自身免疫性内耳疾病：当前知识现状

文献信息

中文摘要

背景/目的： 越来越多的感音神经性听力损失病例由耳蜗内的自身免疫性炎症反应引起，这些反应可局限于内耳，也可与其他系统性自身免疫反应相关。本研究的目的是对过去五年的全球文献进行叙述性综述，总结自身免疫性听力损失的当前知识现状，并了解其病理学和治疗策略。作为一篇叙述性综述，本文旨在描述最有意义且最新的病例报告，这些报告增进了我们对自身免疫性内耳疾病的认识。方法：我们使用关键词“自身免疫性内耳疾病”、“自身免疫性感音神经性听力损失”和“突发性感音神经性听力损失”检索了Scopus、Web of Science和PubMed数据库。筛选范围涵盖过去五年内发表的论文。结果：根据筛选标准，我们确定了122篇相关文章。进一步分析并限定于综述的主要范围后，最终筛选出48篇论文，构成本综述的基础。结论：自身免疫性听力损失似乎是一个日益增加的临床问题。其潜在病理可局限于耳部；然而，它也可能发生在其他自身免疫过程的过程中，并在不同系统和器官中表现出多症状表现。其潜在病理是对抗原（包括位于患者身体组织中的自身抗原）的免疫介导炎症反应。这种病理导致各种功能障碍。诊断具有挑战性，因为尚无特定的诊断标准。炎症标志物通常升高；然而，它们不够特异，无法确定最终诊断。治疗策略因症状而异；然而，免疫抑制药物，主要是皮质类固醇（全身或鼓室内给药），似乎是首选治疗方法。

英文摘要

Background/Objectives: An increasing number of cases of sensorineural hearing loss are caused by autoimmune inflammatory reactions within the cochlea, which can either be limited to the inner ear or associated with other systemic autoimmune reactions. The objective of this work was to conduct a narrative review of the worldwide literature from the past five years, to summarize the current state of knowledge on autoimmune hearing loss, and to understand its pathology and its treatment strategies. As a narrative review, our publication aimed to describe the most interesting and up-to-date case reports that add to our knowledge of autoimmune inner ear conditions. Methods: We searched the Scopus, Web of Science, and PubMed databases using the key phrases "autoimmune inner ear disease", "autoimmune sensorineural hearing loss", and "sudden sensorineural hearing loss". The selection covered papers published within the past five years. Results: Based on the selection criteria, we identified 122 relevant articles. Further analysis and limitation to the main scope of the review led to the final selection of 48 papers, which formed the basis for this review. Conclusions: Autoimmune hearing loss appears to be an increasing clinical problem. The underlying pathology can be limited to the ear; however, it may also occur during the course of other autoimmune processes with multisymptomatic manifestations in different systems and organs. The underlying pathology is an immune-mediated inflammatory response to antigens, including self-antigens located in a patient's body tissues. This pathology leads to various dysfunctions. Diagnosis is challenging, as there are no specific diagnostic criteria. Inflammatory markers are usually elevated; however, they are not specific enough to determine a final diagnosis. Treatment strategies differ, depending on the symptoms; however, immunosuppressive drugs, mainly corticosteroids (systemic or trans tympanic), appear to be a treatment of choice.