The genomic landscape of Ménière's disease: a path to endolymphatic hydrops.

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. CONCLUSION: 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.

Correlation of endolymphatic hydrops and perilymphatic enhancement with the clinical features of Ménière's disease.

OBJECTIVES: To use three-dimensional real inversion recovery (3D-real IR) MRI to investigate correlations between endolymphatic hydrops (EH) grades or the degree of perilymphatic enhancement (PE) and clinical features of Ménière's disease (MD), as previous findings have been inconsistent. METHODS: A total of 273 consecutive patients with definite unilateral MD were retrospectively enrolled from September 2020 to October 2021. All patients underwent 3D-real IR and 3D-T2WI 6 h after intravenous gadolinium injection. MD-related symptom duration and vertigo frequency were recorded. EH grades were evaluated, the signal intensity ratio (SIR) was measured, and correlations between clinical features and EH, PE were assessed respectively. RESULTS: The study included 123 males and 150 females, with a mean age of 53.0 years. A longer duration of vertigo was associated with higher cochlear EH grades, whereas the opposite was true for the duration of aural fullness. A longer time since vertigo onset was associated with higher vestibular EH grades; the opposite was true for the duration of individual vertigo attacks. The multiple regression analysis revealed that age, tinnitus duration, and vestibular EH were risk factors for SIR. Furthermore, the low-frequency hearing threshold (HT) was a risk factor for cochlear and vestibular EH, and the SIR. CONCLUSION: The EH grade and SIR (an indicator for the quantitative evaluation of PE) were correlated with clinical features and HT of MD; thus, imaging can be a valuable tool in planning individualised treatment. CLINICAL RELEVANCE STATEMENT: This study revealed that the grade of endolymphatic hydrops and degree of perilymphatic enhancement positively correlates with the length of time since onset of clinical symptoms and hearing thresholds in patients with Ménière's disease, facilitating the tailored treatment. KEY POINTS: • Relationships between 3-dimensional real inversion recovery features and clinical symptoms in Ménière's disease are unknown. • Symptom duration and hearing thresholds correlated with endolymphatic hydrops grades and degree of perilymphatic enhancement. • MRI features correlate with MD severity; thus, imaging is valuable for planning tailored treatment.

A novel 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 in Meniere's disease patients.

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.

Blood-labyrinth barrier breakdown in Meniere's disease.

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.

Impact of endolymphatic hydrops on the function of the horizontal canal during caloric stimulation in Ménière's disease.

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.

Comparative analysis of vestibular endolymphatic hydrops grading methods and hearing loss in Ménière's disease: a retrospective MRI study using 3D-real inversion recovery sequence.

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.

The value of gadolinium-enhanced MRI in predicting the development of sudden hearing loss into Ménière's disease.

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.

Efficacy of endolymphatic duct blockage in treating Ménière's disease. / å† æ·‹å·´ç®¡å¤¹é—­æœ¯æ²»ç–—æ¢ å°¼åŸƒç— çš„æ•ˆæžœ.

OBJECTIVES: Ménière's disease (MD) is an idiopathic inner ear disorder characterized by recurrent episodes of episodic rotational vertigo, fluctuating hearing loss, tinnitus, and a feeling of ear stuffiness. Endolymphatic sac (ES)-related surgery is used primarily in patients with MD who have failed to respond to pharmacologic therapy. Endolymphatic duct blockage (EDB) is a new procedure for the treatment of MD, and related clinical studies are still scarce. This study aims to investigate the dynamic changes in endolymphatic hydrops (EH) and the long-term surgical outcomes in MD patients undergoing EDB, and to evaluate the impact of different types of ES on the surgical efficacy. METHODS: A retrospective analysis was conducted on 33 patients with refractory MD who underwent EDB. Based on the morphology of their endolymphatic sacs, patients were divided into a normal-type group (n=14) and an atrophic-type group (n=19). The frequency of vertigo symptoms, hearing, vestibular function, and the dynamic changes of gadolinium-enhanced MRI of the inner ear were compared were compared before and after surgery between the 2 groups. RESULTS: Compared with the atrophic-type group, the patients in the normal-type group had a higher rate of complete vertigo control, better cochlear and vestibular function, and a lower endolymph to vestibule volume ratio (all P<0.05). In addition, 7 patients in the normal-type group were found to have reversal of EH, while no reversal of EH was detected in the atrophic-type group after surgery. CONCLUSIONS: The response to EDB treatment varies between normal and atrophic MD patients, suggesting that the 2 pathological types of endolymphatic sacs may have different underlying mechanisms of disease.

Delayed post gadolinium MRI descriptors for Meniere's disease: a systematic review and meta-analysis.

OBJECTIVES: Delayed post-gadolinium magnetic resonance imaging (MRI) detects changes of endolymphatic hydrops (EH) within the inner ear in Meniere's disease (MD). A systematic review with meta-analysis was conducted to summarise the diagnostic performance of MRI descriptors across the range of MD clinical classifications. MATERIALS AND METHODS: Case-controlled studies documenting the diagnostic performance of MRI descriptors in distinguishing MD ears from asymptomatic ears or ears with other audio-vestibular conditions were identified (MEDLINE, EMBASE, Web of Science, Scopus databases: updated 17/2/2022). Methodological quality was evaluated with Quality Assessment of Diagnostic Accuracy Studies version 2. Results were pooled using a bivariate random-effects model for evaluation of sensitivity, specificity and diagnostic odds ratio (DOR). Meta-regression evaluated sources of heterogeneity, and subgroup analysis for individual clinical classifications was performed. RESULTS: The meta-analysis included 66 unique studies and 3073 ears with MD (mean age 40.2-67.2 years), evaluating 11 MRI descriptors. The combination of increased perilymphatic enhancement (PLE) and EH (3 studies, 122 MD ears) achieved the highest sensitivity (87% (95% CI: 79.92%)) whilst maintaining high specificity (91% (95% CI: 85.95%)). The diagnostic performance of "high grade cochlear EH" and "any EH" descriptors did not significantly differ between monosymptomatic cochlear MD and the latest reference standard for definite MD (p = 0.3; p = 0.09). Potential sources of bias were case-controlled design, unblinded observers and variable reference standard, whilst differing MRI techniques introduced heterogeneity. CONCLUSIONS: The combination of increased PLE and EH optimised sensitivity and specificity for MD, whilst some MRI descriptors also performed well in diagnosing monosymptomatic cochlear MD. KEY POINTS: • A meta-analysis of delayed post-gadolinium magnetic resonance imaging (MRI) for the diagnosis of Meniere's disease is reported for the first time and comprised 66 studies (3073 ears). • Increased enhancement of the perilymphatic space of the inner ear is shown to be a key MRI feature for the diagnosis of Meniere's disease. • MRI diagnosis of Meniere's disease can be usefully applied across a range of clinical classifications including patients with cochlear symptoms alone.

Improving diagnostic accuracy for probable and definite Ménière's disease using magnetic resonance imaging.

PURPOSE: To determine whether magnetic resonance imaging (MRI) can improve diagnostic accuracy for definite and probable Ménière's disease (MD) based on perilymphatic enhancement (PE) and endolymphatic hydrops (EH). METHODS: 363 patients with unilateral MD (probable MD, n = 75 and definite MD, n = 288) were recruited. A three-dimensional zoomed imaging technique with parallel transmission SPACE real inversion recovery was performed 6 h after intravenous gadolinium injection to investigate the presence of PE and to evaluate the grading and location of EH. PE and EH characteristics were analyzed and compared between the probable and definite MD groups. RESULTS: The cochlear and vestibular EH grading on the affected side was more severe in the definite MD group than that in the probable MD group (P < 0.001). The EH locations within the inner ear on the affected side also differed between the two groups (χ2 = 81.15, P < 0.001). The signal intensity ratio (SIR) on the affected side was significantly higher in the definite MD group than in the probable MD group (t = 2.18, P < 0.05). The assessment of the combination of PE and EH parameters within the inner ear revealed a higher area under the curve (AUC) in the definite MD group (0.82) compared with the AUCs of the parameters assessed alone. CONCLUSION: The assessment of a combination of PE and EH parameters improved the diagnostic accuracy for probable and definite MD, suggesting that MRI findings may be clinically useful in the diagnosis of MD.

The Diagnostic Value of Multifrequency Tympanometry in Patients with Ménière's Disease: A Prospective Analysis.

INTRODUCTION: Diagnosing Ménière's disease (MD) by its characteristics such as episodes of vertigo, fluctuating hearing loss, and tinnitus with aural fullness remains challenging. Available tests evaluating the presence of endolymphatic hydrops (EH) are often expensive or time assuming. An in-office quick and simple non-invasive diagnostic test is multifrequency tympanometry (MFT). It can measure conductance at 2 kHz probe tones, which was demonstrated to reflect variations in cochlear pressure. Previous studies investigating MFT as a diagnostic test for MD showed conflicting outcomes possibly biased by their retrospective design. METHODS: We prospectively collected MFT results (Y width) in patients with dizziness and compared MFT test results in affected (group 1) and unaffected (group 2) ears of 37 MD subjects and in control ears of 33 non-MD subjects (group 3). RESULTS: The mean value of the Y width in affected ears was 315.6 ± 70.2 daPa compared to 292.3 ± 98.6 daPa in unaffected ears in MD subjects and 259.4. ± 60.6 daPa in the non-MD group. A positive test result (i.e., a Y width of 235 daPa or more) was found in 35 ears in the MD group, 21 times involving the affected ear and 14 times involving the unaffected ear, compared to 16 in the non-MD group. No significant differences between the three groups could be demonstrated (p > 0.05). We found a sensitivity of 58.3% and specificity of 66.3% for detecting EH in an affected ear in MD subjects. CONCLUSION: There is a trend towards increased conductance tympanometry in affected ears. However, we noticed a high false positive rate of MFT and do not support standardized use of MFT as an additional diagnostic tool for detecting EH in MD patients. A negative test result on the contrary is unlikely related to EH.

Characterizing a Joint Reflection-Distortion OAE Profile in Humans With Endolymphatic Hydrops.

OBJECTIVES: Endolymphatic hydrops (EH), a hallmark of Meniere disease, is an inner-ear disorder where the membranes bounding the scala media are distended outward due to an abnormally increased volume of endolymph. In this study, we characterize the joint-otoacoustic emission (OAE) profile, a results profile including both distortion- and reflection-class emissions from the same ear, in individuals with EH and speculate on its potential utility in clinical assessment and monitoring. DESIGN: Subjects were 16 adults with diagnosed EH and 18 adults with normal hearing (N) matched for age. Both the cubic distortion product (DP) OAE, a distortion-type emission, and the stimulus-frequency (SF) OAE, a reflection-type emission, were measured and analyzed as a joint OAE profile. OAE level, level growth (input/output functions), and phase-gradient delays were measured at frequencies corresponding to the apical half of the human cochlea and compared between groups. RESULTS: Normal hearers and individuals with EH shared some common OAE patterns, such as the reflection emissions being generally higher in level than distortion emissions and showing more linear growth than the more strongly compressed distortion emissions. However, significant differences were noted between the EH and N groups as well. OAE source strength (a metric based on OAE amplitude re: stimulus level) was significantly reduced, as was OAE level, at low frequencies in the EH group. These reductions were more marked for distortion than reflection emissions. Furthermore, two significant changes in the configuration of OAE input/output functions were observed in ears with EH: a steepened growth slope for reflection emissions and an elevated compression knee for distortion emissions. SFOAE phase-gradient delays at 40 dB forward-pressure level were slightly shorter in the group with EH compared with the normal group. CONCLUSIONS: The underlying pathology associated with EH impacts the generation of both emission types, reflection and distortion, as shown by significant group differences in OAE level, growth, and delay. However, hydrops impacts reflection and distortion emissions differently. Most notably, DPOAEs were more reduced by EH than were SFOAEs, suggesting that pathologies associated with the hydropic state do not act identically on the generation of nonlinear distortion at the hair bundle and intracochlear reflection emissions near the peak of the traveling wave. This differential effect underscores the value of applying a joint OAE approach to access both intracochlear generation processes concurrently.

Meniere's disease: Pathogenesis, treatments, and emerging approaches for an idiopathic bioenvironmental disorder.

Meniere's disease (MD) is a severe inner ear condition known by debilitating symptoms, including spontaneous vertigo, fluctuating and progressive hearing loss, tinnitus, and aural fullness or pressure within the affected ear. Prosper Meniere first described the origins of MD in the 1860s, but its underlying mechanisms remain largely elusive today. Nevertheless, researchers have identified a key histopathological feature called Endolymphatic Hydrops (ELH), which refers to the excessive buildup of endolymph fluid in the membranous labyrinth of the inner ear. The exact root of ELH is not fully understood. Still, it is believed to involve several biological and bioenvironmental etiological factors such as genetics, autoimmunity, infection, trauma, allergy, and new theories, such as saccular otoconia blocking the endolymphatic duct and sac. Regarding treatment, there are no reliable and definitive cures for MD. Most therapies focus on managing symptoms and improving the overall quality of patients' life. To make significant advancements in addressing MD, it is crucial to gain a fundamental understanding of the disease process, laying the groundwork for more effective therapeutic approaches. This paper provides a comprehensive review of the pathophysiology of MD with a focus on old and recent theories. Current treatment strategies and future translational approaches (with low-level evidence but promising results) related to MD are also discussed, including patents, drug delivery, and nanotechnology, that may provide future benefits to patients suffering from MD.

Temporal changes in endolymphatic hydrops on MRI with or without intervention: A systematic review.

OBJECTIVE: The pathophysiology of Meniere's Disease (MD) involves endolymphatic hydrops (ELH) of the inner ear. Magnetic Resonance Imaging (MRI) has been shown to detect ELH, but changes in ELH have been poorly described using this modality. Our objective was to review MRI-measured changes in ELH over time and after medical and/or surgical intervention in patients with MD. We secondarily aim to associate changes in ELH with changes in MD symptomatology. DATABASES REVIEWED: Medline, Web of Science, and Embase databases. METHODS: A systematic review of articles was performed to identify studies utilizing MRI to measure ELH changes over time, and after medical or surgical treatment. Articles on non-human subjects and without direct measurement of ELH were excluded. RESULTS: Of 532 studies identified, 12 were included, involving 170 patients (mean age 56.3 years). Ten studies were prospective; two were retrospective. Five studies strictly utilized medical means of intervention, four utilized surgical treatments, one utilized both, and two observed temporal changes without treatment. Across all interventions, 72.1 % of patients exhibited the same or worsening ELH on imaging. In studies reporting vertigo outcomes, 95.9 % of patients exhibited improvement after the treatment period. CONCLUSION: Medical and surgical interventions often yield symptomatic relief of vertigo in MD patients despite stable or increasing ELH volume. MRI may have greater clinical utility in diagnosing ELH as opposed to assessing treatment response.

The clinical findings to notice mild elevation of intracranial pressure in an otology clinic.

Mildly elevated intracranial pressure appears to be a distinct pathology separate from idiopathic increased intracranial pressure and migraine. Many patients present with head fullness-pressure and dizziness, which is often suggestive of a clinical diagnosis of vestibular migraine. These patients may additionally have episodic vertigo as seen in endolymphatic hydrops and positional vertigo in addition to feeling dizzy. In most cases, hearing is normal. A long duration of the condition or a presence of associated ear pathologies, i.e., dehiscence, fistula, hypermobile footplate, or history of chronic ear infections can add hearing loss to clinical presentations. Low-pitch pulsatile tinnitus, when present, is an important symptom. The neuroimaging findings such as partially empty sella, blockage of the dural venous sinus or sigmoid sinus wall defects are frequently observed. The condition is diagnosed by measuring the lumbar puncture opening pressure. Typically, patients have normal cerebrospinal fluid chemistry and microscopy. Lowering the pressure during the lumbar puncture will resolve the patient's symptoms temporarily. Medical therapy is typically successful using carbonic anhydrase inhibitors and corrections of medical disorders that may be contributing to the increased pressure. A few require shunting or stenting procedures. In this review article, we define the condition in detail with illustrative cases that we collected from our practices.

Systematic review of the diagnostic value of hydrops MRI in relation to audiovestibular function tests (electrocochleography, cervical vestibular evoked myogenic potential and caloric test).

The objective of this systematic review is to compare the diagnostic value of endolymphatic hydrops (EH) magnetic resonance imaging (MRI) with audiovestibular function tests, including electro cochleography (ECochG), cervical vestibular evoked myogenic potential (cVEMP) and caloric tests for the diagnosis of definite Meniere's disease (DMD). An electronic search was performed in the PubMed, Embase and Cochrane databases in August 2022. Original studies which reported the efficacy of gadolinium MRI for diagnosis of DMD were compared with ECochG, cVEMP and caloric tests from 2007 to 2022 published in English. Two reviewers extracted the methodology and results of MRI and functional tests, assessing them independently. A modified version of the Quality Assessment of Diagnostic Accuracy Studies (QUADAS-2) was used for the assessment of the quality and the risk of bias of each study. The proportion of DMD cases diagnosed by MRI hydrops vs corresponding functional tests were calculated and the relationship between MRI and functional tests were evaluated using the Cohen's Kappa test. Concerning the MRI, the proportion diagnostic of DMD was 0.67 by cochlear EH and 0.80-0.82 by vestibular EH. Regarding the functional test, the propotiojn diagnostic of DMD was 0.48 by ECochG, 0.76 by cVEMP and 0.65 by caloric test. The findings of this systematic review were that the vestibular EH on imaging most effectively assisted in diagnosing DMD. Among the functional tests, cVEMP was the second most effective test. The agreement between imaging and cVEMP was moderate (0.44), indicating a gap between the patients identified by the imaging and functional tests based on the relatively small number of patients.

Is there progression of endolymphatic hydrops in Ménière's disease? Longitudinal magnetic resonance study.

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.

Assessing the usefulness of inner ear contrast-enhanced magnetic resonance imaging and pure-tone audiometry in estimating the prognostic value of hearing improvement in patients with definite Meniere's disease.

PURPOSE: Meniere's disease (MD) is characterized by combined cochlear and vestibular symptoms. However, its underlying cause remains unclear, with low-to-mid-tone hearing impairment being predominantly reported. Moreover, predicting hearing improvement after disease onset is not possible. This study aimed to assess whether hearing improvement in patients with definite MD (DMD) could be predicted using inner ear contrast magnetic resonance imaging (IEC-MRI) and pure-tone audiometry (PTA) at disease attack. MATERIALS AND METHODS: Between April 2020 and March 2022, seven outpatients with DMD were enrolled based on the Bárány Society DMD criteria. The patients were divided into two groups: low-tone hearing loss (LTL) group and low-to-mid-tone hearing loss (LMTL) group. Hearing improvement rates as well as the possible inter-relation between endolymphatic hydrops and hearing improvement were evaluated. RESULTS: Endolymphatic hydrops was found in two of four LTL cases. One of three LMTL cases had prominent lymphedema. All patients with LTL but only one patient with LMTL had hearing improvement. Endolymphatic hydrops was not found to be correlated with hearing improvement. CONCLUSIONS: Estimating hearing improvement in patients with DMD using IEC-MRI was not possible. However, PTA showed better hearing prognosis in LTL than in LMTL. Therefore, estimating hearing improvement using PTA was possible at disease attack.

Endolymphatic hydrops imaging and correlation with clinical characteristics, audiovestibular function and mental impairment in patients with Meniere's disease.

PURPOSE: MR imaging was used to visualize the vestibular and cochlear endolymphatic hydrops in patients with Meniere's disease (MD). The relationship between the degree of hydrops and clinical characteristics, audiovestibular function, anxiety and depression state in MD patients. METHODS: 70 patients with definitely or probably unilateral Meniere's disease received bilateral intratympanic gadolinium administration and MR scanning. The degree of bilateral vestibular and cochlea hydrops were analyzed and evaluated by three-dimensional real inversion recovery (3D-real IR) sequence, and the correlation between the grades of endolymphatic hydrops (EH) and disease course, vertigo grading assessment, the duration of vertigo, hearing loss level, caloric test, vestibular myogenic evoked potential (VEMP), electrocochleogram (EcoG), vertigo disability scale (physical, emotional, functional), anxiety and depression scale were studied. RESULTS: It was found that the vestibule and cochlea EH of the affected and the contralateral ear had different degrees of hydrops and there was no statistical difference between the left and right vestibules. The degree of vestibule EH (V-EH) was significantly positively correlated with the degree of cochlear EH (C-EH). C-EH and hearing loss level were positively correlated with EcoG. There was positive correlation between vestibular EH and hearing loss level, VEMP, caloric test, disease course or vertigo duration. There was a negative relationship between Dizziness Handicap Inventory (Emotion) (DHI(E)) and VEMP. Self-rating Anxiety Scale (SAS) and Self-rating Depression Scale (SDS) scores were positive correlated with DHI(E) and DHI total scores in MD patients. CONCLUSION: Endolymph-enhancing MRI was used as an important imaging method for the diagnosis of labyrinthine hydrops in Meniere's disease. There were certain correlation between EH and the degree of vertigo attack, hearing loss level, vestibular function, and further changes in anxiety and depression emotion.

Limitation of updated MR images on the vestibular hydrops in Meniere's disease.

PURPOSE: This study adopted the cervical and ocular vestibular-evoked myogenic potential (cVEMP and oVEMP) tests in Meniere's disease (MD) patients to correlate them with vestibular endolymphatic hydrops (EH) on MR images. METHODS: A total of 25 patients with unilateral definite MD identified by positive cochlear hydrops on MR images were enrolled. All patients underwent audiometry, cVEMP test and oVEMP test, followed by MR imaging for confirmation. RESULTS: A significantly declining sequence of abnormality rates in MD patients was identified from the audiometry (92%), cVEMP test (52%) to the oVEMP test (40%), which was consistent with a significantly decreasing order of prevalence of EH on MR images running from the cochlea (100%), saccule (56%) to the utricle (52%). The cVEMP test for detecting the saccular hydrops revealed a sensitivity of 62%, while the oVEMP test for assessing the utricular hydrops showed a sensitivity of 70%. However, correlating VEMP results with vestibular hydrops did not show any significant relationship. In addition, mean hearing level (MHL) at four frequencies (500, 1000, 2000, and 3000 Hz) of Grade I cochlear hydrops (51 ± 19 dB) did not significantly differ from Grade II cochlear hydrops (53 ± 19 dB). CONCLUSION: Limitations of the updated MR imaging for visualizing the hydrops comprised: (1) failure to correlate vestibular hydrops with VEMP results, and (2) failure to correlate grade of cochlear hydrops with MHL. The reason is probably because updated MR imaging fails to identify distorted contour of the cochlea/utricle/saccule. Further advanced technique using ultrahigh resolution of fine structures in the inner ear compartments is essential to promote a wider use of MR imaging.