特纳综合征相关耳部疾患的分布特征及管理策略  

Otologic disorders and management strategies in Turner syndrome

作　　者：司瑜[1,2] 熊莹 张丽娜 李湘辉[1,2] 卓师培[1,2] 冯奕思[1,2] 梁立阳 张志钢 Si Yu;Xiong Ying;Zhang Lina;Li Xianghui;Feng Yisi;Liang Liyang;Zhang Zhigang(Department of Otorhinolaryngology Head and Neck Surgery,Sun Yat-Sen Memorial Hospital,Sun Yat-Sen University,Guangzhou 510120,China;Institute of Hearing and Speech-Language Science,Sun Yat-sen University,Guangzhou 510120,China;Department of Pediatrics,Sun Yat-Sen Memorial Hospital,Sun Yat-Sen University,Guangzhou 510120,China)

机构地区：[1]中山大学孙逸仙纪念医院耳鼻咽喉头颈外科,广州510120 [2]中山大学听力与言语研究所,广州510120 [3]中山大学孙逸仙纪念医院儿科,广州510120

出　　处：《中华耳鼻咽喉头颈外科杂志》2022年第5期595-601,共7页Chinese Journal of Otorhinolaryngology Head and Neck Surgery

基　　金：国家自然科学基金(81670921)。

摘　　要：目的分析特纳综合征(Turner syndrome,TS)患者耳部疾患的发生率及危险因素,为TS患者的耳健康提供管理策略。方法本研究是以调查问卷为基础的前瞻性横断面研究,纳入2010年1月至2021年3月在中山大学孙逸仙纪念医院就诊的TS患者(共纳入71例,TS诊断时年龄3~11岁,耳鼻喉科就诊时年龄4~27岁),问卷调查患者不同年龄段耳部疾患的发生情况。横断面研究内容包括耳部形态学及听功能评估,并进一步分析引起耳部疾患及听功能改变的危险因素。采用Prism软件进行数据统计分析。结果问卷调查发现急性中耳炎在3~6岁及7~12岁患者中的发生率显著高于12岁以上患者,分别为33.8%(24/71)、42.9%(30/70)及23.5%(8/34);复发性急性中耳炎在3~6岁患者中的发病比例高达21.1%(15/71),其中约46.6%(7/15)患者会持续到6岁以后。分泌性中耳炎在3~6岁、7~12岁及12岁以上三个年龄段的发生率分别为32.4%(23/71)、34.3%(24/70)及38.2%(13/34),鼓膜穿刺治疗的复发率分别为100%(7/7)、42.9%(3/7)及50.0%(1/2),显著高于鼓膜置管治疗。3~6岁及7~12岁两个年龄段,X染色体结构异常患者急性中耳炎及分泌性中耳炎的患病率低于其他核型患者;大于12岁的患者,急性中耳炎的患病率在各种核型中的差异不明显,分泌性中耳炎在含Y染色体核型中患病率最高。此外,伴随有其他系统疾病患者的急性中耳炎、分泌性中耳炎的发生率明显增加。横断面研究发现本组TS患者中低位耳的发生比例为7.0%(5/71),外耳道狭窄4.2%(3/71),鼓膜形态异常38.0%(27/71),听力异常35.2%(25/71),其中传导性听力损失17例,感音神经性听力损失6例,混合性听力损失2例;其余46例患者听力正常,但其中6例耳声发射异常。咽鼓管功能评估发现咽鼓管功能不良的比例为38%(27/71)。听力下降及咽鼓管功能异常与核型无明显相关性(χ^(2)值分别为2.83和2.84,P值分别为0.418和0.417),但与是否伴随其�Objective To analyze the incidence and risk factors of otologic disorders in patients with Turner syndrome(TS),so as to provide management strategies for ear health.Methods This study is a prospective study based on questionnaires and a cross-sectional study.The TS patients who visited our hospital from 2010 January to 2021 March were included(A total of 71 patients with TS were included in this study.the age of TS diagnosed was 3-to 11-year-old,age of visiting ENT department was 4-to 27-year-old)and the incidence of otologic diseases in different age groups was investigated by questionnaires.The cross-sectional study included ear morphology and auditory function assessment,and further analysis of the risk factors that related to ear disease.Prism was used for data analysis.Results The investigation found that the incidence of acute otitis media in patients aged 3-6 and 7-12 years was higher than that of patients over 12 years old,which was 33.8%(24/71),42.9%(30/70)and 23.5%(8/34),respectively;21.1%(15/71)of patients were recurrent acute otitis media in patients aged 3-6 years,and about 46.6%(7/15)of them persisted beyond 6-year.The prevalence of otitis media with effusion in the three groups was 32.4%(23/71),34.3%(24/70)and 38.2%(13/34),respectively;the recurrence rate of tympanocentesis was 100%(7/7),42.9%(3/7)and 50.0%(1/2),which was significantly higher than that of grommet insertion.For age groups of 3-6 and 7-12 years,the prevalence of acute otitis media and secretory otitis media was lower in the X chromosome structure abnormal patients;while for patients older than 12 years,otitis media with effusion was the highest prevalence in Y-chromosome-containing karyotypes.In addition,the prevalence of acute otitis media and otitis media with effusion in patients with other system diseases were increased significantly.A cross-sectional study found that 7.0%(5/71)of the lower auricular,4.2%(3/71)of the external auditory canal narrow,and 38.0%(27/71)of the tympanic membrane abnormality.35.2%(25/71)had abnormal heari

关 键 词：特纳综合征 中耳炎 听力 

分 类 号：R764[医药卫生—耳鼻咽喉科]