山东大学耳鼻喉眼学报 ›› 2024, Vol. 38 ›› Issue (4): 96-196.doi: 10.6040/j.issn.1673-3770.0.2024.219

• 论著 • 上一篇    下一篇

先天性单侧聋患儿112例临床资料分析

张晓1,刘海红1,刘薇1,李颖1,陈敏1,刘冰1,白杰1,张杰1,郑军2,杨扬1   

  1. 1.国家儿童医学中心/首都医科大学附属北京儿童医院/儿童耳鼻咽喉头颈外科疾病北京市重点实验室 耳鼻咽喉头颈外科, 北京 100045;
    2.首都医科大学附属北京同仁医院耳鼻咽喉头颈外科, 北京 100005
  • 发布日期:2024-07-09
  • 通讯作者: 杨扬. E-mail:yangyang@bch.com.cn

Analysis of clinical data form 112 cases of children with congenital single-sided deafness

ZHANG Xiao1, LIU Haihong1, LIU Wei1, LI Ying1, CHEN Min1, LIU Bing1, BAI Jie1, ZHANG Jie1, ZHENG Jun2, YANG Yang1   

  1. 1. Department of Otorhinolaryngology & Head and Neck Surgery, Beijing Children's Hospital, Capital Medical University National Center for Children's Health, Beijing Key Laboratory for Pediatric Diseases of Otolaryngology Head and Neck Surgery, Beijing 100045, China2. Department of Otorhinolaryngology & Head and Neck Surgery, Beijing Tongren Hospital, Capital Medical University, Beijing 100005, China
  • Published:2024-07-09

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摘要： 目的 探讨先天性单侧聋(single-sided deafness, SSD)患儿就诊原因/发现途径、听力学特点、影像学特点,归纳总结单侧聋患儿临床特点及干预策略。 方法 回顾性分析2020年6月至2022年6月间就诊于北京儿童医院耳鼻喉科诊断为先天性SSD患儿的临床资料,包括患儿一般资料、就诊原因/发现途径,听性脑干反应、纯音测听、耳蜗微音器电位(cochlear microphonics, CM)、耳声发射(otoacoustic emission, OAE)、声导抗等检查;颞骨CT和/或内听道MRI影像学检查,总结其临床特点,并对干预治疗的患儿进行随访。 结果 共112例患儿纳入本研究,其中男66例、女46例;左耳61例、右耳51例;0~13岁,中位数年龄 4岁6个月;OAE:聋耳均未引出;CM:38例聋耳可引出;对侧正常耳OAE、CM均正常引出;声导抗均为A型曲线;因新生儿听力筛查未通过就诊的患儿比例最高(41.1%);影像学显示结构异常75例(66.9%),其中比例最高为单纯蜗神经孔狭窄/蜗神经发育不良62例(82.7%);SSD患儿接受外科干预治疗2例,其中不伴蜗神经发育不良行人工耳蜗植入1例、伴蜗神经发育不良行骨桥植入1例。术后6个月,2例患儿均方根误差(root-mean-square error, RMSE)、定位偏侧BIAS值均有改善,声源定位能力提高。 结论 先天性单侧聋患儿内耳结构异常比例较高,以蜗神经孔狭窄/蜗神经发育不良多见。儿童单侧聋不易被发觉,应重视新生儿听力筛查与入园听力体检及定期听力检查,警惕预防潜在的交通事故风险发生。针对当前先天性SSD干预率极低的现状,应提高对疾病特点的认识,给予个体化、科学性干预。

关键词: 儿童, 先天性单侧聋, 内耳畸形, 听力筛查, 蜗神经

Abstract: Objective To investigate the causes/detection pathways, audiological characteristics, and imaging features of children with congenital single-sided deafness(SSD), and to summarize the clinical characteristics and intervention strategies. Methods We retrospectively analyzed the clinical data of children diagnosed with congenital SSD at the Department of Otorhinolaryngology, Beijing Children's Hospital; from June 2020 to June 2022, including general information of the children, reason for consultation/path of discovery, ABR, pure tone audiometry, CM, OAE, acoustic impedance, temporal bone CT and/or inner auditory canal MRI, summarized the clinical characteristics and followed up the children who received treatment. Results A total of 112 children were enrolled in this study, including 66 males and 46 females; 61 left ears and 51 right ears; 0-13 years old; OAE: none of the deaf ears were elicited; CM: 38 deaf ears could be elicited; the highest proportion of children visited the clinic due to failure of newborn hearing screening(41.1%); 75 cases(66.9%)had structural anomalies shown by imaging, with the proportion the highest percentage was 62 cases(82.7%)with simple cochlear nerve foramen stenosis/cochlear nerve dysplasia; 2 children with SSD underwent surgical interventions, including 1 case of cochlear implantation without cochlear nerve dysplasia and 1 case of bone bridge implantation with cochlear nerve dysplasia. Six months after surgery, both children's RMSE and BIAS improved, and the ability to localize the sound source was improved. Conclusion Children with congenital SSD have a high proportion of structural abnormalies of the inner ear, with stenosis of the cochlear nerve foramen/cochlear nerve hypoplasia being the most common. SSD in children is not easy to detect, and attention should be paid to newborn hearing screening and hearing screening, and vigilance should be exercised to prevent the occurrence of potential traffic accident risks. Given the current situation that the intervention rate of congenital SSD is extremely low, we should improve the understanding of the characteristics of the disease and provide individualised and scientific interventions.

Key words: Children, Congenital single-sided deafness, Inner ear malformation, Hearing screen, Cochlear nerve

中图分类号: 

  • R764.43
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