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<p>Since Prosper Ménière’s original description more than 160&#xa0;years ago, concepts of Ménière’s disease (MD) pathophysiology and treatment have evolved through distinct phases. For much of the twentieth century, the field was dominated by the hydrodynamic paradigm, which attributed symptoms to pressure-driven endolymphatic hydrops and episodic membrane rupture. Over the past three decades, however, accumulating pathological, physiological, genetic, and imaging evidence has undermined this model. This review traces the transition from the classic hydrodynamic era to a&#xa0;modern framework that conceptualizes MD as a&#xa0;disorder characterized by endolymphatic sac (ELS) failure, leading to impaired inner-ear homeostasis. Recent temporal bone studies demonstrate that endolymphatic hydrops reflects proliferative epithelial growth rather than pressure-induced distension, while systematic analyses identify two major disease endotypes—hypoplastic and degenerative—defined by distinct endolymphatic sac pathologies, which are associated with differing clinical phenotypes. Advances in magnetic resonance imaging and computed tomography now allow for in vivo visualization of hydrops and ELS-related anatomy, enabling endotype stratification in living patients. Together, these developments support a&#xa0;new disease model analogous to renal insufficiency, with important implications for mechanistic research, animal modeling, diagnostic criteria, and personalized clinical management of MD.</p>

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Evolution of understanding and clinical practice of Ménière’s disease

  • 期刊论文

  • 发表日期：

    2026年6月2日

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  • Steven D. Rauch

摘要

Since Prosper Ménière’s original description more than 160 years ago, concepts of Ménière’s disease (MD) pathophysiology and treatment have evolved through distinct phases. For much of the twentieth century, the field was dominated by the hydrodynamic paradigm, which attributed symptoms to pressure-driven endolymphatic hydrops and episodic membrane rupture. Over the past three decades, however, accumulating pathological, physiological, genetic, and imaging evidence has undermined this model. This review traces the transition from the classic hydrodynamic era to a modern framework that conceptualizes MD as a disorder characterized by endolymphatic sac (ELS) failure, leading to impaired inner-ear homeostasis. Recent temporal bone studies demonstrate that endolymphatic hydrops reflects proliferative epithelial growth rather than pressure-induced distension, while systematic analyses identify two major disease endotypes—hypoplastic and degenerative—defined by distinct endolymphatic sac pathologies, which are associated with differing clinical phenotypes. Advances in magnetic resonance imaging and computed tomography now allow for in vivo visualization of hydrops and ELS-related anatomy, enabling endotype stratification in living patients. Together, these developments support a new disease model analogous to renal insufficiency, with important implications for mechanistic research, animal modeling, diagnostic criteria, and personalized clinical management of MD.