Search Results (56)

Background/Objectives: The diagnosis of vestibular migraine (VM) and Meniere’s disease (MD) is based mainly on clinical criteria. The aim of this study is to systematically review and investigate the potential role of the video Head Impulse Test (vHIT) in the differential diagnosis between VM and MD. Methods: A systematic review of the English-language literature was conducted, including studies from database inception to November 2023, in accordance with PRISMA guidelines. Medline (via PubMed), Cochrane Database and Scopus were reviewed. The review included studies involving adult patients diagnosed with VM, MD, or healthy control individuals who underwent vHIT and reported data on vHIT abnormalities, gain, and refixation saccades. The AXIS tool was applied for risk of bias assessment in all cross-sectional studies. A random-effects meta-analysis was performed to compare vHIT gains between individuals with VM and those with MD. Results: Eleven cross-sectional observational studies with a case–control comparison design were included, comprising a total of 362 patients with VM, 307 patients with MD, and 135 healthy control subjects. All studies applied the same diagnostic criteria for VM; however, varying criteria were used for the diagnosis of MD. Four studies evaluated the duration of vestibular symptoms, two assessed migraine duration, and six provided a rationale for excluding individuals with overlapping VM and MD diagnoses. Criteria for defining an abnormal vHIT result were specified in six studies. Seven studies reported vHIT gain values for the lateral semicircular canal, while eight presented data on saccade incidence and characteristics. Additionally, four studies were included in the meta-analysis, which yielded a mean difference in the vHIT gain of −0.0203 (95% CI: −0.0789 to 0.0383; p = 0.4968), indicating no statistically significant difference between patients with VM and those with MD. Conclusions: In this review, vHIT gain did not differ significantly between VM and MD groups, suggesting that vHIT gain alone has limited utility in their differential diagnosis. Combined saccade patterns may still prove clinically useful as more robust and consistent data become available. Full article

Background: Serotonin (5-HT) is a neurotransmitter and a hormone that regulates various functions. Serotonin receptors have been studied in animal experiments in the vestibular system, beginning from the inner ear and vestibular nuclei. However, the role of serotonin in the vestibular system and disorders remains to be clarified. Methods: A review of the literature was performed on different databases according to the PRISMA guidelines. Only publications published on humans and in English have been included. A total of 41 articles were included in this review. Results: There are many publications regarding the use of SSRI/SNRI in vestibular disorders. Regarding persistent postural perceptual dizziness (PPPD) and chronic subjective dizziness (CSD) the available evidence supports multimodality treatment incorporating vestibular rehabilitation, serotonergic medications, and cognitive behavior therapy, although most studies have not included a placebo control group. As for vestibular migraine (VM), SNRI and SSRIs were proposed as preventive therapy and demonstrated a reduction in vertigo attacks in patients with Menière’s Disease (MD), especially when symptoms of anxiety disorder were present. Conclusions: Although SSRIs/SNRIs are considered an off-label therapy for vertigo, several studies have assessed their efficacy in vestibular disorders, as indicated in the data published on PPPD, MD, and VM above all. As some studies report that serotonin receptors are also present in the inner ear and vestibular nuclei, it can be postulated that in cases where the natural levels of serotonin are altered, such as in depression and anxiety, the change in serotonin levels may affect vestibular function and play a role in vestibular disorders. Full article

Background and Objectives: Sensorineural hearing loss (SNHL) is influenced by various causes, including thyroid diseases. For example, hypothyroidism and thyroid autoimmunity can damage the inner ear through hormonal, immune, and vascular mechanisms. Vestibular disorders like Ménière’s disease (MD) and benign paroxysmal positional vertigo (BPPV) also show possible associations with thyroid dysfunction. Materials and Methods: A review following PRISMA guidelines searched PubMed, Scopus, and Google Scholar for studies linking thyroid disorders with inner ear dysfunction. Results: Out of 985 screened records, 30 studies met inclusion criteria, involving various thyroid disorders, primarily hypothyroidism and autoimmune thyroiditis. Scientific evidence supports a correlation between hypothyroidism and hearing impairment. However, some studies also suggest a link between hyperthyroidism and inner ear disorders, particularly focusing on the role of autoimmunity in this context. Concerning vestibular dysfunction, the available studies are less abundant and support a significant association between thyroid disease and Meniere’s disease. Conclusions: There is a clear correlation between hypothyroidism and auditory function. A substantial body of literature also supports an association with vestibular disorders, although some discrepancies remain. Further research is needed to elucidate the underlying pathophysiological mechanisms (e.g., autoimmune, vascular, metabolic) involved with this correlation. Full article

Background/Objectives: Skull vibration-induced nystagmus (SVIN) is a rapid bedside test that reveals vestibular asymmetry. Its clinical utility in Ménière’s disease (MD) remains controversial, particularly regarding its association with radiological endolymphatic hydrops (EH). This study aimed to evaluate the relationship between SVIN, audiovestibular parameters, and EH severity in patients with unilateral definite MD. Methods: This prospective observational study was conducted at a tertiary academic referral center and included patients with unilateral MD who underwent SVIN testing (SVT), audiovestibular evaluation (PTA, cVEMP, oVEMP, vHIT, and caloric testing), and 3T MRI with gadolinium-enhanced 3D-FLAIR sequences to quantify EH. Results: In total, 84 patients were included in the study. SVIN was present in 57.14% of patients (n = 48), with ipsilesional nystagmus being the most frequent subtype (64.58%). Patients with SVIN had significantly higher vestibular EH (p = 0.017) and vestibular endolymphatic ratio (REL) in the affected ear (p = 0.019). Disease duration (p = 0.026) and shorter time since last vertigo spell (p = 0.018) were also associated with SVIN presence. REL correlated moderately with disease duration (r = 0.390, p < 0.001), PTA (r = 0.576, p < 0.001), and number of vertigo spells (r = 0.236, p = 0.031), but not with time since last crisis (r = −0.127, p = 0.252). ROC analysis yielded an AUC of 0.735 for REL in predicting SVIN. Conclusions: SVIN correlates with the severity of vestibular EH. This finding indicates a stimulus-locked response of a vestibular asymmetry rather than a purely structural alteration. Full article

Background: Meniere’s disease (MD) is a chronic inner ear disorder affecting approximately 0.2% of the population, with 30% of patients remaining refractory to conventional treatments. The pathophysiology involves endolymphatic hydrops, suggesting that agents affecting fluid homeostasis might provide therapeutic benefit. Sodium-glucose cotransporter 2 (SGLT-2) inhibitors, originally developed for diabetes, offer unique mechanisms including natriuresis and osmotic diuresis that may address the underlying fluid imbalance in MD. Methods: We conducted a retrospective observational study at the Korea University Anam Hospital, analyzing the medical records of patients with definite MD (Bárány Society criteria) who received off-label empagliflozin 10 mg daily between January 2023 and December 2023. Six patients (3 men, 3 women; mean age 55.8 years) with treatment-resistant MD were identified who had failed conventional therapy for at least 3 months. Primary outcomes included changes in pure tone threshold average (PTA), low-frequency threshold average (LFA), vertigo episode frequency, and vertigo severity using visual analog scale (VAS) scores, assessed at baseline and after 3 months of treatment. Results: All patients demonstrated clinically significant improvements in both auditory and vestibular symptoms. Mean PTA improved from 31.4 dB to 20.8 dB (improvement of 10.6 dB, p < 0.05). Low-frequency hearing showed more substantial recovery, with LFA improving from 37.2 dB to 15.6 dB (improvement of 21.6 dB, p < 0.01). Vertigo frequency decreased dramatically from 1.6 episodes per month to 0.1 episodes per month, with four patients experiencing a complete resolution of vertigo episodes. VAS scores for vertigo severity decreased from 5.2 to 0.5. Treatment was well-tolerated, with only minor adverse effects reported in two patients: transient polyuria in one patient and 5 kg weight loss in another, both consistent with the known pharmacological profile of SGLT-2 inhibitors. Conclusions: This preliminary study suggests a potential clinical benefit of repurposing SGLT-2 inhibitors for treatment-resistant MD. However, the retrospective design and inherent limitations prevent definitive conclusions about causality. The significant improvements observed in both hearing thresholds and vestibular symptoms warrant further investigation through randomized controlled trials with objective outcome measures to establish the true efficacy of this therapeutic approach. Full article

Background: Meniere’s disease (MD) is a rare inner ear disorder characterized by endolymphatic hydrops and symptoms such as vertigo and hearing loss, with no curative treatment currently available. XuanYunNing tablets (XYN) have been clinically used to treat MD, but their molecular mechanisms remain unclear. Objective: This study aimed to systematically evaluate the pharmacological effects of XYN in a guinea pig model of MD and to elucidate the underlying molecular mechanisms of both MD pathogenesis and XYN intervention through integrated multi-omics analyses, including transcriptomics, proteomics, and bioinformatics. Methods: A guinea pig model of endolymphatic hydrops was induced by intraperitoneal injection of desmopressin acetate (dDAVP). Pharmacodynamic efficacy was evaluated via behavioral scoring and histopathological analysis. The differentially expressed genes (DEGs) and differentially expressed proteins (DEPs) modulated by XYN treatment were identified using high-throughput transcriptomic and proteomic sequencing. These data were integrated through multi-omics bioinformatic analysis. Key molecular targets and signaling pathways were further validated using RT-qPCR and Western blotting. Results: Pharmacological evaluations showed that guinea pigs in the model group exhibited a 26% increase in endolymphatic hydrops area, while high-dose XYN treatment reduced this area by 19% and significantly improved functional parameters, including overall physiological condition (e.g., weight and general appearance), auricular reflexes to low-, medium-, and high-frequency sound stimuli, nystagmus, and the righting reflex. High-throughput sequencing combined with integrative omics analysis identified 513 potential molecular targets of XYN. Subsequent network and module analyses pinpointed the JAK-STAT signaling pathway as the central axis. Mendelian randomization (MR) analysis further supported a causal relationship between MD and metabolic, immune, and inflammatory traits, reinforcing the central role of JAK-STAT signaling in both MD progression and XYN-mediated intervention. Mechanistic studies confirmed that XYN downregulated IFNG, IFNGR1, JAK1, p-STAT3/STAT3, and AOX at both mRNA and protein levels, thereby inhibiting aberrant JAK-STAT pathway activation in MD model animals. In addition, a total of 125 chemical constituents were identified in XYN by UHPLC-MS analysis. ZBTB20 and other molecules were identified as potential blood-based biomarkers for MD. Conclusions: This study reveals that XYN alleviates MD symptoms by disrupting a pathological cycle driven by JAK-STAT signaling, inflammation, and metabolic dysfunction. These findings support the clinical potential of XYN in the treatment of Meniere’s disease and may inform the development of novel therapeutic strategies. Full article

Background: Generative AI (GenAI) models like ChatGPT have gained significant attention in recent years for their potential applications in healthcare. This study evaluates the concordance of responses generated by ChatGPT (versions 3.5 and 4.0) with the key action statements from the American Academy of Otolaryngology–Head and Neck Surgery (AAO-HNS) clinical practice guidelines (CPGs) for Ménière’s disease translated into Chinese. Methods: Seventeen questions derived from the KAS were translated into Chinese and posed to ChatGPT versions 3.5 and 4.0. Responses were categorized as correct, partially correct, incorrect, or non-answers. Concordance with the guidelines was evaluated, and Fisher’s exact test assessed statistical differences, with significance set at p < 0.05. Comparative analysis between ChatGPT 3.5 and 4.0 was performed. Results: ChatGPT 3.5 demonstrated an 82.4% correctness rate (14 correct, 2 partially correct, 1 non-answer), while ChatGPT 4.0 achieved 94.1% (16 correct, 1 partially correct). Overall, 97.1% of responses were correct or partially correct. ChatGPT 4.0 offered enhanced citation accuracy and text clarity but occasionally included redundant details. No significant difference in correctness rates was observed between the models (p = 0.6012). Conclusions: Both ChatGPT models showed high concordance with the AAO-HNS CPG for MD, with ChatGPT 4.0 exhibiting superior text clarity and citation accuracy. These findings highlight ChatGPT’s potential as a reliable assistant for better healthcare communication and clinical operations. Future research should validate these results across broader medical topics and languages to ensure robust integration of GenAI in healthcare. Full article

Background: Meniere’s disease is characterized by a triad of vertigo episodes, fluctuating hearing loss, and tinnitus. The disease is followed by a loss of quality of life in patients, with the severity depending on the individual and the stage of the disease. Since there are no quantitatively validated tests that connect all elements of the disease, the only source of subjective data that can be analyzed is the disease diary and questionnaires, among which the MDPOSI (Meniere’s Disease Patient-Oriented Symptom-Severity Index) stands out as a designated quality-of-life assessment tool. This study aims to evaluate the differences in the questionnaire depending on the clinical characteristics of the disease. Methods: The study recruited 60 patients, with clinical variables including age, gender, disease laterality, caloric testing results, and PTA results, the presence of spontaneous nystagmus, pathological values of calorimetric testing, or rotatory chair testing abnormalities. Results: The appearance of spontaneous nystagmus showed a significant association with worse hearing threshold values at 500 Hz (p = 0.036, OR 4.416) and higher. Worse SRT scores correlated with Q1 (p = 0.011), Q2 (p = 0.028), Q4 (p = 0.045), Q5 (p = 0.013), and the total MDPOSI score (p = 0.008, 0.339). Multivariate analysis showed that a higher total value of the MDPOSI questionnaire was statistically significantly associated with older age (p = 0.042) and spontaneous nystagmus (p = 0.037). Conclusions: There is a correlation between the clinical characteristics of Meniere’s disease and the MDPOSI questionnaire, making it useful for assessing quality of life and disease progression. Full article

Objectives: Lermoyez syndrome (LS) is a rare variant of endolymphatic hydrops with a unique clinical presentation characterized by reversible sensorineural hearing loss preceding vertigo. This review aims to synthesize available literature on LS to clarify its clinical characteristics, diagnostic approach, management strategies, and outcomes, and to highlight the distinguishing features from Menière’s disease (MD). Methods: A systematic literature review according to PRISMA guidelines was conducted from 1919 to 2025. The extracted data included demographics, symptom profiles, audiovestibular testing, imaging findings, treatment approaches, and patient outcomes. Results: A total of 23 studies were identified, reporting 53 individual cases of LS. Patients ranged from 27 to 85 years of age, with a mean age of 50.34 years and a male predominance (64.1%). The hallmark of LS across cases was a reproducible clinical pattern of unilateral low-frequency hearing loss followed by vertigo and subsequent auditory recovery. Audiometry typically confirmed reversible sensorineural hearing loss, while vestibular tests and imaging were often unremarkable, primarily used to exclude alternative diagnoses. Treatment approaches varied and were often based on MD protocols, including dietary modifications, vasodilators, diuretics, and vestibular suppressants. Prognosis was generally favorable, with most patients experiencing both hearing recovery and symptom resolution. Conclusions: LS remains a clinically distinct but underrecognized inner ear disorder. Its defining feature—the paradoxical improvement in hearing after vertigo—distinguishes it from Menière’s disease and should prompt clinicians to consider LS in differential diagnosis. Due to the rarity of LS and the lack of standardized guidelines, diagnosis and treatment rely on careful clinical assessment and individualized management strategies. Full article

Background/Objectives: Menière’s disease (MD) is a debilitating disorder with episodic and variable ear symptoms. Diagnosis can be challenging, and evidence for therapeutic approaches is low. Furthermore, patients show a unique and fluctuating configuration of audiovestibular impairment. As a psychometric instrument to assess hearing-specific disability is currently lacking, we evaluated a short form of the Speech, Spatial, and Qualities of Hearing Scale (SSQ) in a cohort of patients with MD. Methods: Data was collected in the context of a multicenter prospective patient registry intended for the long-term follow up of MD patients. Hearing was assessed by pure tone and speech audiometry. The SSQ was applied in the German language version with 17 items. Results: In total, 97 consecutive patients with unilateral MD with a mean age of 56.2 ± 5.0 years were included. A total of 55 individuals (57.3%) were female, and 72 (75.0%) were categorized as having definite MD. The average total score of the SSQ was 6.0 ± 2.1. Cronbach’s alpha for internal consistency was 0.960 for the total score. We did not observe undue floor or ceiling effects. SSQ values showed a statistically negative correlation with hearing thresholds and a statistically positive correlation with speech recognition scores of affected ears. Conclusions: The short form of the SSQ provides insight into hearing-specific disability in patients with MD. Therefore, it may be informative regarding disease stage and rehabilitation needs. Full article

Background/Objectives: Tonal thresholds, typically assessed through pure-tone audiometry (PTA), are central to the audiological evaluation of Ménière’s disease (MD). However, they fail to capture the complexity of real-life speech perception. This study aimed to characterize the relationship between PTA and speech recognition performance in unilateral MD and to determine whether a measurable dissociation exists between hearing sensitivity and verbal processing. We also evaluated frequency-specific audiometric patterns and potential threshold cut-off values associated with speech recognition decline. Methods: A total of 254 ears from 127 patients were included in the study across three groups: the Ménière group (affected and unaffected ears, n = 64 each) and the control group (n = 63). The pure-tone thresholds, speech recognition threshold (SRT), and the maximum word recognition scores (Rmax) were assessed in all participants. Results: Although the Ménière affected and control groups showed comparable pure-tone average (PTA) values (46.33 vs. 38.13 dB, p = 0.439), the affected group demonstrated significantly poorer speech performance (Rmax: 76.25% vs. 87.49%, p < 0.001; SRT: 50.64 vs. 38.45 dB, p = 0.009). The Ménière unaffected group exhibited near-ceiling performance (Rmax: 99.38%, SRT: 18.33 dB) and a mean PTA of 19.59 dB. A strong correlation between PTA and SRT was observed only in the Ménière affected group (r = 0.942, p < 0.001), whereas correlations were moderate in the unaffected (r = 0.671, p < 0.001) and control (r = 0.728, p < 0.001) groups. The ROC analysis revealed that PTA predicted impaired speech recognition with high accuracy in unaffected (AUC = 0.956, p < 0.001) and control (AUC = 0.829, p < 0.001) ears but far less so in affected ears (AUC = 0.784; all p < 0.001), confirming a functional tonal–verbal dissociation in MD. Conclusions: This study demonstrates a functional audiometric dissociation in unilateral Ménière’s disease. Affected ears show poorer speech recognition and require higher intensities despite similar PTA values. The predictive link between thresholds and verbal performance is disrupted. These findings support the need for combined tonal and speech-based assessment in clinical practice. Full article

Background/Objectives: To compare the outcomes of low-dose intratympanic gentamicin injection (ITGM) in managing intractable Meniere’s disease (MD) between patients receiving a single injection versus multiple injections, and to explore the optimal number of ITGM repetitions. Methods: This retrospective study was conducted at a single tertiary medical center. Clinical charts of patients diagnosed with definite MD between 2015 and 2020 and given low-dose ITGM for intractable vertigo attacks were reviewed. A total of 33 patients were divided into two groups based on the number of ITGM procedures: the single injection group (SG, n = 14) and the multiple injection group (MG, n = 19). In the MG, additional ITGM was performed up to four times. Audiograms, caloric responses, and video head impulse tests (vHIT) were reviewed at each repetition of ITGM. Results: After the first ITGM, both the SG and MG showed significant decreases in caloric responses and vHIT gains, without deterioration in hearing. In the MG group, a second ITGM was needed on average 8.1 ± 6.4 months after the initial ITGM due to persistent vertigo attacks. After the second ITGM, 8 out of 19 MG patients showed additional benefits in terms of reduced vertigo and further decreases in caloric responses. However, after the third and fourth ITGM, no further significant decline in vestibular function was observed, and there was no improvement in subjective dizziness. In the MG, gradual deterioration of hearing was observed. Conclusions: This finding suggests that performing additional low-dose ITGM in poorly responding or recurrent cases appears reasonable up to the second injection. For those who continue to experience vertigo episodes after two ITGM procedures, alternative therapeutic approaches should be considered to preserve hearing. Full article

Ménière’s Disease (MD) is a chronic inner ear disorder defined by recurring episodes of vertigo, fluctuating sensorineural hearing loss, tinnitus, and/or fullness in the ear. Its prevalence varies by region and ethnicity, with scarce epidemiological data in the Brazilian population. Although most MD cases are sporadic, familial MD (FMD) is observed in 5% to 20% of European cases. Through exome sequencing, we have found a rare missense variant in the OTOG gene in a Brazilian individual with MD with probable European ancestry (chr11:17599671C>T), which was previously reported in a Spanish cohort. Two additional rare missense heterozygous OTOG variants were found in the same proband. Splice Site analysis showed that chr11:17599671C>T may lead to substantial changes generating exonic cis regulatory elements, and protein modelling revealed structural changes in the presence of chr11:17599671C>T, chr11:17576581G>C, and chr11:17594108C>T, predicted to highly destabilize the protein structure. The manuscript aims to replicate genes previously reported in a Spanish cohort, and the main finding is that a Brazilian patient with MD also has variants previously reported in familial MD, supporting OTOG as the most frequently mutated gene in MD. Full article

Vestibular atelectasis (VA) is a rare clinical entity characterized by a collapse of the endolymphatic space resulting in vestibular loss with the possible onset of positional and/or sound/pressure-induced vertigo. It could be idiopathic or secondary to other inner-ear diseases including Meniere’s disease (MD). A collapse of the membranous labyrinth involving the semicircular canals (SCs) and the utricle represents its distinctive histopathological feature. While specific radiological patterns consistent with VA have been described on contrast-enhanced MRI with delayed acquisitions, an impairment of the blood–labyrinthine barrier (BLB) could be detected in several disorders leading to vestibular loss. We conducted a narrative review of the literature on VA focusing on the putative pathomechanisms accounting for positional and sound/pressure-induced nystagmus despite unilateral vestibular loss (UVL) in this condition, providing two novel cases of VA. Both patients presented with a clinical picture consistent with unilateral MD that rapidly turned into progressive UVL and positional and/or sound/pressure-induced vertigo. In both cases, the posterior SC was initially impaired at the video-head impulse test (vHIT) and both cervical and ocular VEMPs were initially reduced. Progressively, they developed unsteadiness with paretic spontaneous nystagmus, an impairment also for the lateral and anterior SCs, caloric hypo/areflexia and VEMPs areflexia. They both exhibited ipsilesional nystagmus to sound/pressure stimuli and in one case a persistent geotropic direction-changing positional nystagmus consistent with a “light cupula” mechanism involving the lateral SC of the affected side. A collapse of the membranous labyrinthine walls resulting in contact between the vestibular sensors and the stapes footplate could explain the onset of nystagmus to loud sounds and/or pressure changes despite no responses to high- and low-frequency inputs as detected by caloric irrigations, vHIT and VEMPs. On the other hand, the onset of positional nystagmus despite UVL could be explained with the theory of the “floating labyrinth”. Both patients received contrast-enhanced brain MRI with delayed acquisition exhibiting increased contrast uptake in the pars superior of the labyrinth, suggesting an impairment of the BLB likely resulting in secondary VA. A small intralabyrinthine schwannoma was detected in one case. VA should always be considered in case of positional and/or sound/pressure-induced vertigo despite UVL. Full article

Background: Meniere’s disease (MD) is a set of rare disorders that affects >4 million people worldwide. Individuals with MD suffer from episodes of vertigo associated with fluctuating sensorineural hearing loss and tinnitus. Hearing loss can involve one or both ears. Over 10% of the reported cases are observed in families, suggesting its significant genetic contribution. The condition is polygenic with >20 genes, and several patterns of inheritance have been reported, including autosomal dominant, autosomal recessive, and digenic inheritance across multiple MD families. Preclinical research using animal models has been an indispensable tool for studying the neurophysiology of the auditory and vestibular systems and to get a better understanding of the functional role of genes that are involved in the hearing and vestibular dysfunction. While mouse models are the most used preclinical model, this review analyzes alternative animal and non-animal models that can be used to study MD genes. Methods: A literature search of the 21 genes reported for familial MD and the preclinical models used to investigate their functional role was performed. Results: Comparing the homology of proteins encoded by these genes to other model organisms revealed Drosophila and zebrafish as cost-effective models to screen multiple genes and study the pathophysiology of MD. Conclusions: Murine models are preferred for a quantitative neurophysiological assessment of hearing and vestibular functions to develop drug or gene therapy. Full article