Objective: To explore the otological and hearing characteristics in children with Turner syndrome (TS), to determine risk factors of hearing loss, and to discuss algorithms for future surveillance. Methods: The clinical data of otolaryngology in children with TS from January 2018 to April 2020 were reviewed retrospectively. A total of 46 female children with TS, aged from 5 to 18 years were enrolled. Karyotypes included 17 cases (37.0%) of monosomy, 16 cases (34.7%) of mosaicism, and 13 cases (28.3%) of abnormal X chromosome structures. The otoscopic characteristics, audiological performance, and otologic diagnoses were evaluated. Multivariate logistic regression and Mann-Whitney U test were used for statistical analysis. Results: The most common otologic comorbidity was otitis media with effusion (OME), including 20 cases (43.5%) with 33 ears (35.9%). 14 cases (30.4%) were diagnosed with hearing loss. The regression analysis revealed that the age (OR=1.345, 95%CI: 1.072-1.760) and comorbidity of OME (OR=9.460, 95%CI: 2.065-60.350) were risk factors associated with hearing loss. In TS with OME, when compared with the mean air conduction threshold, the hearing loss of the group with pars flaccida retractions was significantly higher ((24.3±13.8) dB HL vs. (14.4±4.2) dB HL, U=59.500, P=0.008) than that of the group with none retractions. Conclusion: OME and hearing loss are common in children with TS. As age increases or suffers from OME, the risk of hearing loss increases.
目的： 分析儿童特纳综合征（Turner syndrome，TS）的耳科学表现，研究其听力损失的影响因素。 方法： 回顾性分析2018年1月至2020年4月就诊于深圳市儿童医院耳鼻咽喉科的46例儿童TS的临床资料，均为女性患儿，年龄5~18岁。染色体核型X单体型17例（37.0%），嵌合型16例（34.7%），X染色体结构异常13例（28.3%）。并对耳内镜、听力结果和耳科诊治情况进行分析。应用多因素Logistic回归和Mann-Whitney U检验等方法进行统计学分析。 结果： 46例患儿中最常见的耳科疾病是分泌性中耳炎（otitis media with effusion，OME），共20例（43.5%）33耳（35.9%），听力损失者14例（30.4%），多因素Logistic回归分析显示年龄（OR=1.345，95%CI：1.072~1.760）和患有OME（OR=9.460，95%CI：2.065~60.350）是听力损失的独立影响因素，OME患儿的语言频率气导听阈在鼓膜松弛部凹陷与非凹陷的病例之间比较差异有统计学意义［（24.3±13.8）dB HL比（14.4±4.2）dB HL，U=59.500，P=0.008］。 结论： OME和听力损失在儿童TS中常见，年龄增加或患有OME时，出现听力损失的风险增加。.