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Background: Neuro-otological factors that influence changes in spontaneous nystagmus (SN) during vertigo attacks in Ménière's disease (MD) remain unclear., Objective: To identify neuro-otological factors that might influence the initial direction of SN and the directional change of SN., Methods: A prospective, observational study of 22 patients with definite MD to evaluate the initial direction and directional change of SN during vertigo attacks, endolymphatic hydrops (EH) volume, and the function of horizontal semicircular canal and hearing levels., Results: SN consistently began as irritative in 17 of 22 cases, and 9 of 17 cases showed a definite change in direction after onset. SN consistently began as paralytic in 5 of 22 cases, and 3 of 5 cases showed a definite change in direction after onset. Subjects in the irritative initial SN group had less severe degrees of hearing loss, smaller cochlear and vestibular EH volume than the paralytic initial SN group (P = 0.017, < 0.001, and 0.009, respectively). Subjects in the SN direction change group had significantly smaller maximum slow phase velocity, percentage of caloric weakness and canal paresis than the no SN direction change group (P = 0.001, 0.006, and 0.001, respectively). Simple logistic regression analysis showed that smaller EH volume was significantly associated with initial irritative SN (OR = 0.867, 95% CI 0.762-0.988, P = 0.032) and that the degree of canal paresis was negatively associated with the presence of directional change of SN (OR = 0.022, 95% CI 0.002-0.289, P = 0.004)., Conclusions: The morphology of EH and canal paresis may independently affect the characteristics of SN in patients with MD., (© 2022. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany.)

The aim was to explore and characterize dizziness and vertigo (constant vs episodic) and associated problems in patients with Ménière's disease (MD) to allow characterization of the impact of the disease. The study used a retrospective survey design. A total of 539 people with MD participated in this study. The online questionnaire included 36 items which had mixture of structured and open-ended questions that were focusing on MD symptoms, impact of their symptoms, vestibular rehabilitation, as well as health-related quality of life (HRQoL). Forty-six percent of the patients had episodic vertigo, 6% had constant dizziness, 15% had both episodic vertigo and constant dizziness, and 31% did not have vertigo or dizziness within the last 2 years. Patients with MD without any vertigo rated their HRQoL as 73.9%, those with episodic vertigo as 71.1%, those with constant dizziness as 56.9%, and those with constant dizziness and episodic vertigo as 57.9% indicating significant reduction in HRQoL in constant dizziness patients. Constant dizziness was associated with cognitive visual problems, fatigue, balance problems, vestibular drop attacks and syncope. The impact of balance problem was more severe among those with constant dizziness. The most common balance problem was tripping-off (34%), followed by swaying (25%) or rocking (8%) sensations. In the self-administered rehabilitative training, there were no differences between any of the vertigo or dizziness groups although disease profile of MD differed significantly. We emphasize that constant dizziness in MD constitutes a long-term maladaptation to a vestibular and visual cognitive function causing cognitive dissonance. Different types of vertigo and their associated complaints require different treatment strategies to the manage balance problems and to cope with the disease, but best practices is still under research.

Background: This study aimed to investigate the relationship between the features of endolymphatic hydrops and hearing loss in patients with Bilateral Meniere's Disease., Methods: A retrospective analysis was conducted on 77 patients diagnosed with Bilateral Meniere's Disease. The features of endolymphatic hydrops in the affected ear were evaluated through gadolinium-enhanced inner ear Magnetic resonance imaging. The Spearman correlation coefficient, paired t-tests, and Wilcoxon signed-rank tests were employed for data analysis., Results: The analysis revealed a significant correlation between the degree of endolymphatic hydrops and hearing loss across all frequencies(0.125-8 kHz), including the cochlear, vestibular, and overall degree of endolymphatic hydrops. The strongest correlation between the overall degree of endolymphatic hydrops and hearing loss was observed at low frequencies (r = 0.571, p < 0.05), followed by mid-frequencies (r = 0.508, p < 0.05), and high-frequencies (r = 0.351, p < 0.05), with a correlation of r = 0.463, p < 0.05 for the staging of Meniere's disease. Affected Ears with endolymphatic hydrops both in the cochlea and vestibule exhibited more severe hearing loss and Meniere's disease staging compared to those with isolated endolymphatic hydrops within the same patient., Conclusions: The features of endolymphatic hydrops in patients with Bilateral Meniere's Disease were found to correlate with the severity of hearing loss and the staging of Meniere's disease., (© 2024. The Author(s).)

Objectives: To construct and validate a radiomics nomogram based on T2-sampling perfection with application-optimized contrasts using different flip-angle evolutions (SPACE) images for predicting cochlear and vestibular endolymphatic hydrops (EH) in Meniere's disease patients., Methods: A total of 156 patients (312 affected ears) with bilateral definite Meniere's disease who underwent delayed enhancement MRI scans were enrolled in this study. All ears of the patients were divided into a training set (n = 218) and an internal validation set (n = 94). A radiomics nomogram was constructed from radiomics features extracted from the T2-SPACE images, and a radiomics score was calculated. Performance of the radiomics nomogram was assessed using receiver operating characteristics analysis., Results: Five features were selected for the construction of the cochlear radiomics nomogram, and seven features for the vestibular radiomics nomogram. The radiomics nomograms exhibited robust performance in differentiating between EH-positive and EH-negative statuses in both training and validation cohorts, with the area under the receiver operating characteristics curve values for cochlear and vestibular radiomic nomograms being 0.703 and 0.728 in the training set, and 0.718 and 0.701 in the validation set, respectively., Conclusion: The novel radiomics nomograms based on T2-SPACE images were successfully constructed to predict cochlear and vestibular EH in Meniere's disease. The models showed a solid and superior performance and may play an important role in the EH prediction., Clinical Relevance Statement: We constructed a novel radiomics nomogram, which can be a very useful tool for predicting cochlear and vestibular endolymphatic hydrops in Meniere's disease patients., Key Points: • This is the first T2-SPACE-based nomogram to predict cochlear and vestibular endolymphatic hydrops. • The nomogram is of great value to patients who are unable to undergo delayed enhancement MRI scans., (© 2024. The Author(s), under exclusive licence to European Society of Radiology.)

Purpose: To compare the correlation between different grading methods of vestibular endolymphatic hydrops (EH) and the severity of hearing loss in Ménière's disease (MD), and evaluate the diagnostic value of these methods in diagnosing MD., Methods: This retrospective study included 30 patients diagnosed with MD from June 2021 to August 2023. All patients underwent inner ear MR gadolinium-enhanced imaging using three-dimensional (3D)-real inversion recovery sequences and pure-tone audiometry. The EH levels were independently evaluated according to the classification methods outlined by Nakashima et al. (Acta Otolaryngol Suppl 5-8, 2009. https://doi.org/10.1080/00016480902729827 ) (M1), Fang et al. (J Laryngol Otol 126:454-459, 2012. https://doi.org/10.1017/S0022215112000060 ) (M2), Barath et al. (Am J Neuroradiol 35:1387-1392, 2014. https://doi.org/10.3174/ajnr.A3856 ), (M3), Liu et al. (Front Surg 9:874971, 2022. https://doi.org/10.3389/fsurg.2022.874971 ), (M4), and Bernaerts et al. (Neuroradiology 61:421-429, 2019. https://doi.org/10.1007/s00234-019-02155-7 ) (M5), with a subsequent comparison of interobserver agreement. After achieving a consensus, an analysis was performed to explore the correlations between vestibular EH grading using different methods, the average hearing thresholds at low-mid, high-, and full frequencies and clinical stages. The diagnostic capabilities of these methods for MD were then compared., Results: The interobserver consistency of M2-M5 was superior to that of M1. The EH grading based on M4 showed a significant correlation with the average hearing thresholds at low-mid, high-, and full frequencies and clinical stages. M1, M2, M3, and M5 correlated with some parameters. A receiver operating characteristic curve analysis indicated that M5 significantly outperformed M1, M2, M3, and M4 in terms of diagnostic efficiency for MD., Conclusion: M4 showed the strongest correlation with the degree of hearing loss in patients with MD, whereas M5 showed the highest diagnostic performance., (© 2024. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

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., Competing Interests: The authors have no conflicts of interest to disclose., (Copyright © 2024 Wolters Kluwer Health, Inc. All rights reserved.)

Background: Therapeutic options are limited for patients with intractable Meniere's disease who present with recurrent episodes of vertigo and drop attacks., Aims/objectives: To investigate the effectiveness and safety of simultaneous semicircular canal plugging and vestibular plugging in the treatment of Meniere's disease with drop attacks., Material and Methods: This was a single-center study with a 6-month post-operative follow-up. It included five patients with intractable Meniere's disease who presented with recurrent vertigo and drop attacks. All patients underwent surgery of semicircular canal plugging and vestibular plugging. The main outcome measures were vertigo control, drop attack control, hearing, and vestibular function; they were evaluated pre- and post-operatively., Results: Of the five patients, none had a recurrence of drop attacks; four had no recurrence of vertigo, and one patient had one episode of vertigo. Two patients had residual hearing before surgery, which was preserved postoperatively. All patients showed altered results in vestibular tests., Conclusions: Semicircular canal plugging and vestibular plugging appear to be an effective treatment for intractable Meniere's disease presenting with recurrent vertigo and drop attacks. More studies are needed to confirm these results., Significance: This novel surgical procedure can control vestibular symptoms of Meniere's disease while hopefully preserving the hearing function.

Objective: We compared the signal intensity ratio (SIR) of the cochlear basal turn between Meniere's disease and healthy controls to investigate potential damage of the blood-labyrinth barrier in Meniere's disease., Methods: Thirty patients diagnosed with unilateral definite Meniere's disease and 24 healthy controls were enrolled. 3D-FLAIR scan was conducted to assess the grades of endolymphatic hydrops in Meniere's patients while measuring the SIR of cochlear basal turns in both groups. The differences of bilateral SIR between Meniere's disease and healthy control were compared, and the correlation between the SIR on affected ear in Meniere's disease and the grades of cochlear and vestibular hydrops were analyzed., Results: SIR of affected ear in Meniere's disease exhibited significant increase compared to that of unaffected ear. No significant difference was observed in SIR between the two ears in the healthy control. Furthermore, the SIR of unaffected side in Meniere's disease was higher than that of both ears in healthy controls. The SIR in affected ear of Meniere's disease exhibited positive correlation with hydrops in both cochlea and vestibula., Conclusion: The permeability of blood-labyrinth barrier is increased in Meniere's disease, in combination with the typical criteria of Meniere's disease it may be a good biological marker. Destruction of blood-labyrinth barrier may be one of the causes of endolymphatic hydrops in Meniere's disease., (© 2023. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Purpose: Meniere's disease (MD) is a disabling condition with symptoms, such as hearing loss, dizziness, and tinnitus. Surgery is the last resort option for managing MD when other treatments are not effective. Surgical labyrinthectomy (SL) is less commonly performed than vestibular neurectomy or chemical labyrinthectomy. We sought to assess the efficacy and safety of the SL procedure., Methods: A retrospective study was carried out involving a cohort of 25 patients with disabling and drug-resistant MD, who underwent SL performed by the same surgeon at University Hospital UCL Namur between 2009 and 2019. All patients considered their hearing non-functional and requested a radical therapeutic option. We compared subjective and objective measures before and after surgery, retrieved from patient medical records., Results: The difference between the Dizziness Handicap Inventory scores before and after surgery was statistically significant (p < 0.01). 81% of patients being satisfied with surgery. No post-operative complications occurred. Following SL, 14 patients evolved well, both subjectively and clinically, without any further vestibular workup required, which was offered to the other patients. All had a total deafness on the operated side, as expected, while four benefited from multidisciplinary rehabilitation because of persistent tinnitus. The scientific literature on this topic was reviewed, compared, and discussed., Conclusions: Based on our results, SL represents an efficient and safe approach to achieve vestibular deafferentation in patients with unilateral, disabling, and treatment-resistant MD, with non-functional hearing., (© 2023. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Purpose: When a dizzy patient with episodic vertigo has an abnormal caloric and a normal video head impulse test (vHIT), this caloric-vHIT dissociation provides vital information for a diagnosis of Ménière's disease (MD). Endolymphatic hydrops (EH), a histological marker of MD, is hypothesized to be involved in the caloric-vHIT dissociation in MD through hydropic duct distension of the horizontal semicircular canal (SC). This study was designed to determine the impact of EH on the function of horizontal SC during caloric stimulation., Methods: Caloric test and vHIT were used to evaluate the function of horizontal SC every six months, annual magnetic resonance imaging (MRI) was used to evaluate the degree of EH size in the vestibule, and monthly vertigo and hearing evaluation was done for 12 months. EH shrinkage was defined as the size change of vestibular EH from significant to none., Results: Among 133 MD patients evaluated for eligibility, 67 patients with caloric-vHIT dissociation entered the study. Fifteen participants had EH shrinkage (G-I), while 52 participants had no remarkable EH change (G-II). Average values (IQR) of the maximum slow phase velocity in G-I and G-II were 29.6 (13.0-34.0) and 25.9 (17.3-31.3), respectively, at baseline, 26.1 (9.0-38.0) and 23.6 (18.0-28.3) at 12 months. Two-factor repeated-measures ANOVA showed no significant differences between the groups (P = 0.486). The values of vestibulo-ocular reflex gain of the horizontal SC in G-I and G-II remained above 0.8 during the study period., Conclusions: EH detected by MRI shows limited correlation with caloric stimulation results., (© 2023. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Objective: Report three cases of simultaneous triple semicircular canal occlusion (TSCO) and cochlear implantation (CI) as the treatment of intractable Meniere's disease (MD)., Case Reports: Patients with MD can present occasionally with intractable vertigo and profound sensorineural hearing loss (SNHL). TSCO and CI have been proposed to control vertigo and restore profound deafness in patients with MD separately. However, a few studies have reported simultaneous TSCO and CI in the same surgical procedure for the treatment of MD. In the present study, we described three patients with MD showing incapacitating vertigo and severe SNHL who underwent simultaneous TSCO and CI after examinations of auditory system, vestibular system, and imaging. Their symptoms were significantly alleviated during the follow-up period., Conclusion: The combined TSCO and CI remains a viable treatment option which is effective for the control of vertigo as well as the restoring of hearing in patients with MD., (© 2023. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Purpose: To study the efficacy predictors of endolymphatic sac decompression (ESD) in Meniere's disease (MD), and to establish and verify the prediction model of vertigo after ESD in patients with MD., Methods: The retrospective cohort data of 56 patients with unilateral MD who underwent ESD surgery were recorded. A stepwise regression method was used to select optimal modeling variables, and we established a logistic regression model with the outcome of vertigo after ESD. The bootstrap method was used for internal validation., Results: Potential predictors included sex, age, follow-up duration, disease course, attack duration, frequency of attack, pure-tone threshold average (PTA) of the patient's speech frequency, audiogram type, glycerin test results, MD subtype, and 10-year atherosclerotic cardiovascular disease risk classification. Using the stepwise regression method, we found that the optimal modeling variables were the audiogram type and PTA of the patient's speech frequency. The prediction model based on these two variables exhibited good discrimination [area under the receiver operating characteristic curve: 0.72 (95% confidence interval: 0.57-0.86)] and acceptable calibration (Brier score 0.21)., Conclusion: The present model based on the audiogram type and PTA of the patient's speech frequency was found to be useful in guidance of ESD efficacy prediction and surgery selection., (© 2023. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Purpose: The focus on treating patients with Menière's Disease (MD) lies on the reduction of vertigo attacks and the preservation of sensory function. Endolympathic hydrops is considered as an epiphenomenon in MD, which can potentially be altered by endolymphatic sac surgery (ESS). Purpose of the study was to investigate the influences on vertigo control through manipulation of the perilymphatic system with or without ESS., Methods: Retrospective data analysis of 86 consecutive patients with MD according to current diagnostic criteria after endolymphatic sac surgery alone (ESS alone ; n = 45), cochlear implantation (CI) alone (CI alone ; n = 12), and ESS with CI (ESS + CI; n = 29), treated at a tertiary referral center., Main Outcome Measures: vertigo control, speech perception pre- and postoperatively., Results: Gender, side, and preoperative treatment were similar in all groups. Age was younger in the ESS alone -group with 56.2 ± 13.0 years (CI alone  = 64.2 ± 11.4 years; ESS + CI = 63.1 ± 9.7 years). Definitive MD was present in all the CI alone , in 79.3% of the ESS + CI and in 59.6% of the ESS alone -patients. Likewise, vertigo control rate was 100% in the CI alone , 89.7% in the ESS + CI and 66.0% in the ESS alone -group., Conclusions: Vertigo control was improved in all three groups, however, superior in groups treated with CI, potentially contributed by the manipulation of both the endo- and perilymphatic systems. A more systematic characterization of the patients with larger case numbers and documentation of follow up data would be needed to evaluate a clinical effect more properly., (© 2023. The Author(s).)

Objective: This narrative review examines how speculative belief that the autonomic nervous system causes Meniere's Disease (MD) led otolaryngologists to adopt invasive surgical procedures and medical treatments still offered today., Data Sources: Google Scholar, PubMed., Review Methods: A comprehensive literature review (1860-2022) was performed using the terms "Meniere AND (sympathetic OR sympathectomy OR vasomotor OR cervical ganglion)," returning 5360 items. All abstracts were briefly reviewed, relevant publications selected for further study, and key articles discussed by all authors. As it became clear that betahistine was related to the historical narrative, an additional search was performed using "Betahistine AND Meniere AND (vasomotor OR sympathetic OR sympathectomy OR cervical ganglion OR autonomic)," which yielded 336 results., Results: In the 19th and 20th centuries, growing knowledge of human anatomy led the scientific community to speculate that autonomic dysregulation caused many medical conditions. Excessive sympathetic mediated vasomotor changes were thought to cause hypertension, ischemia, and tissue damage. Clinicians applied the hypothesis to MD, assigning the sympathetic nervous system responsible for vertigo secondary to paroxysmal vasospasm and for hearing loss to poor cochlear nutrition. Despite limited animal experiments and isolated clinical observations, otolaryngologists performed sympathectomies, and, in the 1970s, replaced the procedure with betahistine as an alternative medical treatment., Conclusion: Premature excitement about a plausible hypothesis led to unnecessary and unwarranted operations. Despite absent evidence of sympathetic overactivation in MD, surgeons eagerly adopted sympathectomies, and later betahistine. Rigorous evaluation of the validity of these treatment practices is needed., Level of Evidence: 5 Laryngoscope, 134:535-542, 2024., (© 2023 The American Laryngological, Rhinological and Otological Society, Inc.)

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., (Copyright © 2023 Wolters Kluwer Health, Inc. All rights reserved.)

Background: Managing acute vertigo/dizziness for inpatients requires valid communication between the various healthcare professionals that triage such life-threatening presentations, yet there are no current scaling methods for managing such acute vertigo symptoms for inpatients., Objective: To describe the development and validation of the Krems Acute Vertigo/Dizziness Scale (KAVEDIS), a new instrument for tracking subjective symptoms (vertigo, dizziness) and gait impairment across four unique vestibular diagnoses (Menière's disease, benign paroxysmal positional vertigo, peripheral vestibular hypofunction, and vestibular migraine) over a one-year period after inpatient hospital admission., Methods: Retrospective data collection study from KAVEDIS scale and chart documentation., Results: The KAVEDIS scale can significantly distinguish scores from admission to discharge in three of four vestibular diagnoses. The documented course of subjective vestibular symptoms and gait disturbances were correlated in all four groups., Conclusion: We suggest that KAVEDIS documentation among inpatients admitted with acute vertigo/dizziness may improve communication between the various intervening clinicians and help to raise concern in cases of symptomprogression.

Background: The treatment of refractory elderly Meniere's disease is a challenge., Aim: To investigate the efficacy of tympanostomy tube placement in elderly patients with Meniere's disease., Methods: 31 patients over 60 years old with Meniere's disease who had failed medical treatment and underwent tympanostomy tube placement. Vertigo control, hearing level, tinnitus, and ear fullness were recorded and analyzed preoperatively and postoperatively., Results: All patients completed a 6-month follow-up. At 6 months postoperatively,complete control, substantial control and limited control of vertigo were achieved in 10 (32%), 13 (42%), and 6 (19%) patients respectively. Two patients (6%) reported no substantial improvement. Twelve patients completed a 2-year follow-up. At 2 years postoperatively, complete control, substantial control and limited control of vertigo were achieved in 3 (25%), 5 (42%), and 1 (8%) patients respectively. Three (25%) patients showed no significant improvement in vertigo. The majority of patients reported significant improvement in the functional level scale, the Dizziness Handicap Inventory scores, and ear fullness at 6 months and 2 years postoperatively., Conclusions and Significance: Tympanostomy tube placement may reduce vertigo attacks and improved the quality of life in elderly patients with Meniere's disease.

Purpose: Meniere's disease (MD), a disorder of the inner ear, presents numerous therapeutic challenges, and intratympanic (IT) gentamicin has been proposed for intractable cases. However, controversy regarding dosage and method persists. The purpose of this study was to assess the efficacy and safety of low-dose IT gentamicin on vertigo attacks in MD using a clinical symptomatology-based method, wherein administration was repeated only if vertigo attacks recurred, with a 2-week interval between injections., Materials and Methods: This study included 88 patients with unilateral intractable MD. All patients received one to five IT injections with 0.5 ml of 10 mg of gentamicin (80 mg/2 ml) with an interval of 2 weeks between injections. Vertigo attacks were evaluated before and after therapy and categorized into classes A-F according to the 2015 Equilibrium Committee criteria. Audiovestibular assessments, including Pure Tone Audiometry and Vestibulo-Ocular Reflex evaluations, were performed., Results: Before treatment, patients had an average of 4.4 vertigo attacks/month; after treatment, this average decreased to 0.52. The majority of patients (57 %) reached Class A or B vertigo control with five or fewer gentamicin injections. VOR gain was slightly affected on the healthy side and significantly reduced on the affected side. No hearing deterioration was found in any of the treated patients., Conclusions: Low-dose IT gentamicin administration based on clinical symptomatology can produce a satisfactory control of vertigo attacks after treatment. This protocol primarily affected the vestibular function, as demonstrated by the significant reduction in VOR gain on the affected side, while avoiding cochlear damage. The lack of adverse events and preservation of hearing underscore the safety and efficacy of this method. These findings have significant clinical implications, suggesting that a low-dose, clinical symptomatology-based gentamicin treatment regimen could be an effective and safe strategy for managing unilateral Meniere's disease in a larger population., Competing Interests: Declaration of competing interest Authors declare no conflicts of interest related to this study., (Copyright © 2023 Elsevier Inc. All rights reserved.)

Objective: To investigate ictal nystagmus and audiovestibular characteristics in episodic spontaneous vertigo after cochlear implantation (CI)., Study Design: Retrospective and prospective case series., Patients: Twenty-one CI patients with episodic spontaneous vertigo after implantation were recruited., Interventions: Patient-initiated home video-oculography recordings were performed during one or more attacks of vertigo, using miniature portable home video-glasses. To assess canal and otolith function, video head-impulse tests (vHITs) and vestibular-evoked myogenic potential tests were conducted., Main Outcome Measures: Nystagmus slow-phase velocities (SPVs), the presence of horizontal direction-changing nystagmus, and post-CI audiovestibular tests., Results: Main final diagnoses were post-CI secondary endolymphatic hydrops (48%) and exacerbation of existing Ménière's disease (29%). Symptomatic patients demonstrated high-velocity horizontal ictal-nystagmus (SPV, 44.2°/s and 68.2°/s in post-CI secondary endolymphatic hydrop and Ménière's disease). Direction-changing nystagmus was observed in 80 and 75%. Two were diagnosed with presumed autoimmune inner ear disease (SPV, 6.6°/s and 172.9°/s). One patient was diagnosed with probable vestibular migraine (15.1°/s).VHIT gains were 0.80 ± 0.20 (lateral), 0.70 ± 0.17 (anterior), and 0.62 ± 0.27 (posterior) in the implanted ear, with abnormal values in 33, 35, and 35% of each canal. Bone-conducted cervical and ocular vestibular-evoked myogenic potentials were asymmetric in 52 and 29% of patients (all lateralized to the implanted ear) with mean asymmetry ratios of 51.2 and 35.7%. Reversible reduction in vHIT gain was recorded in three acutely symptomatic patients., Conclusion: High-velocity, direction-changing nystagmus time-locked with vertigo attacks may be observed in post-CI implant vertigo and may indicate endolymphatic hydrops. Fluctuating vHIT gain may be an additional marker of a recurrent peripheral vestibulopathy., Competing Interests: Sources of support and disclosure of funding: A.S.Y. received scholarship funding from the University of Sydney and reports no conflict of interest. B.Y.C.K. was a previous employee of Cochlear Ltd before commencing this study and holds an insignificant (<100) amount of Cochlear Ltd shares. M.S.W. receives funding from the Garnett Passe and Rodney Williams Memorial Foundation, and the National Health and Medical Research Council of Australia and reports no conflict of interest. The remaining authors declare no conflict of interest., (Copyright © 2023, Otology & Neurotology, Inc.)

Objective: To compare the clinical features of sudden hearing loss (SHL) in patients with and without endolymphatic hydrops (EH), and to investigate the association between SHL with EH and Ménière's disease (MD)., Methods: The clinical data of 63 SHL patients with first symptoms were evaluated retrospectively. Patients were separated into two groups based on the results of gadolinium-enhanced magnetic resonance imaging: EH and non-EH groups. Independent sample t-test and U-test were used to compare groups for continuous variables, and the chi-squared test, corrected chi-squared test and Bonferroni correction test were used to compare groups for binary and ordinal variables. The binary logistic regression model was utilised for univariate and multivariate analysis of follow-up patient prognosis., Results: The EH and non-EH groups contained 32 and 31 patients, respectively. The EH group had a higher prevalence of low-tone descending hearing loss. Fifty-one patients were followed for more than 2 years. In the EH group, 11 and 15 patients were diagnosed with sudden sensorineural hearing loss (SSNHL) and MD, respectively, while in the non-EH group, 24 patients were diagnosed with SSNHL and only one with MD. EH, low-tone descending hearing loss and vertigo were risk factors for the diagnosis of MD in a subgroup univariate regression analysis of patients experiencing SHL. EH was found to be a risk factor for the progression of SHL into MD in a multifactor regression analysis., Conclusions: Patients with SHL who have EH are more likely to present with low-tone descending hearing loss. EH is a risk factor for the subsequent development of MD., (© 2023 John Wiley & Sons Ltd.)

Association between vestibular function and immune inflammatory response has garnered increasing interest. Immune responses can lead to anatomical or functional alterations of the vestibular system, and inflammatory reactions may impair hearing and balance. Vestibular disorders comprise a variety of conditions, such as vestibular neuritis, benign paroxysmal positional vertigo, Meniere's disease, vestibular migraine, posterior circulation ischemia, and bilateral vestibular disease. Moreover, some patients with autoimmune diseases develop vestibulocochlear symptom. This paper offers an overview of prevalent vestibular diseases and discusses associations between vestibular dysfunction and immune diseases., (© 2023 Walter de Gruyter GmbH, Berlin/Boston.)

Objective: Identify clinically important factors associated with conservative treatment response in Meniere's disease and incorporate these factors into a composite clinical severity staging system., Study Design: Retrospective cohort., Setting: Tertiary academic medical center., Methods: Adult patients newly diagnosed with Meniere's disease between January 1, 2016 and December 31, 2019 were eligible. Patients with previous treatment for Meniere's disease, prior otologic surgery, or a lack of follow-up data were excluded. Treatment-responsive patients were managed with only conservative therapies (eg, dietary modifications, diuretics) and unresponsive patients underwent more intensive therapies (eg, intratympanic procedures, surgical interventions)., Results: Of 78 patients included in the study, 49 (63%) were responsive to conservative therapies and 29 (37%) were not. Responsive patients had higher proportions of no or mild vertigo (24%, 95% confidence interval [CI]: 3.1%-45.8%) and none or mild comorbidity (27%, 95% CI: 9.2%-44.7%) and a lower proportion of hearing loss (19%, 95% CI: 5.6%-32.4%) compared to unresponsive patients. Conjunctive consolidation of these 3 factors was performed to develop a three-stage system with a treatment response gradient ranging from 100% to 64% to 18% for stage 1 (n = 11), stage 2 (n = 56), and stage 3 (n = 11), respectively., Conclusions: This study identified decreased vertigo severity, reduced comorbidity burden, and absence of hearing loss as factors associated with conservative treatment response in Meniere's disease. A composite clinical severity staging system including these 3 factors can be used to optimize treatment selection and promote patient-centered management of Meniere's disease., (© 2023 American Academy of Otolaryngology-Head and Neck Surgery Foundation.)

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 &lt; 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 &lt; 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., (© 2023 The Author(s). Published by S. Karger AG, Basel.)

Background: Ménière's disease (MD) is a common idiopathic inner ear disorder in otorhinolaryngology characterized by recurrent episodes of vertigo, fluctuating sensorineural hearing loss, tinnitus and ear fullness., Objective: To study the effects of low-sodium diet with adequate water intake on the clinical efficacy in MD., Methods: Fifty patients diagnosed with stage-3 unilateral MD were randomly divided into control group( n  = 25) and experimental group( n  = 25). The control group was given routine medication therapy, and the experimental group was restricted to an sodium intake of 1500 mg/d and a water intake of 35 ml/kg/d based in addition to the routine medication therapy. The two groups were assessed using pure tone audiometry, electrocochleography, Tinnitus Handicap Inventory (THI), and Dizziness Handicap Inventory (DHI)., Results: The pure tone audiometry and electrocochleography showed better improvements after treatment in the experimental group than the control group ( p  < 0.05). The THI improved values in the experimental group were significantly higher than the control group ( p  < 0.001). The DHI improved values in the experimental group were significantly higher than the control group ( p  = 0.004)., Conclusions and Significance: Low-sodium diet with adequate water intake improved the hearing and alleviated vertigo and tinnitus in MD patients.

Objective: We investigated correlations among clinical features, degree of inner ear endolymphatic hydrops (EH), and hippocampal volume (HV) in different stages of Meniere's disease (MD)., Methods: From February 2021 to April 2022, clinical data were collected from 99 patients (39 males, 60 females, mean age: 50.4 ± 10.0 [range: 26-69] years) with unilateral MD admitted to the Department of Vertigo Disease of Shandong ENT Hospital. The left and right ears were affected in 64 and 35 patients, respectively. There were 50 and 49 cases in early (Stages 1, 2) and late stages (Stages 3, 4), respectively. Fifty healthy participants were included as controls. Audiovestibular function test results, EH grading using gadolinium-enhanced magnetic resonance imaging (MRI), and HV determined on MRI were analyzed for patients at different stages of MD., Results: Between-group comparisons of early and late MD revealed significant differences in the disease course, vestibular function (VF), degree of EH, and HV. There were no significant between-group differences based on age, sex, affected side, subjective degree of dizziness, hospital anxiety, or depression. Mean HV in patients with early-stage MD was correlated with the canal paresis value of the caloric test and pure tone hearing threshold, HV in late-stage patients was correlated with vestibular EH., Conclusion: Patients with late-stage MD exhibited severe auditory and VF impairments, increased EH, and atrophy of the HV. More advanced disease was associated with greater vestibular damage and degree of EH., Level of Evidence: 3 Laryngoscope, 134:410-418, 2024., (© 2023 The American Laryngological, Rhinological and Otological Society, Inc.)

Competing Interests: The authors have no conflict of interest and funding sources.

Background: Ménière's disease is an inner ear disorder causing recurrent vertigo, hearing loss, and tinnitus. Diagnosis is based on the variability of the symptoms over time and absence of radiological abnormalities. Medical therapy is effective only in a small percentage of patients. Surgical strategies remain controversial. In this article, we revisit a surgical technique neglected over the years: endolymphatic sac surgery., Methods: Fifty-four patients affected by Ménière's disease underwent endolymphatic duct and sac decompression. According to the American Academy of Otolaryngology-Head and Neck Surgery criteria, vertigo control was evaluated with follow-up at 6 months, 1 year, and 2 years from the intervention. Hearing results were evaluated before the surgery and at 2 years of follow-up using the pure tone average. The results were compared with similar techniques of endolymphatic sac surgery described in the literature., Results: According to the American Academy of Otolaryngology-Head and Neck Surgery criteria classification, 2 years after surgery, 87% patients achieved complete control of vertigo (class A). The hearing remained stable in 93.5% of patients. The results appear compatible with other publications data regarding endolymphatic sac surgeries., Conclusion: The duct and endolymphatic sac decompression allows the control of vertigo and preserves hearing from the pathological effects of Ménière's disease. The revised technique allows the functional restoration of endolymphatic homeostasis.

Objective: To analyze evidence supporting an association between immune-related diseases and Ménière's disease (MD) since it has long been thought to be related to autoimmune disorders and allergies., Data Sources: We retrieved records from Pubmed, Web of Science, Scopus, and Cochrane Library to identify studies published between January 2002 and October 2022., Review Methods: Articles were independently assessed by 2 reviewers and verified by a third reviewer. Published cross-sectional studies, cohort/longitudinal studies, case series, and noncomparative cohort studies were considered eligible for inclusion. We conducted a systematic review and meta-analysis according to a registered protocol on the International Prospective Register of Systematic Reviews and Preferred Reporting Items for Systematic Reviews and Meta-analyses guidelines. Selected studies were classified into 2 groups: epidemiological and genetic association studies. Relative frequencies and odds ratios (ORs) for each autoinflammatory/autoimmune disease or genetic marker reported to be associated with MD., Results: Fifteen studies from 6 countries met our inclusion criteria. Nine are epidemiological studies and 6 are genetic association studies. The epidemiological studies were used to perform 3 different meta-analyses. Airway allergic disease and autoimmune thyroid disease showed a significant association with MD (OR = 2.27 [2.08-2.48] and OR = 1.35 [1.25-1.46]); while rheumatoid arthritis did not (OR = 0.63 [0.28-1.41]). Other comorbidities also showed a significant association with MD like chronic obstructive pulmonary disease, vitiligo, fibromyalgia, arthritis, and psoriasis., Conclusion: Epidemiological evidence supports an association between MD and immune-related disorders in European and Asian populations, with population-specific effects. The evaluation of thyroid diseases, airway allergic diseases, and other inflammatory diseases should be implemented in the clinical management of MD patients., (© 2023 The Authors. Otolaryngology-Head and Neck Surgery published by Wiley Periodicals LLC on behalf of American Academy of Otolaryngology-Head and Neck Surgery Foundation.)

Background: Long-term efficacy of middle ear pressure therapy (MEPT) with the EFET01 device in patients in Japan with definitive Ménière's disease (MD) and delayed endolymphatic hydrops (DEH) was evaluated., Objective: To examine the effects of reducing vertigo attacks and improving hearing of MD and DEH patients by using MEPT with the EFET01 device for two years after treatment., Material and Methods: A retrospective study was conducted of 32 MD patients and 2 DEH patients treated by MEPT with the EFET01 device from December 2018 to April 2021. According to Japan Society for Equilibrium Research (JSER) guidelines, patients were investigated for the frequency of vertigo attacks and change in hearing levels during a period of 6 months before to 18-24 months after start of treatment., Results: The frequency of vertigo attacks significantly decreased in both MD and DEH patients, and hearing level has remained stable in the majority of our patients after treatment., Conclusion: Our study clarified that MEPT with the EFET01 device was effective in controlling vertigo symptoms of MD and DEH. It should be considered a safe option for patients failing medical treatment., Significance: The efficacy of MEPT with the EFET01 was shown over a 2-year follow-up period.

Purpose of Review: Meniere's disease is caused by hydropic changes in the endolymphatic system, and manifests as a collection of vertigo, hearing loss, tinnitus, and aural fullness. Although high-quality clinical practice guidelines exist for the diagnosis and initial management of Meniere's disease, there is no strong consensus for treatment of medically refractory Meniere's disease. This review summarizes treatment options and highlights controversies surrounding surgical treatment of Meniere's disease., Recent Findings: Intratympanic steroid and intratympanic gentamicin injections continue to be widely used as in-office therapies in medically refractory Meniere's disease. Despite historical controversy surrounding the use of endolymphatic sac (ELS) surgery, the use of ELS decompression has been widely adopted by the international neurotologic community due to high vertigo control rate, coupled with low risk of audiovestibular loss. Wider decompression of the sac and surgical manipulation of the endolymphatic duct may impact outcome and are the subject of discussion. An emerging surgical technique called Triple Semicircular Canal Occlusion (TSCO) holds promise as a partially ablative procedure with high vertigo control rate in Meniere's disease. Cochlear implants may be placed in active Meniere's disease patients, or during an ablative surgery such as labyrinthectomy., Summary: For the medically refractory Meniere's disease patient, treatment options include intratympanic steroid injection, endolymphatic sac decompression, medical or surgical labyrinthectomy, and vestibular nerve section. TSCO holds promise as an emerging partially ablative procedure. Cochlear implants maintain an important role in the rehabilitation of hearing loss associated with Meniere's disease., (Copyright © 2023 Wolters Kluwer Health, Inc. All rights reserved.)

Objectives: This study investigated the relationship between quantitative value of endolymphatic hydrops in the affected ear and the hearing threshold in patients with unilateral Meniere's disease., Study Design: Prospective study., Methods: Forty patients with unilateral definite Meniere's disease were diagnosed in outpatient clinic. We recorded their clinical symptoms (duration of vertigo and tinnitus and duration of vertigo attacks), and obtained the hearing thresholds of high, middle, and low by pure tone audiometry. A 3D-FLAIR MRI revealed endolymphatic hydrops and assessed quantitative values of cochlear and vestibular endolymphatic hydrops. We assessed the correlation between hearing thresholds and quantitative values of endolymphatic hydrops., Results: All patients showed varying degrees of endolymphatic hydrops in the affected ear. With the duration of vertigo, tinnitus and vertigo attacks as control variables, partial correlation analysis showed that the correlation coefficients between quantitative value of cochlear hydrops and hearing thresholds of low, middle, and high tone in the affected ear were 0.581, 0.610, and 0.125, respectively. The correlation coefficients between quantitative value of vestibular hydrops and hearing thresholds of low, middle, and high tone in the affected ear were 0.727, 0.569, and 0.201, respectively., Conclusions: The degree of endolymphatic hydrops in Meniere's disease can be revealed and assessed by 3D-FLAIR MRI after intravenous administration of double doses of gadoterate meglumine. Clinical evaluation of endolymphatic hydrops in Meniere's disease can be assisted by low- and middle-tone hearing thresholds, thereby providing anatomical support for the clinical symptoms of Meniere's disease.

Background: Chronic dizziness can cause significant functional impairment. Outcome measures used in this patient population have not been examined systematically. Consequently, providers lack consensus on the ideal outcome measures to assess the impact of their interventions., Objective and Methods: We conducted a scoping review to summarize existing literature on outcomes in chronic dizziness (with a minimum of 6 mo of patient follow-up). Among other details, we extracted and analyzed patient demographics, medical condition(s), and the specific outcome measures of each study., Results: Of 19,426 articles meeting the original search terms, 416 met final exclusion after title/abstract and full-text review. Most studies focused on Ménière's disease (75%) and recurrent benign paroxysmal positional vertigo (21%). The most common outcome measures were hearing (62%) and number of attacks by American Academy of Otolaryngology-Head & Neck Surgery criteria (60%). A minority (35%) looked formally at quality-of-life metrics (Dizziness Handicap Index or other)., Conclusions: Ménière's disease and benign paroxysmal positional vertigo are overrepresented in literature on outcome assessment in chronic dizziness. Objective clinical measures are used more frequently than quality-of-life metrics. Future work is needed to identify the optimal outcome measures that reflect new knowledge about the most common causes of chronic dizziness (including persistent postural-perceptual dizziness and vestibular migraine) and consider what is most important to patients., Competing Interests: The authors disclose no conflicts of interest., (Copyright © 2023, Otology & Neurotology, Inc.)

Objectives: The degree of endolymphatic hydrops in Meniere's disease may be related to hearing loss. However, the results of prior studies have been inconsistent. We aimed to investigate the relationship between endolymphatic hydrops and hearing loss characteristics in Meniere's disease., Methods: This study included 54 patients (62 ears) with Meniere's disease. Patients underwent three-dimensional real inversion recovery sequences for magnetic resonance imaging and pure-tone audiometry. Endolymphatic hydrops were assessed according to Gurkov's criteria (2011). Correlations between different degrees of endolymphatic hydrops and pure-tone audiometry, as well as staging, were analysed., Results: Pure tone audiometry and staging were higher for vestibular endolymphatic hydrops complicated by cochlear ones than isolated cochlear or vestibular hydrops (both p < 0.05). There was no significant correlation between vestibular endolymphatic hydrops and pure-tonal audiometry or staging (all p > 0.05). The degree of hydrops in the middle turn was correlated with the mid-frequency hearing threshold (p < 0.05). The degree of cochlear hydrops was correlated with the audiometry, low-frequency hearing thresholds, mid-frequency hearing thresholds and staging (p < 0.01)., Conclusion: The types and sites of endolymphatic hydrops based on a 3D-real inversion recovery sequence can be used to indicate the degree of hearing loss in patients with Meniere's disease., Level of Evidence: Level III., (Copyright © 2023 Associação Brasileira de Otorrinolaringologia e Cirurgia Cérvico-Facial. Published by Elsevier España S.L.U. All rights reserved.)

Objectives: Our aim in this study was to characterize the morphology of the endolymphatic compartment on histopathology in individuals with Ménière's disease (MD) and to determine why hydrops of the saccule is more pronounced than that of other compartments of the inner ear in MD., Methods: Temporal bones from 9 patients with idiopathic MD and from 10 individuals without MD/endolymphatic hydrops were examined. The inner ear fluid compartments in normal ears, and ears with MD were three-dimensionally reconstructed and their volume was calculated. The thickness of the membranes of the labyrinth was measured, and both ruptures of the membranes and patency of the utriculoendolymphatic (UEV; Bast's) valve were assessed., Results: In ears with MD, the saccule and the cochlear duct were most frequently hydropic; the utricle was involved approximately half as frequently. In ears without MD, the Reissner's membrane and the membranous wall of the saccule were thinner than that of the utricle and of the lateral semicircular canal ( p < 0.01). The lateral semicircular canal did not show signs of hydrops. In all ears with MD in which the utricle exceeded the average volume of normals (6 of 12), the UEV was open or there was a rupture in the utricle., Conclusion: Increases in endolymphatic pressure may cause a primary swelling of the apical cochlear duct and saccule, both of which have relatively thin membranes. Hydrops in the utricle may occur less frequently because of a thicker wall, because of a functioning UEV, and when the saccule has already occupied most of the vestibular perilymphatic space., Competing Interests: The authors disclose no conflicts of interest. B.K.W. is supported by grants from the National Institutes of Health (K23DC018302), the Hearing Health Foundation, and the Katie and Robert Niehaus Foundation and has a patent US10646723 that is unrelated to the current work. B.B. receives royalties from Oxford University Press., (Copyright © 2023, Otology & Neurotology, Inc.)

Background: The etiology of Ménière's disease (MD) is still not completely clear, but it is believed to be associated with endolymphatic hydrops (EH), which is characterized by auditory functional disorders. Vasopressin injection in C57BL/6J mice can induce EH and serve as a model for MD. Optical Coherence Tomography (OCT) has shown its advantages as a non-invasive imaging method for observing EH. Aim Investigating the relationship between hearing loss and EH to assist clinical hearing assessments and indicate the severity of hydrops., Methods: C57BL/6J mice received 50 μg/100g/day vasopressin injections to induce EH. Auditory function was assessed using auditory brainstem response (ABR) and distortion product otoacoustic emissions (DPOAE). OCT was used to visualize the cochlea., Result: OCT observed accumulation of fluid within the scala media in the cochlear apex. ABR showed significant hearing loss after 4 weeks. DPOAE revealed low-frequency hearing loss at 2 weeks and widespread damage across frequencies at 4 weeks., Conclusion: The development of hearing loss in mouse models of MD is consistent with EH manifestations. Significance This study demonstrates the possibility of indirectly evaluating the extent of EH through auditory assessment and emphasizes the significant value of OCT for imaging cochlear structures.

Objective: To clarify whether age impacts the development of endolymphatic hydrops (ELH) in neurotologic patients. Documentation of ELH in living patients on magnetic resonance imaging (MRI) allows analysis of patient age and formation of ELH, which is impossible by means of postmortem temporal bone pathology., Study Design: Retrospective case review., Setting: Tertiary referral center., Patients: Fifty patients (100 ears) with top 3 diagnoses of definite Menière's disease, delayed ELH, or probable Menière's disease., Interventions: Endolymph MRI after intravenous gadolinium injection and pure-tone audiometry., Main Outcome Measures: Cochlear and vestibular ELH confirmed by MRI., Results: The prevalences of ears showing both cochlear and vestibular ELH were similar among age groups <30 years (30%), 30-59 years (25.9%), and ≥60 years (34.4%; p > 0.05; χ2 test). Using logistic regression modeling, mean hearing level at the six frequencies was positively associated with a higher risk of cochlear ELH (odds ratio, 1.3; 95% confidence interval, 1.1-1.5 per 10-dB increment). In the same regression model, age did not impact the outcome of cochlear ELH (odds ratio, 1.0; 95%confidence interval, 0.7-1.4 per 10-year increment). Age did not differ among ears with no ELH (mean ± standard deviation age, 48.6 ± 14.4 yr), ears with only cochlear ELH (59.3 ± 10.7 yr), ears with only vestibular ELH (50.4 ± 16.9 yr), or ears with both cochlear and vestibular ELH (51.5 ± 18.4 yr; p > 0.05, analysis of variance)., Conclusion: Chronological age was not associated with the formation of ELH. Aging per se may not be associated with the development of ELH in neurotologic patients., Competing Interests: The authors disclose no conflicts of interest., (Copyright © 2023, Otology & Neurotology, Inc.)

Background: Orthostatic dizziness/vertigo (ODV) is characterized by lightheadedness owing to postural changes., Aims/objectives: To measure the endolymphatic space (ELS)/total fluid space (TFS) volume ratio and the distribution rate of endolymphatic fluid (ELF) of patients with ODV and compare them with those of control subjects (CS)., Materials and Methods: This study included 22 patients (44 ears) with ODV and 52 controls (104 ears, CS). The ELS/TFS volume ratio (%) and distribution rate (%) of the inner ear components were measured using 3-dimensional magnetic resonance imaging., Results: In the ODV group, the mean ELS/TFS volume ratios of the cochlea, vestibule, and semi-circular canals (SCCs) were 12.1%, 18.6%, and 18.1%, respectively; the mean ELS distribution rates for the cochlea, vestibule, and SCCs were 27.3%, 26.2%, and 46.6%, respectively. The ELS distribution rate of the vestibule was significantly lower ( p  < .01) and the ELS distribution rate of the SCCs was significantly higher in the ODV than in the CS group ( p  < .01)., Conclusions and Significance: The ELS distribution rate in the vestibule + SCCs among patients with ODV did not differ from that in the CS; ELF in the vestibule moved to the SCCs, and a large amount of ELF was distributed only in the SCCs.

Meniere's disease (MD) is a disorder of the inner ear characterized by chronic episodes of vertigo, tinnitus, increased aural pressure, and sensorineural hearing loss. Causes of MD are unknown, but endolymphatic hydrops is a hallmark. In addition, 5%-15% of MD cases have been identified as familial. Whole-genome sequencing studies of individuals with familial MD identified DTNA and FAM136A as candidate genes for autosomal dominant inheritance of MD. Although the exact roles of these genes in MD are unknown, FAM136A encodes a mitochondrial protein, and DTNA encodes a cytoskeletal protein involved in synapse formation and maintenance, important for maintaining the blood-brain barrier. It is also associated with a particular aquaporin. We tested vestibular and auditory function in dtna and fam136a knockout (KO) mice, using RotaRod and startle reflex-based clicker tests, respectively. Three-factor analysis of variance (ANOVA) results indicated that sex, age, and genotype were significantly correlated with reduced mean latencies to fall ("latencies") for male dtna KO mice, while only age was a significant factor for fam136a KO mice. Fam136a KO mice lost their hearing months before WTs (9-11 months vs. 15-20 months). In male dtna KO mice, divergence in mean latencies compared with other genotypes was first evident at 4 months of age, with older males having an even greater decrease. Our results indicate that fam136a gene mutations generate hearing problems, while dtna gene mutations produce balance deficits. Both mouse models should help to elucidate hearing loss and balance-related symptoms associated with MD., (© 2023 The Authors. European Journal of Neuroscience published by Federation of European Neuroscience Societies and John Wiley & Sons Ltd.)

Objectives: The objective of this study is to understand whether the hearing loss laterality in Menière's disease (MD) correlates with migraine symptoms laterality such as headache, neck stiffness, and otalgia., Methods: We performed a retrospective review of prospectively obtained data on patients presenting between September 2015 and October 2021 with definite or probable MD. A custom-designed, comprehensive questionnaire was used to identify patients' migraine-related symptoms. The clinical and audiometric data were used to diagnose patients with definite or probable MD using criteria set by the American Academy of Otolaryngology-Head and Neck Surgery., Results: In total, 113 patients with definite or probable MD were included in the study. The mean age of the patients was 60 ± 15 years with no gender predominance (49.6% male and 50.4% female). A total of 57 (50%) patients were presented with headaches. Among the migraine headache cohort, headache and otalgia were on the same side as the MD ear affected by hearing loss. In addition, in patients who present with otalgia as the primary feature of headache, otalgia was more likely to be on the same side as the ear affected by the hearing loss., Conclusions: The high prevalence of migraine symptoms on the same side of the ear affected by MD among this cohort could suggest a shared pathophysiology in both MD and migraine, possibly involving migraine-related changes in both the cochlea and vestibule., (Copyright © 2023, Otology & Neurotology, Inc.)

Objectives: To evaluate the diagnostic role of combined electrocochleography and pure-tone audiometry monitoring during dehydrating test in Ménière's disease and consider its suitability as a diagnostic tool to differentiate those patients with unclear differential diagnosis and therefore identify those with clear endolymphatic hydrops responsive to dehydrating test. To study the efficacy of dehydrating therapy on vertiginous symptoms and hearing loss in patients with Ménière's disease., Study Design: Prospective case series., Settings: University hospital, secondary referral center., Patients: Thirty patients, 20 women and 10 men, age range of 25 to 75 years, matching the criteria for definite Ménière's disease according to the Barany Society classification., Intervention: Diagnostic. During an active phase of the disease, electrocochleography and pure-tone audiometry were performed, and repeated at 30th, 45th, and 60th minutes after intramuscular injection of 40 mg furosemide and 40 mg methylprednisolone., Main Outcome Measure: Data related to symptoms, electrocochleography, and pure-tone audiometry during the dehydrating test were collected at different times and statistically analyzed., Results: After the administration of dehydrating therapy, we observed that both summating potential and action potential ratio and summating potential and action potential area ratio were normalized in 21 of 30 subjects. Furthermore, pure-tone audiometry thresholds improved significantly. An improvement of ear fullness was also observed, whereas tinnitus unchangeably persisted., Conclusions: The monitoring of the electrocochleography and pure-tone audiometry thresholds during dehydrating tests with furosemide and methylprednisolone could allow to detect an improvement of instrumental features and clinical symptoms related to endolymphatic hydrops, and therefore, it could be used as a diagnostic tool in the identification of those patients affected by Ménière's disease with unclear differential diagnosis., Competing Interests: The authors disclose no conflicts of interest., (Copyright © 2023, Otology & Neurotology, Inc.)

Competing Interests: The authors disclose no conflicts of interest.

Objective: To determine the efficacy of intratympanic OTO-104 for the treatment of Ménière's disease., Study Designs: Three randomized, double-blind, placebo-controlled, multicenter studies of OTO-104 in patients with Ménière's disease., Setting: The United States and throughout Europe., Patients: Individuals with Ménière's disease aged 18 to 85 years., Interventions: All three studies were conducted according to a similar protocol, whereby after a 1-month lead-in period, eligible patients received a single intratympanic injection of either 12 mg OTO-104 (otic formulation of dexamethasone in thermosensitive poloxamer) or placebo (1:1) and were observed for 3 months., Main Outcome Measures: The primary efficacy endpoint was measured by the number of definitive vertigo days (DVDs) at month 3. Secondary objective was OTO-104 safety and tolerability including adverse events, audiometry, tympanometry, and otoscopic examinations., Results: Although OTO-104 demonstrated numerically greater reductions in DVD compared with placebo across all three studies, statistical significance versus placebo (primary efficacy endpoint) was only achieved in one study, the AVERTS-2 study (n = 174, p = 0.029). Secondary vertigo efficacy endpoints were statistically significant at month 3 in that study including vertigo severity, the effect of vertigo on daily activity (days at home sick or bedridden), and vertigo frequency. In the AVERTS-1 study, which did not meet the primary endpoint, a subgroup analysis of the 115 patients (69.7% of study population) who did not previously receive intratympanic steroid injections demonstrated that OTO-104 patients had significantly lower mean DVD at month 3 than patients receiving placebo (1.9 for OTO-104 versus 3.0 for placebo; p = 0.045). Importantly, a significant placebo response was observed across studies in Ménière's disease patients. OTO-104 and the intratympanic injection procedure were well tolerated., Conclusions: In all three high-quality, randomized, double-blind, placebo-controlled, multicenter studies, a single intratympanic injection of 12 mg OTO-104 demonstrated numerically greater reductions in vertigo versus placebo in patients with Ménière's disease, but statistical separation from placebo was demonstrated in only one of the studies. OTO-104 was safe and well tolerated.(Otonomy, Inc. funded; NCT02717442, NCT02612337, NCT03664674)., (Copyright © 2023 The Author(s). Published by Wolters Kluwer Health, Inc. on behalf of Otology & Neurotology, Inc.)

Objectives: The differentiation between Vestibular Migraine (VM) and Meniere's Disease (MD) is difficult because of overlapping symptoms. The study aimed to compare the clinical characteristics and vestibular function test results between VM and MD patients., Methods: Seventy-one patients with definite VM and 31 patients with definite unilateral MD were included. All patients received Caloric Test (CT), Video Head Impulse Test (vHIT) and Vestibular Evoked Myogenic Potential (VEMP) test within 7 days after visiting the hospital. Results of these tests were compared between groups., Results: Most VM patients (64.0%) experienced spontaneous internal vertigo, while most MD patients (66.7%) experienced spontaneous external vertigo. MD patients had more severe vestibular symptoms and autonomic responses compared to VM patients during attacks (p =  0.03, p = 0.00, respectively). The nystagmus intensity of CT-induced was greater in VM patients than in MD patients (p = 0.003). More VM patients had CT intolerance and Central Positional Nystagmus (CPN) compared to MD patients (p = 0.002, p = 0.006, respectively). More MD patients had CT(+) and vHIT saccades wave compared to VM patients (p < 0.001, p = 0.002, respectively). The non-elicitation rate of cervical VEMP was higher, and the ocular VEMP amplitudes were lower in MD patients than in VM patients (p = 0.002, p = 0.018)., Conclusions: Vestibular symptoms during attacks combined with the results of vestibular function tests may be used to differentiate between VM and MD. The diverse nature of vestibular symptoms (especially internal vertigo), history of motion sickness and CT intolerance may provide clues to the diagnosis of VM, whereas spontaneous external vertigo, CT(+) with vHIT(-), and the presence of saccades may provide clues to the diagnosis of MD., (Copyright © 2023. Published by Elsevier España, S.L.U.)

Background: Meniere Disease (MD) is an inner ear syndrome, characterized by episodes of vertigo, tinnitus and fluctuating sensorineural hearing loss. The pathological mechanism leading to sporadic MD is still poorly understood, however an allergic inflammatory response seems to be involved in some patients with MD., Objective: Decipher an immune signature associated with the syndrome., Methods: We performed mass cytometry immune profiling on peripheral blood from MD patients and controls. We analyzed differences in state and differences in abundance of the different cellular subsets. IgE levels were quantified through ELISA on supernatant of cultured whole blood., Results: We have identified two clusters of individuals according to the single cell cytokine profile. These clusters presented differences in IgE levels, immune cell population abundance, including a reduction of CD56 dim 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., Competing Interests: Declaration of Competing Interest The authors declare that they have no competing interests., (Copyright © 2023 The Authors. Published by Elsevier Inc. All rights reserved.)

Objective: To evaluate speech outcomes and facial nerve stimulation (FNS) rates in patients with far advanced otosclerosis (FAO) after cochlear implantation., Methods: A systematic review was performed using standardized methodology of Medline, EMBASE, PubMed, Cochrane, and Web of Science databases. Studies were included if adults with FAO underwent cochlear implantation. Exclusion criteria included concurrent otologic history (e.g., Meniere's disease, superior canal dehiscence), non-English-speaking implant users, case reports, abstracts, and letters/commentaries. Bias was assessed using the Newcastle-Ottawa Scale for cohort studies and the National Institute of Health Scale for case series. The primary outcome measure was speech discrimination and the secondary outcomes were rates of partial insertion and FNS., Results: Twenty-seven studies evaluated cochlear implantation in FAO. Due to the heterogeneity of testing methods, statistical pooling of speech discrimination was not feasible, but qualitative synthesis indicated a positive effect of implantation. Pooled rates of FNS were 18% (95% confidence interval, CI 12%-27%) and the rate of partial insertion was 10% (95% CI 7%-15%)., Conclusion: Cochlear implantation in FAO demonstrates significant gains in speech discrimination scores with higher rates of FNS and partial insertion. Laryngoscope, 133:1288-1296, 2023., (© 2022 The American Laryngological, Rhinological and Otological Society, Inc.)

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.

Introduction: In this article, the bi-fenestral surgical chemical labyrinthectomy is introduced as a surgical demolition technique for treating resisting incurable forms of Meniere's disease in patients aged over 70 and/or with low hearing residues refractory to medical treatment., Materials and Methods: The results on participants fitting the inclusion criteria (n = 16) were reported using anamnesis (frequency of the crisis), Dizziness Handicap Inventory (DHI) and Functional Level Scale (FLS) before and after the intervention., Results: Vertigo control was achieved in all patients of this case series. A difference of 57 and 3.67 in mean DHI (from 68 (SD 16.7) to 11 (SD 14)) and FLS (from 4.68 (SD 0.7) to 0.1 (SD 0.3)) scores respectively were seen after an average of 16.28 months. Contextually tinnitus was reported to improve in seven patients (43.75%), aggravate in three (18.75%) and remain unchanged in the remaining six (37.5%)., Conclusion: Bi-fenestral surgical chemical labyrinthectomy appears a safe, immediate, and effective demolition treatment for vertigo control in a restricted class of patients affected by intractable Meniere disease., (Copyright © 2022. Published by Elsevier España, S.L.U.)

Background: The grading of intracochlear endolymphatic hydrops (EH) in Meniere's disease (MD) varies and lacks uniformity., Aims: To compare the grading consistency and correlation between different grade methods of intracochlear EH and hearing loss., Materials and Methods: Thirty-one patients diagnosed with MD underwent gadolinium-enhanced magnetic resonance imaging. Two radiologists graded the cochlea EH according to M1, M2, M3, or M4. We analysed the grading consistency and correlation between the EH degrees and hearing loss., Results: The weighted kappa coefficients for inter-observer and intra-observer agreements for grading using M1 were good, whereas those for M2, M3, and M4 are excellent (all p  < 0.001). The cochlear EH degree based on M2 was correlated with the low-to-mid frequencies, high frequencies, full frequencies, and MD clinical stage (all p  < 0.05). The degrees based on M1, M3, M4 were only relevant to some of the 4 items., Conclusions: The grading consistency of M2, M3, M4 is relatively higher than that of M1, and M2 shows the strongest correlation with hearing loss., Significance: Our results provide a more accurate method for assessing the clinical severity of MD.

Background: Endolymphatic hydrops (EH) is universal in Ménière´s disease (MD). Given its chronic course, with variable interval before complete clinical picture is installed, it seems relevant to understand the progression of vestibular EH and hemato-perilymphatic barrier disruption in patients with MD and monosymptomatic presentations., Methods: 239 consecutive patients were referred to us with suspected hydropic ear disease. 50 individuals accepted to participate in this study-final longitudinal sample included 24 patients (7 D1, 7 D2, 10 D3). Control group included ten patients. At recruitment, a clinical and MRI re-evaluation was done (3T, intravenous technique) (MR2) and 2 years after MRI was repeated (MR3). Previous MRI (MRI1) were retrospectively evaluated. Patients were classified as definite (D1), possible (D2) and atypical (D3-monosymptomatic) MD. Control group included non-typical symptoms (C2/C3) and 6 asymptomatic (C1). Vestibular endolymphatic ratio (vER) and grading, presence/absence of cochlear EH, asymmetry of cochlear perilymphatic enhancement, and rate of progression of vER were assessed by two independent neuroradiologists and compared between patient and control groups (index ear)., Results: EH was universal and pronounced in D1 and remained stable. vER progression was more variable and higher in some D3 patients (index ear worse) and in D2 (non-index), although this observation was not statistically significant., Conclusions: Considering that many probable and monosymptomatic presentations progress years later into definite MD and given the bilateral tendency of the disease, these findings may indicate that there is an initial accelerated worsening of EH in initial stages of the disease. These data should be confirmed with controlled and larger sample studies., (© 2022. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Background and Purpose: The detection rate of premortem MR imaging endolymphatic hydrops is lower than that of postmortem endolymphatic hydrops in Ménière disease, indicating that current MR imaging techniques may underestimate endolymphatic hydrops. Therefore, we prospectively investigated whether a novel high-resolution MR imaging technique, the 3D zoomed imaging technique with parallel transmission real inversion-recovery (3D-ZOOMit real IR), would improve the detection of endolymphatic hydrops compared with conventional 3D TSE inversion-recovery with real reconstruction., Materials and Methods: Fifty patients with definite unilateral Ménière disease were enrolled and underwent 3D-ZOOMit real IR and 3D TSE inversion-recovery with real reconstruction 6 hours after IV gadolinium injection. The endo- and perilymph spaces were scored separately. The contrast-to-noise ratio, SNR, and signal intensity ratio of the 2 sequences were respectively calculated and compared. The presence of endolymphatic hydrops was evaluated., Results: The endolymphatic space in the cochlea and vestibule was better visualized with 3D-ZOOMit real IR than with conventional 3D TSE inversion-recovery with real reconstruction ( P  < .001). There were differences between the 2 sequences in the evaluation of no cochlear hydrops and cochlear hydrops (both, P  < .017). All contrast-to-noise ratio, SNR, and signal intensity ratio values of 3D-ZOOMit real IR images were statistically higher than those of conventional 3D TSE inversion-recovery with real reconstruction (all, P  < .001)., Conclusions: The 3D-ZOOMit real IR sequences are superior to conventional 3D TSE inversion-recovery with real reconstruction sequences in visualizing the endolymphatic space, detecting endolymphatic hydrops, and discovering contrast permeability., (© 2023 by American Journal of Neuroradiology.)