Bois E, Nassar M, Zenaty D, Léger J, Van Den Abbeele T, Teissier N
Service d'ORL pédiatrique, hôpital Robert-Debré, Paris, université Diderot Paris 7, AP-HP, 48, boulevard Sérurier, 75019 Paris, France.
Service d'ORL pédiatrique, hôpital Robert-Debré, Paris, université Diderot Paris 7, AP-HP, 48, boulevard Sérurier, 75019 Paris, France.
Eur Ann Otorhinolaryngol Head Neck Dis. 2018 Feb;135(1):21-24. doi: 10.1016/j.anorl.2017.08.006. Epub 2017 Sep 21.
Patients with Turner syndrome (TS) have craniofacial malformations, such as Eustachian tube hypoplasia and dysfunction and velar dysfunction, which foster acute otitis media. The aim of this study was to inventory pediatric otologic disorders in patients with TS at their first ENT consultation in our center.
We reviewed the ENT consultation data of pediatric TS patients followed in our center between 2005 and 2015: otoscopy, hearing threshold, and history of acute otitis media or ENT surgery. Data were compared according to karyotype: X monosomy (45,X), mosaic (45,X/46,XX), isochromosome (46,Xi [Xq]), X ring chromosome X (XrX), with Y material, and "other".
Ninety patients, with mean age 11.9years (±4.8years) at first ENT consultation, were included: 29% showed tympanic abnormality on otoscopy, 21% had hearing loss, 24% had history of recurrent acute otitis media; 18% had undergone adenoidectomy, 24% T-tube insertion, and 5.6% tympanoplasty. No particular karyotype was associated with higher risk of hearing loss or acute otitis media.
Patients with TS showed high prevalence of pediatric otologic disorders; they therefore require close and prolonged ENT follow-up.
特纳综合征（TS）患者存在颅面畸形，如咽鼓管发育不全及功能障碍和腭功能障碍，这些会引发急性中耳炎。本研究的目的是梳理在我们中心首次接受耳鼻喉科会诊的TS患者的小儿耳科疾病情况。
我们回顾了2005年至2015年在我们中心随访的小儿TS患者的耳鼻喉科会诊数据：耳镜检查、听力阈值以及急性中耳炎或耳鼻喉科手术史。根据核型对数据进行比较：X单体型（45,X）、嵌合体（45,X/46,XX）、等臂染色体（46,Xi [Xq]）、X环状染色体X（XrX）、含Y物质以及“其他”。
纳入了90例患者，首次耳鼻喉科会诊时的平均年龄为11.9岁（±4.8岁）：29%的患者耳镜检查显示鼓膜异常，21%有听力损失，24%有复发性急性中耳炎病史；18%接受了腺样体切除术，24%进行了T型管置入，5.6%进行了鼓膜成形术。没有特定核型与更高的听力损失或急性中耳炎风险相关。
TS患者小儿耳科疾病的患病率较高；因此他们需要密切且长期的耳鼻喉科随访。
