Diagnosis Of Meniere's Disease Research Articles

Labor market effects of rehabilitation for patients diagnosed with dizziness - a Danish nationwide register-based cohort study.

Both vestibular neuronitis (VN) and Meniere's disease (MD) have great impact on quality of life and are associated with a significant number of sick leave days absent from work. The aim was to assess labor market participation rate one year after hospital diagnosis of VN and MD and the use of rehabilitation measures. Nationwide register-based cohort study including patients with VN (n = 1,341) and MD (n = 843) and control persons matched in 1:5 with a VN cohort control (n = 6,683) and MD cohort control (n = 4,209). Compared to control persons, VN patients were more likely to be single, have higher income, and a higher Charlson comorbidity index score. MD patients had a higher level of education and a higher Charlson index compared to control persons. One year after patients were diagnosed with VN, no significant difference in labor market participation was observed (p = 0.88). However, MD patients had a 10.4% reduced probability of possessing a full-time job one year after diagnosis compared to matched control persons (58.1 ± 0.5% vs. 68.5 ± 0.5%, p < 0.001). Both VN and MD patients consulted otorhinolaryngologists, general practitioners, and physiotherapists more than control persons both before and after the initial diagnosis (p < 0.01). In addition, MD patients also consulted psychologists more frequently before and after diagnosis of the disease (p < 0.01). Intrahospital diagnosed MD increases the risk of leaving the labor market in opposition to VN. Both MD and VN are associated with significant expenses to the Danish health care system from the use of public rehabilitation measures and medical consultations.

Comparing the Saccule-to-Utricle Ratio in Early- Versus Late-Stage Meniere's Disease Patients.

To compare the saccule-to-utricle ratio in early- versus late-stage Meniere's disease (MD) patients based on magnetic resonance imaging (MRI) images. In this retrospective study, we performed 3-dimensional real inversion recovery (3D-real IR) MRI 24 h after intratympanic gadolinium administration in unilateral MD patients at early-stage (n = 56) and late-stage (n = 70). Two radiologists independently graded endolymphatic hydrops (EH) and the saccule-to-utricle ratio inversion (SURI) was compared between the two groups. Furthermore, early-stage MD patients were further divided into two subgroups based on disease duration: ≤6 months (n = 20) and >6 months (n = 36) and the SURI was compared. Among the 56 patients in the early-stage group, 26 cases (46.43%) exhibited an enlarged saccule that is larger than the utricle, showing SURI. In contrast, among the late-stage MD, only four cases (5.71%) showed SURI (p < 0.001). In the early-stage MD subgroup with a disease duration of ≤6 months, the proportion of SURI was 70% (14/20), which was higher than that in the subgroup with a disease duration of >6 months (33.33%, 12/36, p = 0.02). SURI may serve as an effective imaging marker for diagnosis of early-stage MD. Our finding suggests that endolymphatic hydrops in MD may primarily originate from the saccule. Level 3 Laryngoscope, 2024.

Selective Vestibular Neurectomy through the Presigmoid Retrolabyrinthine Approach in the Treatment of Meniere's Disease.

Meniere's disease (MD) is a disabling disease, especially in patients who are refractory to medical therapy. Moreover, selective vestibular neurectomy (VN), in these selected cases, can be considered a surgical alternative which preserves hearing function and facial nerve. We retrospectively studied 23 patients with MD diagnosis and history of failed extradural endolymphatic sac surgery (ELSS) who underwent combined micro-endoscopic selective VN, between January 2019 and August 2023, via a presigmoid retrolabyrinthine approach. All patients were stratified according to clinical features, assessing preoperative and postoperative hearing levels and quality of life. At the maximum present follow-up of 2 years, this procedure is characterized by a low rate of complications and about 90% vertigo control after surgery. No definitive facial palsy or hearing loss was described in this series. One patient required reintervention for a CSF fistula. Statistically significant (p = 0.001) difference was found between the preoperative and the postoperative performance in terms of physical, functional, and emotive scales assessed via the DHI questionnaire. Selective VN via a presigmoid retrolabyrinthine approach is a safe procedure for intractable vertigo associated with MD, when residual hearing function still exists. The use of the endoscope and intraoperative neuromonitoring guaranteed a precise result, saving the cochlear fibers and facial nerve. The approach for VN is a familiar procedure to the otolaryngologist, as is lateral skull base anatomy to the neurosurgeon; therefore, the best results are obtained with multidisciplinary teamwork.

Evolution of Vestibular Findings During and Between the Attacks of Meniere Disease: Update.

The diagnosis of Meniere disease (MD) has based on characteristics of vertigo and findings of audiologic evaluation. This review focuses on the recent findings of the evolution of vestibular function and their underlying physiology during and between the attacks of MD and thus aims to help identify this common disorder with many faces according to the phase. During the attacks, the direction of spontaneous nystagmus changes over time, beating initially toward the affected ear (irritative nystagmus), then toward the healthy ear (paretic nystagmus), and finally back toward the affected ear again (recovery nystagmus). Apart from these direction changes, atypical forms of spontaneous nystagmus, such as downbeat, discordant horizontal-torsional, and aperiodic alternating nystagmus, can be observed. Head impulse tests (HITs) are mostly normal during the irritative/recovery phases, but positive in more than half of patients during the paretic phase. By contrast, caloric tests are usually abnormal irrespective of the phases, although paradoxical caloric hyper-responsiveness can be observed in 18% of patients during the irritative/recovery phases. Thus, dissociation in the findings of caloric tests-HITs can be observed during and between the attacks. Horizontal head shaking tends to augment spontaneous nystagmus during each phase, while skull vibration mostly induces nystagmus beating toward the healthy ear irrespective of the phases. During the attacks, ocular vestibular-evoked myogenic potentials (VEMPs) may be enhanced, whereas cervical VEMPs are usually decreased during stimulation of the involved ear. Recognizing these evolutions of vestibular findings during and between the attacks of MD would provide insights into its pathophysiology and aid in treatments and diagnosis.

Trends in Management of Ménière Disease: A TriNetX Network Database Analysis.

This study investigated management practices for Meniere's disease (MD) and their temporal trends from 2008 to 2022 within the TriNetX network database. Study cohort included adult patients (≥18 years) with the diagnosis of MD from TriNetX's multi-institutional medical records (n = 77,493). MD diagnosis and management were queried based on the international classification of diseases, tenth revision, current procedural terminology, and RXNorm codes. Temporal trends were analyzed using joinpoint regression. There was significant increase in rates of relevant medications prescribed within 12 months of MD diagnosis from 2008 to 2022 (annual percent change[APC]: 1.2 [95% confidence interval, CI: 0.4-1.9]). There were no significant changes in rate of intratympanic injection within 12 months of MD diagnosis (1.7 [95% CI: -1.1 to 4.5]). Rate of endolymphatic sac surgery and labyrinthectomy any time after MD diagnosis gradually decreased from 2008 to 2022 at APC of -8.1 (95% CI: -11.8 to -4.2) and -11.0 (95% CI: -14.0 to -7.7), respectively. Use of relevant medications has significantly increased during the early management of MD and the overall use of surgical treatments has decreased.

Differentiating Meniere's Disease and Vestibular Migraine: Insights from Gadolinium-Enhanced Magnetic Resonance Imaging and Clinical Features.

To explore the predictive factors between Meniere's disease (MD) and vestibular migraine (VM) by Gadolinium-enhanced Magnetic resonance imaging (MRI) of the inner ear and Clinical Features. Eighty-seven patients (50 MD and 37 VM) underwent intratympanic injection of gadolinium and MRI was performed 24 h later. All patients underwent pure tone audiometry and caloric tests. In the MD group, 46 (92%) of 50 patients developed endolymphatic hydrops, although only 2 (5.4%) in the vestibular migraine (VM) group had positive results groups (p < 0.001). The incidence of migraine was 14% in the MD group and 67.7% in the VM group (p < 0.001). Multivariate logistic regression of the two groups of patients indicated that the greater the sum of the maximum slow phase velocity (SPV) of the ipsilateral ear, the higher the risk of VM occurrence (p = 0.009). The incidence of carsickness was positively correlated with the incidence of VM (p < 0.001) and asymmetric hearing loss (AHL) was negatively correlated with the diagnosis of VM (p = 0.045). Gadolinium-enhanced MRI of the inner ear is helpful for the differential diagnosis of VM and MD. Carsickness, decreased AHL, and increased Sum of the maximum SPV in the ipsilateral side (SSPVI) may act as diagnostic predictors of VM. 3 Laryngoscope, 134:426-432, 2024.

Comparison of clinical characteristics and vestibular function test results in patients with vestibular migraine and Menière’s disease

ObjectivesThe 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. MethodsSeventy-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. ResultsMost 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). ConclusionsVestibular 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. Level of evidence4.

A bibliometric and visualization study of Meniere's disease: Current status and global hotspots and emerging trends.

Meniere's disease (MD) is a clinical condition characterized by endolymphatic hydrops. Persistent symptoms negatively affect patients mood, and the underlying etiology remains unclear. It is necessary to comprehensively understand the relevant publications, review the history and current status of research, and analyze hotspots and frontiers of research on MD. We retrieved literature on Meniere's disease from 2003 to 2022 from the Web of Science database and extracted the data. Data visualization and analysis was conducted using Cite Space, VOS viewer, an online web tool, and Microsoft Office Power Point 2019. In total, 2847 publications were analyzed. The number of annual publications was relatively stable, with an accelerated upward trend over the past 5 years. The country with the most publications was USA (751, 26.38%), while the University of Munich contributed more publications than any other institution (117, 4.11%). The article titled "Diagnostic criteria for Meniere's disease" by Lopez-Escamez J et al in 2015 was the most cited and co-cited publication, and also had the top co-cited references with the strongest citation bursts. Naganawa S was the author with the most publications (85, 2.99%). The top 3 journals and co-cited journals were Otology Neurotology, Acta Oto Laryngologica, and Laryngoscope. Recently, the key theme words were "sensorineural hearing loss," "therapy," "intratympanic injection method," "vestibular-evoked myogenic potentials," "vestibular migraine," "magnetic resonance imaging," and "meniere's disease." The US has the largest number of publications and research institutions, many European countries have high-quality journals, and Japan has the highest number of scholars. The international opinion on Meniere's disease is relatively uniform. The stepped-therapy for MD is scientific and clear. Intratympanic injection of steroids and intratympanic injection of gentamicin are commonly used, but steroids are considered safer. Saccular dysfunction may be more common in patients with MD than in those with utricular dysfunctions. It is worth paying attention to study the relationship between MD and vestibular migraine through headache. Progress in magnetic resonance imaging technology is still required for the imaging diagnosis of MD.

2D Measurements of the Angle of the Vestibular Aqueduct Using CT Imaging.

Recently, Bächinger et al. developed a software that measures the angle between the vestibular aqueduct proximal to the vestibule and the distal vestibular aqueduct on computed tomography (CT) scans and found differences in the vestibular aqueduct angle between the hypoplastic and degenerative categories of Meniere's disease (MD). Hypoplastic radiological findings were associated with the development of bilateral MD and hypoplastic changes were not found outside of fetal temporal bones and individuals with MD. The purpose of this study is to examine how the software developed by Bächinger et al. performs when applied to a large dataset of adult patients with varied otologic diagnoses. Adult patients who underwent high resolution flat panel CT scans without intravenous contrast (n = 301) were retrospectively reviewed. Measurements of the angle of the vestibular aqueduct were made using the previously developed software tool. The tool could be applied to measure the vestibular aqueduct angle in most CT scans of the temporal bones (n = 572 ears, 95%). While the majority of ears fell within the normal range of &lt;120 degrees (n = 462, 80%), fourteen ears (2.3%) in 13 patients were found to have vestibular aqueduct angles that meet criteria for hypoplastic MD (&gt;140 degrees). Only one of the 13 patients had a diagnosis of MD and not in the ear in the hypoplastic category. An inconsistent pattern of other otologic diagnoses were found among the 13 individuals meeting criteria for hypoplastic MD. Although prior reports indicate the software has prognostic value in individuals with MD, these results suggest that the software may have lower positive predictive value when applied to a large population of individuals with varied otologic diagnoses.

Cyclophosphamide inhibits the progression of Meniere's disease by reducing the generation of circulating immune complex.

Endolymphatic hydrops is a characteristic pathological manifestation of Meniere's disease (MD) that has been previously associated with autoimmunity. Interest in the circulating immune complex (CIC) has increased due to its reported role in the occurrence of MD. The present study aimed to investigate the potential value of serum CIC concentration in the diagnosis of MD and the therapeutic potential of cyclophosphamide (CTX) for the treatment of MD. In the present study, guinea pigs were immunized with isologous crude inner ear antigens to establish an autoimmune MD model. Pure tone audiometry, Vestibular-evoked myogenic potential test, electrocochleography test and auditory brainstem response was applied in this study for assessing the severity of MD in guinea pigs. ELISA was applied to measure CIC, tumor necrosis factor α (TNF-α) and heat shock protein 70 (HSP70) expression levels in the serum samples of different groups of patients. Western blotting was applied to detect the protein expression of HSP70 in inner ear tissues in guinea pigs. Hematoxylin and eosin staining was applied to visualize the spiral ganglions in spiral ganglions models. CIC expression in the inner ear was detected by immunohistochemistry. In vivo experiments were performed to confirm the therapeutic effects of CTX in MD. Serum concentrations of CIC, TNF-α and HSP70 were found to be significantly higher in patients with MD, which were also associated with increases in hearing classification and the severity of endolymphatic hydrops. Using a guinea pig MD model, ELISA results revealed significantly increased serum CIC, TNF-α and HSP70 concentrations compared with those in the control group. ABR results showed that the thresholds in the CTX group were notably decreased compared with that in the dexamethasone group, whereas CIC concentrations in the serum were reduced following dexamethasone and CTX treatments compared with those after saline treatment. In the inner ear tissues, the CIC concentration in CTX group was lower than that in the dexamethasone group. Similarly, reductions in HSP70 and TNF-α concentrations was also observed in a similar manner. Immunohistochemistry staining found notably lower CIC deposition in the inner ear tissues following CTX treatment than that in dexamethasone group. Taken together, higher CIC expression can be used as a biomarker for MD diagnosis. The efficacy of CTX in MD was found to be higher compared with that in dexamethasone, which may be associated with the effective inhibition of CIC, HSP70 and TNF-α generation.

Novel Magnetic Resonance Imaging-Based Method for Accurate Diagnosis of Meniere's Disease.

Background: Pathologically, Meniere's disease symptoms are considered to be associated with endolymphatic hydrops. Examinations revealing endolymphatic hydrops can be useful for accurate Meniere's disease diagnosis. We previously reported a quantitative method for evaluating endolymphatic hydrops, i.e., by measuring the volume of the endolymphatic space using three-dimensional magnetic resonance imaging (MRI) of the inner ear. This study aimed to confirm the usefulness of our methods for diagnosing Meniere's disease. Here, we extracted new explanatory factors for diagnosing Meniere's disease by comparing the volume of the endolymphatic space between healthy volunteers and patients with Meniere's disease. Additionally, we validated our method by comparing its diagnostic accuracy with that of the conventional method.Methods and Findings: This is a prospective diagnostic accuracy study performed at vertigo/dizziness centre of our university hospital, a tertiary hospital. Eighty-six patients with definite unilateral Meniere's disease and 47 healthy volunteers (25 and 33 males, and 22 and 53 females in the control and patient groups, respectively) were enrolled. All participants underwent 3-Tesla MRI 4 h after intravenous injection of gadolinium to reveal the endolymphatic space. The volume of the endolymphatic space was measured and a model for Meniere's disease diagnosis was constructed and compared with models using conventional criteria to confirm the effectiveness of the methods used. The area under the receiver operating characteristic curve of the method proposed in this study was excellent (0.924), and significantly higher than that derived using the conventional criteria (0.877). The four indices, sensitivity, specificity, positive predictive value, and negative predictive value, were given at the threshold; all of these indices achieved higher scores for the 3D model compared to the 2D model. Cross-validation of the models revealed that the improvement was due to the incorporation of the semi-circular canals.Conclusions: Our method showed high diagnostic accuracy for Meniere's disease. Additionally, we revealed the importance of observing the semi-circular canals for Meniere's disease diagnosis. The proposed method can contribute toward providing effective symptomatic relief in Meniere's disease.

Radiological Configuration of the Vestibular Aqueduct Predicts Bilateral Progression in Meniere's Disease.

Objective: Meniere's disease (MD) progresses from unilateral to bilateral disease in up to 50% of patients, often chronically and severely impairing balance and hearing functions. According to previous studies, 91% of bilateral MD patients demonstrate bilateral hypoplasia of the endolymphatic sac (ES) upon histological and radiological examination of their inner ears. Here, we seek to validate a radiological marker for ES hypoplasia that predicts the risk for future progression to bilateral MD in individual patients.Methods: Patients with unilateral MD and radiological evidence for ES hypoplasia in either the clinically affected inner ear (cohort MDuni-hpuni) or both inner ears (cohort MDuni-hpbi) were included. Given our hypothesis that ES hypoplasia critically predisposes the inner ear to MD, we expected progression to bilateral MD only in the MDuni-hpbi cohort. To investigate eventual progression to bilateral MD, clinical, audiometric, and imaging data were retrospectively collected over follow-up periods of up to 31 years.Results: A total of 44 patients were included in the MD-hpuni (n = 15) and MDuni-hpbi (n = 29) cohorts. In line with our radiology-based predictions, none (0/15) of the MD-hpuni patients exhibited progression to bilateral MD, whereas 20/29 (69%) MD-hpbi patients have already progressed to bilateral MD. Using the Kaplan–Meier estimator, bilateral disease progression would be observed in 100% of MD-hpbi patients 31 years after the initial diagnosis with an estimated median time to bilateral progression of 12 years. The nine MD-hpbi patients who, so far, remained with unilateral disease demonstrated a median time since initial (unilateral) MD diagnosis of only 6 years and are thus still expected to progress to bilateral disease.Conclusion: Progression to bilateral MD adheres to predictions based on the radiological presence or absence of ES hypoplasia. This prognostic tool, if validated by prospective long-term studies, will provide clinically relevant information about a patient's future disease burden and will help to select more personalized treatment regimens.

Comparison of Glycerol Test, ECochG and VEMP Findings in Patients with Meniere's Disease.

Present study aimed to find if multiple audio-vestibular tests can be used to improve in diagnosing Meniere's disease. The study compares the results of four test procedures of glycerol test, Electrocochleography (ECochG), cervical Vestibular Evoked Myogenic Potential (c-VEMP) and ocular Vestibular Evoked Myogenic Potential (o-VEMP) individually and also together to see whether the diagnosis of Meniere's disease can be improved. Experimental group consist of 16 participants (32 ears) diagnosed with Meniere's disease as per American Academy of Otolaryngology (AAO) -Head and Neck Foundation (2020) guidelines. Control group consist of 16 participants with compliant of only vertigo participated in the study. Participants in both group underwent glycerol test, Electrocochleography (ECochG), cervical Vestibular Evoked Myogenic Potential (c-VEMP) and ocular Vestibular Evoked Myogenic Potential (o-VEMP). Glycerol test showed a 38% positive rate for the presence of Meniere's disease. ECochG indicated the presence of endolymphatic hydrops in 62% of the participants. Abnormal finding in c-VEMP and o-VEMP was seen in 31% and 56% of the participants respectively. The combination of two tests such as ECochG and o-VEMP has increased the positive rate upto 87%. Adding one more test of c-VEMP or combining all four audiovestibular tests had a positive rate of 94% only. The present study indicated ECochG is a more sensitive tool in the diagnosis of Meniere disease followed by o-VEMP. Thus, the present study indicated that combination ECochG and o-VEMP could be used to find the presence of Meniere's disease successfully.

Reflection for Meniere’s Disease in the Era of Modern Precision Medicine

Since our understanding of the mechanisms behind many diseases has improved over time, many diseases have been renamed based on their anatomical locations and pathophysiological findings. One such example is Meniere's disease (MD), a very well-known vertiginous condition named after the French physician Prosper Meniere, who had suggested in 1861 that the symptoms of the condition were caused by the inner ear instead of the brain. In contrast to its pathological findings, this symptom-based diagnosis for MD raises important questions: is MD a particular inner ear disorder or is it just an interpretation of an inner ear disorder accompanied by deafness and vertigo? There is no clear-cut criteria for MD inclusion, and diagnostic practices tend to restrict MD as a specific inner ear disease by excluding known inner ear and/or brain etiologies. In contrast to Dr. Meniere's original concept, which stressed that vertigo could be caused by inner ear problems, this exclusion criteria was not justified. The author believes that MD may have been a generic term for an inner ear disease that caused deafness and vertigo. Aside from simply issuing a diagnosis of MD, the author suggests that physicians should inform patients with deafness and vertigo about the diseased site and the associated pathological changes instead.

Meniere’s Disease: New Frontiers in Management for Better Results

Abstract Introduction Meniere's disease (MD) remains a difficult disease to diagnose, especially in the early stages when not all of the symptoms may be present. Sensorineural hearing loss, tinnitus, and recurrent vertigo constitute the hallmark symptoms of MD. Endolymphatic hydrops (EH) has been described as the responsible pathology in MD. Since that description, the medical and surgical treatment of MD have been directed at reducing the volume of endolymph. Unfortunately, these approaches have had equivocal success in the control of vertigo and recovery of hearing. So, a routine treatment directed at resolution of EH may not be suitable for all patients. Treatment has to be directed at the cause of EH whenever possible. Objectives The aim of this study was to define new findings in clinical tests and modes of treatment in MD, to determine the outcome of vertigo and hearing in patients after treatment, and to describe treatment which will prevent long term deterioration of hearing. Materials and Methods Forty-six new patients with a diagnosis of MD were treated with antiviral drugs or diuretics. Drugs were used based on nature of dehydration test. Hearing test including pure tone average (PTA) and speech discrimination (SD) was performed prior to treatment and at 1 to 2 months, 6 months, and 1 year after initiation of treatment. Effect on dizziness was recorded at each evaluation; hearing was judged to be improved, if PTA was lowered by at least 10 to 15 dB or an increase in SD &gt; 20%. Results The antiviral approach has virtually eliminated the use of various surgical methods used in the past. Dehydration test-based treatment protocol with diuretics and antivirals and antimigraine prophylaxis when needed has led to remission of disease in 93.5% of patients. With prompt treatment, inner ear damage can be prevented. Conclusion Orally administered antiviral drugs should be considered in the treatment of MD. Migraine-associated MD patients need migraine prophylaxis and this will lead to improvement in Meniere’s symptoms also. If Intratympanic therapy is considered, then targeted low-dose delivery method of using Gelfoam instillation of gentamicin is preferable according to our study to prevent any significant hearing loss.

Dietary Restriction for The Treatment of Meniere's Disease.

Meniere's disease (MD) is an idiopathic inner ear disorder characterized by spontaneous recurrent vertigo, fluctuating sensorineural hearing loss (SNHL), aural fullness and tinnitus. Endolymphatic hydrops (EH) of the inner ear is currently considered the pathophysiological mechanisms that underlies typical symptoms of MD. MD diagnosis is based on the criteria of the Baràny Society. There are many therapeutic options for MD, but none is considered effective by the scientific community. The first-line treatment commonly includes dietary modification, as low salt diet and reduction of alcohol and caffeine daily intake. Although some studies showed a positive effect of these dietary restrictions, even in the prevention of recurrences, currently there is no uniform consensus on their usefulness. New dietary approach, such SPC-flakes, are being evaluated: further assessments will be needed to validate their use in clinical practice.

Endolymphatic volume in patients with meniere's disease and healthy controls: Three-dimensional analysis with magnetic resonance imaging.

ObjectiveRecently, 3‐Tesla magnetic resonance imaging (MRI) with intravenous gadolinium injection has been used to reveal endolymphatic hydrops (EH). In the present study, we aimed to evaluate EH in patients with Meniere's disease (MD) objectively and quantitatively, and compared the endolymphatic space (ELS) in individuals with MD and healthy controls, to gain understanding of the characteristics of MD.MethodsEighty‐two patients with unilateral MD (uMD), 16 patients with bilateral MD (bMD), and 47 healthy volunteers were enrolled. All participants underwent 3‐T MRI at 4 hours after intravenous gadolinium injection. The volumes of the total fluid space (TFS) and ELS were measured semiautomatically using our workstation, and the percentage of ELS to TFS (ELS percentage) was calculated.ResultsThe ELS percentage was 13.9 in the ears of controls, 18.2 in the contralateral ear of individuals with uMD, 26.1 in the affected ears of these individuals, and 23.0 in both ears of individuals with bMD. The ELS percentages in the affected ear of uMD and the ears of bMD individuals were significantly higher than that in the ears of control individuals (P < .01, one‐way analysis of variance (ANOVA), Tukey's test).ConclusionThe ELS is significantly larger in the affected ears of uMD and in both ears of bMD individuals. Accurate diagnosis of MD can be facilitated by using 3‐T MRI 4 hours after intravenous gadolinium injection and performing volumetric measurements of the ELS.Level of Evidence2b

Evaluation of vestibular evoked myogenic potential, caloric test and cochlear electrogram in the diagnosis of Meniere's disease

Objective:The aim of this study is to evaluate the diagnostic value of vestibular-evoked myogenic potential （cVEMP and oVEMP）, caloric test, and cochlear electrogram （EcochG） in patients with Meniere's disease （MD） and non-Meniere's disease. Method:Sixty-four patients （64 ears） with Unilateral Meniere's disease were enrolled in the study group （MD group）, and 127 cases（254 ears） of non-Meniere's disease patients as non-MD group, including vertigo migraine in 40 cases, benign paroxysmal positional vertigo in 48 cases, benign recurrent vertigo in 13 cases, vestibular paroxysmia in 3 cases, vestibular neuritis in 5 cases and other undiagnosed vertigo in 18 cases. Both group undertake cVEMP, oVEMP, caloric test and ECochG. Use Medcale software to draw ROC curve of ECochG and calculate the area under curve（AUC）, Jordan index and optimal diagnostic cut-off points. Make the cut-off point as the point of -SP/AP, then evaluate the sensitivity, specificity, positive predictive value（PPV）, negative predictive value（NPV） and diagnostic accuracy of cVEMP, oVEMP, caloric test and ECochG in MD group and non-MD group. Result:The AUC of ECochG ROC curve was 0.74, the Jordan index was 0.47 and the cut-off point was 0.4. The sensitivity and specificity of cVEMP（62% and 68%）, oVEMP（61% and 53%） and caloric test（53% and 57%） were all below ECochG（65% and 78%）. The positive predictive value and of ECochG was the highest（61.9%）, the negative predictive value of cVEMP was highest（87.5%）. The diagnostic accuracy of ECochG was highest（74%）, followed with cVEMP（67%）, oVEMP（55%） and caloric test（56%）. Conclusion:Compared with the vestibular function tests, the sensitivity, specificity, diagnostic accuracy and NPV were all higher in ECochG, and the diagnostic benefit can be maximized when -SP/AP value>0.4. So the value of single vestibular function examination in the diagnosis of Meniere's disease is limited. The diagnosis of MD still requires a comprehensive evaluation in combination with medical history, audiological tests and vestibular function examinations.

Systematic review of magnetic resonance imaging for diagnosis of Meniere disease.

The diagnostic criteria for Meniere Disease (MD) are clinical and include two categories: definite MD and probable MD, based on clinical examination and without the necessity of advanced vestibular or audiological testing. The condition is a heterogeneous disorder and it is associated with endolymphatic hydrops (EH), an accumulation of endolymph in the inner ear that causes damage to the ganglion cells. Patients with suspected EH can be examined by Magnetic Resonance Imaging (MRI), offering new insights into these inner ear disorders. Results of imaging studies using the hydrops protocols show conflicting results in MD patients. These discrepancies can be dependent either on the MRI sequence parameters or on the method of hydrops grading or the inclusion criteria to select patients. The visualization of EH can be classified based on a semi-quantitative ratio between endolymph and perilymph liquids, or on the distinction between the saccule and the utricle structures. In addition, MRI can also be used to evaluate whether cochleovestibular nerves can present with imaging signs of axonal loss.In this systematic review, we have selected case-controlled studies to better characterize the potential added value in the diagnosis and management of patients with MD. Using different techniques, studies have identified the saccule as the most specifically involved structure in MD, and saccular hydrops seems to be associated with low to medium-tone sensorineural hearing loss degree. However, early symptoms still appear too subtle for identification using MRI and the reproducibility of the hydrops protocols with various MRI scan manufacturers is debatable, thus limiting expansion of these techniques into clinical practice for the diagnosis of MD at this time.Further research is needed. The future inclusion of semicircular canal hydrops location in the imaging signs and the application of MRI in patients with atypical presentations hold promise.

Excess of Rare Missense Variants in Hearing Loss Genes in Sporadic Meniere Disease.

Meniere's disease (MD) is a clinical spectrum of rare disorders characterized by vertigo attacks, associated with sensorineural hearing loss (SNHL) and tinnitus involving low to medium frequencies. Although it shows familial aggregation with incomplete phenotypic forms and variable expressivity, most cases are considered sporadic. The aim of this study was to investigate the burden for rare variation in SNHL genes in patients with sporadic MD. We conducted a targeted-sequencing study including SNHL and familial MD genes in 890 MD patients to compare the frequency of rare variants in cases using three independent public datasets as controls. Patients with sporadic MD showed a significant enrichment of missense variants in SNHL genes that was not found in the controls. The list of genes includes GJB2, USH1G, SLC26A4, ESRRB, and CLDN14. A rare synonymous variant with unknown significance was found in the MARVELD2 gene in several unrelated patients with MD. There is a burden of rare variation in certain SNHL genes in sporadic MD. Furthermore, the interaction of common and rare variants in SNHL genes may have an additive effect on MD phenotype. This study will contribute to design a gene panel for the genetic diagnosis of MD.