Endolymphatic Hydrops Research Articles

Comparison between 1.5 and 3-Tesla MRI findings in Ménière's disease.

Introduction3 T-MRI of the inner ear has been used to identify the endolymphatic hydrops (EH) phenomenon, and less frequently 1.5 T-MRI. The aim of this study was to assess whether there was agreement between findings of EH at 1.5 T MRI and those obtained at 3.0 T MRI in patients clinically diagnosed with definite Meniere disease (MD).MethodsCross-sectional, blinded study was conducted in a tertiary neurotology ambulatory practice. Thirty patients with clinical diagnosis of unilateral definite MD was included. Two MRI exams (1.5 T and 3.0 T) were performed for each patient and were evaluated by two examiners (E1, E2) who were blinded to the symptomatic ear. An analysis of intra-and inter-examiner agreement was performed. It was determined whether there was an association between MRI findings and disease duration, symptom severity, and MD clinical stage.ResultsE1 found EH at 3 T-MRI in 26 (86.66%) patients and at 1.5 T-MRI in 25 (83.33%). E2 found EH in 25 (83.33%) patients in 3 T-MRI and in 22 (73.33%) at 1.5 T-MRI. The agreement between the examiners’ assessments in relation to the EH was high (0.844) for the 3 T MRI and substantial for the 1.5 T, both statistically significant. There was no statistically significant relationship between EH imaging findings and clinical disease severity and course.Discussion1.5 T and 3.0 T MRI images agreed regarding the findings of absence or presence of cochlear hydrops (CH) and vestibular hydrops (VH). The degrees of CH and VH found at 3.0 T MRI in symptomatic ear were not associated with clinical aspects and the stage of disease.

Endolymphatic hydrops and cochlear synaptopathy after noise exposure are distinct sequelae of hair cell stereociliary bundle trauma

Endolymphatic hydrops, increased endolymphatic fluid within the cochlea, is the key pathologic finding in patients with Meniere’s disease, a disease of episodic vertigo, fluctuating hearing loss, tinnitus, and aural fullness. Endolymphatic hydrops also can occur after noise trauma and its presence correlates with cochlear synaptopathy, a form of hearing loss caused by reduced numbers of synapses between hair cells and auditory nerve fibers. Here we tested whether there is a mechanistic link between these two phenomena by using multimodal imaging techniques to analyze the cochleae of transgenic mice exposed to blast and osmotic challenge. In vivo cochlear imaging after blast exposure revealed dynamic increases in endolymph that involved hair cell mechanoelectrical transduction channel block but not the synaptic release of glutamate at the hair cell–auditory nerve synapse. In contrast, ex vivo and in vivo auditory nerve imaging revealed that synaptopathy requires glutamate release from hair cells but not endolymphatic hydrops. Thus, although endolymphatic hydrops and cochlear synaptopathy are both observed after noise exposure, one does not cause the other. They are simply co-existent sequelae that derive from the traumatic stimulation of hair cell stereociliary bundles. Importantly, these data argue that Meniere’s disease derives from hair cell transduction channel blockade.

3T-3D FLAIR MRI in Menière's disease: associated profiles with clinical symptoms and electroacoustic characteristics.

Diagnosis of Menière's disease relies on clinical symptoms. Injected 3T MRI can show endolymphatic hydrops (EH), but correlation with the clinical status of MD, (probable -PMD or definite-DMD) remains doubtful. We revealed endolymphatic pressure disruption through functional exploration and verified if it was associated with an EH through MRI. We prospectively analyzed 3D3T FLAIR MRI of DMD and PMD patients. All of them underwent electrocochleography (EcoG), distortion-product otoacoustic emissions (DPOAEs), and videonystagmograhy (VNG). Amplitudes of summating potential (SP) and cochlear nerve action potential (AP) were measured on EcoG. DPOAE-phase was collected at 1kHz for the 2f1-f2 DPOAE between sitting and laying position. A SP/AP ≥ 40% and a DPOAE phase-shift > 40° revealed pressure disruption. 39 patients (25 women, 53 y.o. 20-78), were included, with 32 DMD ears and 11 PMD ears. MRI was performed in a median of 21 days [0; 68] from the MD incident. Audiovestibular exploration took place 41 days after the crisis [0;83]. MRI revealed an EH in 71.9% and 27.2% of DMD and PMD, respectively. When combining functional explorations and MRI, testing was positive in 97% for DMD and 82% for PMD. When abnormal (59%), VNG mainly showed hyporeflexia in the diseased ear. In patients suffering from DMD or PMD, with endolymphatic pressure disturbances confirmed by combined DPOAE-phase and EcoG, 3T 3D MRI reveals EH mostly in DMD but rarely in PMD. This seems to confirm that disturbance of endolymphatic pressure precedes EH.

Automated volumetric analysis of the inner ear fluid space from hydrops magnetic resonance imaging using 3D neural networks

Due to the development of magnetic resonance (MR) imaging processing technology, image-based identification of endolymphatic hydrops (EH) has played an important role in understanding inner ear illnesses, such as Meniere’s disease or fluctuating sensorineural hearing loss. We segmented the inner ear, consisting of the cochlea, vestibule, and semicircular canals, using a 3D-based deep neural network model for accurate and automated EH volume ratio calculations. We built a dataset of MR cisternography (MRC) and HYDROPS-Mi2 stacks labeled with the segmentation of the perilymph fluid space and endolymph fluid space of the inner ear to devise a 3D segmentation deep neural network model. End-to-end learning was used to segment the perilymph fluid and the endolymph fluid spaces simultaneously using aligned pair data of the MRC and HYDROPS-Mi2 stacks. Consequently, the segmentation performance of the total fluid space and endolymph fluid space had Dice similarity coefficients of 0.9574 and 0.9186, respectively. In addition, the EH volume ratio calculated by experienced otologists and the EH volume ratio value predicted by the proposed deep learning model showed high agreement according to the interclass correlation coefficient (ICC) and Bland–Altman plot analysis.

Expression of AQP8 in Serum of Patients With Meniere's Disease and Its Value in Evaluating the Degree of Hydrolabyrinth and Predicting Prognosis.

This study aims to explore the role of serum aquaporin 8 (AQP8) expression in evaluating the degree of hydrolabyrinth and predicting prognosis in patients with Meniere's disease. One hundred and five patients diagnosed with Meniere's disease in our hospital were enrolled in the Meniere's disease group. Another 102 healthy subjects were enrolled as the control group. The expression of serum AQP8 mRNA was determined by the quantitative real-time PCR (qRT-PCR) method. Receiver operating characteristic (ROC) curve analysis was carried out to analyse the predictive value of serum AQP8 mRNA expression for poor prognosis in Meniere's disease patients. Multivariate logistic regression was used to analyse the influencing factors of poor prognosis in patients with Meniere's disease. The expression level of serum AQP8 mRNA in the Meniere's disease group was significantly higher than that in the control group (p < 0.05). In the severe hydrops group, serum AQP8 mRNA expression levels were higher than in the mild hydrops group and the no endolymphatic hydrops group. Additionally, the mild hydrops group had higher serum AQP8 mRNA levels than the no endolymphatic hydrops group (p < 0.05). The disease course, proportion of severe hydrops and serum AQP8 mRNA expression were all higher in the poor prognosis group compared to the good prognosis group (p < 0.05). The area under the curve (AUC) for serum AQP8 mRNA in predicting poor prognosis in Meniere's disease patients was 0.812 (95%CI: 0.702-0.922). AQP8 mRNA is associated with the degree of hydrolabyrinth in patients with Meniere's disease and plays an important role in predicting prognosis.

Impairment of Internal Auditory Canal Barrier in Meniere'sDisease.

The aim of this investigation was to explore the potential correlation between the signal intensity ratio (SIR) at the internal auditory canal (IAC) fundus and hearing impairment in Meniere's disease (MD), thereby providing a foundation to further understand the mechanisms underlying hearing loss. Fifty patients diagnosed with unilateral definite MD were enrolled in the study. 3D-FLAIR MRI was conducted 4 h after intravenous administration of gadobutrol to determine the SIR of the bilateral IAC fundus. The difference in the SIR of the IAC fundus between the affected and unaffected sides was assessed, followed by an analysis of its correlation with low-, middle-, and high-tone hearing thresholds. Correlation analysis was also conducted between the degree of endolymphatic hydrops (EH) in the vestibule and cochlea and the SIR on the affected side. The degree of EH in MD can be clearly visualised using 3D-FLAIR MRI. The SIR on the affected side was significantly higher than that on the unaffected side (p = 0.000). Furthermore, a positive correlation was observed between the SIR at the affected and low (r = 0.692, p = 0.000), middle (r = 0.615, p = 0.000) and high-tone (r = 0.440, p = 0.001) hearing thresholds, while the SIR showed no significant correlation with cochlear (r = 0.315, p = 0.088) or vestibular hydrops (r = 0.215, p = 0.244). The IAC fundus barrier may be damaged in patients with MD, representing one of the factors affecting the level of hearing.

Deep Phenotyping of a Mouse Model for Hearing Instability Disorders.

Hearing instability in Slc26a4-insufficiency mice may be due to differential expression of genes related to ion homeostasis and activated macrophages. Hearing instability (HI) disorders, defined by either hearing fluctuation or sudden loss, remain incompletely understood. Recent studies have described a Slc26a4 (pendrin)-insufficiency mouse model (DE17.5) that offers a genetically driven model for HI, although deep audiometric and immunohistologic phenotyping of this model remains poorly characterized. Homozygous DE17.5 mice with (F) and without (NF) HI were delineated by serial auditory brainstem responses (ABR) between postnatal days 30 and 60 and compared with adult phenotypically wild-type Slc26a4-heterozygous controls without evidence of HI (Het). HI was defined as a change in threshold of at least 15 dB in at least two frequencies or at least 20 dB in at least one frequency from the previous week. Stria vascularis (SV) cell type-specific gene expression, endolymphatic hydrops (EH), endocochlear potential (EP), and macrophage activation were analyzed and compared between the cohorts. F mice demonstrated significant reductions in the expression of cell type-specific genes related to ion homeostasis and increased macrophage activation within the SV compared with NF and Het cohorts. Both F and NF DE17.5 homozygous mice demonstrated reductions in EP and increased EH compared with the Het cohort. Deep phenotyping of DE17.5 mice demonstrates changes in EP and EH compared with control; however, the HI phenotype was associated with differential ion homeostasis gene expression and increased macrophage activation in the SV. This provides potential further insights into the underlying pathogenesis and possible immunologic contributions of HI in humans.

Comparative analysis of classic and delayed Ménière's disease: clinical characteristics and endolymphatic hydrops manifestations in an Asian cohort.

To investigate the clinical characteristics and endolymphatic hydrops (ELH) Manifestations in patients with delayed Ménière's disease (DMD) compared to classic Ménière's disease (CMD). A prospective cohort study was conducted involving 523 patients diagnosed with MD. Clinical data, inner ear function, and ELH characteristics were analyzed and compared between CMD and DMD groups. Assessments included audiometry, VEMP, and MRI. DMD prevalence was 6.1%. Bilateral MD was significantly more common in DMD (34.4% vs. 14.5%, P = 0.003). DMD patients had more severe hearing loss in the first involved ear and more deteriorated VEMP in the contralateral ear (P < 0.05). ELH was present in 100% of affected ears, with a higher contralateral ELH proportion in DMD (34.4% vs. 15.0%, P = 0.005). DMD patients exhibit a higher prevalence of bilateral MD, more severe hearing loss, and greater contralateral ELH compared to CMD patients. Both subtypes share similar ELH characteristics, suggesting common underlying mechanisms.

Morphometric evaluation of facial and vestibulocochlear nerves using magnetic resonance imaging in patients with Menière's disease.

Menière's disease (MD) is a condition of unknown etiology, involving genetic predisposition, autoimmune processes, viral infections, cellular apoptosis, and oxidative stress. This study aimed to investigate potential differences in the VIIth and VIIIth cranial nerves in MD patients using Hydrops-MRI (FLAIR) for morphometric evaluations. Using a 3T MRI scanner, constructive-interference-in-steady-state (CISS) and 3D-FLAIR-inversionrecovery (FLAIR) sequences were acquired. We evaluated the morphometrics of the VIIth and VIIIth cranial nerves from the cerebellopontine angle to the internal auditory canal fundus, comparing the non-affected and affected sides. Furthermore, we examined the findings in relation to symptom duration and evaluated feasibility of FLAIR-imaging in morphometry of cranial nerves. A total of 53 MD patients with unilateral symptoms were included. After statistical analysis, no significant differences were found regarding morphometric changes in the affected side compared to the non-affected side of the VIIth and VIIIth cranial nerves. There was also no significant difference between morphometric evaluations of patients with different symptom durations. The morphometric evaluation using Hydrops-MRI-Sequences (FLAIR) showed no significant difference compared to established morphometric highly T2-weighted imaging (CISS). Our data found no differences in nerve morphometry between clinically non-affected and affected sides in unilateral MD patients, nor any correlation with symptom duration. This contrasts with previous findings of correlations between clinical features and endolymphatic hydrops. A disease process starting before clinical symptom onset could be a possible explanation. Morphometric evaluation of brain nerves using Hydrops-MRI-Sequences is practical and provides similar results to T2-weighted imaging, improving patient comfort and reducing MRI scan time. CN = cochlear nerve; CPA = cerebellopontine angle; CSA = cross-sectional area; FN = facial nerve; IAC = internal auditory canal; IVN = inferior vestibular nerve; LD = long diameter; MD = Menière's disease; SD = short diameter; SVN = superior vestibular nerve.

Correlation between the etiology of severe hearing loss and endolymphatic hydrops.

This study aimed to investigate correlation between the presence of endolymphatic hydrops(EH) and factors such as causes of hearing loss, patient age, duration of deafness, and results of vestibular function tests. We retrospectively reviewed medical charts of 128 ears of cochlear implantees who were not considered relevant to Meniere's disease. When comparing group with genetic variants of GJB2, SLC26A4, LMX1A and other genetic mutation group, the proportion of vestibular EH and cochlear EH found in group with genetic variants of GJB2, SLC26A4, LMX1A was significantly higher than group with other genetic etiology (p < 0.01) or the group with all the other causes of hearing loss (p < 0.01). The rate of vestibular and cochlear EH detection was higher in younger patients (41.5% and 35.4%) than in older patients (25.4% and 20.6%). A higher ratio of vestibular and cochlear EH was observed in patients with a longer duration of deafness (37.5% and 31.3%) than those with a shorter duration of deafness (29.7% and 25.0%). The group with vestibular EH showed a higher incidence of abnormal findings in the caloric test (42.9%) than the group without vestibular EH (28.2%). Patients with genetic variants of GJB2, SLC26A4, LMX1A, younger patients, those with longer deaf durations showed a higher prevalence of vestibular and cochlear EH, implying EH appears to be formed as a developmental disorder in association with a certain set of genetic variants, rather than a phenotypic marker as a result of severe to profound hearing loss.

Detecting endolymphatic hydrops in patients with cranial nerve VIII schwannoma using hydrops MR examination.

This study utilized Hydrops MRI in patients with cranial nerve (CN) VIII schwannoma to assess the concomitance with endolymphatic hydrops (EH), aiming to elucidate the mechanism of hydrops formation in these patients. Twenty-six patients diagnosed as CN VIII schwannoma including vestibular schwannoma (VS) in 24 and intracochlear schwannoma (ICS) in 2 were enrolled. Fifteen patients received radiosurgery and 11 patients opted for a wait-and-scan approach. All patients underwent an inner ear test battery, followed by Hydrops MRI. Based on Hydrops MRI, 6 patients (23%) with positive EH were assigned to Group A, while the other 20 patients showing negative EH were assigned to Group B. The abnormality rates of inner ear test battery in Group A ran from the audiometry (100%), cervical vestibular-evoked myogenic potential (cVEMP) test (83%), ocular VEMP (oVEMP) test (67%) to the caloric test (33%), exhibiting a significantly declining sequence. This declining sequence is consistent with the decreasing order in the prevalence of EH from the cochlea (83%), saccule (50%) to utricle (50%). However, Group B did not show such decreasing trend, indicating that Groups A and B did not share the common mechanism. The mean tumor size prior to radiosurgery was 1.91 ± 0.89cm, which significantly reduced to 1.53 ± 0.60cm at a mean interval of 6years following radiosurgery. In contrast, tumor size remained unchanged in those opted for a wait-and-scan approach. It is recommended to utilize Hydrops MRI for patients with CN VIII schwannoma during annual follow-up, particularly those experienced episodic vertigo and/or deteriorated hearing. The Hydrops MRI can not only monitor tumor size, but also detect the presence of EH, so as to guide treatment decision.

Perilymphatic enhancement and endolymphatic hydrops: MRI findings and clinical associations.

In this study, we aimed to summarize magnetic resonance imaging (MRI) findings of perilymphatic enhancement (PE) and endolymphatic hydrops (EH) of the inner ear, which are associated with vestibular and cochlear symptoms. We analyzed data on ears with definite Meniere's disease (MD), sensorineural hearing loss (SNHL), vertigo, and listening difficulties (LiD) from 508 ears of 254 patients who underwent contrast-enhanced 3-Tesla MRI between April 2021 and March 2023. We evaluated the degree of endolymphatic hydrops (EH), signal intensity ratios (SIRs) between the basal turns of the cochlea and cerebellum, and hearing levels for all ears. Ears with definite MD were also assessed for changes in vestibular and cochlear symptoms within 6 months. Ears with definite MD exhibited significantly higher percentages of EH in both the vestibule and cochlea compared with ears with other diseases. Furthermore, ears with MD or sensorineural hearing loss (SNHL) had significantly higher SIRs of PE compared with ears with other diseases or asymptomatic ears. Among patients with definite MD, those experiencing hearing fluctuations or vertigo attacks within the last 6 months had significantly higher SIRs of PE compared with those who did not experience any symptoms. Significant EH in the vestibule and cochlea was a major finding for the imaging diagnosis of definite MD. A high SIR of PE was a good indicator for assessing MD activity, reflecting vestibular and cochlear symptoms and fluctuations.Level of Evidence: 4.

Correlation of Endolysmphatic Duct Signal Intensity With Clinical Features in Otological Diseases.

Bilateral high signal intensity (SI) in the endolymphatic duct (ED) on magnetic resonance imaging (MRI) has been reported as a common characteristic in ears with large vestibular aqueduct syndrome (LVAS). However, the significance of bilateral high SI in the ED remains unknown. The present study aimed to compare the correlation between SI in the ED and the clinical manifestations in various otological disorders and consider the significance of the MRI findings. Retrospective study. University hospital. The study included 2,450 ears from 1,225 patients with various otological disorders. All ears underwent 3T enhanced MRI and were evaluated for the degree of endolymphatic hydrops (EH) and the SI ratios (SIRs; i.e., the calculation between SIs in the ED and those in the cerebellum). The imaging findings were compared with their clinical symptoms. Ears with bilateral high SIRs in the ED tended to have considerably less occurrence of EH in both the cochlea and vestibule than those with bilateral low SIRs. Ears with SIR ≥8 showed significantly elevated hearing thresholds at lower frequencies on pure-tone audiometry, although they exhibited a markedly lower incidence of cochlear EH than those with SIR <8. Moreover, ears with vertigo exhibited notably higher SIRs than those without vertigo. Bilateral high SI in the ED on MRI may reflect pathophysiology underlying sensorineural hearing loss and vestibular symptoms, which are not associated with EH formation.

The characteristics of three-dimensional real inversion recovery MRI sequence in delayed Meniere’s disease

Background Delayed Meniere’s disease (DMD) is characterized by episodic vertigo occurring after a significant delay following longstanding sensorineural hearing loss. However, its pathogenesis and characteristics remain unclear. Objectives To explore the characteristics of endolymphatic hydrops (EH) in DMD and investigate its pathology using MRI. Materials and methods 17 patients diagnosed with DMD were included in this study. A 3D-real-IR MRI sequence was utilized to visualize and grade EH. The signal intensity of the basal cochlear turn was compared to that of the cerebellar white matter to calculate the CC ratio. A paired t-test was employed to assess the differences in the CC ratio between the affected and unaffected sides in patients with unilateral DMD. Results EH was present in 100% of cases on the affected side. Unilateral EH was seen in 82.4%, and bilateral EH in 23.5%. Vestibular EH without cochlear involvement occurred in 29.4%, and cochlear EH without vestibular involvement in 5.9%. The CC ratio was significantly higher on the affected side (p < 0.01). Conclusions and significance A significant grade of vestibular EH was observed in DMD, with bilateral EH being relatively common. The increased contrast on the affected side may reflect the underlying pathology in DMD patients.

The relationship between endolymphatic hydrops features and hearing loss in Bilateral Meniere's disease

BackgroundThis study aimed to investigate the relationship between the features of endolymphatic hydrops and hearing loss in patients with Bilateral Meniere's Disease.MethodsA retrospective analysis was conducted on 77 patients diagnosed with Bilateral Meniere's Disease. The features of endolymphatic hydrops in the affected ear were evaluated through gadolinium-enhanced inner ear Magnetic resonance imaging. The Spearman correlation coefficient, paired t-tests, and Wilcoxon signed-rank tests were employed for data analysis.ResultsThe analysis revealed a significant correlation between the degree of endolymphatic hydrops and hearing loss across all frequencies(0.125–8 kHz), including the cochlear, vestibular, and overall degree of endolymphatic hydrops. The strongest correlation between the overall degree of endolymphatic hydrops and hearing loss was observed at low frequencies (r = 0.571, p < 0.05), followed by mid-frequencies (r = 0.508, p < 0.05), and high-frequencies (r = 0.351, p < 0.05), with a correlation of r = 0.463, p < 0.05 for the staging of Meniere's disease. Affected Ears with endolymphatic hydrops both in the cochlea and vestibule exhibited more severe hearing loss and Meniere's disease staging compared to those with isolated endolymphatic hydrops within the same patient.ConclusionsThe features of endolymphatic hydrops in patients with Bilateral Meniere's Disease were found to correlate with the severity of hearing loss and the staging of Meniere's disease.

Imaging endolymphatic space of the inner ear in vestibular migraine

BackgroundVestibular migraine (VM), the most frequent episodic vertigo, is difficult to distinguish from Ménière’s disease (MD) because reliable biomarkers are missing. The classical proof of MD was an endolymphatic hydrops...

Comparative analysis of vestibular endolymphatic hydrops grading methods and hearing loss in Ménière's disease: a retrospective MRI study using 3D-real inversion recovery sequence.

To compare the correlation between different grading methods of vestibular endolymphatic hydrops (EH) and the severity of hearing loss in Ménière's disease (MD), and evaluate the diagnostic value of these methods in diagnosing MD. This retrospective study included 30 patients diagnosed with MD from June 2021 to August 2023. All patients underwent inner ear MR gadolinium-enhanced imaging using three-dimensional (3D)-real inversion recovery sequences and pure-tone audiometry. The EH levels were independently evaluated according to the classification methods outlined by Nakashima et al. (Acta Otolaryngol Suppl 5-8, 2009. https://doi.org/10.1080/00016480902729827 ) (M1), Fang et al. (J Laryngol Otol 126:454-459, 2012. https://doi.org/10.1017/S0022215112000060 ) (M2), Barath et al. (Am J Neuroradiol 35:1387-1392, 2014. https://doi.org/10.3174/ajnr.A3856 ), (M3), Liu et al. (Front Surg 9:874971, 2022. https://doi.org/10.3389/fsurg.2022.874971 ), (M4), and Bernaerts et al. (Neuroradiology 61:421-429, 2019. https://doi.org/10.1007/s00234-019-02155-7 ) (M5), with a subsequent comparison of interobserver agreement. After achieving a consensus, an analysis was performed to explore the correlations between vestibular EH grading using different methods, the average hearing thresholds at low-mid, high-, and full frequencies and clinical stages. The diagnostic capabilities of these methods for MD were then compared. The interobserver consistency of M2-M5 was superior to that of M1. The EH grading based on M4 showed a significant correlation with the average hearing thresholds at low-mid, high-, and full frequencies and clinical stages. M1, M2, M3, and M5 correlated with some parameters. A receiver operating characteristic curve analysis indicated that M5 significantly outperformed M1, M2, M3, and M4 in terms of diagnostic efficiency for MD. M4 showed the strongest correlation with the degree of hearing loss in patients with MD, whereas M5 showed the highest diagnostic performance.

Differentiating Definite and Probable Ménière Disease: A Comprehensive Evaluation of Audio-Vestibular Function Testing Combined with Inner Ear MRI.

To evaluate the differences between audio-vestibular function testing and inner ear gadolinium magnetic resonance imaging (MRI) in distinguishing definite Ménière disease (DMD) and probable Ménière disease (PMD), and to provide a reference for early clinical diagnosis and intervention. A total of 116 patients diagnosed with DMD (n = 80) and PMD (n = 36) were enrolled. The differences in the results of pure tone audiometry, caloric test, and tympanic injection of gadolinium for contrast-enhanced MRI between the two groups were compared and analyzed. Parameters that could differentiate between the two conditions were identified, and the sensitivity and specificity and the area under the curve (AUC) of individual and combined indices in the differential diagnosis of DMD and PMD were evaluated. The hearing threshold and hearing asymmetry rate of the DMD group were significantly higher than those of the PMD group (p < 0.001), 98.8% and 30.6%, respectively. The abnormal rates of canal paresis (CP) and severity of endolymphatic hydrops in the DMD group were higher than those in the PMD group (p < 0.05). When combined with high-frequency hearing thresholds, hearing asymmetry, hearing curve type, endolymphatic hydrops, and abnormal CP, the diagnostic accuracy of DMD was improved compared to using high-frequency alone (p < 0.05). This study showed that PMD and DMD may represent two different stages in the development of MD disease. The comprehensive assessment of audio-vestibular function testing and inner ear MRI proves beneficial for early diagnosis, potentially contributing to the preservation of inner ear function.

Applications of Machine Learning in Meniere's Disease Assessment Based on Pure-Tone Audiometry.

To apply machine learning models based on air conduction thresholds of pure-tone audiometry for automatic diagnosis of Meniere's disease (MD) and prediction of endolymphatic hydrops (EH). Retrospective study. Tertiary medical center. Gadolinium-enhanced magnetic resonance imaging sequences and pure-tone audiometry data were collected. Subsequently, basic and multiple analytical features were engineered based on the air conduction thresholds of pure-tone audiometry. Later, 5 classical machine learning models were trained to diagnose MD using the engineered features. The models demonstrating excellent performance were also selected to predict EH. The model's effectiveness in MD diagnosis was compared with experienced otolaryngologists. First, the winning light gradient boosting (LGB) machine learning model trained by multiple features demonstrates a remarkable performance on the diagnosis of MD, achieving an accuracy rate of 87%, sensitivity of 83%, specificity of 90%, and a robust area under the receiver operating characteristic curve of 0.95, which compares favorably with experienced clinicians. Second, the LGB model, with an accuracy of 78% on EH prediction, outperformed the other 3 machine learning models. Finally, a feature importance analysis reveals a pivotal role of the specific pure-tone audiometry features that are essential for both MD diagnosis and EH prediction. Highlighted features include standard deviation and mean of the whole-frequency hearing, the peak of the audiogram, and hearing at low frequencies, notably at 250 Hz. An efficient machine learning model based on pure-tone audiometry features was produced to diagnose MD, which also showed the potential to predict the subtypes of EH. The innovative approach demonstrated a game-changing strategy for MD screening and promising cost-effective benefits for the health care enterprise.

Bilateral Horizontal Canal Dysplasia Presenting as Hearing Loss With Recurrent Vertigo Mimicking Meniere's Disease.

We report an extremely rare case with bilateral horizontal semicircular canal (HSC) dysplasia presenting as hearing loss with recurrent vertigo mimicking Meniere's disease in a previously healthy 49-year-old male patient. HSC malformation is one of the common isolated developmental anomalies of the bony labyrinth usually associated with varying degree of hearing loss. He suffered from recurrent episodes of vertigo lasting more than 20 minutes accompanied by left hearing loss and tinnitus for 3 years. Pure-tone audiometry revealed a mild to moderate hearing loss at low frequencies in the left ear. Bithermal caloric testing revealed significant left-sided canal paralysis, but other vestibular testing was normal. Computerized tomography (CT) of the temporal bone showed the left HSC hypoplasia lacking a central bony island fused together with an enlarged vestibule, and right dysplasia, finally diagnosed with bilateral HSC dysplasia. Meniere's disease-like recurrent vertigo attack and hearing loss were suspected to be caused by subsequent endolymphatic hydrops which could be due to HSC dysplasia.