An overload of missense variants in the OTOG gene may drive a higher prevalence of familial Meniere disease in the European population.

Meniere disease is a complex inner ear disorder with significant familial aggregation. A differential prevalence of familial MD (FMD) has been reported, being 9-10% in Europeans compared to 6% in East Asians. A broad genetic heterogeneity in FMD has been described, OTOG being the most common mutated gene, with a compound heterozygous recessive inheritance. We hypothesize that an OTOG-related founder effect may explain the higher prevalence of FMD in the European population. Therefore, the present study aimed to compare the allele frequency (AF) and distribution of OTOG rare variants across different populations. For this purpose, the coding regions with high constraint (low density of rare variants) were retrieved in the OTOG coding sequence in Non-Finnish European (NFE).. Missense variants (AF < 0.01) were selected from a 100 FMD patient cohort, and their population AF was annotated using gnomAD v2.1. A linkage analysis was performed, and odds ratios were calculated to compare AF between NFE and other populations. Thirteen rare missense variants were observed in 13 FMD patients, with 2 variants (rs61978648 and rs61736002) shared by 5 individuals and another variant (rs117315845) shared by two individuals. The results confirm the observed enrichment of OTOG rare missense variants in FMD. Furthermore, eight variants were enriched in the NFE population, and six of them were in constrained regions. Structural modeling predicts five missense variants that could alter the otogelin stability. We conclude that several variants reported in FMD are in constraint regions, and they may have a founder effect and explain the burden of FMD in the European population.

Epidemiology and genetics of Meniere's disease.

PURPOSE OF REVIEW: This review discusses the recent developments on the understanding of epidemiology and genetics of Meniere's disease. RECENT FINDINGS: Meniere's disease has been shown to be associated with several comorbidities, such as migraine, anxiety, allergy and immune disorders. Recent studies have investigated the relationship between environmental factors and Meniere's disease such as air pollution, allergy, asthma, osteoporosis or atmospheric pressure, reporting specific comorbidities in East Asian population. The application of exome sequencing has enabled the identification of genes sharing rare missense variants in multiple families with Meniere's disease, including OTOG and TECTA and suggesting digenic inheritance in MYO7A . Moreover, knockdown of DTNA gene orthologue in Drosophila resulted in defective proprioception and auditory function. DTNA and FAM136A knockout mice have been studied as potential mouse models for Meniere's disease. SUMMARY: While it has attracted emerging attention in recent years, the study of Meniere's disease genetics is still at its early stage. More geographically and ethnically based human genome studies, and the development of cellular and animal models of Meniere's disease may help shed light on the molecular mechanisms of Meniere's disease and provide the potential for gene-specific therapies.

Course of Duration and Trigger Factors of Vertigo Attacks in Patients with Benign Recurrent Vertigo, Menière's Disease, or Vestibular Migraine.

INTRODUCTION: Benign recurrent vertigo (BRV), Menière's disease (MD), and vestibular migraine (VM) show many similarities with regard to the course of vertigo attacks and clinical features. In this paper, we elaborate on the decreasing frequency of vertigo attacks observed in a previous study from our group by exploring changes in the duration and trigger factors of vertigo attacks in patients with BRV, MD, or VM. METHODS: For this 3-year prospective cohort study in our tertiary referral center we recruited patients with a confirmed diagnosis of BRV, MD, or VM by a neurologist and otorhinolaryngologist in our center in 2015-2016. A study-specific questionnaire was used to assess the usual duration of vertigo attacks and their potential triggers every 6 months. Main outcome measures were changes in duration and trigger factors of vertigo attacks in the subgroups of patients with persisting attacks, which were analyzed using repeated measures logistic regression models. RESULTS: 121 patients were included (BRV: n = 44; MD: n = 43; VM: n = 34) of whom 117 completed the 3-year follow-up period and 57 (48.7%) kept reporting vertigo attacks at one more follow-up measurements. None of the diagnosis groups showed statistically significant shortening of attack duration at the subsequent annual follow-up measurements compared to baseline. At baseline, stress and fatigue being reported as triggers for attacks differed significantly between the three groups (stress: BRV 40.9%, MD 62.8%, VM 76.5%, p = 0.005; fatigue: BRV 31.0%, MD 48.8%, VM 68.8%, p = 0.003). In the VM group, a consistent reduction of stress and fatigue as triggers was observed up until the 24- and the 30-month follow-up measurements, respectively, with odds ratios (ORs) ranging from 0.15 to 0.33 (all p < 0.05). In the MD group, a consistent reduction of head movements as trigger was observed from the 24-month measurement onward (ORs ranging from 0.07 to 0.11, all p < 0.05). CONCLUSION: Our study showed no reduction in vertigo attack duration over time in patients with BRV, MD, and VM who remain to have vertigo attacks. In VM and MD patients with persisting vertigo attacks stress, fatigue and head movements became less predominant triggers for vertigo attacks.

Probabilities of Isolated and Co-Occurring Vestibular Disorder Symptom Clusters Identified Using the Dizziness Symptom Profile.

OBJECTIVES: Dizziness is among the most common reasons people seek medical care. There are data indicating patients with dizziness, unsteadiness, or vertigo may have multiple underlying vestibular disorders simultaneously contributing to the overall symptoms. Greater awareness of the probability that a patient will present with symptoms of co-occurring vestibular disorders has the potential to improve assessment and management, which could reduce healthcare costs and improve patient quality of life. The purpose of the current investigation was to determine the probabilities that a patient presenting to a clinic for vestibular function testing has symptoms of an isolated vestibular disorder or co-occurring vestibular disorders. DESIGN: All patients who are seen for vestibular function testing in our center complete the dizziness symptom profile, a validated self-report measure, before evaluation with the clinician. For this retrospective study, patient scores on the dizziness symptom profile, patient age, and patient gender were extracted from the medical record. The dizziness symptom profile includes symptom clusters specific to six disorders that cause vestibular symptoms, specifically: benign paroxysmal positional vertigo, vestibular migraine, vestibular neuritis, superior canal dehiscence, Meniere disease, and persistent postural perceptual dizziness. For the present study, data were collected from 617 participants (mean age = 56 years, 376 women, and 241 men) presenting with complaints of vertigo, dizziness, or imbalance. Patients were evaluated in a tertiary care dizziness specialty clinic from October 2020 to October 2021. Self-report data were analyzed using a Bayesian framework to determine the probabilities of reporting symptom clusters specific to an isolated disorder and co-occurring vestibular disorders. RESULTS: There was a 42% probability of a participant reporting symptoms that were not consistent with any of the six vestibular disorders represented in the dizziness symptom profile. Participants were nearly as likely to report symptom clusters of co-occurring disorders (28%) as they were to report symptom clusters of an isolated disorder (30%). When in isolation, participants were most likely to report symptom clusters consistent with benign paroxysmal positional vertigo and vestibular migraine, with estimated probabilities of 12% and 10%, respectively. The combination of co-occurring disorders with the highest probability was benign paroxysmal positional vertigo + vestibular migraine (~5%). Probabilities decreased as number of symptom clusters on the dizziness symptom profile increased. The probability of endorsing vestibular migraine increased with the number of symptom clusters reported. CONCLUSIONS: Many patients reported symptoms of more than one vestibular disorder, suggesting their symptoms were not sufficiently captured by the symptom clusters used to summarize any single vestibular disorder covered by the dizziness symptom profile. Our results indicate that probability of symptom clusters indicated by the dizziness symptom profile is comparable to prior published work on the prevalence of vestibular disorders. These findings support use of this tool by clinicians to assist with identification of symptom clusters consistent with isolated and co-occurring vestibular disorders.

[Vertigo and dizziness in child: a clinical analysis in 120 cases].

Objective: To investigate the disease composition, clinical features, diagnosis, and treatment characteristics of vertigo in children. Methods: A total of 120 children with vertigo diagnosed and treated in the Department of Otorhinolaryngology, Children's Hospital, Capital Institute of Pediatrics in Beijing from February 2018 to February 2022 were retrospectively analyzed to explore the clinical characteristics of common peripheral vertigo in children and to summarize the experience of diagnosis and treatment. Results: The etiological composition of 120 cases of vertigo in children are as follows: 63 (52.5%) cases of vestibular migraine of childhood (VMC), 19 (15.8%) of recurrent vertigo of childhood (RVC), 11 (9.2%) of probable vestibular migraine of childhood (PVMC), 10 (8.3%) of secretory otitis media (SOM), 6 (5.0%) of persistent postural-perceptual dizziness (PPPD), 4 (3.3%) of benign paroxysmal positional vertigo (BPPV), 2 (1.7%) of vestibular neuritis (VN), 2 (1.7%) of Meniere's disease (MD), 2 (1.7%) of inner ear malformation (IEM), and 1 (0.8%) of vestibular paroxysmal syndrome (VP).The major cause of vertigo in children of different ages was different. SOM was the most important cause in preschool children, followed by RVC and VMC; VMC was the most important cause in school-age children, followed by RVC; and MD and BPPV were exclusive found in adolescents. The incidence rate of PPPD was higher in adolescents than in preschool and school-age children. Children with vertigo had good prognosis in general. Conclusions: VMC, RVC and SOM are the most common causes in vertigo in children, and their proportion was different in different aged children. Transforming abstract feelings into specific information is the skill required for collecting medical history of children with vertigo. Considering the age and cooperation of children, appropriate hearing and vestibular examination techniques are recommended. We should pay more attention to the mental health of children with vertigo and their parents.

Single-cell immune profiling of Meniere Disease patients.

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 CD56dim 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.

Evidence for a left ear bias in incidence of Meniere's disease.

OBJECTIVE: To explore a side of lesion differences in Meniere's disease (MD). DESIGN: A retrospective review (2019-2021) was conducted of patients with definite MD, as defined by 2015 Bárány Society diagnostic criteria. Testing information included pure tone audiometry, tympanometry, and extra-tympanic electrocochleography (ECochG). Normative ECochG data from healthy subjects determined the 95% cut-off value for clinical abnormality. STUDY SAMPLE: 107 patients with definite MD were included in the study and 40 healthy controls. RESULTS: The review identified 75 patients with unilateral MD and 32 patients with bilateral MD according to their clinical histories. 79% of unilateral cases were found to have MD on the L ear. 94% of bilateral MD cases had L ears more affected than R ears. Objective ECochG testing indicated a greater incidence of elevated SP/AP area curve and amplitude ratios in L ears. On binomial testing, all results indicate a highly significant bias of MD to the L side. CONCLUSIONS: Unilateral MD appears more common on the L side than the R, suggesting that the disease process underlying MD is not symmetrical. MD also appears more common in females than males. It appears that there is a physiological asymmetry in the progression/cause of MD.

Evolution of geriatric Meniere's disease during the past two decades.

BACKGROUND: This study compared the incidence of Meniere's disease (MD) in the elderly aged >65 years between the first (2001-2010) and second (2011-2020) decades to investigate the evolution of geriatric MD. METHODS: Totally, 1605 and 2550 patients with definite MD were experienced at the neurotological clinic during the first and the second decades, respectively. All patients were divided into three groups by 30-year age band, namely elderly (aged 65-94 years), adult (aged 35-64 years) and young (aged 5-34 years) groups, and underwent an inner ear test battery. Factors relating to the incidence of MD during the past two decades were analyzed. RESULTS: The elderly MD group comprised 198 (12.4%) of 1605 MD cases during the first decade, and 463 (18.2%) of 2550 MD cases during the second decade, showing a significantly increased incidence of elderly MD. Correlation between annual life expectancy (x) in Taiwan and annual prevalence (y) of the elderly MD in relation to total MD cases revealed y = 0.023x - 1.660 via linear regression analysis. In contrast, the adult MD group significantly differed in terms of age and gender ratio, but not incidence, between the two decades. Conversely, the young MD group exhibited significantly decreased incidence from the first decade (22.3%) to the second decade (13.8%). CONCLUSION: Evolution of geriatric MD during the past two decades reveals an increased incidence of the elderly MD patients, likely due to increased life expectancy coupled with altered life style.

Recurrence Rate and Risk Factors of Recurrence in Benign Paroxysmal Positional Vertigo: a Single-Center Long-Term Prospective Study With a Large Cohort.

OBJECTIVE: This study aimed to assess the long-term recurrence rate and correlations between recurrence and potential risk factors in patients with benign paroxysmal positional vertigo (BPPV). DESIGN: A total of 548 consecutive patients who demonstrated typical posterior or horizontal BPPV between January 2010 and December 2012 were included in this prospective study. All patients were contacted by phone every 6 months for 5 years and were asked to revisit the clinic when they experienced positional vertigo to be reexamined for recurrence. Recurrence of BPPV was defined as having positional vertigo and nystagmus confirmed following a symptom-free period of at least 7 days after complete resolution. We assessed the 5-year recurrence rate of BPPV, and the time point of recurrence in all patients as well as the risk factors of BPPV recurrence, including the clinical characteristics, therapeutic results of BPPV, and various comorbidities. RESULTS: Among the 548 patients, 121 (22.1 %) had at least one recurrence. Of these, 78 patients (54.5%) had only one recurrence within 5 years, while 43 (45.5%) patients experienced two or more recurrences. A recurrence occurred within 1 year in 82 patients (67.8%). The Cox proportional hazard ratio analysis found that head trauma (p = 0.015), Meniere's disease (p = 0.016), the number of canalith repositioning procedures performed (p = 0.037), and the number of previous vertigo attacks (p = 0.038) were significant risk factors of BPPV recurrence as opposed to hypertension or hyperlipidemia. CONCLUSIONS: The recurrence rate of BPPV was 22.1% at 5 years after the initial treatment. About 70% of recurred patients had a recurrence within 1 year. Head trauma, ipsilateral Meniere's disease, the number of canalith repositioning procedures performed, and the number of previous vertigo attacks were significant risk factors of BPPV recurrence.

Prevalence of anxiety and depression in Meniere's disease; a comparative analytical study.

BACKGROUND: Psychological problems are of most significant issues in patients with Meniere's disease (MD). This study aimed to reveal the frequency of anxiety and depression in MD patients compared to healthy individuals and patients with benign positional paroxysmal vertigo (BPPV) referred to a university hospital. METHODS: This case-control study was conducted on individuals between 18 and 65 years old assigned to three groups: the control, MD, and BPPV groups. The data collecting instruments included a demographic information form, along with Beck Anxiety Inventory (BAI) and Beck Depression Inventory-II (BDI-II) questionnaires. Data were analyzed using SPSS software v.23, and the significance level was considered as p < 0.05. RESULTS: A total of 177 participants enrolled. The average scores of anxiety and depression and the severity of anxiety and depression were higher in MD and BPPV groups than in the control group (P < 0.001). The average anxiety score and anxiety severity in the MD group was higher than in the BPPV group (P < 0.001). However, in the case of depression, only severity was higher in the MD group (P < 0.001), and the average depression score showed no significant difference between MD and BPPV groups. After controlling for underlying variables, the impacts of MD (P < 0.001; regression coefficient = 16.5) and also BPPV (P = 0.025; regression coefficient = 4.6) on anxiety were significant compared to the control group. CONCLUSION: Our results suggest that the prevalence of anxiety and depression is higher in MD and BPPV patients than the healthy people, and MD has a higher effect on the incidence of depression and anxiety compared to BPPV.

Risk factors for Meniere disease: a systematic review and meta-analysis.

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).

Evolution of incidence of audiovestibular disorders during the pandemic COVID-19 period.

PURPOSE: Despite sporadic case reports describing hearing problems in patients with coronavirus disease 2019 (COVID-19), whether COVID-19 affects the audiovestibular system remains unclear. This study assessed the evolution of incidence of audiovestibular disorders during the pandemic COVID-19 period. METHOD: Three audiovestibular disorders namely, sudden sensorineural hearing loss (SSHL), autonomic dysfunction, and Meniere's disease (MD) were analyzed and compared from 2016 to 2020. RESULTS: The annual new cases at our clinic comprised overall 2107, 1997, 1984, 2068, and 1829 from 2016 to 2020, respectively, and the respectively annual cases of SSHL were 54, 46, 42, 45 and 38. Accordingly, annual incidences of SSHL in relation to overall cases of audiovestibular disorders were 2.6%, 2.3%, 2.1%, 2.2% and 2.1% from 2016 to 2020, respectively, exhibiting a non-significant difference (p > 0.05). In contrast, incidence of autonomic dysfunction in the year 2020 was 15.3%, which revealed significantly higher than 8.5-13.1% from 2016 to 2019 (p < 0.001). Restated, the incidence of autonomic dysfunction in 2020 displayed a significantly higher percentage than the other 4 years. Conversely, the incidence of MD in 2020 was 9.8%, showing a significant decline compared with the other 4 years (12.6-15.6% from 2016 to 2019, p < 0.001), CONCLUSION: Evolution of incidence of audiovestibular disorders during the pandemic COVID-19 period revealed increase in the incidence of autonomic dysfunction and decrease in that of MD, while incidence of SSHL remained unchanged from 2016 to 2020. Thus, the SARS-CoV-2 may less affect the audiovestibular system.

Vertigo without cochlear symptoms: vestibular migraine or Menière disease?

BACKGROUND: Menière's disease (MD) is an inner ear disorder due to raised endolymphatic pressure (hydrops), characterized by cochlear symptoms associated with episodic vertigo. In delayed hydrops, vertigo attacks begin long after the onset of a hearing loss. Few were published on MD in which the onset of vertigo precedes cochlear symptoms by several months. Vestibular migraine (VM) is also a cause of episodic vertigo and an association between migraine and MD was proposed. Purpose of our retrospective work was to assess clinical features associated with MD in which vertigo precedes by months cochlear symptoms. METHODS: Our sample was composed by 28 subjects reporting episodic vertigo accompanied by migrainous headache or photo-phonophobia, without cochlear symptoms at onset; during follow-up, all patients developed cochlear symptoms leading to a diagnosis of MD. Results of bedside examination were compared with those of 48 VM subjects with diagnosis of VM confirmed in the follow-up. All subjects performed a bedside examination, including head-shaking, positional, and skull vibration test (SVIN). RESULTS: SVIN was more frequent in MD, while positive positional test in VM. In the entire group of 72 subjects, migrainous headache during vertigo and positive positional test were correlated with a final diagnosis of VM. CONCLUSIONS: Our data are not inconsistent with the hypothesis that in patients reporting only photo-phonophobia during vertigo attacks and with a positive SVIN, the clinical manifestations may be predictive for evolution toward a MD, while migrainous headache and positive positional tests more frequently are correlated to VM.

High level of IgE in acute low-tone sensorineural hearing loss: A predictor for recurrence and Meniere Disease transformation.

BACKGROUND: Autoimmunity may play an important role in sudden onset sensorineural hearing loss. However, little is known about the relationship between immunoglobulin E (IgE) and acute low-tone sensorinerual hearing loss (ALHL). OBJECTIVES: To investigate the relationship between IgE level and endolymphatic hydrops and outcomes of ALHL. METHODS: A total of 242 subjects with sudden onset hearing loss, including 115 with ALHL and 127 with idiopathic sudden sensorineural hearing loss (ISSHL), were included in this study. Peripheral venous blood samples of 242 subjects were collected for detection. Clinical data, IgE level, and distribution of allergens were compared between the ALHL and ISSHL groups. The ALHL group received an electrocochleogram (ECochG) test and a follow-up in the outpatient unit or by telephone to evaluate outcomes. RESULTS: Compared to the values in the ISSHL group, a significantly younger onset age (42.30±14.33 years old), higher female onset proportion (72/115, 62.61%), increased total IgE level (median: 66.47, interquartile range: 24.56, 180.96, IU/mL) and specific IgE level (median: 9.42, interquartile range: 1.42, 22.23 IU/mL) were noted in the ALHL group. A clear difference in allergen distribution was noted between the ALHL and ISSHL groups (p=.001). Total IgE and specific IgE levels were factors that contributed to the SP/AP ratio in the electrocochleogram (ECochG) (R2=0.413) in ALHL group. Finally, during the follow-up (17.61±3.46 months) for the ALHL group, 37 subjects recurred, and 17 subjects developed Meniere Disease. In the ROC curve for ALHL recurrence, the area under the curve (AUC) of total IgE was 0.709 and that of specific IgE was 0.679. For MD transformation, the AUC of total IgE was 0.736 and that of specific IgE was 0.716. CONCLUSIONS: High IgE levels correlated with an enhanced SP/AP ratio in ALHL. High IgE levels could be used as a predictor of ALHL recurrence and MD transformation.

Thyroid Dysfunction in Ménière's Disease: A Comprehensive Review.

INTRODUCTION: The precise etiology of Ménière's disease (MD) remains unknown; however, given the association of MD with serum antibodies and human leukocyte antigen (HLA) complex, several studies have proposed a relationship between MD and thyroid disorders. Similarly, multiple hypotheses exist regarding the metabolic disturbances of fluctuating thyroid hormone as a potential contributing agent in the development of MD. METHODS: A total of 171 abstracts were identified and screened by 2 independent reviewers. Based on inclusion and exclusion criteria, 8 studies were selected for final analysis. Due to heterogeneity of clinical data, meta-analysis was not feasible. RESULTS: The prevalence of autoimmune thyroid disease and hypothyroidism in MD varied significantly from 1 to 38%. Notable bias was introduced given the lack of standardization of diagnostic criteria across studies. Articles that described autoimmune thyroid-specific antibodies and HLA types also presented inconclusive results. Multiple studies noted a potential etiologic role of hypothyroidism in MD, which was often confounded by thyroxine supplementation. CONCLUSIONS: Despite a potential correlation in the medical literature between thyroid disorders and MD, there is currently no definitive causal relationship. Although most of the present medical literature focuses on autoimmunity, dysregulated thyroid hormone levels may also be implicated in the association of MD with thyroid disorders.

Epidemiology and Seasonal Variation of Ménière's Disease: Data from a Population-Based Study.

INTRODUCTION: Ménière's disease (MD) is a disease of the inner ear. In Asian countries, there is one previous epidemiologic study that used a retrospective survey of a specific district. Previous studies have demonstrated that weather variables are strongly associated with symptom aggravation in MD. However, no study has reported on seasonal variation of MD. METHODS: We conducted a retrospective population-based study using the Korean national health insurance claims database (NHICD). In the present study, the prevalence and incidence of MD from 2013 to 2017 were calculated. Prevalence and incidence of MD according to sex, age group, and monthly incidence are presented. Seasonal incidence was calculated for four seasons. The seasonal average humidity and atmospheric pressure were also calculated. RESULTS: The incidence rate of MD showed a rapid yearly increase from 2013 to 2017 in Korea. The prevalence and incidence rate by age group continued to increase up to the 70 year olds. The female-to-male ratio was 2.17. The seasonal incidence of MD was relatively high in summer and autumn and relatively low in winter and spring; however, there were no significant differences. DISCUSSION/CONCLUSIONS: The rapid progression to an aging society, increase in medical accessibility, and lifestyle changes could be the reasons for the elevation of the incidence rate in the Korean population. Consistent with a previous study, the incidence of MD was higher in seasons with high humidity and low atmospheric pressure; however, there were no significant differences. Further studies about the relationship between diet and MD, seasonality of MD with long-term analysis, and investigating the underlying mechanisms connecting weather and MD are needed.

Alcohol consumption in Menière's disease patients.

Objectives: Dietary changes are useful in the management of Menière's disease; regarding alcohol, many clinicians recommend to avoid or reduce its consumption. However, there are no researches aimed to evaluate whether habitual alcohol consumption is more prevalent and/or more intense in patients with Menière's disease.Methods: Cross-sectional, observational, case-control study, including three groups: patients with Menière's disease, patients with vertigo of other origins, and control subjects. Alcohol consumption was compared between these three groups. Participants in this study were grouped according to alcohol consumption as follows: categorization A1 (nonalcohol vs. alcohol consumers), categorization A2 (nonalcohol, low, moderate, and high alcohol consumers), and categorization A3 (light alcohol consumers: nonconsumers plus low consumers; heavy alcohol consumers: moderate plus high consumers).Results: A total of 180 subjects were included in this study (72 in group A, 72 in group B, and 36 in control group); 117 were women. The mean age was 52.7 years. Mean alcohol consumption was 41.22 g/week. Average consumption of alcohol in group A (50.42 g/week) was higher than in other two groups (36.53 g/week in B and 32.22 g/week in C), but differences were not statistically significant. In Menière's group, light alcohol consumers showed age at onset of symptoms (49.39 years) lower than heavy alcohol consumers (55.51 years). No relationship was observed between alcohol consumption and uni or bilateral affectation.Discussion: It is possible that alcohol consumption delays the age at onset of Menière's disease. A hypothetical explanation is the inhibitory effect of alcohol on hypothalamic production of vasopressin. A reduced release of this neurohormone would increase diuresis and decrease endolymphatic pressure.

Comparison of associated comorbid conditions in patients with benign paroxysmal positional vertigo with or without migraine history: A large single institution study.

PURPOSE: In the largest reported United States (U.S.) cohort of benign paroxysmal positional vertigo (BPPV), does the presence or absence of migraine correlate with increased associations of other factors? MATERIALS & METHODS: A retrospective chart review from a single U.S. neurotology center. Adult patients with BPPV from 2003 to 2017 classified into two groups: 1) with migraine; 2) without migraine. RESULTS: The prevalence of self-reported migraine history in a BPPV-positive population (n = 1481) was 25.8% (n = 382). Among those with BPPV and migraine, 88.5% were female, 8.1% had diabetes, 22.5% had prior history of BPPV, 8.9% had Meniere's disease and recurrence rate was 38.3%. No statistically significant differences between recurrence or resolution rates between the two groups were demonstrated (p > 0.05). Adjusted logistic regression model found that women and those with a prior history of BPPV had significantly higher odds of having both BPPV and migraine conditions compared to their counterparts. In contrast, older age and diabetes were associated with significantly lower odds of concomitant BPPV and migraine. CONCLUSIONS: The prevalence of migraine among the study sample was 25.8%. Female gender, prior history of BPPV, younger age, and lack of diabetes were independently associated with the concurrent comorbidity of BPPV and migraine. These data further substantiate previously reported rates (from smaller studies); and, demonstrate that recurrence and resolution rates are not worse in those with BPPV and migraine compared to the general BPPV population. Findings support an association between migraine and BPPV and enhances one's ability to accurately counsel patients regarding these diseases.

Vestibular drop attacks in Ménière's disease and its association with migraine.

PURPOSE: We explored the association of vestibular drop attacks (VDA) with or without migraine in patients with Ménière's disease (MD) and compared with patients classified as vestibular migraine (VM). METHOD: The study involved a cross-sectional survey design. Data were collected from 401 members of Finnish Ménière Federation who experienced VDA. RESULTS: In the sample of 401 patients with VDA who did not experience headache, VM was diagnosed among 16.4% participants and migraine not associated with vertigo was experienced among 25.2% of the participants. Severity of postural instability, ability to move, and ability to stand up from chair differed among VDA baseline, VDA migraine and VM patient groups. Syncope associated with VDA was noticed more frequently in VM group. In terms of neurological complaints, except for visual problems, all other symptoms differed significantly between three patient groups. The health-related quality of life also differed between groups with VM group having the worse scores. CONCLUSIONS: The current study suggests that migraine is common in MD patients who experience VDA. We observed no definite complaint that could be ascertained to VM. The coexistence of migraine increased the impact of complaints associated with VDA.

Whole-exome sequencing suggests multiallelic inheritance for childhood-onset Ménière's disease.

The genetic background of Ménière's disease (MD) was studied in one patient with childhood-onset MD and his grandfather affected with middle age-onset MD. Whole-exome sequencing was performed and the data were compared to 76 exomes from unrelated subjects without MD. Thirteen rare inner ear expressed variants with pathogenic estimations were observed in the case of childhood-onset MD. These variants were in genes involved in the formation of cell membranes or the cytoskeleton and in genes participating in cell death or gene-regulation pathways. His grandfather shared two of the variants: p.Y273N in HMX2 and p.L229F in TMEM55B. HMX2 p.Y273N was considered the more likely candidate for MD, as the gene is known to affect both hearing and vestibular function. The variant in the HMX2 gene may affect inner ear development and structural integrity and thus might predispose to the onset of MD. As there was a significant difference in onset between the patients, an accumulation of defects in several pathways is probably responsible for the exceptionally early onset of the disease, and the genetic etiology of childhood-onset MD is most likely multifactorial. This is the first molecular genetic study of childhood-onset MD.