摘要
目的分析儿童先天性单侧听力损失的病因及临床特征,为单侧听力损失患儿的临床诊断提供依据。方法分析164例先天性单侧听力损失的儿童的临床资料,包括临床病史采集、耳鼻咽喉科专科查体、听力损失高危因素调查,以及听力学及颞骨CT扫描或头颅/内听道MRI检查等。结果164例单侧听力损失患儿中,传导性21例(12.8%),感音性137例(83.5%),神经性5例(3.0%),混合性1例(0.6%);其中轻度61例(37.2%),中度33例(20.1%),重度13例(7.9),极重度57例(34.8%)。92例具有听力损失相关高危因素的患儿中,新生儿高胆红素血症38例(41.3%),早产11例(12.0%),巨细胞病毒感染15例(16.3%),合并有多种高危因素15例(16.3%)等;87例单侧听力损失患儿接受影像学检查,影像诊断学结果正常42例,45例影像异常患儿中,外耳畸形4例,中耳畸形2例,内耳畸形11例,蜗神经发育异常5例,合并多种畸形23例。病因未明29例,基因异常1例,染色体异常1例。结论164例先天性单侧听力损失的患儿中,具有听力损失相关高危因素(新生儿高胆红素血症,早产,巨细胞病毒感染,合并有多种高危因素)和耳影像学异常的患儿占比较高,说明听力损失相关高危因素和耳结构先天发育异常与单侧听力损失关系密切,临床上应着重针对听力损失相关高危因素的病史采集,结合专科查体,综合分析听力学及临床特征,必要时完善颞骨影像学检查。
Objective To report the etiology and clinical characteristics of children with unilateral congenital hearing loss,to provide a basis for improving clinical diagnosis.Methods Clinical data of 164 children with unilateral congenital hearing loss were reviewed,including clinical history,otolaryngology examination,risk factors for hearing loss,audiology assessment and temporal bone CT scan or head/internal auditory canal MRI,etc.Results Among the 164 children,diagnoses included conductive deafness(21 cases,12.8%),sensorineural deafness(137 cases,83.5%),neurological deafness(5 cases,3.0%)and mixed deafness(1 case,0.6%).Hearing loss was rated as mild in 61 cases(37.2%),moderate in 33 cases(20.1%),severe in 13 cases(7.9%)and profound in 57 cases(34.8%).Common risk factors for deafness were identified in 92 cases,including neonatal hyperbilirubinemia(38 cases,41.3%),premature birth(11 cases,12.0%),cytomegalovirus infection(15 cases,16.3%)and multiple comorbid high-risk factors(15 cases,16.3%).Imaging examination data were available in 87 children:normal in 42 cases and abnormal in 45 children,including external ear deformity(4 cases),middle ear deformity(2 cases),inner ear malformations(11 cases),abnormal development of the cochlear nerve(5 cases)and combined malformations(23 cases).Etiology was unknown in 29 cases and involved deafness gene abnormality in 1 case and chromosomal abnormality in 1 case.Conclusion A large proportion of this group of 164 children with unilateral congenital hearing loss demonstrated high-risk factors for hearing loss(neonatal hyperbilirubinemia,premature delivery,cytomegalovirus infection,combined with multiple high-risk factors)or abnormal imaging,indicating the roles of these high-risk factors in the development of unilateral congenital hearing loss.Clinically,the focus should be on collection of medical history including high-risk factors,otolayrngological examination,comprehensive audiology and clinical presentation assessment and,if necessary,completed with temporal bone imaging.
作者
葛成阳
孙婧
付勇
GE Chengyang;SUN Jing;FU Yong(Department of Otolaryngology Head and Neck Surgery,Affiliated Children's Hospital,Zhejiang University School of Medicine,Hang Zhou Zhejiang 310000,China)
出处
《中华耳科学杂志》
CSCD
北大核心
2023年第4期481-485,共5页
Chinese Journal of Otology
关键词
单侧听力损失
儿童
病因
临床特征
Unilateral Hearing Loss
Children
Etiology
Clinical Characteristics
