scholarly journals Validation of inner ear MRI in patients with Ménière’s disease by comparing endolymphatic hydrops from histopathologic specimens

2021 ◽  
Vol 11 (1) ◽  
Author(s):  
Young Sang Cho ◽  
Jong Sei Kim ◽  
Min Bum Kim ◽  
Sung Min Koh ◽  
Chang Hee Lee ◽  
...  
Keyword(s):  
Inner Ear ◽  
Temporal Bone ◽  
Semicircular Canal ◽  
Endolymphatic Hydrops ◽  
Semicircular Canals ◽  
Meniere’S Disease ◽  
Ménière’S Disease ◽  
Affected Side ◽  
Mri Scans ◽  
Temporal Bones

AbstractIntravenous gadolinium-enhanced inner-ear magnetic resonance imaging (IV-Gd inner-ear MRI) has been used to visualize endolymphatic hydrops (EH) in clinical diagnosis of Ménière’s disease (MD). However, lack of histological validation has led to several concerns regarding how best to interpret the resulting images. Here, we compared hydropic changes in temporal bone specimens with the results of IV-Gd inner-ear MRI in patients with MD. Histopathologic images of temporal bones from 37 patients with MD and 10 healthy controls were collected from the National Temporal Bone Bank of the Massachusetts Eye and Ear Infirmary in the United States. The EH ratios in the vestibule and cochlea were calculated from temporal bones using the methods used for IV-Gd inner-ear MRI, and the degree to which the saccular and utricular hydrops contributed to vestibular hydrops was measured. The presence of hydropic change in each semicircular canal was assessed using temporal bone images and compared with IV-Gd inner-ear MRI scans of 74 patients with MD. Based on human temporal bone imagery, the EH ratios in the cochlea and the vestibule on the affected side were 0.314 and 0.757, respectively. In the healthy control group, the ratio was 0.064 for the cochlea and 0.289 for the vestibule; these values were significantly different from those for the affected side of MD patients. The values for the affected ear were similar to the ratios from the IV-Gd inner-ear MRI scans in MD patients. In the vestibule, saccular hydrops were more common than utricular hydrops. The average EH ratios in the saccule and utricle were 0.513 and 0.242, respectively. No significant hydropic change from each of three semicircular canals was evident in temporal bone histopathology. However, herniation of otolithic organs (saccule or utricle) into the lateral semicircular canal was found in 44.4% of the patients, with saccular herniation (24.8%) more common than utricular herniation (16.7%). Although IV-Gd inner-ear MRI might not reflect fully the results of actual histopathology due to the limited resolution of MRI and image-processing techniques, the measured EH ratios from temporal bone specimens and IV-Gd inner-ear MRI scans were similar. Hydropic change in the three semicircular canals was not significant at either the ampullated or nonampullated end. Canal invasion of vestibular hydrops seen on MRI also appeared in temporal bone histopathology, and saccular invasion was dominant.

scholarly journals Endolymphatic Hydrops in Patients With Vestibular Migraine and Concurrent Meniere's Disease

2021 ◽  
Vol 12 ◽  
Author(s):  
Sun-Young Oh ◽  
Marianne Dieterich ◽  
Bit Na Lee ◽  
Rainer Boegle ◽  
Jin-Ju Kang ◽  
...  
Keyword(s):  
Inner Ear ◽  
Endolymphatic Hydrops ◽  
Meniere’S Disease ◽  
Vestibular Migraine ◽  
Menière’S Disease ◽  
Meniere's Disease ◽  
Ménière’S Disease ◽  
Menière's Disease ◽  
Volumetric Quantification ◽  
Ménière's Disease

Objective: Intravenous contrast agent enhanced, high-resolution magnetic resonance imaging of the inner ear (iMRI) confirmed that patients with Menière's disease (MD) and vestibular migraine (VM) could present with endolymphatic hydrops (EH). The present study aimed to investigate EH characteristics and their interrelation to neurotologic testing in patients with VM, MD, or VM with concurrent MD (VM-MD).Methods: Sixty–two patients (45 females, aged 23–81 years) with definite or probable VM (n = 25, 19 definite), MD (n = 29, 17 definite), or showing characteristics of both diseases (n = 8) were included in this study. Diagnostic workup included neurotologic assessments including video-oculography (VOG) during caloric stimulation and head-impulse test (HIT), ocular and cervical vestibular evoked myogenic potentials (o/cVEMP), pure tone audiometry (PTA), as well as iMRI. EH's degree was assessed visually and via volumetric quantification using a probabilistic atlas-based segmentation of the bony labyrinth and volumetric local thresholding (VOLT).Results: Although a relevant number of VM patients reported varying auditory symptoms (13 of 25, 52.0%), EH in VM was only observed twice. In contrast, EH in VM-MD was prevalent (2/8, 25%) and in MD frequent [23/29, 79.3%; χ2(2) = 29.1, p < 0.001, φ = 0.7]. Location and laterality of EH and neurophysiological testing classifications were highly associated (Fisher exact test, p < 0.005). In MD, visual semi-quantitative grading and volumetric quantification correlated highly to each other (rS = 0.8, p < 0.005, two-sided) and to side differences in VOG during caloric irrigation (vestibular EH ipsilateral: rS = 0.6, p < 0.05, two-sided). In VM, correlations were less pronounced. VM-MD assumed an intermediate position between VM and MD.Conclusion: Cochlear and vestibular hydrops can occur in MD and VM patients with auditory symptoms; this suggests inner ear damage irrespective of the diagnosis of MD or VM. The EH grades often correlated with auditory symptoms such as hearing impairment and tinnitus. Further research is required to uncover whether migraine is one causative factor of EH or whether EH in VM patients with auditory symptoms suggests an additional pathology due to MD.

Magnetic Resonance Imaging of Ménière’s Disease After Intravenous Administration of Gadolinium

2018 ◽  
Vol 127 (11) ◽  
pp. 777-782 ◽  
Author(s):  
Suming Shi ◽  
Ping Guo ◽  
Wuqing Wang
Keyword(s):  
Magnetic Resonance Imaging ◽  
Magnetic Resonance ◽  
Endolymphatic Hydrops ◽  
Meniere’S Disease ◽  
Meniere's Disease ◽  
Ménière’S Disease ◽  
Resonance Imaging ◽  
Menière's Disease ◽  
Affected Side ◽  
Ménière's Disease

Objectives: A three-dimensional (3D) inversion-recovery (IR) sequence with real reconstruction (3D-real IR) sequence 4 hours after intravenous (IV) gadolinium injection has been used to visualize the endolymphatic hydrops (ELH) in Ménière’s disease (MD). This study was designed to investigate the ELH characteristics in clinically diagnosed definite MD and to explore the pathology of MD with magnetic resonance imaging (MRI). Methods: One hundred fifty-four patients with definite MD were included in this study. All patients underwent the IV method. The grades of ELH were analyzed on each image, regions of interest of the cochlear perilymph and the cerebellum white matter were determined, and the signal intensity ratio of the former to the latter (CC ratio) on both sides in patients with unilateral MD was subsequently evaluated. Results: Endolymphatic hydrops was observed in 148 patients (96.1%) on the clinically affected side; the remaining 6 patients (3.9%) had no apparent ELH. One hundred fifteen patients (74.7%) had unilateral ELH, and 33 patients (21.4%) had bilateral ELH. Eighteen patients (11.7%) had ELH on the clinically silent side. Patients with moderate and significant grades of ELH in the vestibule and cochlea accounted for 88.3% and 90.3%, respectively. The CC ratio of the affected side (1.39 ± 0.37) was higher than that of the unaffected side (1.18 ± 0.29) ( P < .01) in 115 patients with unilateral MD. Conclusions: Moderate and significant grades of ELH are common in MD; however, the proposed diagnostic criteria are not fully consistent with ELH. The elevated contrast effect in the affected side in patients with unilateral MD may better reflect the pathologic condition of MD.

scholarly journals Menière's Disease and Disorders of the Carbohydrate Metabolism Involving the Inner Ear

2019 ◽  
Vol 23 (02) ◽  
pp. 218-220
Author(s):  
Pedro L. Mangabeira Albernaz
Keyword(s):  
Inner Ear ◽  
Carbohydrate Metabolism ◽  
Endolymphatic Hydrops ◽  
Meniere’S Disease ◽  
Menière’S Disease ◽  
Meniere's Disease ◽  
Ménière’S Disease ◽  
Menière's Disease ◽  
Delayed Endolymphatic Hydrops ◽  
Ménière's Disease

Introduction Menière’s disease was described in 1861, but there are still uncertainties regarding its pathophysiology and treatment. Endolymphatic hydrops is recognized as a fundamental pathological characteristic of the disease, as a result of an inadequate absorption of the endolymph. A milder type of endolymphatic hydrops results from an altered chemical composition of the endolymph, due to disorders of the carbohydrate metabolism. Objective To describe the association of both types of hydrops in patients with Menière disease. Methods This was a retrospective study of 98 patients with Menière’s disease, 62 of whom also presented disorders of the carbohydrate metabolism, and 5 patients with delayed endolymphatic hydrops, 2 of whom also presented disorders of the carbohydrate metabolism. Results The follow-up of these patients showed that the correction of the metabolic disorders may help in the clinical treatment of Menière’s disease and of delayed endolymphatic hydrops, but this does not happen in the more severe types of the diseases. Conclusion Patients with Menière’s disease may present simultaneous disorders of the carbohydrate metabolism, affecting the inner ear. The correction of these disorders helps the clinical treatment but does not preclude the progression of the more severe cases of Menière disease.

Meniére's Disease and Endolymphatic Hydrops Clinical-Histopathological Correlations

1980 ◽  
Vol 89 (6_suppl2) ◽  
pp. 2-22 ◽  
Author(s):  
Bernard G. Fraysse ◽  
Antonio Alonso ◽  
William F. House
Keyword(s):  
Histopathological Examination ◽  
Endolymphatic Hydrops ◽  
Meniere’S Disease ◽  
Menière’S Disease ◽  
Meniere's Disease ◽  
Ménière’S Disease ◽  
Menière's Disease ◽  
Histopathological Findings ◽  
Ménière's Disease ◽  
Temporal Bones

The clinical-histopathological correlation between Menière's disease and endolymphatic hydrops was done to explain, as much as possible, causes of symptoms of Menière's disease. Twenty-three temporal bones with endolymphatic hydrops from 17 patients were reviewed and clinical and histopathological findings were correlated. Histopathological examination revealed frequent, severe deformities in the labyrinthine walls and permanent changes in Reissner's membranes. Evidence of rupture was difficult to assess. Of 21 ears of patients with the clinical diagnosis of Menière's disease, 93% had endolymphatic hydrops. A statistical correlation between increased area of the cochlear duct and hearing loss was found. Some correlation was also found between frequency of vertigo and results of electronystagmography with histopathological findings. Consequently, the mechanical effect of endolymphatic hydrops seems to have greater significance in the production of symptoms of Menière's disease than the biochemical effect of ruptures. In a review of ten unusual cases from this series of 17 patients, traumatic neuromas or remnants of vestibular structures were found after an incomplete labyrinthectomy. Histopathological findings of four patients who had undergone endolymphatic subarachnoid shunt surgery are described.

scholarly journals MENIERE’S DISEASE

2021 ◽  
Vol 2 (1) ◽  
pp. 18-21
Author(s):  
Helena Era Millennie ◽  
Badrul Munir ◽  
Zamroni Afif ◽  
Ria Damayanti ◽  
Shahdevi Nandar Kurniawan
Keyword(s):  
Inner Ear ◽  
Endolymphatic Hydrops ◽  
Meniere’S Disease ◽  
Menière’S Disease ◽  
Vestibular Evoked Myogenic Potentials ◽  
Meniere's Disease ◽  
Ménière’S Disease ◽  
Menière's Disease ◽  
Episodic Vertigo ◽  
Ménière's Disease

Meniere’s disease is a disorder of the inner ear resulting in symptoms of episodic vertigo, tinnitus, hearing loss and aural pressure. Although the exact etiology is uncertain, it is associated with raised pressure in the endolymph of the inner ear (endolymphatic hydrops). The diagnosis of Meniere's disease is based on the clinical setting of the patient. This disease usually presents with unilateral ear symptoms but can be also bilateral. Meniere's disease attacks are usually random and episodic (approximately 6-11 per year), with periods of remission that can last from months to years. Investigations are audiometry, electronystagmogram, vestibular evoked myogenic potentials (VEMPs) and imaging.The management consist pharmacological and non pharmacological. Meniere's disease is initially progressive but fluctuates unexpectedly. It is difficult to distinguish natural resolutions from treatment effects.

Fully automated analytic system for measuring endolymphatic hydrops ratio in Ménière's disease using deep learning and MRI (Preprint)

2021 ◽  
Author(s):  
Chae Jung Park ◽  
Young Sang Cho ◽  
Myung Jin Chung ◽  
Yi-Kyung Kim ◽  
Hyung-Jin Kim ◽  
...  
Keyword(s):  
Inner Ear ◽  
Endolymphatic Hydrops ◽  
Meniere’S Disease ◽  
Menière’S Disease ◽  
Meniere's Disease ◽  
Ménière’S Disease ◽  
Mr Images ◽  
Menière's Disease ◽  
Ménière's Disease ◽  
Analysis System

BACKGROUND Recently, analysis of endolymphatic hydrops (EH) using inner ear magnetic resonance imaging (MRI) in Ménière's disease (MD) has been attempted in various studies. In addition, artificial intelligence (AI) has rapidly been incorporated into the medical field. In our previous study, the automated analysis algorithm of EH was completed using the convolutional neural network (CNN). However, several limitations existed, and further studies were conducted to compensate for these limitations. OBJECTIVE The aim of this study is to develop a fully automated analytic system for measuring endolymphatic hydrops, which provides enhanced analysis accuracy and clinical usability in studying Ménière's disease with MRI. METHODS We propose 3into3Inception and 3intoUNet, whose network architectures are based on Inception-v3 and U-Net, respectively. The developed networks were trained for inner ear segmentation using magnetic resonance (MR) images of 124 people and were embedded in a new automated EH analysis system, INner ear Hydrops Estimation via ARtificial InTelligence - version 2 (INHEARIT-v2). After 5-fold cross-validation, an additional test was performed using 60 new unseen MR images to evaluate the performance of our system. INHEARIT-v2 has a new functionality to automatically select representative images from a full MR stack. RESULTS The average segmentation performances of 5-fold cross-validation were measured by the intersection of union, which showed 0.743 ± 0.030 for 3into3Inception and 0.781 ± 0.030 for 3intoUNet. The automatic representative slice selection results of the INHEARIT-v2 differed only within two slices from the expert selection on an unseen dataset. Compared with the ratio measured by experienced physicians, the average interclass correlation coefficient (ICC) for all cases was 0.941; the average ICC of the vestibules was 0.968, and that of cochleae was 0.914. The time required for the fully automated system to accurately analyze the EH ratio in one patient's MRI stack was approximately 3.5 seconds. CONCLUSIONS In this study, a fully automated full-stack MR analysis system of the EH ratio was developed, named INHEARIT-v2, which showed high agreement with experts in an additional test. The system is an upgraded version of INHEARIT and provides higher segmentation performance and includes automatic representative image selection in the MR stack. The new model can help clinicians by providing an objective analysis result and reduce their workload in reading MRIs.

Localization, Frequency, and Severity of Endolymphatic Hydrops and the Pathology of the Labyrinthine Membrane in Meniere's Disease

1987 ◽  
Vol 96 (4) ◽  
pp. 438-445 ◽  
Author(s):  
Taeko Okuno ◽  
Isamu Sando
Keyword(s):  
Endolymphatic Hydrops ◽  
Meniere’S Disease ◽  
Menière’S Disease ◽  
Meniere's Disease ◽  
Ménière’S Disease ◽  
Menière's Disease ◽  
Basal Turn ◽  
Ménière's Disease ◽  
Temporal Bones ◽  
Perilymphatic Space

The localization, frequency, and severity of endolymphatic hydrops in 22 temporal bones of 16 individuals with Meniere's disease were studied histopathologically. Endolymphatic hydrops was more often observed in the pars inferior (22/22) than in the pars superior (13/22) of the temporal bone, and severe hydrops was observed most frequently in the saccule, followed by the cochlea, the utricle, and the three semicircular canals. In the cochlea, the most severe hydrops was observed in the apical turn, followed by the hook portion, the middle turn, and the basal turn. Clinically interesting observations regarding endolymphatic hydrops included bulging into the perilymphatic space of the vestibule. In 17 of 22 bones the saccular membrane bulged into the vestibule laterally and was attached to the footplate of the stapes. In two of 22 bones, Reissner's membrane in the hook portion of the basal turn of the cochlea bulged superiorly into the vestibule, occupying most of the perilymphatic space of the vestibule.

Pathology of Meniere's Disease

1984 ◽  
Vol 93 (4_suppl) ◽  
pp. 31-35 ◽  
Author(s):  
Michael M. Paparella
Keyword(s):  
Endolymphatic Hydrops ◽  
Clinical Signs ◽  
Meniere’S Disease ◽  
Menière’S Disease ◽  
Meniere's Disease ◽  
Ménière’S Disease ◽  
Menière's Disease ◽  
Clear Cut ◽  
Ménière's Disease ◽  
Temporal Bones

From review of recent findings, the pathology of Meniere's disease is described and correlated with clinical signs. Since Meniere's disease can be seen only in humans, assessing its natural history is important. A recent survey of 500 patients demonstrated the three major symptoms to be vestibular, auditory, and aural pressure. Meniere's disease (idiopathic) is distinguished from Meniere's syndrome (symptoms with likely cause), which accounted for approximately one fourth of the patients. Atypical forms include vestibular and cochlear Meniere's disease. A recent review of our temporal bone collection and detailed study of the pathological conditions of 134 temporal bones described in the literature revealed characteristic pathological findings. Patients with clear-cut histories of Meniere's disease may demonstrate little or no endolymphatic hydrops at death. Nevertheless, hydrops of the pars inferior remains the most significant pathological correlate of Meniere's disease. On the basis of these findings, the pathogenesis of the disease (malabsorption of endolymph) and the pathophysiology of the symptoms (physical and chemical) are discussed.

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