Current Understanding and Clinical Management of Meniere's Disease: A Systematic Review

Pérez-Carpena, Patricia; Lopez-Escamez, José A.

doi:10.1055/s-0039-3402065

Cited by 52 publications

(44 citation statements)

References 120 publications

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“…The main risk factor of tinnitus is hearing loss, but it is often associated with other conditions including hyperacusis, anxiety, depression, hypertension, insomnia or migraine [3]. Meniere disease (MD) is a rare inner ear disorder with a significant genetic contribution [4], characterized by episodes of vertigo, tinnitus and sensorineural hearing loss [5]. Although vertigo attacks are considered as the main symptom in the first years of the disease, persistent tinnitus is described as the most troublesome symptom by many MD patients [6,7].…”

Section: Introductionmentioning

confidence: 99%

Burden of rare variants in synaptic genes in patients with severe tinnitus: An exome based extreme phenotype study

et al. 2021

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Background tinnitus is a heterogeneous condition associated with audiological and/or mental disorders. Chronic, severe tinnitus is reported in 1% of the population and it shows a relevant heritability, according to twins, adoptees and familial aggregation studies. The genetic contribution to severe tinnitus is unknown since large genomic studies include individuals with self-reported tinnitus and large heterogeneity in the phenotype. The aim of this study was to identify genes for severe tinnitus in patients with extreme phenotype. Methods for this extreme phenotype study, we used three different cohorts with European ancestry (Spanish with Meniere disease (MD), Swedes tinnitus and European generalized epilepsy). In addition, four independent control datasets were also used for comparisons. Whole-exome sequencing was performed for the MD and epilepsy cohorts and whole-genome sequencing was carried out in Swedes with tinnitus. Findings we found an enrichment of rare missense variants in 24 synaptic genes in a Spanish cohort, the most significant being PRUNE2, AKAP9, SORBS1, ITGAX, ANK2, KIF20B and TSC2 ( p < 2E −04 ), when they were compared with reference datasets. This burden was replicated for ANK2 gene in a Swedish cohort with 97 tinnitus individuals, and in a subset of 34 Swedish patients with severe tinnitus for ANK2, AKAP9 and TSC2 genes ( p < 2E −02 ). However, these associations were not significant in a third cohort of 701 generalized epilepsy individuals without tinnitus. Gene ontology (GO) and gene-set enrichment analyses revealed several pathways and biological processes involved in severe tinnitus, including membrane trafficking and cytoskeletal protein binding in neurons. Interpretation a burden of rare variants in ANK2, AKAP9 and TSC2 is associated with severe tinnitus . ANK2, encodes a cytoskeleton scaffolding protein that coordinates the assembly of several proteins, drives axonal branching and influences connectivity in neurons.

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Section: Introductionmentioning

confidence: 99%

Burden of rare variants in synaptic genes in patients with severe tinnitus: An exome based extreme phenotype study

et al. 2021

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“…However, short-term fluctuations of audiovestibular symptoms do not correlate with gross endolymphatic hydrops changes, suggesting that the fluctuations in hydrops associated with attacks have an amplitude below the limits of current MR resolution. 1…”

Section: Pathophysiologymentioning

confidence: 99%

Menière’s disease

et al. 2020

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Menière’s disease causes paroxysmal rotatory vertigo, due to endolymphatic hydrops, an accumulation of endolymph in the endolymphatic space of the labyrinth. Its major symptoms are attacks of rotatory vertigo lasting minutes to hours, with unilateral hearing loss, tinnitus and aural fullness. As the disease progresses, attacks happen less often, but hearing loss and tinnitus gradually become permanent. Neuro-otological complications may develop, such as benign paroxysmal positional vertigo, vestibular drop attacks and bilateral vestibulopathy. The diagnosis of Menière’s disease is based on the typical clinical picture and typical findings on the audiogram. Furthermore, it is now possible to diagnose it by MR of the inner ear. Long-term management has several steps, including diet, diuretics, intratympanic injection of corticosteroid or gentamicin and surgery (endolymphatic sac surgery, grommet insertion, surgical labyrinthectomy).

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“…3 The etiology remains enigmatic but may involve heredity, auto-immunity, and inflammation. 4 The reason why some membranes in Meniere disease only distend while others rupture remains enigmatic. A doublehit mechanism that involves a change in membrane distensibility is theorized to play a role in this process.…”

Section: Introductionmentioning

confidence: 99%

The Concept of a Rupture Risk Envelope for the Cochleo-Saccular Membranes

Pender

2022

Int Arch Otorhinolaryngol

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Introduction Alterations in endolymphatic pressure have long been suspected of being associated with the development of endolymphatic hydrops and rupture of the membranous labyrinth. More recently, there has been a focus on how membrane mechanics might contribute to membrane rupture. This is suspected to involve the viscoelastoplastic properties of these membranes. Objective To construct a rupture risk envelope for the cochleo-saccular membranes based on viscoelastoplasticity to provide insight into lesion behavior in Meniere disease. Methods Reported deformation data from a collagen model of the cochleo-saccular membranes was utilized. Yield stress was defined as 80% of ultimate failure stress. The yield points at various strain rates were used to construct a rupture risk envelope for the membranes. Results The rupture risk envelope was found to be downward sloping in configuration. At the highest strain rate of 385% per minute, the membrane yield was associated with greater stress (7.0 kPa) and lesser strain (30%); while at the lowest strain rate of 19.2% per minute, there was substantially less membrane yield stress (4.3 kPa) but it was associated with greater strain (44%). Conclusion The concept of a rupture risk envelope based on viscoelastoplasticity provides insight into hydropic lesion behavior in Meniere disease. This concept helps to explain how variations in membrane distensibility might occur as suspected in the double hit theory of lesion generation in Meniere disease. Slowly developing lesions would appear have a lower rupture risk while rapidly developing lesions would appear to have a greater risk of early membrane rupture.

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Current Understanding and Clinical Management of Meniere's Disease: A Systematic Review

Cited by 52 publications

References 120 publications

Burden of rare variants in synaptic genes in patients with severe tinnitus: An exome based extreme phenotype study

Burden of rare variants in synaptic genes in patients with severe tinnitus: An exome based extreme phenotype study

Menière’s disease

The Concept of a Rupture Risk Envelope for the Cochleo-Saccular Membranes

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