Potential treatments and interventions of HHL (Lu et al., 2023)

<b>Purpose: </b>The purpose of this study was to systematically review the research literature with regards to treatments and intervention methods for hereditary hearing loss. Our goal was to provide reference guidelines for the rational use of medication and gene-targeted therapy for patients with hereditary hearing loss and discuss the future development of research in this area.<b>Method:</b> We searched two core databases, PubMed and Web of Science, for relevant literature relating to potential treatments and interventional methods for hereditary hearing loss. Then, we used Microsoft Excel to perform basic statistical analysis of the data, the R language to perform bibliometric analyses, and VOSviewer and CiteSpace to visualize data. In addition, we clustered and descriptively analyzed the data and identified the relative importance of each approach with regard to precise patient outcomes.<b>Results:</b> In this study, we followed the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) standardized screening process and identified a total of 103 research articles. The average annual growth rate of publications in this area was 12.73%. The country with the highest number of publications and citations was the United States; 80 of these publications (associated with 76.92% of funding) were supported by grants from 16 countries. Potential treatments and interventions were clustered according to the stage of research and showed that 8.74% remain in the research design stage, 59.22% are in the clinical validation stage, and 32.04% are being applied in the clinic. The main research focus in this field is cochlear implants and gene therapy.<b>Conclusions:</b> Hereditary hearing loss is in a critical period of transition from preventive to therapeutic research. Gene-targeted interventions represent one of the most promising and effective treatments.<b>Supplemental Material S1.</b> Search formulas and search time frames for each database.<b>Supplemental Material S2.</b> Ranking of authors with &gt; 4 publications and articles fractionalized.<b>Supplemental Material S3.</b> Top 10 countries with average articles cited.Lu, Y., Hu, Y., Wang, S., Pan, S., An, K., Wang, T., He, Y., Tian, C., &amp; Lei, J. (2023). Hereditary hearing loss: A systematic review of potential treatments and interventions. <i>American Journal of Audiology</i>. Advance online publication. https://doi.org/10.1044/2023_AJA-23-00069

**研究目的**：本研究旨在系统梳理遗传性听力损失的治疗与干预方法相关研究文献，以期为遗传性听力损失患者合理用药及靶向基因治疗提供参考规范，并探讨该领域未来研究的发展方向。
**方法**：本研究检索PubMed、Web of Science两大核心数据库中与遗传性听力损失潜在治疗及干预方法相关的文献。随后采用Microsoft Excel对数据进行基础统计分析，使用R语言开展文献计量分析，并借助VOSviewer与CiteSpace实现数据可视化。此外，本研究对数据进行聚类与描述性分析，明确各干预手段针对精准患者预后的相对重要性。
**结果**：本研究遵循系统评价与Meta分析优先报告条目（Preferred Reporting Items for Systematic Reviews and Meta-Analyses, PRISMA）的标准化筛选流程，最终纳入103篇研究文献。该领域文献的年均增长率为12.73%。发表文献与被引频次最高的国家为美国；其中80篇文献（占总文献量的76.92%）获得了16个国家的科研基金资助。本研究按研究阶段对潜在治疗与干预手段进行聚类分析，结果显示8.74%的研究仍处于研究设计阶段，59.22%处于临床验证阶段，32.04%已应用于临床。该领域的主要研究热点为人工耳蜗植入术与基因治疗。
**结论**：遗传性听力损失研究正处于从预防研究向治疗研究转型的关键时期。靶向基因干预是目前最具前景与有效性的治疗手段之一。
**补充材料S1**：各数据库的检索式与检索时限。
**补充材料S2**：发表文献≥4篇的作者排名及文章分数拆分情况。
**补充材料S3**：篇均被引频次前10的国家。
Lu Y、Hu Y、Wang S、Pan S、An K、Wang T、He Y、Tian C、Lei J.（2023）. 遗传性听力损失：潜在治疗与干预手段的系统评价. *美国听力学杂志（American Journal of Audiology）*. 在线优先出版. https://doi.org/10.1044/2023_AJA-23-00069