梅尼爾氏症之病理機轉到治療

Pathophysiology and Management of Ménière’s Disease

梅尼爾氏症（Ménière’s disease , MD）又稱作美尼爾氏症，是一種內耳疾患，病患會有陣發性眩暈、波動性聽損及耳嗚、耳脹等症狀。其典型的病理變化為內耳內淋巴水腫（endolymphatic hydrops, EH），及內耳毛細胞和神經元受損；然而，梅尼爾氏症的詳細致病機轉尚不明確。目前診斷標準以臨床症狀為主，缺乏確定診斷的客觀性檢查，內耳功能檢查及磁振造影檢查（magnetic resonance imaging , MRI）檢查仍是輔助的角色，多用於鑑別診斷（如突發性耳聾及前庭性偏頭痛）。治療上，控制梅尼爾氏症急性發作及預防復發為首要目標，多可靠藥物治療達成，少數頑固性眩暈需要介入性治療，如鼓室內注射，或是手術以抑制眩暈。本文從歷史淵源開始，介紹梅尼爾氏症的流行病學、症狀、機轉、診斷及治療，期望能讓臨床醫師更認識這個常見疾病。

Ménière’s disease (MD) is a disorder of the inner ear that causes episodic vertigo, fluctuating hearing loss, tinnitus and aural fullness. Although the pathognomonic change of MD is endolymphatic hydrops (EH) with damage of the hair cells and neurons in the inner ear, the pathogenic mechanism of MD is still unclear. Currently, the diagnosis of MD relies mainly on clinical symptoms, as objective examinations are insufficient to confirm the diagnosis. Laboratory tests and magnetic resonance imaging (MRI) usually serve as supporting tools. They are mostly helpful in differentiating MD with other inner ear disorders (e.g., sudden deafness, vestibular migraine). The therapeutic goal of MD includes control of acute attack and prevention of recurrences. It can usually be achieved by medical therapy, while few intractable cases may need interventions such as intratympanic injection or surgery. In this article, we introduce the historical origin, epidemiology, clinical manifestations, pathophysiology, diagnosis and treatment of MD, with the aim of acquainting healthcare professionals with this fairly prevalent illness.