UNASSIGNED: Three-tesla MRI with gadolinium-based contrast agents is important in diagnosing Ménière\'s disease. However, contrast agents cannot be used in some patients. By using the compositional difference between the inner ear endolymph and perilymph, we performed basic and clinical research focused on potassium ions and protein to find the optimal parameters for visualizing endolymphatic hydrops on MRI without contrast. We then examined the relationship between severity stage and visualization rate of endolymphatic hydrops.
UNASSIGNED: In phantom experiments simulating the endolymph and perilymph, we explored MRI parameters that could be used to separate endolymph from perilymph by gradually changing the inversion time. We then used these parameters to perform both new non-contrast MRI and contrast MRI on the same day in Ménière\'s disease patients, and we compared the visualization rates of endolymphatic hydrops under the two modalities. Fifty patients were selected from 478 patients with Ménière\'s disease of different severity stages; 12 patients had asthma and allergy to contrast agents.
UNASSIGNED: The higher the disease stage, the higher the endolymphatic hydrops visualization rate. The new non-contrast MRI gave significantly higher (p < .01) visualization rates of endolymphatic hydrops on the affected side in patients at Stage 3 or above than in Stages 1 and 2 combined.
UNASSIGNED: New non-contrast MRI with parameters focusing on the endolymph-perilymph difference in the density of protons surrounding the potassium ions and protein can produce images consistent with endolymphatic hydrops. We believe that this groundbreaking method will be useful for diagnosing Ménière\'s disease in patients.
UNASSIGNED: Clinical studies are at evidence level 3 in non-randomized controlled trials.

OBJECTIVE: To explore the microstate characteristics and underlying brain network activity of Ménière\'s disease (MD) patients based on high-density electroencephalography (EEG), elucidate the association between microstate dynamics and clinical manifestation, and explore the potential of EEG microstate features as future neurobiomarkers for MD.
METHODS: Thirty-two patients diagnosed with MD and 29 healthy controls (HC) matched for demographic characteristics were included in the study. Dysfunction and subjective symptom severity were assessed by neuropsychological questionnaires, pure tone audiometry, and vestibular function tests. Resting-state EEG recordings were obtained using a 256-channel EEG system, and the electric field topographies were clustered into four dominant microstate classes (A, B, C, and D). The dynamic parameters of each microstate were analyzed and utilized as input for a support vector machine (SVM) classifier to identify significant microstate signatures associated with MD. The clinical significance was further explored through Spearman correlation analysis.
RESULTS: MD patients exhibited an increased presence of microstate class C and a decreased frequency of transitions between microstate class A and B, as well as between class A and D. The transitions from microstate class A to C were also elevated. Further analysis revealed a positive correlation between equilibrium scores and the transitions from microstate class A to C under somatosensory challenging conditions. Conversely, transitions between class A and B were negatively correlated with vertigo symptoms. No significant correlations were detected between these characteristics and auditory test results or emotional scores. Utilizing the microstate features identified via sequential backward selection, the linear SVM classifier achieved a sensitivity of 86.21% and a specificity of 90.61% in distinguishing MD patients from HC.
CONCLUSIONS: We identified several EEG microstate characteristics in MD patients that facilitate postural control yet exacerbate subjective symptoms, and effectively discriminate MD from HC. The microstate features may offer a new approach for optimizing cognitive compensation strategies and exploring potential neurobiological markers in MD.

UNASSIGNED: Evaluate the quality of responses from Chat Generative Pre-Trained Transformer (ChatGPT) models compared to the answers for \"Frequently Asked Questions\" (FAQs) from the American Academy of Otolaryngology-Head and Neck Surgery (AAO-HNS) Clinical Practice Guidelines (CPG) for Ménière\'s disease (MD).
UNASSIGNED: Comparative analysis.
UNASSIGNED: The AAO-HNS CPG for MD includes FAQs that clinicians can give to patients for MD-related questions. The ability of ChatGPT to properly educate patients regarding MD is unknown.
UNASSIGNED: ChatGPT-3.5 and 4.0 were each prompted with 16 questions from the MD FAQs. Each response was rated in terms of (1) comprehensiveness, (2) extensiveness, (3) presence of misleading information, and (4) quality of resources. Readability was assessed using Flesch-Kincaid Grade Level (FKGL) and Flesch Reading Ease Score (FRES).
UNASSIGNED: ChatGPT-3.5 was comprehensive in 5 responses whereas ChatGPT-4.0 was comprehensive in 9 (31.3% vs 56.3%, P = .2852). ChatGPT-3.5 and 4.0 were extensive in all responses (P = 1.0000). ChatGPT-3.5 was misleading in 5 responses whereas ChatGPT-4.0 was misleading in 3 (31.3% vs 18.75%, P = .6851). ChatGPT-3.5 had quality resources in 10 responses whereas ChatGPT-4.0 had quality resources in 16 (62.5% vs 100%, P = .0177). AAO-HNS CPG FRES (62.4 ± 16.6) demonstrated an appropriate readability score of at least 60, while both ChatGPT-3.5 (39.1 ± 7.3) and 4.0 (42.8 ± 8.5) failed to meet this standard. All platforms had FKGL means that exceeded the recommended level of 6 or lower.
UNASSIGNED: While ChatGPT-4.0 had significantly better resource reporting, both models have room for improvement in being more comprehensive, more readable, and less misleading for patients.

BACKGROUND: Ménière\'s disease (MD) is a disorder of the inner ear that causes episodic bouts of severe dizziness, roaring tinnitus, and fluctuating hearing loss. To date, no targeted therapy exists. As such, we have undertaken a large whole genome sequencing study on carefully phenotyped unilateral MD patients with the goal of gene/pathway discovery and a move towards targeted intervention. This study was a retrospective review of patients with a history of Ménière\'s disease. Genomic DNA, acquired from saliva samples, was purified and subjected to whole genome sequencing.
RESULTS: Stringent variant calling, performed on 511 samples passing quality checks, followed by gene-based filtering by recurrence and proximity in molecular interaction networks, led to 481 high priority MD genes. These high priority genes, including MPHOSPH8, MYO18A, TRIOBP, OTOGL, TNC, and MYO6, were previously implicated in hearing loss, balance, and cochlear function, and were significantly enriched in common variant studies of hearing loss. Validation in an independent MD cohort confirmed 82 recurrent genes. Pathway analysis pointed to cell-cell adhesion, extracellular matrix, and cellular energy maintenance as key mediators of MD. Furthermore, the MD-prioritized genes were highly expressed in human inner ear hair cells and dark/vestibular cells, and were differentially expressed in a mouse model of hearing loss.
CONCLUSIONS: By enabling the development of model systems that may lead to targeted therapies and MD screening panels, the genes and variants identified in this study will inform diagnosis and treatment of MD.

UNASSIGNED: This study used a national insurance claims database to analyze trends in procedural management of Meniere\'s disease.
UNASSIGNED: Retrospective cohort analysis.
UNASSIGNED: Database study using United States inpatient and outpatient insurance claims submitted from January 2003 to December 2021.
UNASSIGNED: The Merative MarketScan Commercial and Medicare Claims Databases were queried for adults (≥18 years) with a diagnosis of Meniere\'s Disease according to International Classification of Diseases codes. Patients receiving procedures per Current Procedural Terminology codes for endolymphatic sac surgery, vestibular nerve section, labyrinthectomy, and intratympanic dexamethasone or gentamicin were identified. Temporal trends were analyzed by calculating annual percent change (APC) in the proportion of patients receiving procedures using Joinpoint regression.
UNASSIGNED: A total of 16,523 unique patients with MD receiving procedural management were identified. From 2003 to 2021, the proportion of patients managed with intratympanic dexamethasone increased (APC 1.76 [95% CI 1.53-1.98], P < .001). The proportion of patients receiving intratympanic gentamicin increased from 2003 to 2015 (APC 4.43 [95% CI 1.29-7.66], P = .008) but decreased from 2015 to 2021 (APC -10.87 [95% CI -18.31 to -2.76], P = .013). The proportion of patients receiving endolymphatic sac surgery (APC: -10.20 [95% CI -11.19 to -9.20], P < .001) and labyrinthectomy (APC: -6.29 [95% CI -8.12 to -4.42], P < .001) decreased from 2003 to 2021.
UNASSIGNED: From 2003 to 2021, there has been an increase in the use of intratympanic dexamethasone and a decrease in the use of intratympanic gentamicin, endolymphatic sac surgery, and labyrinthectomy for procedural management of Meniere\'s Disease.

OBJECTIVE: To design and validate a disease-specific quality of life instrument for Meniere\'s disease.
METHODS: We used a sequential process of expert input, patient focus groups, and analyses of responses to draft questionnaires to create a 24-item Meniere\'s disease quality of life (MenQOL) instrument. The MenQOL and the SF-36v2 were administered to a cohort of 50 patients with Meniere\'s disease and 60 comparison patients with tinnitus, vertigo, or hearing loss from other causes identified at a tertiary academic center. We performed exploratory factor analysis, Cronbach\'s α, between group comparisons of total MenQOL scores, and regression analyses between the MenQOL and SF-36v2 to evaluate the instrument\'s factor structure, internal consistency, face validity, and external validity. Segregation of the instrument into domains was assessed by exploratory factor analysis.
RESULTS: Exploratory factor analysis revealed that the MenQOL has a single domain. Cronbach\'s α = 0.914 indicated high internal consistency for the instrument as a whole. Mean MenQOL scores showing significantly worse quality of life among patients with Meniere\'s disease than comparison participants (52.5 ± 15.8 vs. 43.2 ± 12.6; p = 0.0051), indicating good construct validity. Significant inverse relationships in bivariate linear regressions between total MenQOL scores and SF-36v2 physical (slope = -0.94, p < 0.0001) and mental (slope = -1.16, p < 0.0001) composite scores showed acceptable concurrent validity.
CONCLUSIONS: We have described the initial development of the MenQOL, a simple, valid patient-reported outcome measure that, subject to further study, may be used to assess the effects of treatment on disease-specific quality of life in patients with Meniere\'s disease.
METHODS: 3 Laryngoscope, 2024.

OBJECTIVE: To evaluate the sensitivity and the specificity of summating potential (SP)/action potential (AP) area under the curve (AUC) ratio by a transtympanic electrode and a click stimulus (TT-CS), SP/AP AUC ratio by an extratympanic electrode and a click stimulus (ET-CS) and SP amplitude value by a transtympanic electrode and tone burst stimulus (TT-TBS) in regard of Ménière\'s disease (MD) diagnosis. This is the first study that compares SP amplitude value performed by a TT-TBS and the SP/AP AUC ratio performed by a TT-CS.
METHODS: Retrospective comparative study.
METHODS: Ninety-five patients met the inclusion criteria for electrocochleography (ECochG) testing in a tertiary care center.
METHODS: The sensitivity and specificity of our different ECochG protocols were calculated in regard of the diagnosis of MD.
RESULTS: The patients\' mean age was 54 years old (female predominance). The sensitivity and the specificity of SP/AP area ratio by a TT-CS were 88.5% and 70.0%, respectively. On the other hand, the sensitivity and specificity for the SP amplitude value by a TT-TBS were 60.0% and 55.6%, respectively. SP/AP area ratio by TT-CS was statistically better than SP amplitude value by TT-TBS to detect MD disease (P = .016). However, no difference was identified between SP/AP area ratio by ET-CS and SP amplitude value by a TT-TBS (P = .573).
CONCLUSIONS: SP/AP area ratio by click stimulation has higher sensitivity and specificity to detect MD compared to SP amplitude value by tone burst stimulation. ECochG would be extremely useful in the diagnosis of MD if we use the SP/AP area ratio (sensitivity: 88.5%); therefore, it changes the bad reputation of ECochG sensitivity using SP/AP amplitude ratio (sensitivity: 51.7%) for the diagnosis of MD.

BACKGROUND: Gentamicin is a vestibulotoxic antibiotic often used in patients with Ménière\'s disease for its vestibular ablative effects. Gentamicin\'s effect on the horizontal semicircular canal does not always correlate with the degree of vertigo control achieved by patients; its effect on the vertical semicircular canals remains unknown. We sought to examine the effect of intratympanic gentamicin on vertical semicircular canal function in patients with Ménière\'s disease using video head impulse testing.
METHODS: A retrospective case series was carried out at a tertiary academic center. Patients with Ménière\'s disease who received ≥1 intratympanic gentamicin injection from 2019-2022 and had video head impulse testing performed were included. Outcomes of interest were vertical semicircular canal function following intratympanic gentamicin, correlations between vertical semicircular canal function and horizontal semicircular canal function, and residual symptoms following injection.
RESULTS: Ten patients met inclusion criteria. Twenty percent had abnormal V-SCC function prior to any injection and 40% following the first injection. There was an association between abnormal vertical and horizontal semicircular canal function following the first intratympanic gentamicin injection, though the relationship did not reach statistical significance (p = 0.058). While patients with abnormal vertical semicircular canal function following the first injection were less likely to report ongoing vertigo attacks, the relationship was not statistically significant (p = 0.260).
CONCLUSIONS: Intratympanic gentamicin leads to changes in vertical semicircular canal function in at least a proportion of patients with Ménière\'s disease. Further study is required to better assess correlations between vertical semicircular canal function and symptom control following intratympanic gentamicin.

OBJECTIVE: To characterize the estimated prevalence and clinicodemographic features of Ménière\'s disease (MD) using current diagnostic criteria.
METHODS: A cross-sectional study was undertaken at our tertiary academic referral center. All patients seen in Otolaryngology clinic with ICD-10 diagnoses of MD, from January 1, 2013 to July 31, 2022 were identified. Chart review was undertaken to determine the estimated prevalence of MD meeting AAO-HNS diagnostic criteria. Clinicodemographic features were evaluated against a comparator group without MD seen in our health system.
RESULTS: Of 806 ICD-10 diagnoses of MD, we identified 480 MD cases meeting diagnostic criteria (168 definite). Mean age at presentation for MD cases was 49 years. Forty-seven percent of cases were male. A significantly higher proportion of MD cases than comparators were white (76% vs. 66%, p < 0.001). Mean time since MD symptom onset was 6.7 years, with a mean attack duration of 4.6 h; 7.5% of MD cases reported a positive family history, and 7% had bilateral disease. The odds of reporting migraine were significantly greater among MD patients than comparators (OR 1.74 [1.26-2.42]); the odds of having autoimmune conditions were lower (OR 0.45 [0.28-0.74]); and the odds of reporting allergies were no different (OR 0.96 [0.74-1.25]) versus comparator patients after controlling for demographic characteristics.
CONCLUSIONS: Among MD diagnoses, there is a low estimated prevalence of MD meeting diagnostic criteria, and an even lower prevalence of definite MD. Compared to a comparator group of patients seen for any disorder, patients with MD are more likely to be white, male, and have a history of migraine.
METHODS: 4 Laryngoscope, 134:3310-3315, 2024.

OBJECTIVE: Fluctuating sensorineural hearing loss (SNHL) has multiple etiologies, most commonly Ménière\'s disease (MD), recurrent sudden SNHL, and autoimmune inner ear disorders. Fluctuating SNHL has rarely been described as a symptom of spontaneous intracranial hypotension (SIH).
METHODS: A 39-year-old previously healthy female presented with \"Ménière\'s like\" symptoms responsive to steroid treatment, which worsened during the day and improved in the supine position. Conservative treatment for MD consisting of low salt and caffeine diet and betahistine medication yielded no improvement. Secondary revision of brain imaging scans showed signs indicative of SIH, and a spinal cerebrospinal fluid leak was ultimately found and treated by a novel technique of transvenous fistula embolization by means of Onyx® glue, leading to gradual clinical improvement and near-complete resolution of symptoms.
CONCLUSIONS: SIH should be considered as part of the differential diagnosis of fluctuating SNHL. Clinical and radiological features should be known and sought. We suspect that early diagnosis and treatment can lead to cure and prevent permanent auditory damage.