Audiological Findings in Osteogenesis Imperfecta
Audiological Findings in Osteogenesis Imperfecta
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DOI：
10.3766/jaaa.19.8.3
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发表时间：
2008-09-01
影响因子：
1.2
通讯作者：
Shapiro, Jay
中科院分区：
文献类型：
--
作者：
Pillion, Josepb P.;Shapiro, Jay
背景：成骨不全症（OI）是一种遗传性骨和结缔组织疾病，与轻微创伤后终生频繁发生骨折有关。成骨不全患者经常报告听力损失。目的：本研究是一项回顾性研究，对按年龄分组的成骨不全患者样本进行鼓室导抗测试、声反射和瞬态诱发耳声发射测量。本研究的目的是检查 OI 类型、年龄和 OI 个体样本中听力学结果之间的关系。 研究设计：该研究检查了听力测量、所有类型的听力损失和 OI 类型之间的相关性。 研究样本：该研究纳入了 41 名 OI 患者。将患者分为两组进行分析：一组年龄小于 20 岁（n = 21），另一组年龄在 20 岁及以上（n - 20）。结果：62% 的耳朵出现各种病因的听力损失。 41% 的耳朵出现感音神经性或混合性听力损失，21% 的耳朵出现传导性听力损失。结果表明，与同龄儿童相比，年轻的成骨不全患者出现与中耳炎相关的中耳功能障碍的风险更大。与听骨功能障碍相关的鼓室导抗异常在老年患者中更常见。所有类型的听力损失在老年患者组 (88%) 中比年轻患者组 (38%) 更为普遍。结论：无论年龄如何，听力损失并不少见。因此，建议对各个年龄段的成骨不全患者进行密切的听力学监测。
Background: Osteogenesis imperfecta (OI) is an inherited bone and connective tissue disorder associated with the lifelong occurrence of frequent fractures following even mild trauma. Hearing loss is frequently reported in patients with OI.Purpose: This investigation is a retrospective study of measurements of tympanometry, acoustic reflexes, and transient evoked otoacoustic emissions in a sample of patients with OI grouped according to age. The purpose of the study was to examine the relationship between the type of OI, age, and audiological findings in a sample of individuals with OI.Research Design: The study examined the correlation between audiometric measures, hearing loss of all types, and type of OI.Study Sample: Forty-one patients with OI were included in the study. The patients were divided into two groups for analysis: one group less than 20 years of age (n = 21) and the other group aged 20 and over(n - 20).Results: Hearing loss of all etiologies was observed in 62% of ears. Sensorineural or mixed hearing loss was observed in 41% and conductive hearing loss in 21% of ears. Results indicate that the younger patients with OI were subject to a greater risk of middle ear dysfunction associated with otitis media than is typical for children of comparable age. Tympanometric abnormalities associated with ossicular dysfunction were more often found in the older age group of patients. Hearing loss of all types was more prevalent in the older group of patients (88%) than in the younger patient group (38%).Conclusions: Hearing loss was not uncommon regardless of age. Therefore, close audiological monitoring of patients with OI is recommended across the age spectrum.