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Ménière’s disease (MD) is characterized by an abnormal dilatation of the endolymphatic compartment called endolymphatic hydrops and is associated with fluctuating hearing losses and vertigo. Corticosteroid treatment is typically administered for its anti-inflammatory effects to MD patients. However, we recently described for the first time a direct interaction of two corticosteroids (dexamethasone and cortisol) with human AQP2 which strongly inhibited water fluxes. From these initial studies, we proposed an AQPs Corticosteroids Binding Site (ACBS). In the present work, we tested the interaction of 10 molecules associated to the steroid family for this putative ACBS. We observed a wide diversity of affinity and inhibitory potential of these molecules toward AQP2 and discussed the implications for inner ear physiology. Among the tested compounds, cholecalciferol, calcitriol and oestradiol were the most efficient AQP2 water permeability inhibitors.

Introduction Degenerative changes in the otolithic organs have been theorized to be caused by the mechanical obstruction to endolymphatic flow, possibly resulting in endolymphatic hydrops (ELH). Otolin-1 is an otoconial matrix protein that crosses the blood labyrinth barrier and has been found in the serum of healthy and diseased patients. Objective To measure the serum levels of Otolin-1 in Meniere disease (MD) patients and compared them with the healthy individuals. Methods This pilot, cross-sectional study was performed at our tertiary care referral center to compare the serum Otolin-1 levels of healthy individuals with those of MD patients. The blood samples were obtained during patients' visit to the vertigo clinic following remission of an acute episode. The data was analyzed using the Stata/SE version 12.0 (StataCorp. College Station, TX, USA). Comparison between the serum Otolin-1 levels in the two groups was performed using the unpaired t -test. A p -value of 0.05 was considered to be statistically significant. Results The participants were divided into two groups, with 31 MD patients, and 30 age and gender-matched members of the control group. The serum levels of Otolin-1 in MD patients (247.6, ± 44.2 pg/ml) were not found to be significantly different from those of the control group (236.2, ± 43.5 pg/ml) (p = 0.31). Conclusion The current study reveals that the serum levels of Otolin-1 are not significantly different between the patients with MD in the interictal phase and the control group's healthy ones. [ABSTRACT FROM AUTHOR]

Objective: Bilateral Ménière's disease is classified according to the time of appearance of symptoms in each ear into synchronous and metachronous types. A descriptive longitudinal study, involving 59 bilateral Ménière's disease patients, was carried out to assess the two forms of bilateral Ménière's disease. Method: Data on symptomatic chronology in each ear, auditory evolution and evolution of vertiginous crisis, among other aspects, were obtained, analysed and compared. Possible risk factors for Ménière's disease becoming bilateral were analysed after conducting nested case–control studies in a cohort. Results: The metachronous form was seen in 76.3 per cent of cases, and the time it took for the disease to become bilateral took a median time of seven years. The symptomatic triad was the most frequent symptomatic debut for the first ear in both forms. Synchronous debut presented a greater average hearing loss. Suffering from migraine and a symptomatic onset with a greater number of symptoms appear to be possible predictors of conversion to bilateral Ménière's disease. Conclusion: Bilateral Ménière's disease temporal models presented differences. The study of them helps to better understand, prevent and predict the behaviour of these patients. [ABSTRACT FROM AUTHOR]

Meniere's disease (MD) is a chronic condition affecting the inner ear whose precise etiology is currently unknown. We propose the hypothesis that MD is a migraine-related phenomenon which may have implications for future treatment options for both diseases. The association between MD and migraine is both an epidemiological and a mechanistic one, with up to 51% of individuals with MD experiencing migraine compared to 12% in the general population. The presence of endolymphatic hydrops in those with MD may be the factor that unites the two conditions, as hydropic inner ears have an impaired ability to maintain homeostasis. Migraine headaches are theorized to cause aura and symptoms via spreading cortical depression that ultimately results in substance P release, alterations in blood flow, and neurogenic inflammation. Chronically hydropic inner ears are less able to auto-regulate against the changes induced by active migraine attacks and may ultimately manifest as MD. This same vulnerability to derangements in homeostasis may also explain the common triggering factors of both MD attacks and migraine headaches, including stress, weather, and diet. Similarly, it may explain the efficacy of common treatments for both diseases: current migraine treatments such as anti-hypertensives and anti-convulsants have shown promise in managing MD. Though the etiology of both MD and migraine is likely multifactorial, further exploration of the association between the two conditions may illuminate how to best manage them in the future. MD is likely a manifestation of cochleovestibular migraine, which occurs as a result of migraine related changes in both the cochlea and vestibule.

COVID-19 is a disease caused by a new type of SARS virus. COVID-19 clinical symptoms are primarily related to the respiratory system but may also be involved in many others, including the nervous system. Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) can be an aetiological factor responsible for balance disorders. Symptoms such as vertigo may coexist with other symptoms or be an isolated manifestation of the disease. Among other balance dysfunctions associated with COVID-19 are BBPV (benign paroxysmal positional vertigo), vestibular neuritis, acute labyrinthitis, acute cerebellitis, as well as Ménière’s disease. MRI, Videonystagmographic (VNG) and the Dix-Hallpike test are used to make a diagnosis. Improvements were observed after treatment with oral corticosteroids and vestibular physiotherapy along with intratympanic injection of dexamethasone. Antiemetics, antihistamines, anti-cholinergics and benzodiazepines drugs may be helpful in relieving symptoms. In addition, drugs used in scheduled treatment of COVID-19, such as azithromycin and hydroxychloroquine, may cause balance disorders due to their potential side effects. The following article presents cases of COVID-positive patients with balance disorders. The aim of this article was to raise awareness that nonspecific symptoms such as balance disorders may be the only manifesta-tion of COVID-19, which is important due to early diagnosis and administration of treatment.

BACKGROUND: In the present study, we investigated the localization of otopetrin-2-a member of the otopetrin family that encodes proton-selective ion channels-in the human macula utricle using immunohistochemistry. METHODS: Macula utricle were acquired at surgery from patients who required transmastoid labyrinthectomy for intractable vertigo due to Menieres disease (MD; n = 3) and/or vestibular drops attacks (VDA; n = 2) and from temporal bones (n = 2) acquired at autopsy from individuals with no balance disorders. Immunofluorescence staining with otopetrin-2 (rabbit affinity purified polyclonal antibody) and GFAP (mouse monoclonal antibody) to identify vestibular supporting cells was made in formalin fixed cryostat sections or whole microdissected utricle (for flat mount preparations). Secondary antibodies against rabbit and mouse were used for the identification of both proteins. Digital fluorescent images were obtained using a high-resolution laser confocal microscope. RESULTS: Using cryostat sections and flat mount preparations otopetrin-2 immunofluorescence was seen as punctated signal throughout the supporting cells cytoplasm. GFAP immunofluorescence was present in the supporting cell cytoplasm. The distribution of otopetrin-2 was similar in the macula utricle obtained from MD, VDA, or autopsy normative patients. CONCLUSIONS: Otopetrin-2 was localized in supporting cells in a similar fashion that otopetrin-1 previously reported in the mouse macula utricle. The differential expression of otopetrin-2 in the supporting cells of the human macula utricle suggest an important role in the vestibular sensory periphery homeostasis and otolith maintenance.

Emerging evidence suggests substantial overlap between the symptoms of Meniere's disease (MD) and migraine-related cochlear/vestibular disorders. We report a 5-year-old girl with a 6-month history of left-sided hearing loss followed by daily episodes of vertigo, headache, and vomiting who met the criteria for definite MD. The patient became symptom-free and gained near normal hearing levels after starting on a 6-week migraine diet/lifestyle regimen with riboflavin and magnesium. We believe that the symptoms of MD may be primarily due to a vestibular migraine phenomenon. Pediatric MD patients may benefit from migraine lifestyle/dietary changes with control of both cochlear and vestibular symptoms.

Congenital cytomegalovirus (cCMV) infection can cause fluctuating hearing loss and vestibulopathy. The pathogenesis is unknown. This report describes a 13-year old boy with cCMV and severe hearing loss in the right ear since age 3, presenting with fluctuating hearing loss in the left ear and vestibular symptoms. 3D fluid attenuated inversion recovery (FLAIR) MRI showed endolymphatic hydrops in the acutely affected ear. This is the first description of a child with cCMV subjected to this imaging technique, raising the question whether endolymphatic hydrops could play a role in the development of late-onset symptoms and demonstrating the possibilities of this MRI sequence.

Objective: The heterogeneity of Ménière's disease is presently defined by a variety of subtypes. This study introduced three different subtypes of unilateral Ménière's disease based on the evolution of vertigo crises from their inception. Method: A longitudinal descriptive study of 327 unilateral Ménière's disease patients was performed. In a subgroup of patients followed from the onset of the disease, 3 subtypes of unilateral Ménière's disease were defined according to the vertiginous crises suffered during the first 10 years of the disorder. Results: Data was available for 87 patients with unilateral Ménière's disease from the start of their disease (26.6 per cent of the original sample). These patients were grouped into three models according to their symptomatic evolution. Model 3 was associated with a worse hearing prognosis, a greater number of Tumarkin's otolithic crises and the need for surgery. Model 1 presented less hearing loss. Conclusion: Unilateral Ménière's disease models based on the evolution of vertiginous crises present differences according to aspects such as hearing loss, vertiginous crisis, Tumarkin's otolithic crisis and the need for surgery. [ABSTRACT FROM AUTHOR]

Background and Aim: The Acceptance Noise Level (ANL) which determines the tolerable noise level while listening to running speech, is related to factors such as hearing loss and speech presentation level. This study aims to investigate the effect of speech presentation level on the ANL in people with unilateral Meniere's Disease (MD) compared to the normal-hearing people. Methods: In this study, 33 people aged 32-60 years with unilateral MD and 38 normal-hearing people aged 20-46 years participated. The ANL growth was evaluated unilaterally at four different speech presentation levels: Most Comfortable Level (MCL), -10 dB lower than the MCL, +10 dB higher than the MCL, and a range between MCL and uncomfortable level. Results: In MD patients, the ANL of the affected ear was significantly different from that of the contralateral ear (p<0.010). Compared to the normal-hearing people, the pattern of ANL growth in MD patients was significantly different, but the ANL growth patterns were similar between the affected and contralateral ears. Conclusion: ANL and its growth pattern are different between the affected and contralateral ears of patients with unilateral MD, but are similar compared to normal-hearing people. Despite the apparently normal hearing thresholds, the performance of the contralateral ear in noise at various speech presentation levels in patients with unilateral MD seems to be similar to that of the affected ear. [ABSTRACT FROM AUTHOR]

Vertigo/dizziness is a subjective sensation of rotation, usually peripheral in origin, which could be physiological or pathological. It needs to be differentiated from imbalance or disequilibrium, which usually has a central or cervicogenic etiology, and also from other vertigo mimics like presyncope or seizure disorder. Vertigo/dizziness is a symptom whose etiology can be multifactorial with various clinical overlaps among different vertigo syndromes due to multiple levels of involvement in the vestibular pathway. Hence, an otolaryngologist faces a great challenge in finding the etiology. Localization of the exact site of vertigo can be challenging for many specialists. Meniere disease, migraine, or benign paroxysmal positioning vertigo are the most common causes of recurrent vertigo. Posterior circulation ischemia or stroke can also cause vertigo or imbalance. This review discusses the role of thorough history, and clinical examination, which includes Dix-Hallpike test, other vestibular and auditory function tests and audiograms, imaging comprising of MRI of brain and spine, and high-resolution computed tomography scan of temporal bone. The current review also describes cervicogenic vertigo, which is treated and studied mainly by physiotherapists, with lack of clarity of this entity among otolaryngologists and other specialists. The multidisciplinary approach for vertigo entails active participation of a team comprising of otolaryngologists, physiotherapists, general physicians, neurologists, orthopedician, and neurosurgeons, based on clinical features and investigations, is described in the review. [ABSTRACT FROM AUTHOR]

Genetic sensorineural hearing loss and Meniere disease have been associated with rare variations in the coding and non-coding region of the human genome. Most of these variants were classified as likely pathogenic or variants of unknown significance and require functional validation in cellular or animal models. Given the difficulties to obtain human samples and the raising concerns about animal experimentation, human-induced pluripotent stem cells emerged as cellular models to investigate the interaction of genetic and environmental factors in the pathogenesis of inner ear disorders. The generation of human sensory epithelia and neuron-like cells carrying the variants of interest may facilitate a better understanding of their role during differentiation. These cellular models will allow us to explore new strategies for restoring hearing and vestibular sensory epithelia as well as neurons. This review summarized the use of human-induced pluripotent stem cells in sensorineural hearing loss and Meniere disease and proposed some strategies for its application in clinical practice. [ABSTRACT FROM AUTHOR]

Purpose: To investigate the correlation between vestibular hydrops (VH), cochlearhydrops (CH), vestibular aqueduct non-visibility (VANV), and visually increased perilymphatic enhancement (VIPE) with the findings of pure-tone audiometry (PTA) in Meniere’s disease (MD) patients.Methods: In this cross-sectional study, 53 ears belonging to 48 patients were divided into two groups and evaluated. In group “MD patients,” there were 24 ears of 19 patients diagnosed with the definite MD (14 patients with unilateral and 5 patients withbilateral involvements). The “control group” consisted of 29 non-symptomatic ears belonging to patients diagnosed with unilateral sudden sensory-neural hearing loss or unilateral schwannoma. All the patients underwent 2 sessions of temporal bone MRI using the same 3T system: an unenhanced axial T1, T2, and 3D-FLAIR MRI, an intravenous gadolinium-enhanced axial T1 fat-sat, and 4 h after the injection, an axial 3D-T2 cube and 3D-FLAIR session. VH, CH, VANV, and VIPE were assessed. Subsequently, the correlation between EH indices and PTA findings (in three frequency domains of low, middle, and high) were evaluated, and the predictive value of MRI was calculated.Results: VH was significantly correlated with the hearing threshold in the low, middle, and high-frequency domains. CH was also correlated with the hearing threshold in the low and middle domains. Contrarily, VIPE was not associated with hearing thresholds, and VANV was only correlated with the hearing threshold in low frequencies.Conclusion: The grade of VH, CH, and VANV were significantly correlated with the hearing thresholds in PTA.

Ménière’s disease (MD) is a multifactorial disorder with typical symptoms of recurrent vertigo, tinnitus, fluctuating hearing loss, and sensations of ear fullness. This disease greatly reduces the quality of life for patients. Unfortunately, it is difficult to diagnose and predictthe prognosis using only diagnostic methods, including audiometry. Therefore, since the mid-2000s, various efforts have been made to directly identify endolymphatic hydrops (EH), a histologic hallmark of MD, through magnetic resonance imaging (MRI) of the inner ear. Various studies have revealed significant correlation among degree of EH on inner ear MRI, patient symptoms, and test results. Although there are some limitations, inner ear MRI is expected to be widely used for differential diagnosis of MD, recurrent low-frequency hearing loss, non-specific vertigo, and vestibular migraine. In addition, as an automated analysis system of EH using the convolutional neural network algorithm has been developed,the usefulness of inner ear MRI is increasing. This algorithm can generate results that are highly consistent with those generated by manual calculation and can do so more quickly. Although there are some limitations to be overcome, inner ear MRI is expected to be widely used for differential diagnosis of various EH-related diseases in the not-too-distantfuture.

Objective To elucidate all the aspects that neurosurgeons should know about the Ménière disease (MD). Methods Review of guidelines, books, and studies from 1933 to 2021, from basic to translational research, using human and animal endolymphatic sac (ES) tissue or cells, as well as reviews, case reports, and papers about surgical experience. This article is divided into three parts. In this last part, we review the MD. Results The MD is one of the most common pathologies in the ES. It was first described by Prosper Ménière in 1861 with its clinical triad: dizziness, tinnitus, and hearing loss. A lot of theories relating ES to the MD have been proposed. Some of them postulate that it is caused by a narrowing and shortening in the endolymphatic duct, and others relate it to severe inflammation on the ES. Mostly due to the lack of understanding of this pathology, the diagnosis is mainly clinical, despite histopathology being helpful to confirm the diagnosis. The treatment of the MD can be done in 3 different ways: pharmacological, nonpharmacological, and surgical. Conclusion The MD is one of the most common pathologies in the inner ear and has been largely studied over the years. The latest diagnosis guidelines must help in the classification and give better basis for diagnosis and treatment, which, despite not being curative yet, has improved over the years. Pharmacological treatment based on the possible etiologies, allied with proper diet and routine exercise, is showing promising results.

Objective:To evaluate the change in quality of life (QOL) of patients with Meniere's disease (MD) after treatment with migraine prophylaxis therapy.Methods:Patients with definite MD were given the Meniere's Disease Outcomes Questionnaire-Retrospective (MDOQ-R) after migraine prophylactic therapy to assess QOL. Changes in physical, emotional, and social parameters affected by MD were calculated, along with a global pre- and posttreatment QOL scores.Results:The MDOQ-R was given to 27 consecutive patients with definite MD. Patients who had at least an 18-month follow-up were included, resulting in 25 questionnaires. The mean change in QOL score was 25 ± 16 (range, -3 to 55), P = .02. Quality of life was improved in 23 (92%) of the respondents in every metric measured, unchanged in 1 (4%), and poorer in 1 (4%) of patients after migraine prophylaxis treatment.Conclusions:Majority of MD patients who had all failed diuretic therapy responded positively to medications used for migraine prophylaxis, as indicated by a significant improvement in QOL. This study may further suggest a correlation between the pathophysiologic basis of disease in MD and vestibular migraine. Patients with MD may be successfully managed with medications intended to treat migraine.

Purpose: To assess the effect of oral splint therapy on audio-vestibular symptoms in patients with Menière's disease (MD) and temporomandibular disorder (TMD). Methods: Retrospective case–control study. Treatment group: 37 patients with MD and TMD who received gnatological treatment. Control group: 26 patients with MD and TMD who had never received gnatological treatment. The number of vertigo spells in 6 months (primary endpoint), pure-tone audiometry average (PTA), MD stage, functional level, Dizziness handicap Index (DHI), Tinnitus handicap Index (THI) and Aural Fullness Scale (AFS) were compared at baseline and after 24 months according to groups. Analysis of Covariance was used to determine the treatment effect. Results: Groups were comparable for demographic, clinical data, baseline PTAs and the number of vertigo spells. Analysis of covariance showed a significant effect of gnathological treatment on number of vertigo spells ( η p 2 = 0.258, p < 0.001), PTA ( η p 2 = 0.201, p < 0.001), MD stage ( η p 2 = 0.224, p < 0.001), functional level ( η p 2 = 0.424, p < 0.001), DHI ( η p 2 = 0.421, p < 0.001), THI ( η p 2 = 0.183, p < 0.001), but not for AFS ( η p 2 = 0.005, p = 0.582). The treatment group showed vertigo control of class A in 86.5% and class B in 13.5% of patients. In the control group, vertigo control was of class A in 19.2% of patients and class B in 11.5%, class C in 30.8%, class D in 11.5%, class E in 19.2% and class F in 7.7%. Classes of vertigo control differed significantly (X2 test, p < 0.001). Conclusions: Oral splint therapy could represent a viable treatment in patients with TMD and uncontrolled MD disease. The effects are maintained at least after 2 years. [ABSTRACT FROM AUTHOR]

Objective: This study aimed to assess the clinical implications of delayed-acquisition post-gadolinium magnetic resonance imaging in identifying endolymphatic hydrops in Ménière's disease. Method: This study was a systematic review using Medline and Embase and following the Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines with predetermined criteria, namely Ménière's disease, post-gadolinium magnetic resonance imaging and endolymphatic hydrops. The Quality Assessment of Diagnostic Accuracy Studies-2 tool was used to assess bias. Results: Eleven studies were included; they all used 3T magnetic resonance imaging, with three-dimensional fluid-attenuated inversion recovery being the most common sequence. Intravenous gadolinium administration was more widely used compared with the intratympanic route. As for the timing of acquisition, 4 hours post-administration was universally used for the IV gadolinium and 24 hours was used for the intratympanic gadolinium. Despite patient-selection associated bias, all studies reported adequate visualisation of the endolymphatic spaces. Conclusion: The use of delayed-acquisition magnetic resonance imaging is increasingly supported in visualising the endolymphatic spaces in Ménière's disease. Although the accessibility of 3T magnetic resonance imaging questions its wider applicability, it is a promising tool for the near future. [ABSTRACT FROM AUTHOR]

Meniere Disease (MD) is an idiopathic inner ear disease with complex etiology and pathogenesis, which is still unclear. With the development in gene analysis technology, the genetic research of MD has attracted extensive attention, resulting in a large number of studies on the research of the relationship between human genes and MD. This paper aims to review the studies on this topic in recent years. The studies mainly focused on the genetics of familial MD and the correlation between MD and potentially related functional genes. The results of these studies have demonstrated the complexity and diversity of the pathogenesis of MD with both genetic and epigenetic alterations, suggesting that MD might be related to inflammation, immunity, aqua and ion balance in the lymphatic fluid, virus infection, metabolism, and abnormal function of nerve conduction. The finding of rare mutations in TECTA, MYO7A and OTOG genes and other genes such as CDH23, PCDH15 and ADGRV1 in the same families suggest that the integrity of the stereocilia and their interaction with the tectorial and otolithic membranes could be involved in the pathophysiology of familial MD. [ABSTRACT FROM AUTHOR]

Objective: To investigate gravity perception disturbance (GPD) in patients with Meniere disease (MD), we classified GPD type based on the results of the head‐tilt perception gain (HTPG) and the head‐upright subjective visual vertical (HU‐SVV) evaluated by the head‐tilt SVV (HT‐SVV) test in patients with unilateral MD. Methods: We conducted the HT‐SVV test on 115 patients with unilateral MD and 115 healthy controls. Among the 115 patients, the period from the first vertigo episode to the examination (PFVE) was known for 91 patients. Results: The HT‐SVV test classified 60.9% and 39.1% of patients with unilateral MD as GPD and non‐GPD, respectively. GPD was classified according to HTPG/HU‐SVV combinations as follows: Type A GPD (21.7%, normal HTPG/abnormal HU‐SVV), Type B GPD (23.5%, abnormal HTPG/normal HU‐SVV), and Type C GPD (15.7%, abnormal HTPG/abnormal HU‐SVV). As the PFVE became longer, patients with non‐GPD and Type A GPD decreased; however, those with Types B and C GPD increased. Conclusion: This study provides novel information on unilateral MD from the perspective of gravity perception by classifying GPD based on the results of the HT‐SVV test. This study's findings suggest that overcompensation for vestibular dysfunction in patients with unilateral MD exhibited by large HTPG abnormalities may be strongly associated with persistent postural‐perceptual dizziness. Level of Evidence: 3b [ABSTRACT FROM AUTHOR]

Vertigo and dizziness are among the most common reasons for patients to seek medical help and approximately 3.5 % of all emergency department visits are related to them. The acutely dizzy patient can present a diagnostic and management dilemma for emergency departments and physicians in charge of those patients. Hence, it is of great importance to distinguish the life-threatening conditions, which require acute treatment, from “benign”, self-limiting conditions. While the traditional approach to dizziness relies on “symptom quality” or “type”, the new approach is based on symptom timing, triggers, duration, associated symptoms and patient history. Based on timing and triggers, this new approach divides patients into three key categories: acute vestibular syndrome, spontaneous episodic vestibular syndrome and triggered episodic vestibular syndrome. The acute vestibular syndrome (AVS) is defined as the acute onset of persistent dizziness associated with nausea or vomiting, gait instability, nystagmus, and head-motion intolerance lasting longer than 24 hours. AVS is provoked by a variety of central and peripheral causes, the most common of which are vestibular neuritis and acute stroke (posterior circulation). The HINTS (Head Impulse, Nystagmus, Test of Skew) plus protocol is a powerful tool for distinguishing a possible posterior stroke from peripheral vestibular dysfunction and it has been highly recommended recently. The most common causes of peripheral vertigo are benign paroxysmal positioning vertigo (BPPV), vestibular neuritis and Ménière’s disease. In this article we bring a review of new approaches to vertigo which contribute to faster and easier differentiation of benign and life-threatening conditions. A review of the most common types of peripheral vertigo is also included. [ABSTRACT FROM AUTHOR]

Background: Meniere's disease arises when an abnormal fluid accumulation results in heightened pressure within the inner ear or labyrinth. Its symptoms encompass vertigo, tinnitus, hearing loss, and a sensation of fullness in the ear. Various triggers for Meniere's disease are known, from smoking and alcohol consumption to recent viral illnesses, allergies, and anxiety. Meniere's disease presenting as a postinfectious inflammatory condition secondary to parasitic disease is unknown., Case Presentation: A 37-year-old Hispanic male patient, native to southern Honduras, presented with dizziness and tinnitus. These symptoms progressed to include left-sided sensorineural hearing loss. The patient was clinically diagnosed with Meniere's disease and referred to an otolaryngologist. Laboratory work-up revealed a positive result for the anti-heat shock proteins antibody and abnormal electrocochleography, more prominent on the left than the right. His vestibulonystagmogram displayed both central and peripheral findings, and bithermal caloric irrigations suggested abnormal peripheral function with a 57% weakness in the left ear. Despite dietary and lifestyle modifications, as well as treatment with oral steroids, his symptoms persisted. More detailed history revealed that he had been treated for a tapeworm infection at the age of 14 years in his Honduran village. Subsequent magnetic resonance imaging scans of the brain highlighted postinflammatory calcification in the right parietal lobe and generalized volume loss, more substantial than what would be expected for the patient's age. The patient was also referred to rheumatology and neurology, where he was diagnosed with neurocysticercosis., Conclusion: To the authors' knowledge, this is the first report suggesting that Meniere's disease could be possibly triggered by or concurrent with neurocysticercosis. This highlights the need for a comprehensive examination to identify any coexisting neurological conditions in patients presenting with Meniere's disease., Competing Interests: Declarations. Ethics approval and consent to participate: Consent was obtained from patient whose medical presentation is featured in this report. His identity is fully anonymized. Consent for publication: Written informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal. Competing interests: The authors declare that they have no competing interests., (© 2025. The Author(s).)

Meniere disease (MD) is a rare set of conditions associated with the accumulation of endolymph in the cochlear duct and the vestibular labyrinth with a decrease of endocochlear potential. It is considered a chronic inflammatory disorder of the inner ear with a multifactorial origin. The clinical syndrome includes several groups of patients with a core phenotype: sensorineural hearing loss, episodes of vertigo, and tinnitus with a non-predictable course. Genetic factors and the innate immune response seem to play a central role in the pathophysiology of the condition. Autoimmune MD should be diagnosed if a patient fulfills the diagnostic criteria for MD and one of the following autoimmune disorders: autoimmune thyroid disease, psoriasis, autoimmune arthritis, ankylosing spondylitis, or systemic lupus erythematosus. We summarize the evidence to support autoimmune MD as an endophenotype in bilateral MD associated with the allelic variant rs4947296 and nuclear factor-kappa B (NF-κB)-mediated inflammation, the role of cytokines (particularly interleukin-1β and tumor necrosis factor-α) in defining a subset of patients with autoinflammation, and the potential role of cytokines as biomarkers to distinguish between patients with MD and vestibular migraine. Finally, we also introduce a list of potential drugs that could regulate the immune response in MD with potential for repurposing in clinical trials.

Purpose: To summarized the latest evidence of risk factors for developing MD. Methods: We searched Medline, the Cochrane Central Register of Controlled Trials (CENTRAL), Embase, CBM, VIP, WanFANG, and CNKI, and ClinicalTrials.gov. till June 2021 for cohort and case–control studies investigating risk factors for MD. The exposure group was participants with a clinical diagnosis of MD which was made according to the diagnostic scale of the Equilibrium Committee of the American Academy of Otolaryngology-Head and Neck Surgery (AAO-HNS), the control group was participants without MD. The outcome was determined by incidence or prognostic of MD. Paired reviewers independently screened citations, assessed bias risk of included studies using the Newcastle–Ottawa Scale. Odds ratios (OR), hazard ratios(HR), relative risk(RR) and 95% confidence interval (CI) were calculated for dichotomous data. The statistical analyses were carried out with the use of Review Manager 5.3. The level of statistical heterogeneity for pooled data were assessed by using I2 statistics and Q-test. Results: 768 abstracts and articles were identified by our search, of which 25 studies (n = 1, 471, 944) were included. There were 18 cohort studies, 7 case–control studies. Three distinct subgroups (age, sex, sleep) were identified. There were a total of two studies involving age, two studies involving gender and two studies involving sleep disorder. High to moderate methodological quality established that age [hazard ratios (HR) 2.21, 95% CI 1.85–2.65, I2 = 0%] and sleep disorder[HR 1.68, 95% CI 1.47–1.93, I2 = 0%] were risk factors for MD. While there was little evidence showing that sex was not a risk factor for MD [HR 1.61, 95% CI 0.91–2.84, I2 = 74%]. Conclusion: The current evidence supports the suggestion that age and sleep disorder are risk factors for MD. Sex, gene, and hypothyroidism are tentative risk factors but conflicting/inconclusive results. Funding: No external funding. Registration: CRD42021248199 (Prospero). [ABSTRACT FROM AUTHOR]

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% perminute, themembrane 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. [ABSTRACT FROM AUTHOR]

Objectives The aims of this study were to investigate the effects of intratympanic injections of isosorbide on vestibular function in animal models of endolymphatic hydrops and to find a new treatment option for the acute onset of vertigo in Ménière disease (MD). Methods Seventy male guinea pigs received intratympanic injection of isosorbide (IT-ISB). The animals were divided into three study groups: control, a chronic hydrops model, and an acute hydrops model. Intracochlear drug concentrations were measured using high-performance liquid chromatography. Vestibular function was analyzed using an animal rotator test with bidirectional sinusoidal harmonic acceleration before and after IT-ISB administration. Histological changes were also investigated. Results ISB successfully permeated the perilymph through the round window membrane (RWM) at all three concentrations (25%, 50%, and 100%). In the chronic hydrops model, while IT-ISB histologically induced a reduction of endolymphatic hydrops, vestibular function was unchanged. In the acute hydrops model, no endolymphatic hydrops was histologically observed, and vestibular symmetry was also preserved after IT-ISB. Conclusion ISB passed through the RWM into the perilymphatic space even at lower concentrations. IT-ISB histologically reduced hydrops in the chronic model and preserved symmetrical vestibular function in the acute model. IT-ISB could be a treatment candidate for acute attacks of vertigo in MD.

ObjectiveMal de debarquement syndrome (MdDS) is a balance disorder that typically starts after an extended exposure to passive motion, such as a boat or plane ride. Management is typically supportive (e.g. physical therapy), and symptoms that persist beyond 6 months have been described as unlikely to remit. This study was conducted to evaluate the response of patients with MdDS to management with migraine prophylaxis, including lifestyle changes and medical therapy.Study designProspective review.SettingAmbulatory setting at a tertiary care medical center.MethodsClinical history, detailed questionnaires, and audiograms were used to diagnose patients with MdDS. Those patients with the diagnosis of the MdDS were placed on our institutional vestibular migraine management protocol. Treatment response was assessed with a quality-of-life (QOL) survey and visual analog scale.ResultsFifteen patients were diagnosed with MdDS, with a predominance of females (73%) and a mean age of 50 ± 13 years. Eleven patients (73%) responded well to management with a vestibular migraine protocol, which included lifestyle changes, as well as pharmacotherapy with verapamil, nortriptyline, topiramate, or a combination thereof. In comparison, a retrospective control group of 17 patients demonstrated a lower rate of improvement when treated with vestibular rehabilitation and physical therapy.ConclusionManagement of MdDS as vestibular migraine can improve patients' symptoms and increase the QOL. Nearly all the patients suffering from MdDS had a personal or family history of migraine headaches or had signs or symptoms suggestive of atypical migraine.Level of evidence4 Laryngoscope, 127:1670-1675, 2017.

A clinical diagnosis of Ménière’s disease (MD) is made based on medical history and audiometry findings. The 1995 American Academy of OtolaryngologyHead and Neck Surgery (AAO-HNS) guidelines requires histopathological confirmation of endolymphatic hydrops (EH) for a diagnosis of “certain” MD. Symptoms such as dizziness and ear fullness are important diagnostic features; however, the descriptions provided by patients are frequently vague and nonspecific. A recently developed magnetic resonance imaging (MRI) protocol to document EH is, therefore, useful for the evaluation of inner ear status in patients with MD. In this study, patients with MD were assessed using MRI and the HYDROPS (HYbriD of Reversed image Of Positive endolymph signal and native image of positive perilymph Signal) protocol to investigate the eectiveness of MRI for visualization of the endolymphatic space in the diagnosis of MD by correlating clinical laboratory parameters with the grade of EH. Of the 123 patients with MD recruited in this study, 80 had definite MD, 11 had probable MD, and 32 had possible MD based on the 1995 AAOHNS guidelines. The EH grade based on HYDROPS MRI was determined independently by two otorhinolaryngologists and compared with several clinical parameters, including the diagnostic scale of MD (1995 AAO-HNS guidelines), pure tone average (PTA), low tone average (LTA), canal paresis (CP) on the caloric test, and disease duration. Cochlear hydrops and vestibular hydrops were detected in 58 and 80% of 80 definite MD ears, in 33 and 58% of 12 probable MD ears, and in 5 and 27% of 37 possible MD ears, respectively. The proportion of higher hydrops grades increased significantly with grade according to the MD diagnostic scale (p < 0.0001). Both PTA and LTA were significantly higher in patients with hydrops grade 2 than hydrops grade 0 in both the cochlea and the vestibule. CP was significantly higher in patients with grade 2 than grade 0 vestibular hydrops. Disease duration was not associated with hydrops grade. Radiological evaluation of MD using the HYDROPS protocol is useful for evaluation of the extent and severity of EH in the diagnosis of MD based on its pathophysiological mechanism. [ABSTRACT FROM AUTHOR]

Aircraft accidents caused by dizziness of aviation workers are so common that they account for 5% to 10% of the total, and in most cases, have fatal consequences. Therefore, it is essential to diagnose and treat the dizziness of aviation workers properly. Common diseases caused by abnormal vestibular function include benign paroxysmal positional vertigo, Meniere’s disease, and vestibular neuritis. In this paper, we first proposed an overview of these diseases’ pathophysiology, diagnosis, and treatment. Also, we evaluated the importance of these diseases in aerospace medicine and presented aerospace medical dispositions for aviation medical examiners.

The ultrastructural organization of the blood labyrinthine barrier (BLB) was investigated in the human vestibular endorgan, the utricular macula, using postmortem specimens from individuals with documented normal auditory and vestibular function and surgical specimens from patients with intractable Meniere's disease. Transmission electron microscopic analysis of capillaries located in the normal human utricular stroma showed vascular endothelial cells with few pinocytotic vesicles, covered by a smooth and uniform basement membrane surrounded by pericyte processes. Meniere's disease specimens revealed differential ultrastructural pathological changes in the cellular elements of the microvasculature. With moderate degeneration of the BLB, there were numerous vesicles within the vascular endothelial cells (VECs), with increased numbers at the abluminal face, pericyte process detachment and disruption of the perivascular basement membrane surrounding the VECs. With severe degeneration of the BLB, there was severe vacuolization or frank apparent necrosis of VECs and loss of subcellular organelles. A higher severity of BLB degenerative changes was associated with a higher degree of basement membrane thickening and edematous changes within the vestibular stroma. This study presents the first ultrastructural analysis of the capillaries constituting the BLB in the human vestibular macula utricle from normal and Meniere's disease.

COVID-19 is a disease caused by a new type of SARS virus. COVID-19 clinical symptoms are primarily related to the respiratory system but may also be involved in many others, including the nervous system. Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) can be an aetiological factor responsible for balance disorders. Symptoms such as vertigo may coexist with other symptoms or be an isolated manifestation of the disease. Among other balance dysfunctions associated with COVID-19 are BBPV (benign paroxysmal positional vertigo), vestibular neuritis, acute labyrinthitis, acute cerebellitis, as well as Ménière’s disease. MRI, Videonystagmographic (VNG) and the Dix-Hallpike test are used to make a diagnosis. Improvements were observed after treatment with oral corticosteroids and vestibular physiotherapy along with intratympanic injection of dexamethasone. Antiemetics, antihistamines, anticholinergics and benzodiazepines drugs may be helpful in relieving symptoms. In addition, drugs used in scheduled treatment of COVID-19, such as azithromycin and hydroxychloroquine, may cause balance disorders due to their potential side effects. The following article presents cases of COVID-positive patients with balance disorders. The aim of this article was to raise awareness that nonspecific symptoms such as balance disorders may be the only manifestation of COVID-19, which is important due to early diagnosis and administration of treatment. [ABSTRACT FROM AUTHOR]

ObjectivesIn evaluating the historical context of the first description of Menière's disease, its association with migraine headaches is compelling. We have outlined the events and observations of Prosper Menière, which led him to establish a link between migraine headaches and his eponymous disease.Study designProsper Menière's original French writings were translated by our group and used to recount his observations and thoughts. Miles Atkinson's English translations were used as a reference. Additional otological texts of the era were also reviewed as it relates to Menière's disease.MethodsProsper Menière wrote a series of four articles 1 year before his death. In one of these articles, he makes references to migraine headaches on several occasions. These original writings were analyzed, and the physical findings he described were interpreted based on their relation to migraine headaches.ResultsThe passages in his published articles provide historical insight into Menière's observations. His writings describe in detail symptoms of migraine headaches uniquely evident in his patient population. Through his observations, he recognized that in addition to exhibiting symptoms of tinnitus, hearing loss and vertigo his patients also suffered from migraine headaches.ConclusionsAlthough his colleagues discounted Menière's theory concerning migraine headaches, he continued to make deductive inferences and publish his findings, leading to the association of migraine headaches and Menière's disease. Today, this association continues to be debated, adding to Prosper Menière's legacy.

Meniere's Disease (MD) is an inner ear disorder characterized by spontaneous recurrent vertigo, fluctuating sensorineural hearing loss, aural fullness and low-pitch tinnitus. Therapeutic management of MD includes dietary restriction and medical therapy. A minority of cases is characterized by frequent vertigo attacks, progressive hearing loss and persistent tinnitus even through the continuous medical treatments; this condition is called intractable MD and requires a therapeutic escalation from non-invasive medical treatment to surgical intervention. Invasive procedures include endolymphatic sac surgery, vestibular nerve section and labyrinthectomy. These procedures have a very high success rate on symptom control but may have a severe impact on the hearing function. However, the simultaneous combined approach of demolitive surgery and cochlear implantation may be a valid approach to treat symptoms of intractable MD and preserve hearing function. In the present study, we review current literature focusing on intractable MD to describe and discuss advantages and disadvantages of established and newly proposed surgical treatments for intractable MD. [ABSTRACT FROM AUTHOR]

Purpose: Heavily T2-weighted 3D FLAIR (hT2w-3D-FLAIR) sequence with constant flip angle (CFA) has been reported as being more sensitive to low concentrations of gadolinium (Gd) enabling endolymphatic hydrops (EH) visualization. The purpose of this study was to compare signal-to-noise (SNR) ratio, detection rate of EH, and increased perilymphatic enhancement (PE) as well as diagnostic accuracy in diagnosing definite Menière's disease (MD), using 3D-SPACE FLAIR versus conventional 3D-TSE FLAIR. Methods: This retrospective study included 29 definite MD patients who underwent a 4-h delayed intravenous (IV) Gd-enhanced 3D-TSE FLAIR and 3D-SPACE FLAIR MRI between February 2019 and February 2020. MR images were qualitatively and quantitatively analyzed twice by 2 experienced head and neck radiologists. Qualitative assessment included grading of cochlear and vestibular EH and visual comparison of PE. Quantitative assessment of PE was performed by placing a region of interest (ROI) and ratio calculation in the basal turn of the cochlea and the brainstem. Results: The intra- and inter-reader reliability for grading of EH and PE was excellent (0.7 < kappa < 0.9) for 3D-SPACE FLAIR and exceeded the values for 3D-TSE FLAIR (0.5 < kappa < 0.9) The combination of EH and visual assessment of PE has the highest diagnostic accuracy in diagnosing definite MD on 3D-SPACE FLAIR with a sensitivity of 0.91 and a specificity of 0.98 resulting in a sensitivity raise of 6% compared to 3D-TSE FLAIR. Conclusion: Four-hour delayed IV Gd-enhanced 3D-SPACE FLAIR sequence has a higher sensitivity and reproducibility than 3D-TSE FLAIR for the visualization of EH and increased PE in definite MD patients. [ABSTRACT FROM AUTHOR]

Objectives: 3D-fluid attenuation inversion recovery (FLAIR) collected 4 h after intravenous gadolinium injection can delineate the perilymphatic space (PLS) from the endolymphatic space (ELS) to capture endolymphatic hydrops, the pathological counterpart of Ménière's disease. We aimed to optimize visualization of such inner ear internal anatomy using 3D-FLAIR without injection. Methods: 3D-FLAIR signal from different fluid compartments such as PLS and ELS was first simulated. Then, twenty-two healthy subjects were scanned at 3.0-T MRI with non-injected 3D-FLAIR using variable T2 preparations (T2Preps) (OFF, 200, 400, and 600 ms) and variable inversion times (TIs) (from 224 to 5000 ms) and different resolutions (1.0 × 1.0 × 1.5, 0.6 × 0.6 × 0.8, and 0.6 × 0.6 × 0.6 mm3). The relative contrast between PLS and ELS and the visibility of the saccule and utricle were assessed. Additionally, non-injected 3D-FLAIR with the optimal setting was tested in a Ménière patient and compared with gadolinium-injected 3D-FLAIR. Results: The PLS and ELS were differentiated when T2Prep was used but not without. The relative contrast was larger with T2Prep at 400 ms than at 200 or 600 ms (0.72 ± 0.22 vs. 0.44 ± 0.11, p = 0.019; and 0.72 ± 0.22 vs. 0.46 ± 0.28, p = 0.034, respectively). The saccule and utricle were best delineated in 87. % cases with T2Prep = 400 and TI = 2100 ms at the highest resolution. Visualization of the saccule and utricle in the optimized non-injected 3D-FLAIR was similar to conventional injected 3D-FLAIR in a patient. Conclusions: Combining a specific T2Prep and TI in non-injected 3D-FLAIR could separate PLS and ELS and even the saccule and utricle, paving the way toward future application to diagnose Ménière's disease. Key Points: • MRI can capture the internal anatomy of inner ear without injection of contrast media. • Specific parameters consisting of a T2 preparation of 400 ms and an inversion time of 2100 ms must be used to visualize the saccule and utricle on non-injected 3D-FLAIR. [ABSTRACT FROM AUTHOR]

Background: Both vestibular schwannoma (VS) and Meniere's disease (MD) patients underwent hydrops MRI to clarify the relationship between VS and endolymphatic hydrops (EH)., Methods: Eighty patients with VS or MD underwent an inner ear test battery followed by hydrops MRI, and were then divided into 3 groups. Group A comprised 58 MD patients (62 ears) with positive EH but negative VS. Group B included 18 VS patients (18 ears) with negative EH, while Group C consisted of 4 patients (4 ears) who had VS concomitant with EH. Another 14 MD patients who tested negative for EH on hydrops MRI were initially excluded from this cohort, but were later included for comparison., Results: The decreasing prevalence of EH at the cochlea, saccule and utricle in Group A was identified in 59 (95%), 42 (68%) and 40 (65%) ears, respectively, mimicking a declining sequence of abnormality rates running from audiometry (86%), cervical vestibular-evoked myogenic potential (cVEMP) test (55%) to the ocular (oVEMP) test (53%). However, such decreasing trend was not identified in Groups B and C. In Groups C and A combined, 4 (6%) of 62 EH patients had concomitant VS. Conversely, 4 (18%) of 22 VS patients in Groups C and B combined had concurrent EH., Conclusion: A very low (6%) rate of VS in EH patients indicates that VS in EH patients may be coincidental. In contrast, EH was identified in 18% prevalence of VS patients, mirroring the 22% prevalence of cochlear EH demonstrated in VS donors through histopathological studies., Competing Interests: Declaration of competing interest The authors declare that they have no conflict of interest., (Copyright © 2024 Formosan Medical Association. Published by Elsevier B.V. All rights reserved.)

Aviation medical examiners should always keep two principles in mind when evaluating patients with vertigo: Does this disease render him or her incapacitated? And does this disease recur? Meniere's disease accompanies vertigo and hearing symptoms such as hearing loss and tinnitus and is particularly important because it can render the patient incapacitated and has recurrent consequences. In this case, we intended to investigate the course of a patient who was evaluated with symptoms of dizziness and tinnitus and to review the patient's judgment result according to the principles of aeromedical evaluation.

Objectives/hypothesisTo better understand the features of migraine in Meniere's disease (MD).Study designRetrospective review of prospectively obtained surveys in an outpatient clinic of a tertiary medical center.MethodsDetailed questionnaires on headaches and dizziness were given to consecutive patients presenting with dizziness. The responses were verified by the clinician with the patient. The data, in addition to the clinical history and audiogram, were used to diagnose patients with migraine headaches and MD using criteria set by the International Headache Society (IHS) and the American Academy of Otolaryngology-Head and Neck Surgery, respectively. The prevalence of migraine-like symptoms in those patients with MD, who did not fit the diagnostic criteria for migraine, was evaluated.ResultsThirty-seven patients with definite MD were included. There was a predominance of females (female/male:26/11). Mean age of patients was 52 ± 14 years. Nineteen patients (51%) had migraine headaches. Fifteen patients fulfilled the criteria for definite vestibular migraine. Of those who did not fulfill the IHS migraine criteria, a majority had characteristics such as a family history of migraine, visual motion sensitivity, or lifelong motion sickness that were highly suggestive of a migraine disorder.ConclusionsA majority of patients with MD have migraine headaches as defined by the IHS. Sensitivity to visual motion, light and sound, head motion, smells, weather changes, or medication was present in 95% of all patients with definite MD and 82% of non-IHS migraine MD patients. This may suggest that MD may be an atypical variant of migraine.

Semicircular canal and otolith functions came to be evaluated recently, but comprehensive and comparative analysis of canal and otolith dysfunction in common vestibular disorders is lacking. We aimed to analyze the abnormal rates of canal and otolith function in common vestibular disorders. We enrolled 302 patients who were managed for 2 months in a dizziness clinic. Results of caloric, video head impulse test (vHIT), and cervical and ocular vestibular evoked myogenic potential (cVEMP and oVEMP) tests were analyzed and compared among various diagnoses. Vestibular disorders diagnosed included recurrent vestibulopathy (RV, 27%), vestibular migraine (VM, 21%), benign paroxysmal positional vertigo (BPPV, 17%), Meniere's disease (MD, 11%), vestibular neuritis (VN, 10%), orthostatic dizziness (7%), and central lesions (3%). Lateral canal dysfunction was found most in VN (100%) and less commonly in definite MD (75%), RV (46%) and definite VM (29%). Abnormal caloric results were more common than abnormal vHIT in all disorders. Otolith dysfunction was found more frequently than lateral canal dysfunction in most vestibular disorders except VN. An abnormal cVEMP was more frequent in definite MD than the other disorders. Isolated otolith dysfunction without lateral canal dysfunction was the most found in BPPV, followed by definite VM, RV, and definite MD in decreasing order. Various patterns of involvement in canal and otoliths were revealed in vestibular disorders, suggesting different pathogenesis. [ABSTRACT FROM AUTHOR]