Search Results (113)

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 evaluate the clinical characteristics and diagnostic significance of dissociation between bithermal caloric test and video head impulse test (vHIT) in patients presenting with dizziness. Methods: We retrospectively reviewed 644 patients who underwent bithermal caloric testing and vHIT at a university-affiliated general hospital. Patients were classified into concordant and discordant groups based on test results. The discordant group was further subdivided into those with abnormal caloric test and normal vHIT, and those with normal caloric test and abnormal vHIT. Demographic data, vestibular function test outcomes, and clinical diagnoses were analyzed. Results: Discordant results were observed in 36.5% of patients. Among these, 31.8% had abnormal caloric responses with normal vHIT, and 4.7% had normal caloric responses with abnormal vHIT. Most patients in both discordant subgroups were diagnosed with peripheral vestibular disorders, such as Ménière’s disease and unilateral vestibulopathy. The abnormal caloric/normal vHIT pattern was more common and associated with low-frequency dysfunction. The normal caloric/abnormal vHIT pattern, though less frequent, also involved predominantly peripheral etiologies. Conclusions: Dissociation between caloric and vHIT results is not uncommon and provides important diagnostic insights. Employing both tests in a complementary manner enhances the identification of frequency-specific vestibular deficits and supports more accurate diagnosis and management of vestibular disorders. 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

Background/Objectives: Vestibular dysfunction is one of the main complications after cochlear implant (CI) surgery, and there are currently no standardized protocols for vestibular assessment in CI candidates. Our objectives were to investigate the incidence of vestibular impairment after CI surgery, anamnestic (age, known systemic pathologies and cause of deafness) and surgical (intraoperative complications, malposition of the CI) risk factors, and the role of vestibular assessment in the selection of the suitable ear for implantation. Methods: We included 68 adult patients (80 ears) affected by moderate-to-profound SNHL undergoing CI. The dizziness handicap inventory (DHI), the video head impulse test (VHIT), the caloric test, and dynamic posturography (DP) were used to study the vestibular function and balance before and one month after CI. The DHI was also administered 24 h after surgery. Results: Despite significative impairment 24 h after surgery (29.6 ± 30), the mean DHI score returned to preoperative values (17.9 ± 26) after one month. Dizziness persisted in case of age ≥ 65 years old, surgical difficulties, simultaneous bilateral CI, Meniere’s disease and otosclerosis, comorbidities ≥ 3, anxiety/depression, and neurological diseases. The VHIT significantly worsened in 25% of ears, while the caloric test SPV nystagmus significantly decreased in 30% of ears. In cases of preoperative unilateral weakness, the implantation of the better ear was significantly related to higher DHI scores. Only 4/68 patients had a significant persistent reduction in the postural composite score after surgery, with an increased risk of falls. Conclusions: Medical history and vestibular assessment predict the risk of vestibular damage and help to choose the CI’s side and to manage vertigo after surgery. 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

This review focuses on the contribution of medical imaging in the diagnosis of peripheral vestibular disorders. This is a narrative review based on a focused literature search conducted using PubMed and the Cochrane Library. Imaging is not usually recommended in initial consultations for vestibular disorders because only 5–10% of MRI scans reveal findings directly related to the disease. The study is a review of the literature that highlights the utility and limitations of imaging such as computed tomography (CT) and magnetic resonance imaging (MRI). It follows the diagnostic approach from history and physical examination to laboratory tests and imaging. Some conditions like vestibular neuritis and benign paroxysmal positional vertigo (BPPV) have limited imaging utility due to the fine details required. Conversely, high-resolution CT and MRI are important for diagnosing Meniere’s disease, acoustic neuroma, and superior canal dehiscence. The role of imaging varies a lot among specific conditions. Advances in imaging technology, particularly high-resolution MRI, promise enhanced diagnostic capabilities. Full article

Background/Objectives: The association between vestibular symptoms and psychological distress has been previously studied, mainly with the use of questionnaires. The purpose of this study is to compare the consumption of psychotropic drugs between a group of patients with vertigo and a control group. Methods: A prospective cross-sectional, observational, case–control study was carried out, including 506 patients (232 with Ménière’s disease, 79 with vestibular migraine, 34 with vestibular neuritis, and 161 with benign paroxysmal positional vertigo). In total, 253 participants were included in the control group. Both groups were comparable regarding age, sex, and history of previous psychiatric diseases. Results: The percentage of patients with vertigo who consumed psychotropic drugs (41.3%) was higher than the percentage of the control group who did so (26.9%) (Fisher’s exact test, p < 0.0001; OR = 1.914, CI95% (1.377; 2.662)). The mean number of psychotropic drugs consumed was also higher (Mann–Whitney test, p = 0.0003) in cases (0.68 ± 0.959) than in controls (0.47 ± 0.889). This higher consumption in the group of patients with vertigo was found for all pharmacological groups studied, being especially relevant regarding “anxiolytics and hypnotics and sedatives” and “antidepressants”. No statistically significant differences in the consumption of psychotropic drugs between types of vestibular disorders were observed. The longer the symptoms were present, the higher the prevalence of psychotropic drug use was observed. Conclusions: A relationship between vertigo and consumption of psychotropic drugs was found. Recording the consumption of these drugs is proposed as an objective method to better understand the psychological distress that patients with vertigo may suffer from. 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

Objectives: to retrospectively evaluate the clinical relevance of the 1000/500 Hz inter-frequency amplitude ratio (IFAR) in cervical and ocular vestibular evoked myogenic potentials (cVEMPs and oVEMPs) in patients with unilateral definite Ménière’s disease (MD) to identify the pathological ear. Method: cVEMPs and oVEMPs results obtained at 500 Hz and 1000 Hz were retrospectively analyzed in 28 patients with unilateral definite MD. 1000/500 Hz IFAR were calculated and compared for each ear. Spearman correlation tests between patient age and 1000/500 Hz IFAR were also performed. Results: No significant difference was observed between the 1000/500 Hz IFAR calculated in both pathological and healthy ears when the cVEMPs were performed (p = 0.74; Wilcoxon signed-rank test). 1000/500 Hz IFAR results obtained in healthy and pathological ears were also not different for oVEMPs (p = 0.73; Wilcoxon signed-rank test). Analysis of modified 1000/500 Hz IFARs for healthy and pathological ears showed no difference in both cVEMPs and oVEMPs (p = 0.44; p = 0.95, respectively; Wilcoxon signed-rank test). There was a significant positive correlation between IFARs, modified IFARs, and patient age for cVEMPs (p = 0.017; p = 0.012, respectively, Spearman’s correlation test). A significant positive correlation was also found between modified IFARs and the subject age in oVEMPs (p = 0.019, Spearman’s correlation test). Conclusions: We did not observe any significant increase of 1000/500 Hz IFARs and 1000/500 Hz modified IFARs in ears affected by definite MD compared to healthy ears. Moreover, our research suggests that the age of the participants may influence IFAR results, which may lead to misdiagnosis in the elderly. It is, therefore, essential to conduct further prospective studies in larger cohorts, stratifying results by participant age, to better understand the role of 1000/500 Hz IFAR values in the diagnosis of MD. Full article