Patient Perspective
Heather Davies | VeDA (Vestibular Disorders Association) ambassador, author, podcast host
• Davies shares her personal experience with Ménière's disease, describing severe episodes of vertigo, tinnitus, and hearing issues that forced her to leave her nursing career and drastically change her life.
• After being encouraged to "dream another dream," she found purpose by creating the Ménière's Muse podcast, which provides a platform for vestibular disorder patients to share their stories and build community.
• Despite describing Ménière's as both a "blessing and a curse," Davies emphasizes the importance of finding hope, adapting to new limitations, and recognizing that medical research provides critical support to those suffering from vestibular disorders.
History of Ménière’s Disease
Robin Bigelow, M.D. | The House Institute Foundation
• Prosper Ménière revolutionized understanding of vertigo in 1861 by linking it to inner ear disease rather than brain disorders, describing what became known as Ménière's disease with symptoms of fluctuating hearing loss, tinnitus, and dizzy spells.
• Over the past 150 years, numerous treatments for Ménière's disease have been attempted—from early remedies like bloodletting, leeches, and iodine to various surgical interventions including endolymphatic sac surgery, nerve sectioning, and labyrinthectomy—reflecting the challenging nature of treating this condition.
Treatment: State of the Art Clinical Care; Clinical Unmet Needs
Habib Rizk, M.D. | Medical University of South Carolina (MUSC)
• Current clinical care for Ménière's disease lacks evidence-based treatments, with dietary modifications, diuretics, and betahistine commonly used despite limited scientific support, though clinicians observe patient improvements that suggest effectiveness beyond placebo effects.
• There's significant overlap between Ménière's disease and vestibular migraine, with research suggesting multiple subclassifications or "endophenotypes" of Ménière's disease that may require different treatment approaches, including those with migraine features.
• Clinical trials for Ménière's disease face significant challenges including significant placebo response rates, inconsistent outcome measures, and the need for“better trial designs that account for disease subtypes and fluctuating symptoms, with researchers suggesting alternative approaches like the STAR*D design used in depression clinical trials, to maximize recruitment accrual, compliance, and longitudinal follow-up.
Migraine and Ménière’s Disease
Jeffrey Sharon, M.D. | University of California, San Francisco (UCSF)
• Migraine affects 10-15% of the global population and shows surprising connections with vestibular disorders, with more than half of Ménière's disease patients meeting criteria for migraine, suggesting a relationship that dates back to Prosper Ménière's original observations in 1861.
• Research shows biological links between migraine and vestibular function through CGRP (calcitonin gene-related peptide), a neuroinflammatory peptide expressed in the vestibular periphery that affects balance in animal models, with preliminary clinical trial data showing CGRP-blocking drugs may reduce dizziness symptoms.
• There are multiple lines of evidence connecting migraine and Ménière's disease: epidemiological overlap, shared symptoms and triggers, correlation in symptom laterality, potential pathophysiological mechanisms involving trigeminal nerve innervation of inner ear blood vessels, and genetic patterns in affected families. This suggests that exploring migraine medications as treatments for Ménière's disease is warranted.
Imaging/MRI
Amy Juliano, M.D. | Mass Eye and Ear
• MRI imaging techniques have evolved to visualize endolymphatic hydrops in Ménière's disease patients by using contrast agents that permeate into perilymph but not endolymph, allowing radiologists to objectively identify enlarged cochlear ducts, saccules, and utricles as visible signs of the condition.
• Research has identified two distinct endotypes of Ménière's disease: hypoplastic (where the endolymphatic sac never developed properly) and degenerative (where the endolymphatic sac developed but then degenerated), which can be distinguished on imaging by examining the vestibular aqueduct angle or the thickness of retrolabyrinthine bone.
• Modern radiological reports for Ménière's disease patients now include assessment of endolymphatic structure sizes, perilymph enhancement (which correlates with sensorineural hearing loss), and anatomical features that help subtype the disease, providing valuable prognostic information for treatment planning.
Panel Discussion: Management of Ménière’s/Patient Stories
Moderated by John Oghalai, M.D. (USC) with Habib Rizk, M.D. (MUSC), Jeffrey Sharon, M.D. (UCSF), William Slatterly, M.D. (House Institute Foundation)
• The panelists discussed approaches to diagnosing and treating Ménière's disease, emphasizing the importance of careful history-taking to distinguish "classic" Ménière's (vertigo episodes with concurrent hearing loss, tinnitus, and aural fullness) from other conditions like vestibular migraine.
• First-line treatments typically include low-salt diet, migraine diet, and diuretics, with steroids considered for persistent symptoms or significant hearing fluctuations; the panelists had varying practices regarding when to order MRIs and vestibular testing.
• Several potential triggers or contributing factors were discussed, including barometric pressure changes, allergies, stress, and genetic factors, with some panelists routinely performing genetic testing in challenging cases.
• The experts emphasized the need for better research on Ménière's disease subtypes and etiology, with consensus that it's likely multiple different conditions with similar presentations, requiring more precise diagnosis and individualized treatment approaches.
Molecular Basis
Andreas Eckhard, M.D. | Harvard Medical School/Mass Eye and Ear
• Evidence that endolymphatic hydrops may be a compensatory response to injury rather than the cause of symptoms challenges the traditional view of Ménière's disease as primarily a fluid pressure disorder.
• Research shows that the endolymphatic sac, which makes up about a third to half of the inner ear's epithelial surface, is consistently damaged in Ménière's disease patients, and this damage represents a major injury to the inner ear.
• This suggests a new model where Ménière's disease follows a pattern of maladaptive progression similar to chronic kidney disease: with initial injury, compensatory mechanisms (including cell proliferation in hydropic membranes), a phase of fluctuating function, and eventual burnout.
Genetics
Jose Antonio Lopez-Escamez, M.D., Ph.D. | University of Sydney, Australia
• Ménière's disease can be seen not a single disease but has multiple subtypes, including three immune response-related subgroups (autoimmune, autoinflammatory, and IgE-associated) that can be identified through measuring cytokines and IgE in blood tests, while approximately 40% of patients may have a genetic origin with normal immune responses.
• Genetic research has identified about 20 genes associated with familial Ménière's disease, with the three most common being OTOG (encoding otogelin), MYO7A (encoding myosin), and TECTA (encoding alpha-tectorin), and a newly discovered gene GJD3 (encoding Connexin 31.9), with most genetic cases involving the stereocilia and tectorial membrane.
• These findings suggest Ménière's disease often results from a combination of genetic predisposition (a "fragile ear") plus environmental triggers like noise exposure, with approximately 9% of European cases being familial, and an estimated 28% of sporadic cases potentially having genetic origins as well.
Immunology and Ménière’s Disease
Andrea Vambutas, M.D. | Northwell Health, New York
• A challenge to traditional classifications of Ménière's disease is that it may exist on a continuum with autoimmune inner ear disease, with some cases potentially being autoinflammatory rather than autoimmune in nature.
• Research shows different cytokine profiles among patients, particularly finding that corticosteroid-responsive patients have high TNF levels that drop with treatment, while corticosteroid-resistant patients have low TNF but high IL-1 levels and may respond to anakinra (an IL-1 receptor antagonist).
• A phase two clinical trial using anakinra for corticosteroid-resistant Ménière's disease patients is being conducted, with preliminary results showing promising signals, building on their earlier research that found a 70% response rate in autoimmune inner ear disease patients treated with anakinra.
Social and Psychological Triggers
Joanna Wolfson, Ph.D. | New York University Langone Health
• The significant psychological impact of Ménière's disease is that 50-60% of patients experience anxiety or depression due to factors like the traumatic and unpredictable nature of symptoms, uncertainty about future progression, and the "hidden disability" quality that others cannot see or understand.
• The presentation explains how Ménière's symptoms and psychological distress create a vicious cycle (symptoms trigger fight-or-flight responses that can exacerbate dizziness and other symptoms), and recommends psychological interventions including explaining the stress-symptom cycle, teaching relaxation techniques, using cognitive-behavioral therapy approaches to reframe thoughts, and helping patients gradually return to enjoyable activities with an acceptance of their "new normal."
Panel Discussion: Barriers to Ménière’s Disease Research
Moderated by Divya Chari, M.D. (Harvard Medical School) with Andrea Vambutas, M.D. (Northwell Health), Andreas Eckhard, M.D. (Harvard/Mass Eye and Ear), Jose Antonio Lopez-Escamez, M.D., Ph.D. (University of Sydney)
• The panelists emphasized the need for more precise patient selection using biomarkers (such as IL-1 beta and TNF levels) to differentiate Ménière's disease from similar conditions like vestibular migraine and to identify distinct subgroups within Ménière's patients.
• Clinical trials for Ménière's disease face significant challenges due to the disease's fluctuating nature and significant placebo response rates, making it difficult to distinguish treatment effects from the natural course of the disease.
• Research has evolved from viewing Ménière's as a purely mechanical problem to understanding its molecular and genetic components, with significant advances in identifying familial clusters and developing humanized transgenic animal models.
• Despite affecting relatively few people, Ménière's disease doesn't qualify for "rare disease" funding benefits, creating additional obstacles for research funding and drug development.
