Endolymphatic Hydrops in Ménière's Disease

Endolymphatic hydrops represents the defining histopathological feature of Ménière’s disease, characterised by episodic vertigo, fluctuating sensorineural hearing loss, tinnitus and aural fullness. This condition arises from excessive accumulation of endolymph within the membranous labyrinth, leading to distension of cochlear and vestibular structures. Although first described over a century ago, the underlying mechanisms remain multifactorial, encompassing genetic predispositions, autoimmune and inflammatory processes, ion-transport dysfunction and potential mechanical obstruction of endolymphatic pathways. Advances in high-resolution magnetic resonance imaging have enabled in vivo visualisation and grading of hydrops, refining diagnostic accuracy and revealing subclinical involvement. At the cellular level, dysregulation of inflammatory mediators, aberrant activation of the NLRP3 inflammasome in vestibular immune cells and excitotoxic injury from neurotransmitter imbalances have been implicated in hair cell degeneration and vestibular nerve damage. The global burden of Ménière’s disease underscores the need for integrated approaches that combine immunomodulation, targeted drug delivery and precise phenotyping to mitigate symptoms and preserve auditory and vestibular function.

Research from Nature Portfolio

Recent studies have elucidated the role of serum/glucocorticoid-inducible kinase 1 as a critical regulator of innate immune responses in endolymphatic hydrops. Loss of this kinase in murine models leads to aberrant activation of the NLRP3 inflammasome in resident vestibular macrophages, driving IL-1β–mediated hair cell injury, neural compromise and exacerbated fluid accumulation. Pharmacological or genetic inhibition of NLRP3 restores cochlear and vestibular function, identifying inflammasome modulation as a promising immunotherapeutic strategy for controlling hydrops and its audiovestibular sequelae.

Endolymphatic Hydrops in Ménière's Disease publication trend

The graph below shows the total number of articles in endolymphatic hydrops in ménière's disease across all publications each year (not limited to Nature Index journals).

Technical terms

Endolymphatic Hydrops: Excessive accumulation of endolymph in the membranous labyrinth causing distension and pressure changes.

NLRP3 inflammasome: A multiprotein complex that activates proinflammatory cytokines in response to cellular stress.

Serum/glucocorticoid-inducible kinase 1 (SGK1): A serine/threonine kinase modulating inflammatory pathways and cellular ion homeostasis.

IL-1β: A proinflammatory cytokine involved in immune activation and tissue injury.

Glutamate excitotoxicity: Neuronal damage caused by excessive stimulation from the neurotransmitter glutamate.

Glutaminase (GLS): An enzyme converting glutamine to glutamate, implicated in neurotransmitter balance.

Mendelian randomization: A genetic epidemiology method that infers causal relationships using genetic variants as instrumental variables.

References

1. Serum/glucocorticoid-inducible kinase 1 deficiency induces NLRP3 inflammasome activation and autoinflammation of macrophages in a murine endolymphatic hydrops model. Nature Communications (2023).
2. Meniere's disease: Pathogenesis, treatments, and emerging approaches for an idiopathic bioenvironmental disorder. Environmental Research (2023).
3. IL-1β promotes glutamate excitotoxicity: indications for the link between inflammatory and synaptic vesicle cycle in Ménière’s disease. Cell Death Discovery (2024).
4. Exploring causal correlations between inflammatory cytokines and Ménière’s disease: a Mendelian randomization. Frontiers in Immunology (2024).
5. Detection and Grading of Endolymphatic Hydrops in Menière Disease Using MR Imaging. American Journal of Neuroradiology (2014).