Temporal Bone Histopathology of Atypical Cogan Syndrome.

Cogan's syndrome is a rare disorder first clinically defined in 1945, characterized by nonsyphilitic interstitial keratitis and progressive audiovestibular symptoms. Later, patients with audiovestibular dysfunction and various types of inflammatory eye disease were classified as having atypical Cogan's syndrome. The etiology and pathogenesis of Cogan's syndrome remain largely unknown. Here, we report a case of atypical Cogan's syndrome with a histological assessment of the temporal bone during the acute disease period. Temporal bone histology was compared to age- and gender-matched normal control, and our findings revealed endolymphatic hydrops and degenerative changes in various parts of the inner ear. Laryngoscope, 2024.

An unenhanced 3D-FLAIR sequence using long repetition time and constant flip angle to image endolymphatic hydrops.

OBJECTIVES: To evaluate a three-dimensional fluid-attenuated inversion recovery (3D-FLAIR) sequence using a long repetition time (TR) and constant flip angle (CFA) in differentiating between perilymph and endolymph in a phantom study, and unenhanced endolymphatic hydrops (EH) imaging in a patient study. METHODS: Three solutions in similar ion and protein concentrations with endolymph, perilymph, and cerebrospinal fluid were prepared for variable flip angle (VFA) 3D-FLAIR (TR 10,000 ms) and CFA (120°) 3D-FLAIR using different TR (10,000, 16,000, and 20,000 ms). Fifty-two patients with probable or definite Meniere's disease received unenhanced CFA (120°) 3D-FLAIR using a long TR (20,000 ms) and 4-h-delay enhanced CFA (120°) 3D-FLAIR (TR 16,000 ms). Image quality, signal-to-noise ratio (SNR), and contrast-to-noise ratio (CNR) of them were compared. Agreement in the evaluation of the EH degree between them was analyzed. RESULTS: In the phantom study, CNRs between perilymphatic and endolymphatic samples of VFA 3D-FLAIR (TR 10,000 ms) and CFA 3D-FLAIR (TR 10,000, 16,000, and 20,000 ms) were 6.66 ± 1.30, 17.90 ± 2.76, 23.87 ± 3.09, and 28.22 ± 3.15 (p < 0.001). In patient study, average score (3.65 ± 0.48 vs. 4.19 ± 0.40), SNR (34.56 ± 9.80 vs. 51.40 ± 11.27), and CNR (30.66 ± 10.55 vs. 45.08 ± 12.27) of unenhanced 3D-FLAIR were lower than enhanced 3D-FLAIR (p < 0.001). Evaluations of the two sequences showed excellent agreement in the cochlear and vestibule (Kappa value: 0.898 and 0.909). CONCLUSIONS: The CFA 3D-FLAIR sequence using a long TR could be used in unenhanced EH imaging with high accuracy. CLINICAL RELEVANCE STATEMENT: Unenhanced imaging of endolymphatic hydrops is valuable in the diagnosis and follow-up of patients, especially those who cannot receive contrast-enhanced MRI. KEY POINTS: Ion and protein concentration differences can be utilized in differentiating endolymph and perilymph on MRI. Endolymphatic and perilymphatic samples could be differentiated in vitro on this 3D-FLAIR sequence. This unenhanced 3D-FLAIR sequence is in excellent agreement with the enhanced constant flip angle 3D-FLAIR sequence.

Relationship Between Cochlear Lateral Wall Changes and Endolymphatic Hydrops in Otitis Media.

OBJECTIVES: Despite otitis media and various disease processes being associated with endolymphatic hydrops (EH), an exact explanation of the pathophysiology has yet to be reported. This study aimed to investigate the changes in the cochlear lateral wall structures and their potential correlation with the presence and severity of cochlear EH in acute and chronic otitis media cases. The investigations were conducted in both chinchilla animal model and human temporal bone specimens. METHODS: We studied a total of 15 chinchilla and 25 human temporal bones from our collection, which were categorized into acute otitis media, chronic otitis media (COM), and control groups. Through quantitative analysis, we measured the area of cochlear lateral wall structures and observed the presence and the degree of EH using light microscopy. RESULTS: No significant changes were determined in the area of the spiral ligament (p > 0.05) across the species. However, a significant (p < 0.05) decrease in the mean area of the stria vascularis in the basal turn was identified in COM groups compared to controls of both species. Chinchilla model additionally exhibited pathology extending to the lower mid turn. A negative correlation was found between the mean strial area and the severity of EH in both the animal model and human samples. CONCLUSIONS: COM associated with significant changes in the stria vascularis that may lead to significant increase in the degree of EH. The presented animal model exhibited parallel findings with human samples, suggesting its viability as a valuable model for future studies. LEVEL OF EVIDENCE: N/A Laryngoscope, 2024.

Efficacy of 100 hz sound stimulation on saccular dysfunction in meniere's disease.

BACKGROUND: Although various medical remedies have been attempted to alleviate the symptoms of Meniere's disease (MD), the risk-benefit ratios of these various treatments remain debatable. OBJECTIVE: We investigated the efficacy of sound stimulation of 100 Hz for treating vestibular dysfunction in patients with Meniere's disease (MD). MATERIALS AND METHODS: Patients with definitive MD with intractable vestibular symptoms and endolymphatic hydrops (EH) in the inner ear were evaluated. The experimental group received sound stimulation of 75 dB at a frequency of 100 Hz for 5 min, and the control group received sound stimulation of 75 dB at a frequency of 250 Hz for 5 min. Cervical vestibular-evoked myogenic potentials (cVEMPs) were measured before and after each sound stimulation, and the results of the clinical tests were compared between the two patient groups. RESULTS: Significant increases in cVEMP amplitudes were observed after sound stimulation of 100 Hz in ears with vestibular endolymphatic hydrops, although no such improvement was observed in the control group. CONCLUSION: Sound stimulation of 75 dB at a frequency of 100 Hz leads to improvement in cVEMP amplitude in patients with definitive MD. Adequate sound stimulation might be a new method for treating vestibular dysfunction associated with MD.

Potential Application of Hydrops MR Imaging: A Systematic Review.

BACKGROUND: Diagnostic dilemma between clinical Meniere's disease and radiological endolymphatic hydrops (EH) has emerged since the introduction of hydrops magnetic resonance imaging (MRI). The aim of this study is to explore the potential application of hydrops MRI on diagnosing the EH. METHODS: This review was developed from peer-reviewed articles published in those journals listed on journal of citation reports. The MEDLINE database of the US National Library of Medicine, Scopus, and Google Scholar were used to collect articles based on the guidelines (PRISMA 2020 statement) for reporting reviews. RESULTS: Initially, 470 articles were retrieved from 1983 to 2023, and 80 relevant articles were ultimately selected. The sensitivity (69%-92%) and specificity (78%-96%) values varied from each laboratory for detecting EH via hydrops MRI, probably due to candidate selection and the grading system employed. CONCLUSION: The application of hydrops MRI allows (1) differentiation between EH and sudden sensorineural hearing loss; (2) determination of the affected side of EH; and (3) confirmation of the diagnosis of EH concomitant with other disorders. Notably, not all differentials for EH can be visualized on MR images. One of the existing gaps to be filled is that updated hydrops MRI fails to identify distortion, that is, rupture, collapse, fistula, or fibrosis of the inner ear compartments, akin to what histopathological evidence can demonstrate. Hence, enhanced ultrahigh resolution of hydrops MRI is required for demonstrating fine structures of the inner ear compartments in the future.

Sudden Sensorineural Hearing Loss in Patients Aged from 15 to 40 Years.

Objectives: The purpose of this study was to investigate the hearing characteristics and causes of sudden sensorineural hearing loss (SSNHL) in patients aged from 15 to 40 years, focusing on audiological outcomes one year after the diagnosis. Methods: The medical records of individuals with SSNHL who were referred to our tertiary-level audiologic center were reviewed. All patients had undergone comprehensive diagnostic evaluations, including high-resolution 3D-FLAIR delayed magnetic resonance imaging (MRI), cone beam computed tomography (CBCT), and screening for coagulation, infectious, and autoimmune diseases. Results: Overall, 56 patients (mean age 28.1 ± 7.6 years) were included in the study. The hearing threshold in the affected ear improved significantly from 56.0 ± 18.0 dB at the diagnosis to 46.9 ± 22.3 dB after one year (p = 0.02). The degree of hearing loss, audiometric configurations, hearing improvements, and adherence to hearing treatments showed considerable variability among patients. Aural fullness, tinnitus, and hyperacusis were the predominant symptoms associated with SSNHL, and their prevalence decreased significantly over time. The diagnostic protocol led to the identification of the specific cause of SSNHL in 75% (42/56) of patients. The known etiology was found to be otological (39.3%), infectious (21.4%), autoimmune (7.1%), vascular (5.4%), or neoplastic (1.8%). In particular, Menière's disease (n = 12), isolated cochlear endolymphatic hydrops (n = 6), HSV-1 (n = 5), and EBV (n = 4) infections were the most frequent causes of SSNHL. Conclusions: The identification of the specific etiology of SSNHL may facilitate a more personalized approach to management and treatment.

Relationship between vestibular schwannoma and endolymphatic hydrops.

BACKGROUND: Both vestibular schwannoma (VS) and Meniere's disease (MD) patients underwent hydrops MRI to clarify the relationship between VS and endolymphatic hydrops (EH). METHODS: Eighty patients with VS or MD underwent an inner ear test battery followed by hydrops MRI, and were then divided into 3 groups. Group A comprised 58 MD patients (62 ears) with positive EH but negative VS. Group B included 18 VS patients (18 ears) with negative EH, while Group C consisted of 4 patients (4 ears) who had VS concomitant with EH. Another 14 MD patients who tested negative for EH on hydrops MRI were initially excluded from this cohort, but were later included for comparison. RESULTS: The decreasing prevalence of EH at the cochlea, saccule and utricle in Group A was identified in 59 (95%), 42 (68%) and 40 (65%) ears, respectively, mimicking a declining sequence of abnormality rates running from audiometry (86%), cervical vestibular-evoked myogenic potential (cVEMP) test (55%) to the ocular (oVEMP) test (53%). However, such decreasing trend was not identified in Groups B and C. In Groups C and A combined, 4 (6%) of 62 EH patients had concomitant VS. Conversely, 4 (18%) of 22 VS patients in Groups C and B combined had concurrent EH. CONCLUSION: A very low (6%) rate of VS in EH patients indicates that VS in EH patients may be coincidental. In contrast, EH was identified in 18% prevalence of VS patients, mirroring the 22% prevalence of cochlear EH demonstrated in VS donors through histopathological studies.

A systematic review of the clinical evidence for an association between type I hypersensitivity and inner ear disorders.

Inner ear disorders have a variety of causes, and many factors can contribute to the exacerbation of cochlear and vestibular pathology. This systematic review aimed to analyze clinical data on the coexistence and potential causal interaction between allergic diseases and inner ear conditions. A search of PubMed and Web of Science identified 724 articles, of which 21 were selected for full-text analysis based on inclusion and exclusion criteria. The epidemiologic evidence found overwhelmingly supports an association between allergic disease and particular inner ear disorders represented by a high prevalence of allergic reactions in some patients with Ménière's disease (MD), idiopathic sudden sensorineural hearing loss (ISSHL), and acute low-tone hearing loss (ALHL). In addition, patients with MD, ISSHL, and ALHL had higher levels of total serum IgE than healthy subjects. Finally, in some cases, changes in cochlear potential may have been induced by antigen exposure, while desensitization alleviated allergy and inner ear-related symptoms. The exact mechanism of interaction between the auditory/vestibular and immune systems is not fully understood, and further clinical and basic research is needed to understand the relationship between the two systems fully.

A hidden pathophysiology of endolymphatic hydrops: case report of a patient with spontaneous intracranial hypotension presenting with sudden sensorineural hearing loss with vertigo.

Spontaneous intracranial hypotension (SIH) is characterized by decreased cerebrospinal fluid (CSF) volume due to leakage through the dural membrane. We present the case of a patient with SIH manifested by fluctuating low-frequency hearing loss, tinnitus, and vertigo. In this patient, endolymphatic hydrops in the cochlea and saccule were visualized by means of a special sequence of inner ear magnetic resonance imaging scans, with a gadolinium-based contrast agent administered intravenously. Endolymphatic hydrops is a potential underlying pathophysiology of SIH-associated hearing impairment. We hypothesize that SIH may be a rare cause of 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.

PURPOSE: 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. METHODS: 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. RESULTS: 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. CONCLUSION: M4 showed the strongest correlation with the degree of hearing loss in patients with MD, whereas M5 showed the highest diagnostic performance.

Can Multifrequency Tympanometry Be Used in the Diagnosis of Meniere's Disease? A Systematic Review and Meta-Analysis.

(1) Background: Ménière's disease (MD) is a disease of the inner ear, presenting with episodes of vertigo, hearing loss, and tinnitus.The aim of this study is to examine the role of multifrequency tympanometry (MFT) in the diagnosis of MD. (2) Methods: A systematic review of MEDLINE (via PubMed), Scopus, Google Scholar, and the Cochrane Library was performed, aligned with the PRISMA guidelines. Only studies that directly compare ears affected by Ménière's disease with unaffected or control ears were included. Random-effects model meta-analyses were performed. (3) Results: Seven prospective case-control studies reported a total of 899 ears, 282 of which were affected by Ménière's disease (affected ears-AE), 197 unaffected ears in patients with MD (UE), and 420 control ears (CE) in healthy controls. No statistically significant differences between the groups were observed regarding resonant frequency (RF). The pure tone audiometry average of the lower frequencies (PTA basic) was significantly greater in affected ears when compared with unaffected ears. The conductance tympanogram at 2 kHz revealed a statistically significantly greater G width of 2 kHz in the affected ears when compared to both unaffected and control ears, while control ears had a statistically significant lesser G width of 2 kHz compared to both the other two groups. (4) Conclusions: MFT, and specifically G width at 2 kHz, could be an important tool in the diagnosis of MD.

Regression of Endolymphatic Hydrops in Patient With Meniere's Disease Treated With Vestibular Neurectomy: A Case Report.

Vestibular neurectomy is an effective method eliminating vertigo attacks in patients suffering from disabling Meniere's disease with no clinical improvement despite conservative and intratympanic therapy. Magnetic resonance imaging allows in vivo evaluation of changes manifesting in the inner ear after treatment; however, downgrading of the endolymphatic hydrops after vestibular neurectomy had not been previously described in the literature. In the present article, a case of a patient with unilateral severe Meniere's disease treated with selective vestibular nerve section from middle fossa approach was described. Clinical symptoms and audiovestibular tests were evaluated before and 13 months after the surgery. Complete resolution of vertigo episodes and hearing preservation was achieved. Magnetic resonance imaging was performed before and after the surgery using a 3 Tesla scanner with dedicated protocol after intravenous administration of gadolinium contrast agent. In the follow-up examination, regression of the cochlear and vestibular endolymphatic hydrops was visualized, which may suggest processes occurring in the labyrinth as a result of the vestibular efferent fibers section.

Direct Visualization of Tracer Permeation into the Endolymph in Human Patients Using MR Imaging.

PURPOSE: The endolymph of the inner ear, vital for balance and hearing, has long been considered impermeable to intravenously administered gadolinium-based contrast agents (GBCAs) due to the tight blood-endolymph barrier. However, anecdotal observations suggested potential GBCA entry in delayed heavily T2-weighted 3D-real inversion recovery (IR) MRI scans. This study systematically investigated GBCA distribution in the endolymph using this 3D-real IR sequence. METHODS: Forty-one patients suspected of endolymphatic hydrops (EHs) underwent pre-contrast, 4-h, and 24-h post-contrast 3D-real IR imaging. Signal intensity in cerebrospinal fluid (CSF), perilymph, and endolymph was measured and analyzed for temporal dynamics of GBCA uptake, correlations between compartments, and the influence of age and presence of EH. RESULTS: Endolymph showed a delayed peak GBCA uptake at 24h, contrasting with peaks in perilymph and CSF at 4h. Weak to moderate positive correlations between endolymph and CSF contrast effect were observed at both 4 (r = 0.483) and 24h (r = 0.585), suggesting possible inter-compartmental interactions. Neither the presence of EH nor age significantly influenced endolymph enhancement. However, both perilymph and CSF contrast effects significantly correlated with age at both time points. CONCLUSION: This study provides the first in vivo systematic confirmation of GBCA entering the endolymph following intravenous administration. Notably, endolymph uptake peaked at 24h, significantly later than perilymph and CSF. The lack of a link between endolymph contrast and both perilymph and age suggests distinct uptake mechanisms. These findings shed light on inner ear fluid dynamics and their potential implications in Ménière's disease and other inner ear disorders.

A novel radiomics nomogram based on T2-sampling perfection with application-optimized contrasts using different flip-angle evolutions (SPACE) images for predicting cochlear and vestibular endolymphatic hydrops in Meniere's disease patients.

OBJECTIVES: To construct and validate a radiomics nomogram based on T2-sampling perfection with application-optimized contrasts using different flip-angle evolutions (SPACE) images for predicting cochlear and vestibular endolymphatic hydrops (EH) in Meniere's disease patients. METHODS: A total of 156 patients (312 affected ears) with bilateral definite Meniere's disease who underwent delayed enhancement MRI scans were enrolled in this study. All ears of the patients were divided into a training set (n = 218) and an internal validation set (n = 94). A radiomics nomogram was constructed from radiomics features extracted from the T2-SPACE images, and a radiomics score was calculated. Performance of the radiomics nomogram was assessed using receiver operating characteristics analysis. RESULTS: Five features were selected for the construction of the cochlear radiomics nomogram, and seven features for the vestibular radiomics nomogram. The radiomics nomograms exhibited robust performance in differentiating between EH-positive and EH-negative statuses in both training and validation cohorts, with the area under the receiver operating characteristics curve values for cochlear and vestibular radiomic nomograms being 0.703 and 0.728 in the training set, and 0.718 and 0.701 in the validation set, respectively. CONCLUSION: The novel radiomics nomograms based on T2-SPACE images were successfully constructed to predict cochlear and vestibular EH in Meniere's disease. The models showed a solid and superior performance and may play an important role in the EH prediction. CLINICAL RELEVANCE STATEMENT: We constructed a novel radiomics nomogram, which can be a very useful tool for predicting cochlear and vestibular endolymphatic hydrops in Meniere's disease patients. KEY POINTS: • This is the first T2-SPACE-based nomogram to predict cochlear and vestibular endolymphatic hydrops. • The nomogram is of great value to patients who are unable to undergo delayed enhancement MRI scans.

Clinical significance of posterior expansion of the utricular endolymphatic space.

BACKGROUND: Posterior expansion of the utricular endolymphatic space (ES) is a finding occasionally observed, and often occurs in bilateral ears. The clinical significance of posterior expansion of the utricular ES is not clear. OBJECTIVES: To investigate the clinical significance of posterior expansion of the utricular ES detected on magnetic resonance imaging (MRI). MATERIALS AND METHODS: Among 957 patients who underwent contrast-enhanced MRI to investigate the presence of endolymphatic hydrops (EH), clinical findings in cases with posterior expansion of the utricular ES were evaluated and the significance of their existence is considered. RESULTS: Posterior expansion of the utricular ES was detected in 30 ears from 20 cases (7 males, 13 females; mean age 48.7 years). The ears with posterior expansion had a significantly lower incidence of vestibular EH than those without, and this tendency was more prominent in cases in bilateral ears. Some cases with posterior expansion had vestibular symptoms and were diagnosed with Ménière's disease, although EH was not detected in their vestibules. CONCLUSION AND SIGNIFICANCE: Posterior expansion of the utricular ES was associated with Ménière's disease without vestibular EH. Posterior expansion of the utricular ES might not be associated with the formation of EH but may occur independently.

Correlation of endolymphatic hydrops and perilymphatic enhancement with the clinical features of Ménière's disease.

OBJECTIVES: To use three-dimensional real inversion recovery (3D-real IR) MRI to investigate correlations between endolymphatic hydrops (EH) grades or the degree of perilymphatic enhancement (PE) and clinical features of Ménière's disease (MD), as previous findings have been inconsistent. METHODS: A total of 273 consecutive patients with definite unilateral MD were retrospectively enrolled from September 2020 to October 2021. All patients underwent 3D-real IR and 3D-T2WI 6 h after intravenous gadolinium injection. MD-related symptom duration and vertigo frequency were recorded. EH grades were evaluated, the signal intensity ratio (SIR) was measured, and correlations between clinical features and EH, PE were assessed respectively. RESULTS: The study included 123 males and 150 females, with a mean age of 53.0 years. A longer duration of vertigo was associated with higher cochlear EH grades, whereas the opposite was true for the duration of aural fullness. A longer time since vertigo onset was associated with higher vestibular EH grades; the opposite was true for the duration of individual vertigo attacks. The multiple regression analysis revealed that age, tinnitus duration, and vestibular EH were risk factors for SIR. Furthermore, the low-frequency hearing threshold (HT) was a risk factor for cochlear and vestibular EH, and the SIR. CONCLUSION: The EH grade and SIR (an indicator for the quantitative evaluation of PE) were correlated with clinical features and HT of MD; thus, imaging can be a valuable tool in planning individualised treatment. CLINICAL RELEVANCE STATEMENT: This study revealed that the grade of endolymphatic hydrops and degree of perilymphatic enhancement positively correlates with the length of time since onset of clinical symptoms and hearing thresholds in patients with Ménière's disease, facilitating the tailored treatment. KEY POINTS: • Relationships between 3-dimensional real inversion recovery features and clinical symptoms in Ménière's disease are unknown. • Symptom duration and hearing thresholds correlated with endolymphatic hydrops grades and degree of perilymphatic enhancement. • MRI features correlate with MD severity; thus, imaging is valuable for planning tailored treatment.

Assessing the optimal MRI descriptors to diagnose Ménière's disease and the added value of analysing the vestibular aqueduct.

OBJECTIVES: To evaluate the diagnostic performance and reliability of MRI descriptors used for the detection of Ménière's disease (MD) on delayed post-gadolinium MRI. To determine which combination of descriptors should be optimally applied and whether analysis of the vestibular aqueduct (VA) contributes to the diagnosis. MATERIALS AND METHODS: This retrospective single centre case-control study evaluated delayed post-gadolinium MRI of patients with Ménièriform symptoms examined consecutively between Dec 2017 and March 2023. Two observers evaluated 17 MRI descriptors of MD and quantified perilymphatic enhancement (PLE) in the cochlea. Definite MD ears according to the 2015 Barany Society criteria were compared to control ears. Cohen's kappa and diagnostic odds ratio (DORs) were calculated for each descriptor. Forward stepwise logistic regression determined which combination of MRI descriptors would best predict MD ears, and the area under the receiver operating characteristic curve for this model was measured. RESULTS: A total of 227 patients (mean age 48.3 ± 14.6, 99 men) with 96 definite MD and 78 control ears were evaluated. The presence of saccular abnormality (absent, as large as or confluent with the utricle) performed best with a DOR of 292.6 (95% confidence interval (CI), 38.305-2235.058). All VA descriptors demonstrated excellent reliability and with DORs of 7.761 (95% CI, 3.517-17.125) to 18.1 (95% CI, 8.445-39.170). Combining these saccular abnormalities with asymmetric cochlear PLE and an incompletely visualised VA correctly classified 90.2% of cases (sensitivity 84.4%, specificity 97.4%, AUC 0.938). CONCLUSION: Either absent, enlarged or confluent saccules are the best predictors of MD. Incomplete visualisation of the VA adds value to the diagnosis. CLINICAL RELEVANCE STATEMENT: A number of different MRI descriptors have been proposed for the diagnosis of Ménière's disease, but by establishing the optimally performing MRI features and highlighting new useful descriptors, there is an opportunity to improve the diagnostic performance of Ménière's disease imaging. KEY POINTS: • A comprehensive range of existing and novel vestibular aqueduct delayed post-gadolinium MRI descriptors were compared for their diagnostic performance in Ménière's disease. • Saccular abnormality (absent, confluent with or larger than the utricle) is a reliable descriptor and is the optimal individual MRI predictor of Ménière's disease. • The presence of this saccule descriptor or asymmetric perilymphatic enhancement and incomplete vestibular aqueduct visualisation will optimise the MRI diagnosis of Ménière's disease.

Association Between Vestibular Aqueduct Morphology and Meniere's Disease.

OBJECTIVE: To investigate the relationship between vestibular aqueduct (VA) morphology and Meniere's disease (MD) using ultrahigh-resolution computed tomography (U-HRCT). METHODS: Retrospective data were collected from 34 patients (40 ears) diagnosed with MD in our hospital who underwent temporal bone U-HRCT with isotropic 0.05-mm resolution, magnetic resonance with gadolinium-enhanced, and pure-tone audiometry; 34 age- and sex-matched controls (68 ears) who underwent U-HRCT were also included. VA patency was qualitatively classified as locally not shown (grade 1), locally faintly shown (grade 2), or clearly shown throughout (grade 3). The width of the outer orifice and VA length and angle were quantitatively measured. Differences in VA morphology between the MD and control groups were analyzed. The correlations between VA morphology and the degrees of hearing loss and endolymphatic hydrops (EH) were also analyzed. RESULTS: VA was classified as grades 1-3 in 11, 17, and 12 ears in the MD group and 5, 26, and 37 ears in the control group, respectively. The patency differed significantly between the groups (p < 0.01). The width of the outer orifice and length of VA were significantly smaller in the MD group than those in the control group (p < 0.05). Both VA patency and length were correlated with the degree of EH in the cochlea and the vestibule (p < 0.05). No difference was found between VA morphology and the degree of hearing loss (p > 0.05). CONCLUSION: The morphological characteristics of VA were found to be associated with the occurrence of MD and the degree of EH. LEVEL OF EVIDENCE: 4 Laryngoscope, 134:3349-3354, 2024.

Improvement of vertigo symptoms and acoustic power absorbance in cases with endolymphatic hydrops.

Objective: The pathophysiology and symptoms underlying Meniere's disease (MD) manifest as endolymphatic hydrops (EH), potentially impacting acoustic power absorbance in vestibular EH. The longitudinal effects of middle ear pressure therapy (MEPT) and conservative therapies for EH by magnetic resonance imaging (MRI) and on acoustic power absorbance on wideband acoustic immittance (WAI) were evaluated, and their changes were compared with clinical symptoms. Methods: Eleven patients with definite MD or delayed endolymphatic hydrops (DEH), resistant to conservative therapies and who continued MEPT for 1 year, were included. Vertigo scores, hearing levels, acoustic power absorbance on WAI, and degrees of EH on 3-T MRI were evaluated and compared before and after the treatments. Results: One year after the start of MEPT, all cases showed symptomatic improvement in vertigo score; however, the degrees of EH showed no improvements except in one case. In the affected ears with EH, their absorbances on WAI improved, particularly at 1580-1905 or 2400-2953 Hz (p < .05). Conclusion: Alleviation of vestibular symptoms with the therapy of MD was not necessarily associated with improved EH. Vestibular symptoms could be related to the change in the impedance of inner ear pressure, which was proven by the normalization of acoustic power absorbance. Assessments of acoustic power absorbance may provide useful information for physiological conditions and causative factors of vertigo in ears with EH. Level of evidence: 4.

Role of conventional MD staging in modern era of hydrops MR imaging.

Objective/Hypothesis: This study correlated stage of Meniere's disease (MD) with MR imaging of endolymphatic hydrops (EH) to assess the role of MD staging in modern era. Study Design: Retrospective study. Methods: Fifty-four MD patients (60 ears) underwent an inner ear test battery and were further confirmed by MR imaging. Sixty MD ears were divided into stages I-IV, and hydrops MR images at each stage were compared. Results: Hydrops MRI demonstrated that EH at the cochlea with respective Grades 0/I/II were 3/7/1 ears for stage I, 0/5/3 ears for stage II, 1/6/26 ears for stage III and 0/2/6 ears for stage IV. Significant relationship was not identified between MD stage and grades of cochlear hydrops. Similarly, no significant relationship was shown between MD stage and grades of vestibular (saccular/utricular) hydrops. The optimal cutoff value of four-tone average for predicting severe type (Grade II-III) cochlear/vestibular EH was 48 dB, which was within the stage III. Hence, prevalence of severe type (Grade II) cochlear EH in stages III (79%) and IV (75%) was significantly higher than stages I (9%) and II (38%). Similarly, severe type saccular/utricular EH in stages III (64%) and IV (75%) also showed significantly higher than stages I (18%) and II (25%). Conclusion: Although conventional MD staging fails to correlate with the grades of EH on hydrops MRI, late-stage MD may indicate heightened EH severity in the cochlea and vestibule. Level of Evidence: 4.