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Clinical Immunology (Orlando, Fla.) Jul 2023Meniere Disease (MD) is an inner ear syndrome, characterized by episodes of vertigo, tinnitus and fluctuating sensorineural hearing loss. The pathological mechanism...
BACKGROUND
Meniere Disease (MD) is an inner ear syndrome, characterized by episodes of vertigo, tinnitus and fluctuating sensorineural hearing loss. The pathological mechanism leading to sporadic MD is still poorly understood, however an allergic inflammatory response seems to be involved in some patients with MD.
OBJECTIVE
Decipher an immune signature associated with the syndrome.
METHODS
We performed mass cytometry immune profiling on peripheral blood from MD patients and controls. We analyzed differences in state and differences in abundance of the different cellular subsets. IgE levels were quantified through ELISA on supernatant of cultured whole blood.
RESULTS
We have identified two clusters of individuals according to the single cell cytokine profile. These clusters presented differences in IgE levels, immune cell population abundance, including a reduction of CD56 NK-cells, and changes in cytokine expression with a different response to bacterial and fungal antigens.
CONCLUSION
Our results support a systemic inflammatory response in some MD patients that show a type 2 response with allergic phenotype, which could benefit from personalized IL-4 blockers.
Topics: Humans; Meniere Disease; Vertigo; Cytokines; Hearing Loss, Sensorineural; Syndrome; Immunoglobulin E
PubMed: 37178857
DOI: 10.1016/j.clim.2023.109632 -
Journal of Immunology Research 2018Immune-mediated inner ear disease can be primary, when the autoimmune response is against the inner ear, or secondary. The latter is characterized by the involvement of... (Review)
Review
Immune-mediated inner ear disease can be primary, when the autoimmune response is against the inner ear, or secondary. The latter is characterized by the involvement of the ear in the presence of systemic autoimmune conditions. Sensorineural hearing loss is the most common audiovestibular symptom associated with systemic autoimmune diseases, although conductive hearing impairment may also be present. Hearing loss may present in a sudden, slowly, rapidly progressive or fluctuating form, and is mostly bilateral and asymmetric. Hearing loss shows a good response to corticosteroid therapy that may lead to near-complete hearing restoration. Vestibular symptoms, tinnitus, and aural fullness can be found in patients with systemic autoimmune diseases; they often mimic primary inner ear disorders such as Menière's disease and mainly affect both ears simultaneously. Awareness of inner ear involvement in systemic autoimmune diseases is essential for the good response shown to appropriate treatment. However, it is often misdiagnosed due to variable clinical presentation, limited knowledge, sparse evidence, and lack of specific diagnostic tests. The aim of this review is to analyse available evidence, often only reported in the form of case reports due to the rarity of some of these conditions, of the different clinical presentations of audiological and vestibular symptoms in systemic autoimmune diseases.
Topics: Autoimmune Diseases; Ear, Inner; Hearing Loss, Conductive; Hearing Loss, Sensorineural; Humans; Meniere Disease; Tinnitus
PubMed: 30211232
DOI: 10.1155/2018/5798103 -
The Cochrane Database of Systematic... Feb 2023Ménière's disease is a condition that causes recurrent episodes of vertigo, associated with hearing loss and tinnitus. Corticosteroids are sometimes administered... (Meta-Analysis)
Meta-Analysis Review
BACKGROUND
Ménière's disease is a condition that causes recurrent episodes of vertigo, associated with hearing loss and tinnitus. Corticosteroids are sometimes administered directly into the middle ear to treat this condition (through the tympanic membrane). The underlying cause of Ménière's disease is unknown, as is the way in which this treatment may work. The efficacy of this intervention in preventing vertigo attacks, and their associated symptoms, is currently unclear.
OBJECTIVES
To evaluate the benefits and harms of intratympanic corticosteroids versus placebo or no treatment in people with Ménière's disease.
SEARCH METHODS
The Cochrane ENT Information Specialist searched the Cochrane ENT Register; Central Register of Controlled Trials (CENTRAL); Ovid MEDLINE; Ovid Embase; Web of Science; ClinicalTrials.gov; ICTRP and additional sources for published and unpublished trials. The date of the search was 14 September 2022.
SELECTION CRITERIA
We included randomised controlled trials (RCTs) and quasi-RCTs in adults with a diagnosis of Ménière's disease comparing intratympanic corticosteroids with either placebo or no treatment. We excluded studies with follow-up of less than three months, or with a cross-over design (unless data from the first phase of the study could be identified). DATA COLLECTION AND ANALYSIS: We used standard Cochrane methods. Our primary outcomes were: 1) improvement in vertigo (assessed as a dichotomous outcome - improved or not improved), 2) change in vertigo (assessed as a continuous outcome, with a score on a numerical scale) and 3) serious adverse events. Our secondary outcomes were: 4) disease-specific health-related quality of life, 5) change in hearing, 6) change in tinnitus and 7) other adverse effects (including tympanic membrane perforation). We considered outcomes reported at three time points: 3 to < 6 months, 6 to ≤ 12 months and > 12 months. We used GRADE to assess the certainty of evidence for each outcome. MAIN RESULTS: We included 10 studies with a total of 952 participants. All studies used the corticosteroid dexamethasone, with doses ranging from approximately 2 mg to 12 mg. Improvement in vertigo Intratympanic corticosteroids may make little or no difference to the number of people who report an improvement in their vertigo at 6 to ≤ 12 months follow-up (intratympanic corticosteroids 96.8%, placebo 96.6%, risk ratio (RR) 1.00, 95% confidence interval (CI) 0.92 to 1.10; 2 studies; 60 participants; low-certainty evidence) or at more than 12 months follow-up (intratympanic corticosteroids 100%, placebo 96.3%; RR 1.03, 95% CI 0.87 to 1.23; 2 studies; 58 participants; low-certainty evidence). However, we note the large improvement in the placebo group for these trials, which causes challenges in interpreting these results. Change in vertigo Assessed with a global score One study (44 participants) assessed the change in vertigo at 3 to < 6 months using a global score, which considered the frequency, duration and severity of vertigo. This is a single, small study and the certainty of the evidence was very low. We are unable to draw meaningful conclusions from the numerical results. Assessed by frequency of vertigo Three studies (304 participants) assessed the change in frequency of vertigo episodes at 3 to < 6 months. Intratympanic corticosteroids may slightly reduce the frequency of vertigo episodes. The proportion of days affected by vertigo was 0.05 lower (absolute difference -5%) in those receiving intratympanic corticosteroids (95% CI -0.07 to -0.02; 3 studies; 472 participants; low-certainty evidence). This is equivalent to a difference of approximately 1.5 days fewer per month affected by vertigo in the corticosteroid group (with the control group having vertigo on approximately 2.5 to 3.5 days per month at the end of follow-up, and those receiving corticosteroids having vertigo on approximately 1 to 2 days per month). However, this result should be interpreted with caution - we are aware of unpublished data at this time point in which corticosteroids failed to show a benefit over placebo. One study also assessed the change in frequency of vertigo at 6 to ≤ 12 months and > 12 months follow-up. However, this is a single, small study and the certainty of the evidence was very low. Therefore, we are unable to draw meaningful conclusions from the numerical results. Serious adverse events Four studies reported this outcome. There may be little or no effect on the occurrence of serious adverse events with intratympanic corticosteroids, but the evidence is very uncertain (intratympanic corticosteroids 3.0%, placebo 4.4%; RR 0.64, 95% CI 0.22 to 1.85; 4 studies; 500 participants; very low-certainty evidence).
AUTHORS' CONCLUSIONS
The evidence for intratympanic corticosteroids in the treatment of Ménière's disease is uncertain. There are relatively few published RCTs, which all consider the same type of corticosteroid (dexamethasone). We also have concerns about publication bias in this area, with the identification of two large RCTs that remain unpublished. The evidence comparing intratympanic corticosteroids to placebo or no treatment is therefore all low- or very low-certainty. This means that we have very low confidence that the effects reported are accurate estimates of the true effect of these interventions. Consensus on the appropriate outcomes to measure in studies of Ménière's disease is needed (i.e. a core outcome set) in order to guide future studies in this area, and enable meta-analysis of the results. This must include appropriate consideration of the potential harms of treatment, as well as the benefits. Finally, we would also highlight the responsibility that trialists have to ensure results are available, regardless of the outcome of their study.
Topics: Adult; Humans; Adrenal Cortex Hormones; Dexamethasone; Meniere Disease; Tinnitus; Vertigo
PubMed: 36847608
DOI: 10.1002/14651858.CD015245.pub2 -
Laryngo- Rhino- Otologie Aug 2017The BEMED study (BMJ 2016; 352: DOI 10.1136) was designed as multi-centric, double-blind, plaebo-controlled study in patients with Menière's disease. It should compare... (Comparative Study)
Comparative Study Randomized Controlled Trial
The BEMED study (BMJ 2016; 352: DOI 10.1136) was designed as multi-centric, double-blind, plaebo-controlled study in patients with Menière's disease. It should compare a low-level (2 × 24 mg/d) vs. high-level (3 × 48 mg/d) betahstine intake vs. placebo. The primary endpoint was the "number of vertigo attacks lasting longer than 20 min as documented in a patient's diary". The main finding of the study was that betahistine did not significantly better reduced the number of vertigo attacks than placebo. Therefore, the BEMED study should be critically discussed in the present paper.
Topics: Adolescent; Adult; Aged; Aged, 80 and over; Betahistine; Dose-Response Relationship, Drug; Double-Blind Method; Female; Germany; Humans; Male; Meniere Disease; Middle Aged; Young Adult
PubMed: 28850992
DOI: 10.1055/s-0043-113690 -
The Cochrane Database of Systematic... Feb 2013This is an update of a Cochrane review first published in The Cochrane Library in Issue 1, 2010.Ménière's disease is characterised by three major symptoms: vertigo,... (Meta-Analysis)
Meta-Analysis Review
BACKGROUND
This is an update of a Cochrane review first published in The Cochrane Library in Issue 1, 2010.Ménière's disease is characterised by three major symptoms: vertigo, deafness, and tinnitus or aural fullness, all of which are discontinuous and variable in intensity. A number of surgical modalities, of varying levels of invasiveness, have been developed to reduce the symptoms of Ménière's disease, but it is not clear whether or not these are effective.
OBJECTIVES
To assess the effectiveness of surgical options for the treatment of Ménière's disease. All surgical interventions used in the treatment of Ménière's disease, either to alter the natural history of the disease or to abolish vestibular function, were considered for this review.
SEARCH METHODS
We searched the Cochrane Ear, Nose and Throat Disorders Group Trials Register; the Cochrane Central Register of Controlled Trials (CENTRAL); PubMed; EMBASE; CINAHL; Web of Science; BIOSIS Previews; Cambridge Scientific Abstracts; ICTRP and additional sources for published and unpublished trials. The date of the most recent search was 7 November 2012.
SELECTION CRITERIA
Randomised or quasi-randomised controlled studies of a surgical modality versus a placebo therapy in Ménière's disease.
DATA COLLECTION AND ANALYSIS
Two authors independently assessed trial quality and extracted data. We contacted study authors for further information.
MAIN RESULTS
The only surgical intervention which has been evaluated in randomised controlled trials and met the inclusion criteria was endolymphatic sac surgery. We identified two randomised trials, involving a total of 59 patients; one comparing endolymphatic sac surgery with ventilation tubes and one with simple mastoidectomy. Neither study reported any beneficial effect of surgery either in comparison to placebo surgery or grommet insertion.
AUTHORS' CONCLUSIONS
The two trials included in this review provide insufficient evidence of the beneficial effect of endolymphatic sac surgery in Ménière's disease.
Topics: Endolymphatic Sac; Humans; Mastoid; Meniere Disease; Middle Ear Ventilation; Randomized Controlled Trials as Topic
PubMed: 23450562
DOI: 10.1002/14651858.CD005395.pub3 -
Scientific Reports Jun 2021Meniere's disease is thought to be a disorder of the inner ear function, affected by genetic and environmental factors. Several recent studies have shown that air...
Meniere's disease is thought to be a disorder of the inner ear function, affected by genetic and environmental factors. Several recent studies have shown that air pollution could affect middle and inner ear diseases. The purpose of this study was to investigate the relationship between the Meniere's disease occurrence and air pollution status in Korea. This study used a time-stratified case-crossover design. Hospital visit data by Meniere's disease were collected from the Korea National Health Insurance Service-National Sample Cohort (NHIS-NSC) database. Daily air pollution data for sulfur dioxide (SO), nitrogen dioxide (NO), carbon monoxide (CO), ozone (O), and particulate matter (PM: ≤ 10 μm in diameter, and PM: ≤ 2.5 μm in diameter) were collected from the National Ambient air quality Monitoring Information System (NAMIS) database. We used two-stage analysis to assess the association between degree of air pollution and the occurrence of Meniere's disease. In the first stage, region-specific analysis was conducted to estimate the odds ratios (ORs) of Meniere's disease risk associated with each air pollutant exposure by using conditional logistic regression for matched case-control sets in 16 regions. In the second stage, region-specific ORs from the first stage were combined and the pooled effect estimates were derived through fixed and random effect meta-analysis. Subgroup analysis was conducted for age, sex, seasonality, and urbanization of residence. In total, 29,646 (32.1% males and 67.9% females) Meniere's disease cases were identified from Korea NHIS-NSC database between 2008 and 2015. Overall, SO, NO, CO, and PM showed significant correlation with Meniere's disease risk at immediate lags, and weaker correlation at delayed lags, whereas O showed slightly negative correlation at the immediate lag (lag0) and PM did not show strong correlation (SO: 1.04 [95% confidence interval: 1.01, 1.06]; NO: 1.08 [1.06, 1.11]; CO: 1.04 [1.02, 1.06]; O: 0.96 [0.93, 0.99]: statistically significant ORs at lag0 are listed). These positive and negative associations between Meniere's disease and each air pollutant were generally stronger in the age of 40-64, female, summer (June-August) season, and urban subgroups. Our results showed that hospital visits for Meniere's disease were associated with the measured concentrations of ambient air pollutants SO, NO, CO, and PM. Further studies are required to confirm these associations and find their mechanisms.
Topics: Adult; Aged; Air Pollutants; Air Pollution; Case-Control Studies; Cross-Over Studies; Female; Humans; Male; Meniere Disease; Middle Aged; Nitrogen Dioxide; Ozone; Particulate Matter; Republic of Korea; Seasons; Sulfur Dioxide; Young Adult
PubMed: 34162905
DOI: 10.1038/s41598-021-92355-0 -
European Annals of Otorhinolaryngology,... Sep 2015Four studies assessing vestibular compensation in Menière's disease patients undergoing unilateral vestibular neurotomy, using different analysis methods, are reviewed,... (Review)
Review
OBJECTIVES
Four studies assessing vestibular compensation in Menière's disease patients undergoing unilateral vestibular neurotomy, using different analysis methods, are reviewed, with a focus on the different strategies used by patients according to their preoperative sensory preference.
MATERIAL AND METHODS
Four prospective studies performed in a university tertiary referral center were reviewed, measuring the pattern of vestibular compensation in Menière's disease patients before and after unilateral vestibular neurotomy on various assessment protocols: postural syndrome assessed on static posturography and gait analysis; perceptual syndrome assessed on subjective visual vertical perception; and oculomotor syndrome assessed on ocular cyclotorsion.
RESULTS
Vestibular compensation occurred at variable intervals depending on the parameter investigated. Open-eye postural control and gait/walking returned to normal one month after neurotomy. Fine balance analysis found that visual perception of the vertical and ocular cyclotorsion impairment persisted at long-term follow-up. Clinical postural disturbance persisted only when visual afferents were cut off (eyes closed). These impairments were the expression of a postoperative change in postural strategy related to the new use of visual and non-visual references.
CONCLUSIONS
Understanding pre-operative interindividual variation in balance strategy is critical to screening for postural instability and tailoring vestibular rehabilitation.
Topics: Adaptation, Physiological; Humans; Meniere Disease; Neurosurgical Procedures; Proprioception; Vestibular Nerve; Vision, Ocular; Walking
PubMed: 26026684
DOI: 10.1016/j.anorl.2015.04.003 -
Journal of the Association For Research... Jun 2023Meniere disease (MD) is a rare disorder of the inner ear defined by sensorineural hearing loss (SNHL) associated with episodes of vertigo and tinnitus. The phenotype is... (Review)
Review
Meniere disease (MD) is a rare disorder of the inner ear defined by sensorineural hearing loss (SNHL) associated with episodes of vertigo and tinnitus. The phenotype is variable, and it may be associated with other comorbidities such as migraine, respiratory allergies, and several autoimmune disorders. The condition has a significant heritability according to epidemiological and familial segregation studies. Familial MD is found in 10% of cases, the most frequently found genes being OTOG, MYO7A, and TECTA, previously associated with autosomal dominant and recessive non-syndromic SNHL. These findings suggest a new hypothesis where proteins involved in the extracellular structures in the apical surface of sensory epithelia (otolithic and tectorial membranes) and proteins in the stereocilia links would be key elements in the pathophysiology of MD. The ionic homeostasis of the otolithic and tectorial membranes could be critical to suppress the innate motility of individual hair cell bundles. Initially, focal detachment of these extracellular membranes may cause random depolarization of hair cells and will explain changes in tinnitus loudness or trigger vertigo attacks in early stages of MD. With the progression of the disease, a larger detachment will lead to an otolithic membrane herniation into the horizontal semicircular canal with dissociation in caloric and head impulse responses. Familial MD shows different types of inheritance, including autosomal dominant and compound recessive patterns and implementation of genetic testing will improve our understanding of the genetic structure of MD.
Topics: Humans; Meniere Disease; Tinnitus; Ear, Inner; Vertigo; Hearing Loss, Sensorineural
PubMed: 37022572
DOI: 10.1007/s10162-023-00896-0 -
European Annals of Otorhinolaryngology,... Dec 2021Vestibular Evoked Myogenic Potentials (VEMP) are commonly recorded in patients experiencing vertigo or chronic instability. This test evaluates the patient's otolith...
Vestibular Evoked Myogenic Potentials (VEMP) are commonly recorded in patients experiencing vertigo or chronic instability. This test evaluates the patient's otolith function and is often combined with both Videonystagmography and Video Head Impulse Test. VEMP is a simple, reproducible test, in the absence of any pre-existing conductive hearing loss. Cervical VEMP explore both saccular function and the inferior vestibular nerve, whereas ocular VEMP assess utricular function and the superior vestibular nerve. In combination with previously described tests, VEMP allows characterization of vertigo and provides support for the diagnosis of superior semicircular canal dehiscence syndrome, Menière's disease, vestibular neuritis, vestibular schwannoma or idiopathic bilateral vestibulopathy. A good knowledge of these electrophysiological tests is essential in order to precisely assess the presence or absence of vestibular function impairment. We describe the test recording technique and the most common pitfalls in interpretation of the results. We then outline the results observed in various diseases impacting vestibular function.
Topics: Head Impulse Test; Humans; Meniere Disease; Vertigo; Vestibular Evoked Myogenic Potentials; Vestibule, Labyrinth
PubMed: 33483282
DOI: 10.1016/j.anorl.2021.01.001 -
F1000Research 2018Ménière's disease (MD) represents a heterogeneous group of relatively rare disorders with three core symptoms: episodic vertigo, tinnitus, and sensorineural hearing... (Review)
Review
Ménière's disease (MD) represents a heterogeneous group of relatively rare disorders with three core symptoms: episodic vertigo, tinnitus, and sensorineural hearing loss involving 125 to 2,000 Hz frequencies. The majority of cases are considered sporadic, although familial aggregation has been recognized in European and Korean populations, and the search for familial MD genes has been elusive until the last few years. Detailed phenotyping and cluster analyses have found several clinical predictors for different subgroups of patients, which may indicate different mechanisms, including genetic and immune factors. The genes associated with familial MD are , , , , , and . At least two mechanisms have been involved in MD: (a) a pro-inflammatory immune response mediated by interleukin-1 beta (IL-1β), tumor necrosis factor alpha (TNFα), and IL-6, and (b) a nuclear factor-kappa B (NF-κB)-mediated inflammation in the carriers of the single-nucleotide variant rs4947296. It is conceivable that microbial antigens trigger inflammation with release of pro-inflammatory cytokines at different sites within the cochlea, such as the endolymphatic sac, the stria vascularis, or the spiral ligament, leading to fluid imbalance with an accumulation of endolymph. Computational integration of clinical and "omics" data eventually should transform the management of MD from "one pill fits all" to precise patient stratification and a personalized approach. This article lays out a proposal for an algorithm for the genetic diagnosis of MD. This approach will facilitate the identification of new molecular targets for individualized treatment, including immunosuppressant and gene therapy, in the near future.
Topics: Genetic Testing; Genetic Therapy; Hearing Loss, Sensorineural; Humans; Immunosuppressive Agents; Meniere Disease; Molecular Targeted Therapy; Precision Medicine
PubMed: 30430003
DOI: 10.12688/f1000research.14417.1