¹⁾			1.000

年龄	2 000Hz		Z值	P	3 000Hz		Z值	P
年龄	WS组	对照组	Z值	P	WS组	对照组	Z值	P
<3岁	10.2(6.8~15.5)	15.4(11.5~20.0)	3.82	<0.001	11.6(8.0~17.4)	16.9(11.4~23.1)	3.08	0.002
3~5岁	9.2(4.6~11.0)¹⁾	17.2(11.2~21.7)	6.78	<0.001	9.9(6.6~13.7)	19.2(15.3~22.9)	6.68	<0.001
≥6岁	8.1(2.5~10.7)¹⁾	16.5(12.6~19.2)	4.76	<0.001	9.3(2.2~13.8)	17.6(15.4~22.6)	5.21	<0.001
H值	8.77	0.54			4.83	2.34
P	0.012	0.765			0.089	0.311
年龄	4 000Hz		Z值	P	5 000Hz		Z值	P
年龄	WS组	对照组	Z值	P	WS组	对照组	Z值	P
<3岁	14.2(9.3~20.5)	20.9(14.6~25.1)	3.23	0.001	15.7(10.1~20.3)	22.7(13.3~27.9)	3.59	<0.001
3~5岁	11.5(8.7~15.8)	23.4(14.4~28.3)	5.69	<0.001	14.7(9.3~20.5)	24.8(18.2~30.8)	5.75	<0.001
≥6岁	11.0(5.3~14.2)¹⁾	25.8(22.4~26.5)	6.41	<0.001	12.7(6.1~18.1)	29.1(20.1~30.8)	4.95	<0.001
H值	6.39	5.71			2.75	5.07
P	0.041	0.058			0.253	0.079

威廉综合征儿童畸变产物耳声发射特征：单中心76例患儿回顾性分析

Williams syndrome children's distorted product otoacoustic emission characteristics: a retrospective analysis of 76 patients at a single center

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