巴西耳科学会与巴西神经耳科学会工作组：梅尼埃病

文献信息

中文摘要

目的： 批判性回顾关于梅尼埃病病理生理学和诊断的现有证据，并提供由巴西耳科学会与巴西神经耳科学会工作组制定的基于共识的建议。
方法： 成立了一个由耳科学和神经耳科学专家组成的工作组，负责审查科学文献并制定文件的主题章节。内容通过面对面和远程会议进行讨论，直至达成共识。建议根据证据水平和推荐强度，使用标准化的治疗干预和诊断方法评估系统进行分级。
结果： 梅尼埃病是一种异质性临床疾病，其特征为反复发作的眩晕、波动性感音神经性听力损失、耳鸣和耳闷胀感。内淋巴积水是最一致的病理组织学发现；然而，它本身并不能解释该疾病的临床表现。当前证据表明涉及多因素机制，包括遗传易感性、免疫功能障碍、炎症过程以及内耳离子稳态的改变。诊断仍主要基于临床，依据国际标准，系列听力测试起着关键作用。电生理测试和影像学在特定情况下可能有用。临床变异性支持亚表型的概念，具有潜在的诊断和预后意义。
结论： 梅尼埃病应被视为一种异质性、多因素的临床实体。诊断需要仔细的临床评估和纵向随访。对病理生理机制理解的进展以及亚表型的识别可能有助于更个体化的诊断和治疗策略。

英文摘要

OBJECTIVE: To critically review the available evidence on the pathophysiology and diagnosis of Menière's disease and to provide consensus-based recommendations developed by a task force of the Brazilian Society of Otology and the Brazilian Academy of Neurotology.
METHODS: A task force consisting of experts in otology and neurotology was established to review the scientific literature and develop thematic sections of the document. The content was discussed in both in-person and remote meetings until consensus was achieved. Recommendations were graded according to level of evidence and strength of recommendation using standardized systems for the evaluation of therapeutic interventions and diagnostic methods.
RESULTS: Menière's disease is a heterogeneous clinical condition characterized by recurrent episodes of vertigo, fluctuating sensorineural hearing loss, tinnitus, and aural fullness. Endolymphatic hydrops represents the most consistent histopathologic finding; however, it does not alone explain the clinical expression of the disease. Current evidence indicates the involvement of multifactorial mechanisms, including genetic predisposition, immune dysfunction, inflammatory processes, and changes in inner ear ion homeostasis. Diagnosis remains primarily clinical, based on international criteria, with serial audiometry playing a key role. Electrophysiologic testing and imaging may be useful in selected situations. Clinical variability supports the concept of subphenotypes with potential diagnostic and prognostic implications.
CONCLUSION: Menière's disease should be recognized as a heterogeneous and multifactorial clinical entity. Diagnosis requires careful clinical evaluation and longitudinal follow-up. Advances in the understanding of pathophysiologic mechanisms and the identification of subphenotypes may contribute to more individualized diagnostic and therapeutic strategies.