双膦酸盐治疗对儿童成骨不全症听力和中耳功能障碍的影响
The Effect of Bisphosphonate Therapy on Hearing and Middle Ear Dysfunction in Pediatric Osteogenesis Imperfecta.
文献信息
| PMID | 42763553 |
|---|---|
| 原文 | 在 PubMed 查看原文 ↗ |
| 发表日期 | 2026 |
| 作者 | Kamron Zand |
| 作者单位 | Creighton University School of Medicine, Omaha, NE. |
| 期刊 | Journal of the American Academy of Audiology |
| SCI 分区 | Q3 |
| IF | 1.6 |
| 研究类型 | 临床研究 · 临床 |
| 所属专科 | 耳科 |
中文摘要
背景： 成骨不全症（OI）是一种异质性遗传性结缔组织疾病，其特征是骨基质形成受损。在28%至65%的OI患者中可观察到听力损失。大多数OI患者出现传导性听力损失（CHL）；然而，混合性和感音神经性（SNHL）听力损失也有描述。
目的： 本回顾性研究的主要目的是描述儿童OI中中耳功能障碍（MED）和听力损失（HL）的发生率，并探讨双膦酸盐治疗对HL的疗效。
研究设计： 对儿童OI患者的回顾性病历审查。
研究样本： 纳入2000年1月至2021年1月期间接受治疗的儿童OI患者。HL根据纯音测听定义，而MED通过226 Hz鼓室导抗图确定。患者按OI类型分类，如果在测试前1年内开始使用双膦酸盐，则被认为正在使用双膦酸盐。
干预： 未进行直接干预。探讨了使用双膦酸盐与未使用双膦酸盐之间的比较。
数据收集与分析： 对分类数据采用卡方检验，以探讨是否存在与二乘二变量的显著关联。
结果： 本研究纳入144例患者。在这些患者中，12.5%有HL。鼓室导抗图显示25%有MED。此外，82.6%目前正在使用双膦酸盐。在所有OI类型中，未观察到当前使用双膦酸盐与HL或MED之间存在关联。然而，对I型OI患者的亚组分析表明，无MED与当前双膦酸盐治疗在统计学上相关（p = 0.025）。
结论： 双膦酸盐与儿童I型OI患者无MED相关。由于其病程较轻，双膦酸盐通常不用于许多I型OI患者。通过减少MED及其相关合并症，我们的结果表明双膦酸盐的使用可能带来显著的治疗获益。
临床相关性声明： HL在OI个体中很常见。我们的研究表明，在较早年龄开始使用双膦酸盐可能延缓导致HL的中耳变化。
英文摘要
BACKGROUND: Osteogenesis imperfecta (OI) is a heterogeneous inherited connective tissue disorder characterized by impaired bone matrix formation. Hearing loss is observed in 28 to 65 percent of patients with OI. Most patients with OI experience conductive hearing loss (CHL); however, mixed and sensorineural (SNHL) hearing loss has also been described.
PURPOSE: The primary aim of this retrospective study was to characterize the rates of middle ear dysfunction (MED) and hearing loss (HL) with pediatric OI and investigate the efficacy of bisphosphonate therapy in HL.
RESEARCH DESIGN: Retrospective chart review of pediatric patients with OI.
STUDY SAMPLE: Pediatric patients with OI treated between January 2000 and January 2021 were included. HL was defined according to pure tone audiometry, while MED was determined by tympanometry at 226 Hz. Patients were categorized by the OI type and considered to be using bisphosphonates if they had been initiated within 1 year prior to testing.
INTERVENTION: No direct intervention was performed. Comparisons between bisphosphate use and no bisphosphonate use were explored.
DATA COLLECTION AND ANALYSIS: The chi-square test was utilized for categorical data to investigate whether a significant association with two-by-two variables existed.
RESULTS: This study included 144 patients. Of these patients, 12.5 percent had HL. Tympanometry revealed that 25 percent had MED. Additionally, 82.6 percent were currently using bisphosphonates. Across all OI types, no association between current bisphosphonates and HL or MED was observed. However, a subanalysis of patients with type I OI indicated that the absence of MED was statistically associated with current bisphosphonate therapy (p = 0.025).
CONCLUSION: Bisphosphonates are associated with the absence of MED in pediatric patients with type I OI. Because of its mild disease course, bisphosphonates are not commonly used for many patients with type I OI. By reducing MED and its associated comorbidities, our results suggest that bisphosphonate use may confer a significant therapeutic benefit.
CLINICAL RELEVANCE STATEMENT: HL is common in individuals with OI. Our study suggests that initiating bisphosphates at an earlier age may delay middle ear changes that contribute to HL.