在患有 osteogenesis imperfecta 的儿科患者中听力损失的表征
Chelsea Cleveland1, Jamil Hayden1, Tekin Baglam1
1Department of Otolaryngology-Head and Neck Surgery, University Hospitals Rainbow Babies and Children's Hospital, Cleveland, OH, USA.
International journal of pediatric otorhinolaryngology
|July 19, 2024
概括
患有 osteogenesis imperfecta (OI) 的儿童有明显更高的听力损失风险. 这项研究发现,患有OI的儿科患者患有任何形式的听力损失的可能性几乎是五倍,导电性听力损失是最常见的.
科学领域:
- 遗传学和遗传性疾病
- 耳鼻喉科 耳鼻喉科 耳鼻喉科
- 儿科健康 儿科健康
背景情况:
- 骨质发生不完善 (Osteogenesis Imperfecta,简称OI) 是一种影响1型原蛋白的普遍遗传性疾病.
- 听力损失是OI的一个显著并发症,影响了46-58%的患者,之前的研究主要集中在成人身上.
- OI可能导致导电性,神经传感或混合性听力损失.
研究的目的:
- 为了研究骨质发育不完善 (OI) 和儿科患者的听力损失之间的关系.
- 量化不同类型的OI诊断儿童听力损失的患病率和风险.
主要方法:
- 使用了TriNetx分析网络,这是一个大规模的非识别电子健康记录数据库.
- 18岁或更年轻的OI诊断患者的查询数据.
- 排除了先天性细胞大脑病毒,内耳形或噪音引起的听力损失的病例.
主要成果:
- 10.07%的儿科OI患者 (n=3256) 患有某种形式的听力损失.
- 导电性听力损失的患病率为5.71%,神经传感性听力损失为3.01%,混合性听力损失为1.35%.
- 相对风险显示OI患者与对照人群相比,所有听力损失类型的风险显著增加,混合听力损失风险最高 (13.86).
结论:
- 这项研究代表了迄今为止关于OI和儿科听力损失的最大分析.
- 患有OI的儿童患者患有任何听力损失的可能性增加了近五倍.
- 导电性听力损失是OI儿童中最常见的类型,而混合性听力损失具有最高的相对风险.
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