Purpose
There is still a clinical-radiologic discrepancy in patients with Menière’s disease (MD). Therefore, the purpose of this study was to investigate the reliability of current MRI endolymphatic ...hydrops (EH) criteria according to Baráth in a larger study population and the clinical utility of new imaging signs such as a supplementary fourth low-grade vestibular EH and the degree of perilymphatic enhancement (PE) in patients with Menière’s disease (MD).
Methods
This retrospective study included 148 patients with probable or definite MD according to the 2015 American Academy of Otolaryngology, Head and Neck Surgery criteria who underwent a 4-h delayed intravenous Gd-enhanced 3D-FLAIR MRI between January 2015 and December 2016. Vestibular EH, vestibular PE, cochlear EH, and cochlear PE were reviewed twice by three experienced readers. Cohen’s Kappa and multivariate logistic regression were used for analysis.
Results
The intra- and inter-reader reliability for the grading of vestibular-cochlear EH and PE was excellent (0.7 < kappa < 0.9). The two most distinctive characteristics to identify MD are cochlear PE and vestibular EH which combined gave a sensitivity and specificity of 79.5 and 93.6%. By addition of a lower grade vestibular EH, the sensitivity improved to 84.6% without losing specificity (92.3%). Cochlear EH nor vestibular PE showed added-value.
Conclusions
MRI using vestibular-cochlear EH and PE grading system is a reliable technique. A four-stage vestibular EH grading system in combination with cochlear PE assessment gives the best diagnostic accuracy to detect MD.
Endolymphatic hydrops (ELH), which is a pathological feature of Meniere's disease (MD), is characterized by an extended endolymphatic space (ELS).
We aimed to perform a quantitative volumetric ...analysis of inner-ear ELH in patients with unilateral MD (uMD).
This study included 97 patients with definite uMD and 49 control subjects (CS). The ELS/total fluid space (TFS) volume ratio (%) and the distribution rate of the inner-ear components in the ELS (%) were measured using 3D magnetic resonance imaging and compared between patients with uMD and CS.
Compared to CS, patients with uMD had significantly higher mean ELS/TFS volume ratios for inner-ear components. The mean distribution rate of the inner-ear components in the ELS was not significantly different between the two groups.
In patients with uMD, ELH was noted to be spread throughout the entire inner ear and the endolymph was evenly distributed in the total ELS. These findings should be useful as a standard reference for further research.
Celotno besedilo
Dostopno za:
DOBA, IJS, IZUM, KILJ, NUK, OILJ, PILJ, PNG, SAZU, UILJ, UKNU, UL, UM, UPUK, VSZLJ
Objective
We investigated correlations among clinical features, degree of inner ear endolymphatic hydrops (EH), and hippocampal volume (HV) in different stages of Meniere's disease (MD).
Methods
From ...February 2021 to April 2022, clinical data were collected from 99 patients (39 males, 60 females, mean age: 50.4 ± 10.0 range: 26–69 years) with unilateral MD admitted to the Department of Vertigo Disease of Shandong ENT Hospital. The left and right ears were affected in 64 and 35 patients, respectively. There were 50 and 49 cases in early (Stages 1, 2) and late stages (Stages 3, 4), respectively. Fifty healthy participants were included as controls. Audiovestibular function test results, EH grading using gadolinium‐enhanced magnetic resonance imaging (MRI), and HV determined on MRI were analyzed for patients at different stages of MD.
Results
Between‐group comparisons of early and late MD revealed significant differences in the disease course, vestibular function (VF), degree of EH, and HV. There were no significant between‐group differences based on age, sex, affected side, subjective degree of dizziness, hospital anxiety, or depression. Mean HV in patients with early‐stage MD was correlated with the canal paresis value of the caloric test and pure tone hearing threshold, HV in late‐stage patients was correlated with vestibular EH.
Conclusion
Patients with late‐stage MD exhibited severe auditory and VF impairments, increased EH, and atrophy of the HV. More advanced disease was associated with greater vestibular damage and degree of EH.
Level of Evidence
3 Laryngoscope, 134:410–418, 2024
This cross‐sectional study investigated the correlations among clinical features, degree of inner ear hydrops, and hippocampal volume in patients with different stages of Meniere's disease (MD) at a vertigo center. We found that the left and right hippocampal volumes of patients with MD were smaller than those of the control group, suggesting that the reduction in hippocampal volume was related to MD. Patients with late‐stage MD exhibited severe impairment of auditory and vestibular functions, increased endolymphatic hydrops, and atrophy of the hippocampal volume. More advanced disease was associated with greater vestibular damage and degree of endolymphatic hydrops.
Meniere’s disease (MD), a syndromal inner ear disease, is commonly associated with a pathological accumulation of endolymphatic fluid in the inner ear, termed “idiopathic” endolymphatic hydrops ...(iEH). Although numerous precipitating/exacerbating factors have been proposed for MD, its etiology remains elusive. Here, using immunohistochemistry and in situ protein–protein interaction detection assays, we demonstrate mineralocorticoid-controlled sodium transport mechanisms in the epithelium of the extraosseous portion of the endolymphatic sac (eES) in the murine and human inner ears. Histological analysis of the eES in an extensive series of human temporal bones consistently revealed pathological changes in the eES in cases with iEH and a clinical history of MD, but no such changes were found in cases with “secondary” EH due to other otological diseases or in healthy controls. Notably, two etiologically different pathologies—degeneration and developmental hypoplasia—that selectively affect the eES in MD were distinguished. Clinical records from MD cases with degenerative and hypoplastic eES pathology revealed distinct intergroup differences in clinical disease presentation. Overall, we have identified for the first time two inner ear pathologies that are consistently present in MD and can be directly linked to the pathogenesis of EH, and which potentially affect the phenotypical presentation of MD.
To explore the differences between endolymphatic duct blockage, endolymphatic sac drainage and endolymphatic sac decompression surgery in the reversal of endolymphatic hydrops (EH) in patients with ...intractable Meniere's disease (MD).
A total of 27 MD patients receiving endolymphatic duct blockage surgery (n = 10), endolymphatic sac drainage surgery (n = 9) and endolymphatic sac decompression surgery (n = 8) underwent gadolinium-enhanced inner ear magnetic resonance imaging (MRI) scans prior to, 2 weeks after and at > 12 months following surgery.
In the group with endolymphatic duct blockage, the second MRI revealed no changes in EH, whereas the third MRI revealed a reversal of vestibular EH in 3 patients and a downgrading of cochlear hydrops in 2 of these 3 patients, who presented with an improvement in their hearing and complete control of vertigo. In the group with endolymphatic sac drainage, the second MRI showed a reversal of EH in 4 patients, and no changes in EH in the remaining 5 patients, whereas the third MRI showed that those 4 patients who presented with a reversal of EH at the second MRI stage remained unchanged except a recurrence of vestibular hydrops in 1 patient. All 4 patients exhibited a complete control of vertigo, but hearing improved in 1, worsened in 1 and remained unchanged in 2. In the group with endolymphatic sac decompression, both the second and third MRI examination revealed no reversal of EH.
The present study has shown that both endolymphatic duct blockage surgery and endolymphatic sac drainage surgery have the potential to reduce EH in certain MD patients, but none of the patients receiving endolymphatic sac decompression surgery showed reversal of their EH.
Meniere's disease is a poorly understood, disabling syndrome causing spells of vertigo, hearing fluctuation, tinnitus, and aural fullness. In this paper, we present a review of the histopathology, ...cytochemistry, and imaging of Meniere's disease. Histopathology is significant for neuroepithelial damage with hair cell loss, basement membrane thickening, and perivascular microvascular damage. Cytochemical alterations are significant for altered AQP4 and AQP6 expression in the supporting cell, and altered cochlin and mitochondrial protein expression. Current developments include imaging techniques to determine the degree and presence of endolymphatic hydrops, and future studies will endeavor to correlate the observance of hydrops with clinical findings.
Meniere disease (MD) is a rare disorder of the inner ear defined by sensorineural hearing loss (SNHL) associated with episodes of vertigo and tinnitus. The phenotype is variable, and it may be ...associated with other comorbidities such as migraine, respiratory allergies, and several autoimmune disorders. The condition has a significant heritability according to epidemiological and familial segregation studies. Familial MD is found in 10% of cases, the most frequently found genes being
OTOG
,
MYO7A
, and
TECTA
, previously associated with autosomal dominant and recessive non-syndromic SNHL. These findings suggest a new hypothesis where proteins involved in the extracellular structures in the apical surface of sensory epithelia (otolithic and tectorial membranes) and proteins in the stereocilia links would be key elements in the pathophysiology of MD. The ionic homeostasis of the otolithic and tectorial membranes could be critical to suppress the innate motility of individual hair cell bundles. Initially, focal detachment of these extracellular membranes may cause random depolarization of hair cells and will explain changes in tinnitus loudness or trigger vertigo attacks in early stages of MD. With the progression of the disease, a larger detachment will lead to an otolithic membrane herniation into the horizontal semicircular canal with dissociation in caloric and head impulse responses. Familial MD shows different types of inheritance, including autosomal dominant and compound recessive patterns and implementation of genetic testing will improve our understanding of the genetic structure of MD.
Emerging evidence suggests substantial overlap between the symptoms of Meniere's disease (MD) and migraine-related cochlear/vestibular disorders. We report a 5-year-old girl with a 6-month history of ...left-sided hearing loss followed by daily episodes of vertigo, headache, and vomiting who met the criteria for definite MD. The patient became symptom-free and gained near normal hearing levels after starting on a 6-week migraine diet/lifestyle regimen with riboflavin and magnesium. We believe that the symptoms of MD may be primarily due to a vestibular migraine phenomenon. Pediatric MD patients may benefit from migraine lifestyle/dietary changes with control of both cochlear and vestibular symptoms.
Objective/Hypothesis
This study aimed at investigating the correlation between a battery of diagnostic symptoms of definite Meniere's disease (MD) and the degree of endolymphatic hydrops (EH) in the ...inner ear.
Study Design
Prospective study.
Methods
Fifty‐four patients diagnosed with unilateral definite MD were enrolled in the study. The hearing levels of all patients at the low, middle, and high frequencies were evaluated. Three‐dimensional fluid‐attenuated inversion recovery (3D‐FLAIR) and three‐dimensional real inversion recovery (3D‐real IR) magnetic resonance imaging (MRI) were performed 24 hours after bilateral intratympanic injection of gadolinium to assess the presence and grading of EH.
Results
Various degrees of EH were observed in the vestibule and/or each turn of the cochlea in the affected ears of all patients. The duration of MD disease and low‐tone and middle‐tone hearing thresholds were proportional to the extent of EH in the vestibule and cochlear. However, no significant correlation was demonstrated between EH and other aspects of symptoms such as high‐tone hearing loss, tinnitus, and aural fullness. Of all subjects, 16.7% exhibited bilateral EH on MRI exam who were diagnosed with unilateral MD based on diagnostic criteria.
Conclusions
EH in the inner ear of MD patients exhibits a progressive deteriorative trend over time. Low‐tone and middle‐tone hearing thresholds can indirectly reflect the severity of EH in the cochlea. EH may not be the primary cause of tinnitus and aural fullness in patients with MD. Patients diagnosed with unilateral MD should maintain long‐term regular follow‐ups for the possibility of developing bilateral EH.
Level of Evidence
4 Laryngoscope, 126:974–979, 2016
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