Correlation between magnetic resonance imaging classification of endolymphatic hydrops and clinical manifestations and audiovestibular test results in patients with definite Ménière's disease.

OBJECTIVES: The purpose was to evaluate magnetic resonance imaging (MRI) classification of endolymphatic hydrops with clinical features, audiological and vestibular tests in patients with definite unilateral Ménière's disease (MD). METHODS: Thirty-eight patients were enrolled in this study. The severity of the main clinical symptoms, audiovestibular tests, and MRI, performed 4 hours after intravenous injection of gadobutrol to visualize inner ear compartments, were evaluated. Endolymphatic space dilatation was assessed using Barath and Bernaerts grading systems, and the correlation between the grade of the hydrops and clinical features was evaluated. RESULTS: Using the Barath system, cochlear hydrops was visualized in 81.6% of affected ears, while vestibular was 63.2%. Sensitivity increased to 94.7% using Bernaerts' modification. Vestibular hydrops involving the utricle was present only among patients with cochlear and saccular endolymphatic space dilatation. There was a significant relationship between the hearing level and the vestibular hydrops degree in the Bernaerts scale. The grade of the hydrops correlated neither with the duration of MD nor with the severity of main clinical symptoms. Our study proved MRI to be a sensitive diagnostic tool in MD. The endolymphatic hydrops' grade correlates with the hearing level, which confirms endolymphatic space dilatation's role in hearing loss. CONCLUSIONS: In our study, two similar MRI grading systems were used; however, several differences were found compared to one another. The Bernaerts scale was more sensitive than the Barath scale, and several relationships between the radiological and clinical data were found. Therefore, several MRI evaluating scales and correlating them with the clinical features are needed. The increased perilymphatic enhancement of the cochlea and an extra low-grade vestibular hydrops distinguished in the Bernaerts scale may increase MD diagnosis sensitivity. Magnetic resonance findings in MD support the clinical diagnosis and may help to understand MD pathophysiology better. This study adds to the knowledge and diagnostics in MD for healthcare to improve patients' treatment.

Relationship between video head impulse test, ocular and cervical vestibular evoked myogenic potentials, and compartmental magnetic resonance imaging classification in menière's disease.

OBJECTIVES/HYPOTHESIS: Currently, it is possible to assess in vivo the morphology of each compartment of the endolymphatic spaces 4 hours after an intravenous administration of gadolinium on magnetic resonance imaging (MRI). The aim of this study was to assess the correlation between otolithic and ampullar functions (cervical vestibular evoked myogenic potential [cVEMP], ocular vestibular evoked myogenic potential [oVEMP], video head impulse test [VHIT]) and delayed inner ear MRI based on a compartmental, anatomically based classification that included the cochlea, the saccule, the utricle, and the ampullas. STUDY DESIGN: Retrospective case-control study. METHODS: In this retrospective study, we performed three-dimensional fluid-attenuated inversion recovery sequences with delayed acquisition in 26 healthy subjects and 31 definite Menière's disease (MD) patients. Each subject was then graded on MRI on the basis on cochlear, saccular, utricular, and ampullar hydrops in MD patients. All patients underwent pure-tone audiometry, VHIT, cVEMP, and oVEMP testing. RESULTS: Cochlear, saccular, utricular, and ampullar hydrops were found on MRI in 88%, 91%, 50%, and 8.5% respectively. We found no significant correlation between the presence of saccular hydrops versus cVEMP, utricular hydrops versus oVEMP, and ampullar hydrops versus VHIT. However, the severity of endolymphatic hydrops on MRI was correlated to the degree of hearing loss. CONCLUSIONS: We proposed a compartmental, anatomically based classification for endolymphatic hydrops on MRI, which included the whole vestibular compartment. Using this classification, we observed increasing morphological changes as the disease evolved, affecting first the saccule, then the utricle, and finally the ampullas. The severity of vestibular endolymphatic hydrops is only correlated to hearing loss severity. LEVEL OF EVIDENCE: 3 Laryngoscope, 130:E444-E452, 2020.

On the classification of hydropic ear disease (Menière's disease).

More than 150 years after its initial description by Prosper Menière, the disease named after him is still at the center of scientific debates. Two recent developments have specifically created a breeding ground for controversy: (1) Since its first description 10 years ago, magnetic resonance imaging diagnosis of endolymphatic hydrops in living patients has seen an increasing and worldwide application. (2) The Bárány Society Classification Committee published diagnostic criteria for Menière's disease in 2015 and proposed a concept of the disease that has elicited widespread criticism. In order to promote the understanding of the underlying controversies and arguments, this article gives an overview of and discusses relevant classification proposals for Menière's disease, including the new classification system of hydropic ear disease.

Menière and Friends: Imaging and Classification of Hydropic Ear Disease.

BACKGROUND: Over 75 years ago, endolymphatic hydrops was discovered as the pathologic correlate of Menière's disease. However, this pathologic finding could be ascertained only in postmortem histologic studies. Due to this diagnostic dilemma and the variable manifestation of the various audiovestibular symptoms, diagnostic classification systems based on clinical findings have been used hitherto. METHODS: A review of the literature of magnetic resonance (MR) imaging of hydropic ear disease. RESULTS: Recent developments of high resolution MR imaging of the inner ear have now enabled us to visualize in vivo endolymphatic hydrops in patients with suspected Menière's disease. The existing knowledge from temporal bone histologic studies and from the emerging evidence on imaging based evaluation of patients with suspected Menière's disease indicate that endolymphatic hydrops not only is responsible for the full-blown clinical triad of simultaneous attacks of auditory and vestibular dysfunction, but also for other clinical presentations such as "vestibular" and "cochlear Menière's disease." CONCLUSION: As a consequence, we propose the term "Hydropic Ear Disease" as a new terminology which is based on symptomatic and imaging characteristics of these clinical entities to clarify and simplify their diagnostic classification.

MRI of endolymphatic hydrops in patients with Meniere's disease: a case-controlled study with a simplified classification based on saccular morphology.

OBJECTIVES: Endolymphatic hydrops (EH) can be studied in patients by MRI. With the semi-quantitative grading system, previous imaging studies showed discrepancies in the occurrence and grading of EH in patients with Meniere's disease (MD). Here, we compared the inversion of the saccule to utricle area ratio (SURI) with the semi-quantitative method of grading conventionally used to diagnose MD. METHODS: Imaging was carried out on a 3-T MRI scanner. We performed 3D-FLAIR sequences 4 h after a single intravenous dose of contrast agent. Two radiologists independently studied the morphology of the inner ear structures in the healthy subjects and MD patients. Each subject was then graded on the basis of the EH semi-quantitative analysis and on saccular morphology using axial and sagittal reference slices in the vestibule plane. RESULTS: Thirty healthy subjects and 30 MD patients had MRI scans. Using the semi-quantitative method, we found no significant difference in the number of subjects with EH between the two groups. SURI was found in 15 out of 30 MD patients and in none of the 30 healthy subjects. In three MD patients the saccule was not visible. CONCLUSION: SURI is currently the most specific criterion for imaging diagnosis of MD. KEY POINTS: • Half of MD patients presented with inversion of the saccule to utricle ratio. • Saccular analysis is crucial when assessing patients with Meniere's disease. • In some patients, the saccule is not visible, suggestive of intra-labyrinthine fistulae.

Indication of the side of delayed endolymphatic hydrops by vestibular evoked myogenic potential and caloric test.

OBJECTIVE: Delayed endolymphatic hydrops (DEH) can be clinically classified into ipsi- and contralateral types. This study aims to investigate the relationship between the results of vestibular evoked myogenic potential (VEMP) and caloric testing and the clinical type of DEH. METHODS: The data of 33 patients with DEH who underwent both VEMPs and caloric testing were retrospectively examined. The type of DEH was judged from its clinical and audiological course. Theasymmetry ratios of the VEMPs and canal paresis on the caloric test were measured. RESULTS: The results of VEMP and the caloric testing were consistent with the type of DEH in 61 and 76% of DEH patients, respectively. Combined use of VEMP and caloric testing correctly identified the type of DEH in 82% of patients. CONCLUSIONS: The combined use of VEMP and caloric testing can indicate the type of DEH at a reasonably high rate, but inconclusively.

Magnetic resonance imaging of the inner ear in Meniere's disease.

Recent magnetic resonance imaging (MRI) techniques have made it possible to examine the compartments of the cochlea using gadolidium-chelate (GdC) as a contrast agent. As GdC loads into the perilymph space without entering the endolymph in healthy inner ears, the technique provides possibilities to visualize the different cochlear compartments and evaluate the integrity of the inner ear barriers. This critical review presents the recent advancements in the inner ear MRI technology, contrast agent application and the correlated ototoxicity study, and the uptake dynamics of GdC in the inner ear. GdC causes inflammation of the mucosa of the middle ear, but there are no reports or evidence of toxicity-related changes in vivo either in animals or in humans. Intravenously administered GdC reached the guinea pig cochlea about 10 minutes after administration and loaded the scala tympani and scala vestibuli with the peak at 60 minutes. However, the perilymphatic loading peak was 80 to 100 minutes in mice after intravenous administration of GdC. In healthy animals the scala media did not load GdC. In mice in which GdC was administered topically onto the round window, loading of the cochlea peaked at 4 hours, at which time it reached the apex. The initial portions of the organ to be filled were the basal turn of the cochlea and vestibule. In animal models with endolymphatic hydrops (EH), bulging of the Reissner's membrane was observed as deficit of GdC in the scala vestibuli. Histologically the degree of bulging correlated with the MR images. In animals with immune reaction-induced EH, MRI showed that EH could be limited to restricted regions of the inner ear, and in the same inner ear both EH and leakage of GdC into the scala media were visualized. More than 100 inner ear MRI scans have been performed to date in humans. Loading of GdC followed the pattern seen in animals, but the time frame was different. In intravenous delivery of double-dose GdC, the inner ear compartments were visualized after 4 hours. The uptake pattern of GdC in the perilymph of humans between 2 hours and 7 hours after local delivery needs to be clarified. In almost all patients with probable or suspected Ménière's disease, EH was verified. Specific algorithms with a 12-pole coil using fluid attenuation inversion recovery sequences are recommended for initial imaging in humans.