Neuroimaging Insights: Structural Changes and Classification in Ménière's Disease.

OBJECTIVES: This study aimed to comprehensively investigate the neuroanatomical alterations associated with idiopathic Ménière's disease (MD) using voxel-based morphometry and surface-based morphometry techniques. The primary objective was to explore nuanced changes in gray matter volume, cortical thickness, fractal dimension, gyrification index, and sulcal depth in MD patients compared with healthy controls (HC). Additionally, we sought to develop a machine learning classification model utilizing these neuroimaging features to effectively discriminate between MD patients and HC. DESIGN: A total of 55 patients diagnosed with unilateral MD and 70 HC were enrolled in this study. Voxel-based morphometry and surface-based morphometry were employed to analyze neuroimaging data and identify structural differences between the two groups. The selected neuroimaging features were used to build a machine learning classification model for distinguishing MD patients from HC. RESULTS: Our analysis revealed significant reductions in gray matter volume in MD patients, particularly in frontal and cingulate gyri. Distinctive patterns of alterations in cortical thickness were observed in brain regions associated with emotional processing and sensory integration. Notably, the machine learning classification model achieved an impressive accuracy of 84% in distinguishing MD patients from HC. The model's precision and recall for MD and HC demonstrated robust performance, resulting in balanced F1-scores. Receiver operating characteristic curve analysis further confirmed the discriminative power of the model, supported by an area under the curve value of 0.92. CONCLUSIONS: This comprehensive investigation sheds light on the intricate neuroanatomical alterations in MD. The observed gray matter volume reductions and distinct cortical thickness patterns emphasize the disease's impact on neural structure. The high accuracy of our machine learning classification model underscores its diagnostic potential, providing a promising avenue for identifying MD patients. These findings contribute to our understanding of MD's neural underpinnings and offer insights for further research exploring the functional implications of structural changes.

The value of four stage vestibular hydrops grading and asymmetric perilymphatic enhancement in the diagnosis of Menière's disease on MRI.

PURPOSE: There is still a clinical-radiologic discrepancy in patients with Menière's disease (MD). Therefore, the purpose of this study was to investigate the reliability of current MRI endolymphatic hydrops (EH) criteria according to Baráth in a larger study population and the clinical utility of new imaging signs such as a supplementary fourth low-grade vestibular EH and the degree of perilymphatic enhancement (PE) in patients with Menière's disease (MD). METHODS: This retrospective study included 148 patients with probable or definite MD according to the 2015 American Academy of Otolaryngology, Head and Neck Surgery criteria who underwent a 4-h delayed intravenous Gd-enhanced 3D-FLAIR MRI between January 2015 and December 2016. Vestibular EH, vestibular PE, cochlear EH, and cochlear PE were reviewed twice by three experienced readers. Cohen's Kappa and multivariate logistic regression were used for analysis. RESULTS: The intra- and inter-reader reliability for the grading of vestibular-cochlear EH and PE was excellent (0.7 < kappa < 0.9). The two most distinctive characteristics to identify MD are cochlear PE and vestibular EH which combined gave a sensitivity and specificity of 79.5 and 93.6%. By addition of a lower grade vestibular EH, the sensitivity improved to 84.6% without losing specificity (92.3%). Cochlear EH nor vestibular PE showed added-value. CONCLUSIONS: MRI using vestibular-cochlear EH and PE grading system is a reliable technique. A four-stage vestibular EH grading system in combination with cochlear PE assessment gives the best diagnostic accuracy to detect MD.

On the classification of hydropic ear disease (Menière's disease).

More than 150 years after its initial description by Prosper Menière, the disease named after him is still at the center of scientific debates. Two recent developments have specifically created a breeding ground for controversy: (1) Since its first description 10 years ago, magnetic resonance imaging diagnosis of endolymphatic hydrops in living patients has seen an increasing and worldwide application. (2) The Bárány Society Classification Committee published diagnostic criteria for Menière's disease in 2015 and proposed a concept of the disease that has elicited widespread criticism. In order to promote the understanding of the underlying controversies and arguments, this article gives an overview of and discusses relevant classification proposals for Menière's disease, including the new classification system of hydropic ear disease.

Extended phenotype and clinical subgroups in unilateral Meniere disease: A cross-sectional study with cluster analysis.

OBJECTIVES: To define clinical subgroups by cluster analysis in patients with unilateral Meniere disease (MD) and to compare them with the clinical subgroups found in bilateral MD. DESIGN: A cross-sectional study with a two-step cluster analysis. SETTINGS: A tertiary referral multicenter study. PARTICIPANTS: Nine hundred and eighty-eight adult patients with unilateral MD. MAIN OUTCOME MEASURES: best predictors to define clinical subgroups with potential different aetiologies. RESULTS: We established five clusters in unilateral MD. Group 1 is the most frequently found, includes 53% of patients, and it is defined as the sporadic, classic MD without migraine and without autoimmune disorder (AD). Group 2 is found in 8% of patients, and it is defined by hearing loss, which antedates the vertigo episodes by months or years (delayed MD), without migraine or AD in most of cases. Group 3 involves 13% of patients, and it is considered familial MD, while group 4, which includes 15% of patients, is linked to the presence of migraine in all cases. Group 5 is found in 11% of patients and is defined by a comorbid AD. We found significant differences in the distribution of AD in clusters 3, 4 and 5 between patients with uni- and bilateral MD. CONCLUSIONS: Cluster analysis defines clinical subgroups in MD, and it extends the phenotype beyond audiovestibular symptoms. This classification will help to improve the phenotyping in MD and facilitate the selection of patients for randomised clinical trials.

Die Behandlung des M. Menière mit Betahistin: Kritische Anmerkungen zur BEMED-Studie.

The BEMED study (BMJ 2016; 352: DOI 10.1136) was designed as multi-centric, double-blind, plaebo-controlled study in patients with Menière's disease. It should compare a low-level (2 × 24 mg/d) vs. high-level (3 × 48 mg/d) betahstine intake vs. placebo. The primary endpoint was the "number of vertigo attacks lasting longer than 20 min as documented in a patient's diary". The main finding of the study was that betahistine did not significantly better reduced the number of vertigo attacks than placebo. Therefore, the BEMED study should be critically discussed in the present paper.

Cluster Analysis to Identify Clinical Subtypes of Ménière's Disease.

OBJECTIVE: To identify distinct clinical subtypes of Ménière's disease by analyzing data acquired from a UK registry of patients who have been diagnosed with Ménière's disease. STUDY DESIGN: Observational study. METHODS: Patients with Ménière's disease were identified at secondary/tertiary care clinics. Cluster analysis was performed by grouping participants sharing similar characteristics and risk factors into groups based on a defined measure of similarity. RESULTS: A total of 411 participants were recruited into this study. Two main clusters were identified: participants diagnosed with ear infections (OR = 0.30, p < 0.014, 95% CI: 0.11-0.78) were more likely to be allocated in Cluster 1 (C1). Participants reporting tinnitus in both ears (OR = 11.89, p < 0.001, 95% CI: 4.08-34.64), low pitched tinnitus (OR = 21.09, p < 0.001, 95% CI: 7.47-59.54), and those reporting stress as a trigger for vertigo attacks (OR = 14.94, p < 0.001, 95% CI: 4.54-49.10) were significantly more likely to be in Cluster 2 (C2). Also, participants diagnosed with Benign Paroxysmal Positional Vertigo (OR = 13.14, <0.001, 95% CI: 4.35-39.74), autoimmune disease (OR = 5.97, p < 0.007, 95% CI: 1.62-22.03), depression (OR = 4.72, p < 0.056, 95% CI: 0.96-23.24), migraines (OR = 3.13, p < 0.008, 95% CI: 1.34-7.26), drug allergy (OR = 3.25, p < 0.029, 95% CI: 1.13-9.34), and hay fever (OR = 3.12, p < 0.009, 95% CI: 1.33-7.34) were significantly more likely to be clustered in C2. CONCLUSIONS: This study supports the hypothesis that Ménière's disease is a heterogeneous condition with subgroups that may be identifiable by clinical features. Two main clusters were identified with differing putative etiological factors. LEVEL OF EVIDENCE: 3 Laryngoscope, 134:3286-3292, 2024.

Diagnostic and therapeutic strategies for Meniere's disease of the Japan Society for Equilibrium Research.

OBJECTIVE: We provided diagnostic and therapeutic strategies for Meniere's disease in accordance with Japanese Clinical Practice Guideline of Meniere's disease and delayed endolymphatic hydrops 2nd ed. Tokyo: Kanehara Shuppan; 2020 edited by the Japan Society for Equilibrium Research. METHODS: The Committee for Clinical Practice Guidelines was entrusted with a review of the scientific literature on the above topic. Clinical Questions (CQs) concerning the treatment for Meniere's disease were produced, and the literature according to each of them including CQ was searched. The recommendations are based on the literature review and the expert opinion of a subcommittee. RESULTS: Diagnosis criteria of Meniere's disease are classified into Meniere's disease with typical cochlear and vestibular symptoms, and atypical Meniere's disease with either cochlear symptoms or vestibular symptoms. Treatment of Meniere's disease was composed of lifestyle changes, medications such as anti-vertigo drugs and diuretics, middle ear positive pressure treatment, and selective destruction of the vestibule. CONCLUSION: Meniere's disease is diagnosed based on clinical histories and examination findings after processes of differential diagnosis. Treatment option of the disease should be selected in order of invasiveness, according to the severity of the disease and the response to each treatment.

Use of ICF in assessing the effects of Meniere's disorder on life.

OBJECTIVES: We sought to determine the value of the World Health Organization's International Classification of Functioning, Disability and Health (ICF) in subjects with Meniere's disorder in relation to their quality of life. METHODS: We asked 228 members of the Finnish Meniere Federation to report the effects that Meniere's disorder had on their lives. The replies were classified on the basis of the ICF classification and related to the EuroQol 5D score and disease-specific impact. Logistic regression and decision tree analyses were used to determine the relationships. RESULTS: Seventy percent of the patients listed impairments, 39% activity limitations, 47% participation restrictions, 16% effects on environmental contextual factors, and 28% effects on personal contextual factors. The EuroQol 5D score was explained by reported vertigo, anxiety, fatigue, restriction of life, and communication problems. The disease-specific impact was explained by episodes of vertigo, fatigue, communication problems, inability to work, restriction of life, and uncertainty of life. Both analysis models provided the same outcome variables, although the decision tree separated the results better (80%) into correct classes than did logistic regression analysis (60%). CONCLUSIONS: Self-reported participation restriction, activity limitation, and personal contextual factors describe the limitations of general life in subjects with Meniere's disorder. The use of the ICF classification provides an instrument that can be used in enablement of subjects with Meniere's disorder.

[The 'dizziness handicap inventory': initial psychometric evaluation of the German version]. / Das "Dizziness Handicap Inventory": Erste psychometrische Kennwerte einer Deutschen Version.

BACKGROUND: Dizziness has a clear impact on quality of life of patients. Standardized instruments such as the "Dizziness Handicap Inventory" (DHI) help clinicians assess this impact systematically. The purpose of this study was to analyse the psychometric quality of a German version of the DHI. METHODS: One hundred and five patients with dizziness as their primary complaint have completed the DHI and undergone vestibular examination. The structure of the DHI was determined with a factor analysis, a principal component analysis with a Varimax rotation. To evaluate the reliability, internal consistency (Cronbach's alpha) was estimated. RESULTS: A three-factor solution was extracted. The factors obtained from the German version of the DHI related to (1) activity and participation limitations (2) emotional problems and (3) motion sensitivity in everyday life of patients. Overall, there was a close correspondence of the factors of the German and the original version. The correlation analysis indicated a close relationship between the DHI-scores and the frequency of dizziness attacks. CONCLUSION: The German version of the DHI exhibited a multidimensional structure and good psychometric quality to assess the impact of dizziness in every day life.

Magnetic resonance imaging of Ménière's disease: early clinical experience in a UK centre.

BACKGROUND: Recent developments in magnetic resonance imaging have enabled demonstration of endolymphatic hydrops, and the clinical application of these imaging studies in Ménière's disease is being explored. OBJECTIVE: To evaluate our centre's experience to date of hydrops magnetic resonance imaging in patients with episodic vertigo. METHODS: Magnetic resonance imaging was performed using a high-resolution three-dimensional fluid-attenuated inversion recovery sequence on a 3 Tesla scanner at 4 hours following double-dose gadolinium administration. RESULTS: The study included 31 patients, 28 of whom had a clinical diagnosis of Ménière's disease. In unilateral Ménière's disease, magnetic resonance imaging was able to lateralise endolymphatic hydrops to the clinically symptomatic ear in all cases. Mild hydrops was often seen in clinically asymptomatic ears. CONCLUSION: There is a good correlation between the clinical symptoms and lateralisation of hydropic changes on magnetic resonance imaging. Further refinements of imaging techniques and grading system will likely improve the diagnostic accuracy and clinical utilisation of hydrops magnetic resonance imaging.

Clinical significance of the summating potential-action potential ratio and the action potential latency difference for condensation and rarefaction clicks in Meniere's disease.

OBJECTIVES: This study was aimed to elucidate the diagnostic significance of the summating potential (SP)-action potential (AP) ratio and the AP latency difference between condensation and rarefaction clicks (AP con-rar difference) in Meniere's disease. METHODS: The AP and SP were recorded transtympanically in 67 patients with definite Meniere's disease. The SP/AP ratio and the AP con-rar difference were assessed in terms of 1) their interrelationship, 2) their relationship to hearing level, and 3) the rate of occurrence of abnormal values according to the stages of Meniere's disease. RESULTS: No correlation was found between the SP/AP ratio and the AP con-rar difference. Neither the SP/AP ratio in general nor the AP con-rar difference was correlated with the hearing level. However, enhanced values of the SP/AP ratio (0.35 or higher) were moderately correlated with the hearing level (r = 0.51), and their occurrence rate was 55.2%. An increased AP con-rar difference (0.13 ms or longer) was not correlated with the hearing level, and its occurrence rate was 50.2%; it appeared most frequently at stage 3 (p <0.05). CONCLUSIONS: An enhanced SP/AP ratio might not always indicate the presence of endolymphatic hydrops associated with an increase in endolymphatic pressure. An increased AP con-rar difference might reflect the presence of a biased basilar membrane resulting from an increased endolymphatic pressure, and hence it is diagnostically essential to simultaneously evaluate the SP/AP ratio and the AP con-rar difference.

[Menière's disease : evidence and controversies]. / M. Menière : Evidenzen und Kontroversen.

Menière's disease is recognized as the idiopathic form of recurrent inner ear disease with the trias of hearing loss, tinnitus and vertigo with aural fullness and Menière's syndrome as the non-idiopathic form. Subentities with unknown pathogenesis are Lermoyez' s syndrome and Tumarkin crises. A common pathogenetic factor is the disturbance of endolymphatic and perilymphatic osmotic and hydrostatic pressure due to defined etiologies or to idiopathic attacks. Etiologies of Menière's syndrome can be pathologic middle ear pressure, anomalies of the vestibular and cochlear aqueducts, round window topography, patency of the ductus peruniens and the utriculo-endolymphatic valve. Indications for treatment are assessed according to the AAO-HNS guidelines and the neurotological function tests and dizziness inventories. Betahistine is recommended as first choice medical treatment as on-label or high dosage administration. In case of medical treatment failure and if hearing is worth saving, endolymphatic shunt surgery (ELS) is the first choice. If deafferentiation of the labyrinth is needed vestibular neurectomy (VE) should be performed. Local gentamicine administration has good long-term results but macula function can often recover and hearing is often deteriorated. Future aspects for the treatment are new experimental results with gene transfer to vestibular hair cells.

Could there be any merit in lumping primary open-angle glaucoma, idiopathic intracranial hypertension and Meniere's disease into a novel and discrete category of fluid tension disorders?

Open-angle glaucoma, idiopathic intracranial hypertension, and Meniere's disease are disorders managed by different specialties in medicine viz. ophthalmology, neurology, and otorhinolaryngology respectively. By working in silos, the similarity of these disorders is overlooked. Close inspection of these disorders reveals the presence of signs and symptoms triggered by fluid under high pressure within relatively closed chambers. There is a similarity in the capillary production of fluid, which then circulates and drains into the venous system. Management practices that reduce fluid production, decrease fluid pressure or enhance fluid drainage are employed for the treatment of all three disorders. A search for a unifying mechanism explaining the pathophysiology of all three disorders may unlock effective and perhaps curative measures for these disorders.

Contemporary role of endolymphatic mastoid shunt surgery in the era of transtympanic perfusion strategies.

OBJECTIVES: Although there exist undisputed methods to permanently silence the aberrant end organ, controversy surrounds the durable efficacy of non-ablative interventions. This study provides a contemporary review of our institution's clinical experience in performing endolymphatic mastoid shunt surgery (EMSS) in patients with medically refractory endolymphatic hydrops, or Meniere's disease. METHODS: Between 1984 and 2002, 1,612 patients were referred to our institution with a diagnosis of Meniere's disease. Of these referrals, 1,172 patients met the criteria for Meniere's disease. Although 553 patients responded to medical management, 486 patients underwent EMSS and 133 patients had refractory disease that required chemical or surgical obliterative interventions. The retrospective study utilizes data collected on 226 patients who were followed for a minimum of 5 years. RESULTS: Overall, 78% patients responded favorably to EMSS, according to the functional level scale and class categories delineated by the American Academy of Otolaryngology-Head and Neck Surgery 1995 guidelines for control of vertigo. According to the Arenberg anatomic classification for endolymphatic sac location, EMSS achieved adequate control of vertigo in 86% of type I cases, 90% of type II cases, and 82% of type III cases. CONCLUSIONS: Endolymphatic mastoid shunt surgery is a relatively safe, effective procedure for the long-term control of vertigo in patients with medically refractory Meniere's disease.

On machine learning classification of otoneurological data.

A dataset including cases of six otoneurological diseases was analysed using machine learning methods to investigate the classification problem of these diseases and to compare the effectiveness of different methods for this data. Linear discriminant analysis was the best method and next multilayer perceptron neural networks provided that the data was input into a network in the form of principal components. Nearest neighbour searching, k-means clustering and Kohonen neural networks achieved almost as good results as the former, but decision trees slightly worse. Thus, these methods fared well, but Naïve Bayes rule could not be used since some data matrices were singular. Otoneurological cases subject to the six diseases given can be reliably distinguished.

Video-head impulse test results in patients with Menière's disease related to duration and stage of disease.

BACKGROUND: The video-head impulse test employs the vestibulo-ocular reflex (VOR) to assess vestibular function. To this day, no consensus has been reached among scientists in terms of whether or not vHIT results change in MD patients as the disease progresses. OBJECTIVE: To assess whether the vHIT is more often abnormal in later stages of MD compared to earlier stages. METHODS: We retrospectively analyzed patients with 'definite' MD who had undergone a vHIT and caloric test between 2012 and 2015. Patients were evaluated based on duration of disease in years (≤1, >1≤5, >5≤10, >10) and stage of disease (stage I and II versus III and IV). For the vHIT, an abnormal vestibulo-ocular reflex was defined as a gain cut-off value of≤0.8 and presence of correction saccades including subanalyses using a cut-off value of≤0.9. RESULTS: In 89 definite MD patients (42 (47%) male, mean age 55±5 (SD)), data on both the caloric test and the vHIT were available. The risk of an abnormal vHIT was 25% in patients with a duration of disease over 10 years compared to 22% in the patients with a disease duration of 10 years or less (risk difference 3%, 95% CI:- 28% to 35%), p = 0.82). The risk for an abnormal vHIT in the Stage I and Stage II was 17% compared to 26% in Stage III and IV (risk difference 9%, 95% CI:- 30% to 11%). When using a cut-off value of 0.9 we also did not demonstrate a relationship between the duration of disease and the proportion of abnormal vHIT test results. CONCLUSIONS: There is no relationship between the proportion of abnormal vHIT test results in patients with MD in either duration or stage of disease.

Clinical Subtyping of Ménière's Disease.

Fundamentally, Ménière's disease is a constellation of symptoms and, as such, may represent the final common pathway for a number of disease processes, as opposed to being the consequence of a single isolated pathology. Within this type of consideration, much can be learned regarding the etiology, presentation, prognosis, and treatment of these individual conditions by applying subtyping techniques currently employed to better understand similar disease processes that are encountered in other allied fields of medicine. This commentary proposes the principles, required processes, and benefits of subtyping for Ménière's disease.

Survey of Ménière's disease in a subspecialty referral practice.

OBJECTIVES: To define the prevalence of definite Ménière's disease (MD) among patients presenting with characteristic symptoms and examine the utility of published diagnostic guidelines. STUDY DESIGN AND SETTING: Retrospective review in an academic referral practice. RESULTS: The prevalence of definite MD in these 295 individuals was 64%. The next-largest group (23%) consisted of patients with only cochlear symptoms. Those initially classified as probable are usually reclassified as definite with extended follow-up. Of those with definite MD, the mean duration of disease at last follow-up was 7.6 years, 56% were female, 19% had bilateral disease, and 34% required surgical management for vertigo. CONCLUSIONS: The 1995 AAO-HNS guidelines are useful for classification of MD according to certainty of diagnosis and severity of disease, though some modifications could be considered. SIGNIFICANCE: Application of consistent diagnostic criteria is essential for epidemiological, genetic, or outcomes studies of Ménière's disease.