大前庭水管综合征患儿听力损失进展影响因素分析

The Analysis of Influencing Factors of Progressive Hearing Loss in Children with Large Vestibular Aqueduct Syndrome

邓琳;程晓华;黄丽辉;刘辉;刘冬鑫;文铖;李悦;杨晓喆;鲜军舫

1:首都医科大学附属北京同仁医院耳鼻咽喉头颈外科

2:北京市耳鼻咽喉科研究所

3:耳鼻咽喉头颈科学教育部重点实验室(首都医科大学)

4:首都医科大学附属北京同仁医院放射科

摘要
目的 探讨大前庭水管综合征(large vestibular aqueduct syndrome, LVAS)患儿听力损失进展的影响因素,为识别进行性听力损失高风险患儿及其听力监测建议提供依据。方法 研究对象为2017年1月至2023年1月确诊为LVAS的49例(95耳)患儿,所有受试者均接受两次及以上听力测试,根据听力进展情况分为稳定组(55耳)和进展组(40耳)。通过单因素和多因素Cox回归分析,确定听力进展的影响因素,分析变量包括:年龄,性别,耳别,初次和末次0.5~4.0 kHz各频率听阈及平均听阈,前庭水管中点、外口、不完全分隔Ⅱ型(incomplete partition type 2,IP-Ⅱ)以及SLC26A4基因型(A型:c.919-2A>G纯合突变,B型:含c.919-2A>G复合杂合或杂合突变,C型:不含c.919-2A>G突变位点,D型:未行基因检测)。筛选变量通过Kaplan-Meier生存分析进一步验证。结果 初次平均听阈每降低1 dB,听力损失进展发生风险增加7.03%(P=0.02)。伴与不伴IP-Ⅱ耳蜗畸形患儿听力损失进展的风险比为5.11(95%CI为1.81~14.45,P=0.002),基因型C型与A型的风险比为6.13(95%CI为2.07~18.13,P=0.001)。Kaplan-Meier生存分析提示这3项因素均具有统计学意义。结论 0.5~4 kHz初次平均听阈低、伴IP-Ⅱ耳蜗畸形和不含c.919-2A>G突变位点是LVAS患儿听力损失进展的影响因素,具有这些进行性听力损失高风险的LVAS患儿建议缩短听力监测周期。
关键词
大前庭水管综合征;进行性听力损失;儿童;Cox回归分析
基金项目(Foundation):
国家自然科学基金(82071064);; 首都卫生发展科研专项(首发2022-2-1092)
作者
邓琳;程晓华;黄丽辉;刘辉;刘冬鑫;文铖;李悦;杨晓喆;鲜军舫
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