Department of Otorhinolaryngology, Hospital Universitario San Cecilio, Instituto de Investigación Biosanitaria de Granada-ibs. GRANADA, Granada, Spain.
Otology & Neurotology Group CTS495, Department of Genomic Medicine - Centro de Genómica e Investigación Oncológica - Pfizer/Universidad de Granada/Junta de Andalucía (Genyo), PTS, Granada, Spain.
Semin Neurol. 2020 Feb;40(1):138-150. doi: 10.1055/s-0039-3402065. Epub 2019 Dec 30.
Meniere's disease (MD) is a set of uncommon disorders with core phenotype of tinnitus, episodic vertigo, and sensorineural hearing loss. MD shows a genetic predisposition and a family history is found in 10% cases, with an autosomal dominant inheritance pattern. It is a multifactorial condition whose onset and development are triggered by the combined effect of genetic and environmental factors. Histopathological studies have associated MD with the accumulation of endolymph in the cochlea and the vestibular organs. However, endolymphatic hydrops does not fully explain the persistence of tinnitus, hearing loss progression, or the frequency of vertigo attacks.There are several comorbidities associated with MD, such as migraine, anxiety, autoimmune, and autoinflammatory disorders, adding more complexity to the phenotype. This "extended phenotype" can make the diagnosis and clinical management more complex, but it could also lead to a better characterization, understanding, and treatment of MD patients.We have conducted a systematic review on MD to update current knowledge, focusing on its mechanisms, diagnosis, comorbidities, and practical management.
梅尼埃病（MD）是一组罕见疾病，其核心表型为耳鸣、阵发性眩晕和感音神经性听力损失。MD 显示出遗传易感性，有 10%的病例存在家族史，呈常染色体显性遗传模式。它是一种多因素疾病，其发病和发展是由遗传和环境因素的综合作用引发的。组织病理学研究将 MD 与内耳和前庭器官中的内淋巴积聚联系起来。然而，内淋巴积水并不能完全解释耳鸣的持续存在、听力损失的进展或眩晕发作的频率。MD 与多种合并症相关，如偏头痛、焦虑症、自身免疫和自身炎症性疾病，这为表型增加了更多的复杂性。这种“扩展表型”可以使 MD 的诊断和临床管理更加复杂，但也可以促进对 MD 患者更好地特征描述、理解和治疗。我们对 MD 进行了系统综述，以更新当前的知识，重点关注其发病机制、诊断、合并症和实际管理。
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