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Endolymphatic Duct Blockage as a Surgical Treatment Option for Ménière's Disease
Published on: April 28, 2023
Evolution of understanding and clinical practice of Ménière's disease
1Department of Otolaryngology-Head and Neck Surgery, Massachusetts Eye and Ear, Harvard Medical School, 243 Charles Street, 02114, Boston, MA, USA. Steven_Rauch@meei.harvard.edu.
Abstract:
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.
Insights
Ménière
Area of Science:
- Otolaryngology
- Inner Ear Physiology
- Pathophysiology
Background:
- Ménière's disease (MD) concepts evolved from a hydrodynamic model to a new framework.
- The classic model attributed symptoms to endolymphatic hydrops and membrane rupture.
- Recent evidence challenges the hydrodynamic paradigm, suggesting a shift in understanding MD.
Purpose of the Study:
- To review the evolution of Ménière's disease pathophysiology and treatment concepts.
- To present a modern framework for MD based on endolymphatic sac (ELS) failure.
- To discuss implications for research, diagnosis, and clinical management.
Main Methods:
- Review of pathological, physiological, genetic, and imaging evidence over three decades.
- Analysis of temporal bone studies and systematic reviews.
- Examination of advances in MRI and CT imaging for in vivo visualization.
Main Results:
- Endolymphatic hydrops is linked to proliferative epithelial growth, not pressure distension.
- Two MD endotypes identified: hypoplastic and degenerative, based on ELS pathology.
- In vivo imaging enables ELS-related anatomy visualization and endotype stratification.
Conclusions:
- Ménière's disease is conceptualized as an endolymphatic sac (ELS) failure disorder.
- This new model impacts MD research, animal modeling, diagnostics, and personalized treatment.
- The analogy to renal insufficiency provides a framework for understanding MD pathophysiology.
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