Chelsea Cleveland, Jamil Hayden, Tekin Baglam, Todd Otteson
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Patients in this group with diagnosis of sensorineural, conductive, or mixed hearing loss were recorded. Patients with diagnoses of congenital cytomegalovirus, congenital inner ear malformations, and noise-induced hearing loss were excluded from analysis.</p></div><div><h3>Results</h3><p>Out of 3256 patients 18 years old or younger with OI, 10.07 % (95 % CI: 9.06–11.16) had a history of any form of hearing loss, 5.71 % (95 % CI: 4.94–6.57) had conductive hearing loss, 3.01 % (95 % CI: 2.45–3.66) had sensorineural hearing loss, and 1.35 % (95 % CI: 0.98–1.81) had mixed hearing loss. Relative risks for diagnosis of any type of hearing loss, conductive hearing loss, sensorineural hearing loss, and mixed hearing loss were calculated: 5.90 (95 % CI 5.32–6.53), 5.08 (95 % CI 4.42–5.84), 6.18 (95 % CI 5.09–7.51), and 13.86 (95 % CI 10.33–18.59) respectively.</p></div><div><h3>Discussion</h3><p>This study is the largest to date that describes a relationship between OI and conductive, sensorineural, and mixed hearing loss. Pediatric patients with OI are almost five times as likely to have any type of hearing loss. There was a significant increased risk in each subgroup, but conductive hearing loss was the most common for hearing loss in children with OI. 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引用次数: 0
摘要
导言成骨不全症（OI）是一种影响 1 型胶原蛋白的常见遗传性疾病。成骨不全症的后遗症多种多样，但听力损失是一种重要的并发症，46%-58%的患者会出现不同程度的听力损失。以往的研究表明，OI 患者可能患有传导性、感音神经性或混合性听力损失。这些研究主要集中在成人人群中。方法在 TriNetx Analytics Network（一个联合健康研究网络，汇集了全美 7800 多万名患者的去标识化电子健康记录数据）中查询了 18 岁或以下诊断为 OI 的患者。这组患者中被诊断为感音神经性听力损失、传导性听力损失或混合性听力损失的患者被记录在案。结果在 3256 名 18 岁及以下的 OI 患者中，10.07%（95% CI：9.06-11.16）有 OI 病史。在 3256 名 18 岁及以下的 OI 患者中，10.07%（95% CI：9.06-11.16）有任何形式的听力损失病史，5.71%（95% CI：4.94-6.57）有传导性听力损失，3.01%（95% CI：2.45-3.66）有感音神经性听力损失，1.35%（95% CI：0.98-1.81）有混合性听力损失。计算了诊断出任何类型听力损失、传导性听力损失、感音神经性听力损失和混合性听力损失的相对风险：本研究是迄今为止描述 OI 与传导性听力损失、感音神经性听力损失和混合性听力损失之间关系的最大规模研究。患有 OI 的小儿患者出现任何类型听力损失的几率几乎是 OI 患者的五倍。每个亚组的风险都明显增加，但传导性听力损失是患有开放性损伤的儿童中最常见的听力损失。与对照组相比，风险最高的亚型是混合性听力损失。
Characterization of hearing loss in pediatric patients with osteogenesis imperfecta
Introduction
Osteogenesis imperfecta (OI) is a common heritable disorder affecting type 1 collagen. The sequelae of OI vary, but hearing loss is a significant complication with 46–58 % of patients having some degree of hearing loss. Previous studies have suggested patients with OI may have conductive, sensorineural, or mixed hearing loss. Majority of these studies focus on the adult population.
Objectives
Identify a relationship between OI and hearing loss in the pediatric population.
Methods
The TriNetx Analytics Network, a federated health research network that aggregates the de-identified electronic health record data of over 78 million patients across the United States, was queried for patients 18 years old or younger with a diagnosis of OI. Patients in this group with diagnosis of sensorineural, conductive, or mixed hearing loss were recorded. Patients with diagnoses of congenital cytomegalovirus, congenital inner ear malformations, and noise-induced hearing loss were excluded from analysis.
Results
Out of 3256 patients 18 years old or younger with OI, 10.07 % (95 % CI: 9.06–11.16) had a history of any form of hearing loss, 5.71 % (95 % CI: 4.94–6.57) had conductive hearing loss, 3.01 % (95 % CI: 2.45–3.66) had sensorineural hearing loss, and 1.35 % (95 % CI: 0.98–1.81) had mixed hearing loss. Relative risks for diagnosis of any type of hearing loss, conductive hearing loss, sensorineural hearing loss, and mixed hearing loss were calculated: 5.90 (95 % CI 5.32–6.53), 5.08 (95 % CI 4.42–5.84), 6.18 (95 % CI 5.09–7.51), and 13.86 (95 % CI 10.33–18.59) respectively.
Discussion
This study is the largest to date that describes a relationship between OI and conductive, sensorineural, and mixed hearing loss. Pediatric patients with OI are almost five times as likely to have any type of hearing loss. There was a significant increased risk in each subgroup, but conductive hearing loss was the most common for hearing loss in children with OI. The highest risk subtype when compared to controls was mixed hearing loss.
期刊介绍：
Accounts of Chemical Research presents short, concise and critical articles offering easy-to-read overviews of basic research and applications in all areas of chemistry and biochemistry. These short reviews focus on research from the author’s own laboratory and are designed to teach the reader about a research project. In addition, Accounts of Chemical Research publishes commentaries that give an informed opinion on a current research problem. Special Issues online are devoted to a single topic of unusual activity and significance.
Accounts of Chemical Research replaces the traditional article abstract with an article "Conspectus." These entries synopsize the research affording the reader a closer look at the content and significance of an article. Through this provision of a more detailed description of the article contents, the Conspectus enhances the article's discoverability by search engines and the exposure for the research.
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