Objective: Evaluation of the effectiveness and posttreatment effects of intratympanic gentamicin and corticosteroids in treating patients with Ménière\'s disease (MD). Methods: Based on PubMed and Embase databases, randomized controlled trials using intratympanic injections of 4 drugs (gentamicin, methylprednisolone, dexamethasone, and placebo) for the treatment of MD were searched from 1995 to October 2023, and the literature was screened according to inclusion and exclusion criteria, and data were netted for meta-analysis using Stata 17. Results: A total of 13 studies were selected, involving 559 participants, with follow-up time ranging from 3 to 28 months. Meta-analysis showed that there was no statistically significant difference in pure-tone average between gentamicin and dexamethasone [standardized mean difference (SMD) = 0.09, 95% confidence interval (CI) (-0.42, 0.24), P < .05]. Compared to placebo, intratympanic injection of gentamicin [risk ratio (RR) = 1.18, 95% CI (0.43, 1.93)], methylprednisolone [RR = 0.88, 95% CI (0.07, 1.70)], and dexamethasone [RR = 0.70, 95% CI (-0.01, 1.41)] all showed better efficacy in treating vertigo. For the treatment of tinnitus, the SUCRA ranking results showed that dexamethasone was the most effective, followed by methylprednisolone and gentamicin. Conclusion: Pharmacological intervention is more effective than placebo in treating MD. Although gentamicin treatment shows significant effects in treating vertigo, corticosteroid combination therapy is markedly superior to gentamicin in controlling hearing loss and vertigo symptoms.

BACKGROUND: Diagnostic dilemma between clinical Meniere\'s disease and radiological endolymphatic hydrops (EH) has emerged since the introduction of hydrops magnetic resonance imaging (MRI). The aim of this study is to explore the potential application of hydrops MRI on diagnosing the EH.
METHODS: This review was developed from peer-reviewed articles published in those journals listed on journal of citation reports. The MEDLINE database of the US National Library of Medicine, Scopus, and Google Scholar were used to collect articles based on the guidelines (PRISMA 2020 statement) for reporting reviews.
RESULTS: Initially, 470 articles were retrieved from 1983 to 2023, and 80 relevant articles were ultimately selected. The sensitivity (69%-92%) and specificity (78%-96%) values varied from each laboratory for detecting EH via hydrops MRI, probably due to candidate selection and the grading system employed.
CONCLUSIONS: The application of hydrops MRI allows (1) differentiation between EH and sudden sensorineural hearing loss; (2) determination of the affected side of EH; and (3) confirmation of the diagnosis of EH concomitant with other disorders. Notably, not all differentials for EH can be visualized on MR images. One of the existing gaps to be filled is that updated hydrops MRI fails to identify distortion, that is, rupture, collapse, fistula, or fibrosis of the inner ear compartments, akin to what histopathological evidence can demonstrate. Hence, enhanced ultrahigh resolution of hydrops MRI is required for demonstrating fine structures of the inner ear compartments in the future.

(1) Background: Ménière\'s disease (MD) is a disease of the inner ear, presenting with episodes of vertigo, hearing loss, and tinnitus.The aim of this study is to examine the role of multifrequency tympanometry (MFT) in the diagnosis of MD. (2) Methods: A systematic review of MEDLINE (via PubMed), Scopus, Google Scholar, and the Cochrane Library was performed, aligned with the PRISMA guidelines. Only studies that directly compare ears affected by Ménière\'s disease with unaffected or control ears were included. Random-effects model meta-analyses were performed. (3) Results: Seven prospective case-control studies reported a total of 899 ears, 282 of which were affected by Ménière\'s disease (affected ears-AE), 197 unaffected ears in patients with MD (UE), and 420 control ears (CE) in healthy controls. No statistically significant differences between the groups were observed regarding resonant frequency (RF). The pure tone audiometry average of the lower frequencies (PTA basic) was significantly greater in affected ears when compared with unaffected ears. The conductance tympanogram at 2 kHz revealed a statistically significantly greater G width of 2 kHz in the affected ears when compared to both unaffected and control ears, while control ears had a statistically significant lesser G width of 2 kHz compared to both the other two groups. (4) Conclusions: MFT, and specifically G width at 2 kHz, could be an important tool in the diagnosis of MD.

Low-frequency air-bone gap (ABG) associated with pulsatile tinnitus (PT) and normal impedance audiometry represents a common finding in patients with third window syndromes. Other inner disorders, including Meniere\'s disease (MD), perilymphatic fistula and intralabyrinthine schwannoma, might sometimes result in a similar scenario. On the other hand, PT is frequently associated with dural arteriovenous fistula (DAVF), while conductive hearing loss (CHL) is extremely rare in this clinical setting. A 47-year-old patient was referred to our center with progressive left-sided PT alongside ipsilateral fullness and hearing loss. She also experienced headache and dizziness. Otoscopy and video-oculographic examination were unremarkable. Conversely, a detailed instrumental audio-vestibular assessment revealed low-frequency CHL with normal impedance audiometry, slight left-sided caloric weakness, slightly impaired vestibular-evoked myogenic potentials on the left and normal results on the video-head impulse test, consistent with an MD-like instrumental profile. Gadolinium-enhanced brain MRI revealed an early enhancement of the left transverse sinus, consistent with a left DAVF between the left occipital artery and the transverse sinus, which was then confirmed by angiography. A trans-arterial embolization with Onyx glue was performed, resulting in a complete recession of the symptoms. Post-operatively, the low-frequency ABG disappeared, supporting the possible role of venous intracranial hypertension and abnormal pressure of inner ear fluids in the onset of symptoms and offering new insights into the pathomechanism of inner ear CHL.

OBJECTIVE: To analyze, by means of a systematic review and meta-analysis, the proportion of patients with Meniere\'s disease who have altered caloric test and vHIT, as well as to determine the prevalence of altered caloric test and normal vHIT dissociation in the diagnosis of Meniere\'s disease.
METHODS: The literature search had no restriction regarding the period of publication on the following indexed data platforms: PubMed, PubMed PMC, BVS-Bireme, Web of Science, Embase and Cochrane Library. Articles that evaluated patients with Meniere\'s disease who underwent caloric test and vHIT were included. Two researchers independently conducted the analysis of the articles, promoting the selection and capture of data, following the recommendations of the PRISMA method, and complying with the criteria for articles inclusion and exclusion defined in the research protocol. In case of disagreement during the selection process, a third researcher was included for analysis.
RESULTS: From a total of 427 initial studies, the researchers selected 12 articles, published between 2014 and 2021, with a total of 708 patients evaluated, with a mean age of 52.72 years old. The prevalence of patients with Meniere\'s disease with altered caloric reflex test was 64% (95% CI 57%‒71%), while the prevalence of altered vHIT was only 28% (95% CI 16%-40%). The prevalence of the altered caloric test + normal vHIT dissociation was 47% (95% CI 37%-57%).
CONCLUSIONS: The video head impulse test and the caloric test are valuable tools for vestibular assessment. The dissociation of findings between these two tests in patients with Meniere\'s disease was more prevalent in this meta-analysis and may be a result of the tonotopy of specialized hair cells in the ampullary crest. The prevalence of altered caloric test was 64% and anormal vHIT was 28%. The dissociation caloric asymmetry and normal vHIT was observed in 47% of the patients.
METHODS:

BACKGROUND: Meniere\'s disease (MD) is a complex disease that can severely affect the quality of life. In this systematic review and meta-analysis, we aimed to investigate the effect of vestibular rehabilitation (VR) versus control/other interventions on the quality of life in patients with MD.
METHODS: We searched six electronic databases (PubMed/MEDLINE, Web of Science, EMBASE, Scopus, ProQuest, CENTRAL) from inception to September 30, 2022 with no language restriction for publications comparing the effect of VR with control/ other interventions in patients with MD. The primary outcome was quality of life assessed by dizziness handicap inventory (DHI).
RESULTS: Overall, three studies with a total of 465 patients were included in the meta-analysis. All the included studies reported immediate-term DHI scores. A medium effect (standardized mean difference [SMD] = - 0.58, 95% confidence interval [CI] - 1.12; - 0.05) was observed favoring the use of VR to improve DHI scores in patients with MD in the immediate term. Moreover, there was severe heterogeneity in immediate DHI scores among the included studies (χ2 = 22.33, P = 0.00, I2 = 82.1%).
CONCLUSIONS: VR rehabilitation can improve the quality of life in patients with MD immediately after treatment. Since all the included studies had a high risk of bias and none had long-term follow-ups, further high-quality research is required to determine the short-, intermediate-, and long-term effects of VR compared to control/other interventions.

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.

Meniere\'s disease is an inner ear disorder characterized by the presence of endolymphatic hydrops in the inner ear and symptomatology of recurrent and debilitating vertigo attacks, tinnitus, aural fullness, and fluctuating sensorineural hearing loss. Although many therapeutic options for MD have been proposed during years, no consensus has been reached by the scientific community. In the last decade, many therapeutic options have been proposed, as intratympanic steroid, intratympanic gentamicin, and intravenous glycerol. Recently, the role of the antisecretory factor in the diet of MD patients have been investigated. Surgery is recommended for intractable MD; some authors proposed new approaches including transcanal endoscopic infracochlear vestibular neurectomy, new marsupiliazation technique in sac surgery, and tenotomy of the stapedius and tensor tympani muscles.

To investigate literature about pharmacological, surgical, and diagnostic innovations for Meniere\'s Disease (MD).
Meniere\'s disease is an inner ear disorder characterized by the presence of endolymphatic hydrops in the inner ear and symptomatology of recurrent and debilitating vertigo attacks, tinnitus, aural fullness, and fluctuating sensorineural hearing loss. Although many therapeutic options for MD have been proposed during years, no consensus has been reached by the scientific community. In the last decade, many therapeutic options have been proposed, as intratympanic steroid, intratympanic gentamicin, and intravenous glycerol. Recently, the role of the antisecretory factor in the diet of MD patients have been investigated. Surgery is recommended for intractable MD; some authors proposed new approaches including transcanal endoscopic infracochlear vestibular neurectomy, new marsupiliazation technique in sac surgery, and tenotomy of the stapedius and tensor tympani muscles.

Familial Meniere Disease (FMD) is a rare inner ear disorder characterized by episodic vertigo associated with sensorineural hearing loss, tinnitus and/or aural fullness. We conducted a systematic review to find sequencing studies segregating rare variants in FMD to obtain evidence to support candidate genes for MD. After evaluating the quality of the retrieved records, eight studies were selected to carry out a quantitative synthesis. These articles described 20 single nucleotide variants (SNVs) in 11 genes (FAM136A, DTNA, PRKCB, COCH, DPT, SEMA3D, STRC, HMX2, TMEM55B, OTOG and LSAMP), most of them in singular families-the exception being the OTOG gene. Furthermore, we analyzed the pathogenicity of each SNV and compared its allelic frequency with reference datasets to evaluate its role in the pathogenesis of FMD. By retrieving gene expression data in these genes from different databases, we could classify them according to their gene expression in neural or inner ear tissues. Finally, we evaluated the pattern of inheritance to conclude which genes show an autosomal dominant (AD) or autosomal recessive (AR) inheritance in FMD.