Department of Biomedical Engineering, The City College of New York, New York, NY, USA.
Department of Otolaryngology, Northwestern University Feinberg School of Medicine, Chicago, IL, USA.
Sci Rep. 2022 Oct 12;12(1):17125. doi: 10.1038/s41598-022-20169-9.
About 70% of people with osteogenesis imperfecta (OI) experience hearing loss. There is no cure for OI, and therapies to ameliorate hearing loss rely on conventional treatments for auditory impairments in the general population. The success rate of these treatments in the OI population with poor collagenous tissues is still unclear. Here, we conduct a systematic review and meta-analysis on the efficacy of treatments addressing hearing loss in OI. This study conforms to the reporting standards of the Preferred Reporting Items for Systematic Reviews and Meta-analyses (PRISMA). Data sources include published articles in Medline via PubMed, Web of Science, Scopus, and Embase, from their inception to November 2020. Studies included individuals with OI undergoing a hearing loss treatment, having pre- and postoperative objective assessment of hearing function at a specified follow-up length. Our search identified 1144 articles, of which 67 were reviewed at full-text screening. A random-effects meta-analysis was conducted on the selected articles (n = 12) of people with OI that underwent stapes surgery. Success was assessed as the proportion of ears with a postoperative Air-Bone Gap (ABG) ≤ 10 dB. A systematic review was conducted on the remaining articles (n = 13) reporting on other treatments. No meta-analysis was conducted on the latter due to the low number of articles on the topic and the nature of single case studies. The meta-analysis shows that stapes surgeries have a low success rate of 59.08 (95% CI 45.87 to 71.66) in the OI population. The systematic review revealed that cochlear implants, bone-anchored hearing aids, and other implantable hearing aids proved to be feasible, although challenging, in the OI population, with only 2 unsuccessful cases among the 16 reviewed single cases. This analysis of published data on OI shows poor clinical outcomes for the procedures addressing hearing loss. Further studies on hearing loss treatments for OI people are needed. Notably, the mechanisms of hearing loss in OI need to be determined to develop successful and possibly non-invasive treatment strategies.
约 70%的成骨不全症（OI）患者会出现听力损失。OI 目前尚无治愈方法，改善听力损失的疗法依赖于普通人群中针对听觉损伤的常规治疗。这些治疗方法在胶原组织较差的 OI 人群中的成功率尚不清楚。在此，我们对针对 OI 听力损失的治疗方法进行了系统评价和荟萃分析。本研究符合系统评价和荟萃分析的首选报告项目（PRISMA）的报告标准。数据来源包括通过 PubMed、Web of Science、Scopus 和 Embase 发布的文章，从成立到 2020 年 11 月。研究包括接受听力损失治疗的 OI 患者，在特定随访长度下对听力功能进行术前和术后客观评估。我们的检索共确定了 1144 篇文章，其中有 67 篇在全文筛选中进行了回顾。对 12 篇关于接受镫骨手术的 OI 患者的选定文章进行了随机效应荟萃分析。成功的评估标准是术后气骨导差（ABG）≤10 dB 的耳朵比例。对其余 13 篇报告其他治疗方法的文章进行了系统评价。由于关于该主题的文章数量较少且性质为单病例研究，因此未对后者进行荟萃分析。荟萃分析表明，镫骨手术在 OI 人群中的成功率较低，为 59.08%（95%CI 45.87-71.66）。系统评价显示，人工耳蜗、骨锚式助听器和其他植入式助听器在 OI 人群中被证明是可行的，尽管具有挑战性，但在综述的 16 个单病例中仅有 2 例失败。对 OI 听力损失治疗的已发表数据进行的分析表明，针对听力损失的手术程序的临床效果不佳。需要进一步研究 OI 患者的听力损失治疗方法。值得注意的是，需要确定 OI 听力损失的机制，以开发成功且可能非侵入性的治疗策略。
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