Delayed post gadolinium MRI descriptors for Meniere's disease: a systematic review and meta-analysis.

OBJECTIVES: Delayed post-gadolinium magnetic resonance imaging (MRI) detects changes of endolymphatic hydrops (EH) within the inner ear in Meniere's disease (MD). A systematic review with meta-analysis was conducted to summarise the diagnostic performance of MRI descriptors across the range of MD clinical classifications. MATERIALS AND METHODS: Case-controlled studies documenting the diagnostic performance of MRI descriptors in distinguishing MD ears from asymptomatic ears or ears with other audio-vestibular conditions were identified (MEDLINE, EMBASE, Web of Science, Scopus databases: updated 17/2/2022). Methodological quality was evaluated with Quality Assessment of Diagnostic Accuracy Studies version 2. Results were pooled using a bivariate random-effects model for evaluation of sensitivity, specificity and diagnostic odds ratio (DOR). Meta-regression evaluated sources of heterogeneity, and subgroup analysis for individual clinical classifications was performed. RESULTS: The meta-analysis included 66 unique studies and 3073 ears with MD (mean age 40.2-67.2 years), evaluating 11 MRI descriptors. The combination of increased perilymphatic enhancement (PLE) and EH (3 studies, 122 MD ears) achieved the highest sensitivity (87% (95% CI: 79.92%)) whilst maintaining high specificity (91% (95% CI: 85.95%)). The diagnostic performance of "high grade cochlear EH" and "any EH" descriptors did not significantly differ between monosymptomatic cochlear MD and the latest reference standard for definite MD (p = 0.3; p = 0.09). Potential sources of bias were case-controlled design, unblinded observers and variable reference standard, whilst differing MRI techniques introduced heterogeneity. CONCLUSIONS: The combination of increased PLE and EH optimised sensitivity and specificity for MD, whilst some MRI descriptors also performed well in diagnosing monosymptomatic cochlear MD. KEY POINTS: • A meta-analysis of delayed post-gadolinium magnetic resonance imaging (MRI) for the diagnosis of Meniere's disease is reported for the first time and comprised 66 studies (3073 ears). • Increased enhancement of the perilymphatic space of the inner ear is shown to be a key MRI feature for the diagnosis of Meniere's disease. • MRI diagnosis of Meniere's disease can be usefully applied across a range of clinical classifications including patients with cochlear symptoms alone.

The Diagnostic Value of Multifrequency Tympanometry in Patients with Ménière's Disease: A Prospective Analysis.

INTRODUCTION: Diagnosing Ménière's disease (MD) by its characteristics such as episodes of vertigo, fluctuating hearing loss, and tinnitus with aural fullness remains challenging. Available tests evaluating the presence of endolymphatic hydrops (EH) are often expensive or time assuming. An in-office quick and simple non-invasive diagnostic test is multifrequency tympanometry (MFT). It can measure conductance at 2 kHz probe tones, which was demonstrated to reflect variations in cochlear pressure. Previous studies investigating MFT as a diagnostic test for MD showed conflicting outcomes possibly biased by their retrospective design. METHODS: We prospectively collected MFT results (Y width) in patients with dizziness and compared MFT test results in affected (group 1) and unaffected (group 2) ears of 37 MD subjects and in control ears of 33 non-MD subjects (group 3). RESULTS: The mean value of the Y width in affected ears was 315.6 ± 70.2 daPa compared to 292.3 ± 98.6 daPa in unaffected ears in MD subjects and 259.4. ± 60.6 daPa in the non-MD group. A positive test result (i.e., a Y width of 235 daPa or more) was found in 35 ears in the MD group, 21 times involving the affected ear and 14 times involving the unaffected ear, compared to 16 in the non-MD group. No significant differences between the three groups could be demonstrated (p > 0.05). We found a sensitivity of 58.3% and specificity of 66.3% for detecting EH in an affected ear in MD subjects. CONCLUSION: There is a trend towards increased conductance tympanometry in affected ears. However, we noticed a high false positive rate of MFT and do not support standardized use of MFT as an additional diagnostic tool for detecting EH in MD patients. A negative test result on the contrary is unlikely related to EH.

Characterizing a Joint Reflection-Distortion OAE Profile in Humans With Endolymphatic Hydrops.

OBJECTIVES: Endolymphatic hydrops (EH), a hallmark of Meniere disease, is an inner-ear disorder where the membranes bounding the scala media are distended outward due to an abnormally increased volume of endolymph. In this study, we characterize the joint-otoacoustic emission (OAE) profile, a results profile including both distortion- and reflection-class emissions from the same ear, in individuals with EH and speculate on its potential utility in clinical assessment and monitoring. DESIGN: Subjects were 16 adults with diagnosed EH and 18 adults with normal hearing (N) matched for age. Both the cubic distortion product (DP) OAE, a distortion-type emission, and the stimulus-frequency (SF) OAE, a reflection-type emission, were measured and analyzed as a joint OAE profile. OAE level, level growth (input/output functions), and phase-gradient delays were measured at frequencies corresponding to the apical half of the human cochlea and compared between groups. RESULTS: Normal hearers and individuals with EH shared some common OAE patterns, such as the reflection emissions being generally higher in level than distortion emissions and showing more linear growth than the more strongly compressed distortion emissions. However, significant differences were noted between the EH and N groups as well. OAE source strength (a metric based on OAE amplitude re: stimulus level) was significantly reduced, as was OAE level, at low frequencies in the EH group. These reductions were more marked for distortion than reflection emissions. Furthermore, two significant changes in the configuration of OAE input/output functions were observed in ears with EH: a steepened growth slope for reflection emissions and an elevated compression knee for distortion emissions. SFOAE phase-gradient delays at 40 dB forward-pressure level were slightly shorter in the group with EH compared with the normal group. CONCLUSIONS: The underlying pathology associated with EH impacts the generation of both emission types, reflection and distortion, as shown by significant group differences in OAE level, growth, and delay. However, hydrops impacts reflection and distortion emissions differently. Most notably, DPOAEs were more reduced by EH than were SFOAEs, suggesting that pathologies associated with the hydropic state do not act identically on the generation of nonlinear distortion at the hair bundle and intracochlear reflection emissions near the peak of the traveling wave. This differential effect underscores the value of applying a joint OAE approach to access both intracochlear generation processes concurrently.

Meniere's disease: Pathogenesis, treatments, and emerging approaches for an idiopathic bioenvironmental disorder.

Meniere's disease (MD) is a severe inner ear condition known by debilitating symptoms, including spontaneous vertigo, fluctuating and progressive hearing loss, tinnitus, and aural fullness or pressure within the affected ear. Prosper Meniere first described the origins of MD in the 1860s, but its underlying mechanisms remain largely elusive today. Nevertheless, researchers have identified a key histopathological feature called Endolymphatic Hydrops (ELH), which refers to the excessive buildup of endolymph fluid in the membranous labyrinth of the inner ear. The exact root of ELH is not fully understood. Still, it is believed to involve several biological and bioenvironmental etiological factors such as genetics, autoimmunity, infection, trauma, allergy, and new theories, such as saccular otoconia blocking the endolymphatic duct and sac. Regarding treatment, there are no reliable and definitive cures for MD. Most therapies focus on managing symptoms and improving the overall quality of patients' life. To make significant advancements in addressing MD, it is crucial to gain a fundamental understanding of the disease process, laying the groundwork for more effective therapeutic approaches. This paper provides a comprehensive review of the pathophysiology of MD with a focus on old and recent theories. Current treatment strategies and future translational approaches (with low-level evidence but promising results) related to MD are also discussed, including patents, drug delivery, and nanotechnology, that may provide future benefits to patients suffering from MD.

Endolymphatic hydrops imaging and correlation with clinical characteristics, audiovestibular function and mental impairment in patients with Meniere's disease.

PURPOSE: MR imaging was used to visualize the vestibular and cochlear endolymphatic hydrops in patients with Meniere's disease (MD). The relationship between the degree of hydrops and clinical characteristics, audiovestibular function, anxiety and depression state in MD patients. METHODS: 70 patients with definitely or probably unilateral Meniere's disease received bilateral intratympanic gadolinium administration and MR scanning. The degree of bilateral vestibular and cochlea hydrops were analyzed and evaluated by three-dimensional real inversion recovery (3D-real IR) sequence, and the correlation between the grades of endolymphatic hydrops (EH) and disease course, vertigo grading assessment, the duration of vertigo, hearing loss level, caloric test, vestibular myogenic evoked potential (VEMP), electrocochleogram (EcoG), vertigo disability scale (physical, emotional, functional), anxiety and depression scale were studied. RESULTS: It was found that the vestibule and cochlea EH of the affected and the contralateral ear had different degrees of hydrops and there was no statistical difference between the left and right vestibules. The degree of vestibule EH (V-EH) was significantly positively correlated with the degree of cochlear EH (C-EH). C-EH and hearing loss level were positively correlated with EcoG. There was positive correlation between vestibular EH and hearing loss level, VEMP, caloric test, disease course or vertigo duration. There was a negative relationship between Dizziness Handicap Inventory (Emotion) (DHI(E)) and VEMP. Self-rating Anxiety Scale (SAS) and Self-rating Depression Scale (SDS) scores were positive correlated with DHI(E) and DHI total scores in MD patients. CONCLUSION: Endolymph-enhancing MRI was used as an important imaging method for the diagnosis of labyrinthine hydrops in Meniere's disease. There were certain correlation between EH and the degree of vertigo attack, hearing loss level, vestibular function, and further changes in anxiety and depression emotion.

Assessing the usefulness of inner ear contrast-enhanced magnetic resonance imaging and pure-tone audiometry in estimating the prognostic value of hearing improvement in patients with definite Meniere's disease.

PURPOSE: Meniere's disease (MD) is characterized by combined cochlear and vestibular symptoms. However, its underlying cause remains unclear, with low-to-mid-tone hearing impairment being predominantly reported. Moreover, predicting hearing improvement after disease onset is not possible. This study aimed to assess whether hearing improvement in patients with definite MD (DMD) could be predicted using inner ear contrast magnetic resonance imaging (IEC-MRI) and pure-tone audiometry (PTA) at disease attack. MATERIALS AND METHODS: Between April 2020 and March 2022, seven outpatients with DMD were enrolled based on the Bárány Society DMD criteria. The patients were divided into two groups: low-tone hearing loss (LTL) group and low-to-mid-tone hearing loss (LMTL) group. Hearing improvement rates as well as the possible inter-relation between endolymphatic hydrops and hearing improvement were evaluated. RESULTS: Endolymphatic hydrops was found in two of four LTL cases. One of three LMTL cases had prominent lymphedema. All patients with LTL but only one patient with LMTL had hearing improvement. Endolymphatic hydrops was not found to be correlated with hearing improvement. CONCLUSIONS: Estimating hearing improvement in patients with DMD using IEC-MRI was not possible. However, PTA showed better hearing prognosis in LTL than in LMTL. Therefore, estimating hearing improvement using PTA was possible at disease attack.

Visualization of the saccule and utricle with non-contrast-enhanced FLAIR sequences.

OBJECTIVES: 3D-fluid attenuation inversion recovery (FLAIR) collected 4 h after intravenous gadolinium injection can delineate the perilymphatic space (PLS) from the endolymphatic space (ELS) to capture endolymphatic hydrops, the pathological counterpart of Ménière's disease. We aimed to optimize visualization of such inner ear internal anatomy using 3D-FLAIR without injection. METHODS: 3D-FLAIR signal from different fluid compartments such as PLS and ELS was first simulated. Then, twenty-two healthy subjects were scanned at 3.0-T MRI with non-injected 3D-FLAIR using variable T2 preparations (T2Preps) (OFF, 200, 400, and 600 ms) and variable inversion times (TIs) (from 224 to 5000 ms) and different resolutions (1.0 × 1.0 × 1.5, 0.6 × 0.6 × 0.8, and 0.6 × 0.6 × 0.6 mm3). The relative contrast between PLS and ELS and the visibility of the saccule and utricle were assessed. Additionally, non-injected 3D-FLAIR with the optimal setting was tested in a Ménière patient and compared with gadolinium-injected 3D-FLAIR. RESULTS: The PLS and ELS were differentiated when T2Prep was used but not without. The relative contrast was larger with T2Prep at 400 ms than at 200 or 600 ms (0.72 ± 0.22 vs. 0.44 ± 0.11, p = 0.019; and 0.72 ± 0.22 vs. 0.46 ± 0.28, p = 0.034, respectively). The saccule and utricle were best delineated in 87. % cases with T2Prep = 400 and TI = 2100 ms at the highest resolution. Visualization of the saccule and utricle in the optimized non-injected 3D-FLAIR was similar to conventional injected 3D-FLAIR in a patient. CONCLUSIONS: Combining a specific T2Prep and TI in non-injected 3D-FLAIR could separate PLS and ELS and even the saccule and utricle, paving the way toward future application to diagnose Ménière's disease. KEY POINTS: • MRI can capture the internal anatomy of inner ear without injection of contrast media. • Specific parameters consisting of a T2 preparation of 400 ms and an inversion time of 2100 ms must be used to visualize the saccule and utricle on non-injected 3D-FLAIR.

Optimized 3D-FLAIR sequences to shorten the delay between intravenous administration of gadolinium and MRI acquisition in patients with Menière's disease.

OBJECTIVES: The aim of this study was to shorten the 4-h delay between the intravenous administration of gadolinium and MRI acquisition for hydrops evaluation using an optimized 3D-FLAIR sequence in patients with Menière's disease. METHODS: This was a single-center prospective study including 29 patients (58 ears), recruited between November 2020 and February 2021. All patients underwent a 3-T MRI with an optimized 3D-FLAIR sequence without contrast then at 1 h, 2 h, and 4 h after intravenous administration of gadobutrol. The signal intensity ratio was quantitatively assessed with the region of interest method. We also evaluated the volume of endolymphatic structures (saccule, utricle) then the presence of endolymphatic hydrops and blood-labyrinthine barrier impairment at each acquisition time. RESULTS: For all ears, the signal intensity ratio was significantly non-inferior at 2 h compared to 4 h, with a mean geometric signal intensity ratio at 0.83 (95% CI: 0.76 to 0.90, one-sided p < .001 for non-inferiority at -30% margin). Mean volume equivalence of saccule and utricle between 2 and 4 h was proven at a ± 0.20 standardized deviation equivalence margin. Intra-rater agreements (Cohen's kappa) were all greater than 0.90 for all endolymphatic hydrops location and blood-labyrinthine-barrier impairment between the 2- and 4-h assessments. CONCLUSIONS: We demonstrated that using an optimized 3D-FLAIR sequence we could shorten the acquisition from 4 to 2 h with a high reliability for the diagnosis of endolymphatic hydrops and blood-labyrinthine-barrier impairment. CLINICAL TRIAL REGISTRATION: Clinical trial no: 38RC15.173 KEY POINTS: • Magnetic resonance imaging with delayed 3D-FLAIR sequences allows the diagnosis of endolymphatic hydrops in patients with definite Menière's disease. • An optimized 3D-FLAIR sequence with a long TR of 16000 ms and a constant flip angle allows for reducing the delay between intravenous injection of gadobutrol and MRI acquisition from 4 to 2 h to diagnose endolymphatic hydrops. • Reducing this delay between intravenous injection and MRI acquisition could have implications for clinical practice for both patients and imaging departments.

Intraoperative Electrocochleography in Subjects Affected by Vestibular Schwannoma and Ménière's Disease: Comparison of Results.

OBJECTIVES: Histologic reports of temporal bones of ears with vestibular schwannomas (VSs) have indicated findings of endolymphatic hydrops (ELH) in some cases. The main goal of this investigation was to test ears with VSs to determine if they exhibit electrophysiological characteristics similar to those of ears expected to experience ELH. DESIGN: Fifty-three subjects with surgically confirmed VS aged ≥18 and with normal middle ear status were included in this study. In addition, a second group of adult subjects (n = 24) undergoing labyrinthectomy (n = 6) or endolymphatic sac decompression and shunt (ELS) placement (n = 18) for poorly controlled vestibular symptoms associated with Meniere's disease (MD) participated in this research. Intraoperative electrocochleography (ECochG) from the round window was performed using tone burst stimuli. Audiometric testing and word recognition scores (WRS) were performed preoperatively. ECochG amplitudes, cochlear microphonic/auditory nerve neurophonic (ANN) in the form of the "ongoing" response and summation potential, were analyzed and compared between the two groups of subjects. In addition, to evaluate any effect of auditory nerve function, the auditory nerve score was calculated for each subject. Pure-tone averages were obtained using the average air conduction thresholds at 0.5, 1, and 2 kHz while WRS was assessed using Northwestern University Auditory Test No. 6 word lists. RESULTS: In the VS group the average pure-tone averages and WRS were 59.6 dB HL and 44.8%, respectively, while in the MD group they were 52.3 dB HL and 73.8%. ECochG findings in both groups revealed a reduced trend in amplitude of the ongoing response with increased stimulus frequency. The summation potential amplitudes of subjects with VS were found to be less negative than the MD subjects for nearly all test frequencies. Finally, the VS group exhibited poorer amounts of auditory nerve function compared to the MD group. CONCLUSIONS: The current findings suggest cochlear pathology (e.g., hair cell loss) in both groups but do not support the hypothesis that VSs cause ELH.

Endolymphatic hydrops in the unaffected ear of patients with unilateral Ménière's disease.

PURPOSE: Current studies show that frequency tuning modification is a good marker for the detection of endolymphatic hydrops (EH) employing magnetic resonance imaging (MRI) in patients with Ménière's disease (MD). The purpose of the present study is to analyze the auditory and vestibular function with audiometric and vestibular-evoked myogenic potentials (VEMP) responses, respectively, in both the affected and unaffected ears of patients with unilateral MD using MRI as diagnostic support for the degree of EH. METHODS: We retrospectively reviewed the medical records of 76 consecutive patients with unilateral definite MD (age 55 (28-75); 39 women, 37 men). MRI was used through intravenous gadolinium administration, audiometry, and VEMPs. Functional tests were performed up to a week after the MRI. All were followed up one year after imaging utilizing clinical, auditory, and vestibular testing to rule out bilateral involvement. RESULTS: In the unaffected ear, the mean pure-tone average is normal even in cases with hydrops and, for a similar severity of hydrops is significantly lower than in the affected ear. Significant differences for the amplitude of the response at 0.5 kHz, at 1 kHz between the affected and unaffected ears were found to be lower in the affected ears. The relative amplitude ratio (1 Kz-0.5 kHz) was significantly lower in the affected ear and in the case of the oVEMP response depends on the degree of EH. The response in the unaffected ear was not modified by the presence or the degree of hydrops. CONCLUSION: In the unaffected ear, hydrops is not associated with hearing deterioration. For a similar degree of hydrops, hearing loss is significantly greater in the affected ear. The endolymphatic hydrops in the vestibule induces a frequency bias in the VEMP response only in the affected ear and not in the unaffected ear. Because of these findings we consider that hydrops does not represent an active disorder in the unaffected ear.

Evaluation of Patients with Delayed Endolymphatic Hydrops by Videonystagmography, Vestibular-Evoked Myogenic Potentials, and Electrocochleography.

INTRODUCTION: The categorization of delayed endolymphatic hydrops (DEH) based on the ear which produces vertigo may sometimes cause misdiagnosis. OBJECTIVES: The aim of this study was investigating the vestibular-evoked myogenic potentials (VEMPs), electrocochleography (ECoG), and videonystagmography (VNG) in cases with DEH to determine the ear that originates symptoms. METHODS: In this cross-sectional study, 34 patients - 20 males and 14 females - with profound unilateral sensorineural deafness and vertigo attacks were recruited and evaluated by the ECoG, VNG, and VEMPs tests. RESULTS: The average age was around 43; the summating potential/action potential was abnormal in 29.4% of patients in their normal auditive ear. In 32.4, 17.6, and 50% of cases with a deaf ear, absent, normal, and abnormal VEMPs results were sequentially observed, respectively. In normal-hearing ears, absent, normal, and abnormal VEMPs were observed in 23.5, 50, and 26.5%, respectively. In the normal-hearing ear, the distribution of abnormal VEMPs was 26.5%, and in the deaf ear, this parameter was abnormal in 50% of the opposite ear (p value = 0.00021). In the VNG test, among patients with a normal-hearing ear, results in 27 and 7 patients were sequentially normal and hypofunction. CONCLUSION AND SIGNIFICANCE: The probability of a hypofunction VNG test in a normal-hearing ear might be greater when the VEMPs results of the contralateral deaf ear are normal. In patients with a normal-hearing ear, the distribution of abnormal VEMPs in the contralateral deaf ear is greater, although the intact side may also manifest abnormality in VEMPs tests. The initial evaluation should begin in a deaf ear as well as for the normal-hearing ear ere utilizing ablation surgery.

Relationship between Endolymphatic Hydrops and Symptoms of Meniere Disease in Acoustic Hearing.

HYPOTHESIS: The endolymphatic hydrops (EH) does not affect hearing loss significantly at low frequencies, whereas the hydrops affects the diplacusis. BACKGROUND: There have been many arguments whether the EH cause the Meniere disease. Despite a lot of experimental studies to investigate the Meniere disease, there have been little modeling studies, which are helpful to understand the mechanism. METHODS: A 3D finite element model of the human cochlea and the middle ear was used for investigation of the relationship between EH and hearing loss at low frequencies and diplacusis (2 specific symptoms of Meniere disease). While the cochlear geometry was simplified as a tapered box shape, the middle ear was based on the real geometry obtained from µCT images. EH is implemented by prestress on the basilar membrane surface in the simulation. RESULTS: The EH did not cause significant hearing loss at low frequencies in both air- and bone-conducted hearing. Rather, this disorder caused a shift in best frequency (BF) position to the base at low frequencies below about 250 Hz. The BF shift can explain the diplacusis because a low-frequency sound can be perceived as a slightly higher frequency so that Meniere patients can perceive 2 different frequency sounds corresponding to a given single-frequency sound. CONCLUSION: The EH cannot be a sufficient condition for Meniere disease, whereas the hydrops can cause the diplacusis.

[Various types of vertigo and balance disorders in patients with Meniere's disease]. / Razlichnye formy golovokruzheniya i narusheniya ravnovesiya pri bolezni Men'era.

Vertigo and balance disorders in Meniere's disease (MD) may have various etiology. The aim of the review is discussing pathogenetic mechanisms of the typical vertiginous paroxysms in MD, resulting from endolymphatic hydrops as well as analysis of etiology, pathogenesis, clinical course and basic treatment of paroxysmal and permanent forms of vertigo and balance disorders, caused by other conditions, associated with MD. We discussed the course of MD complicated by vestibular migraine, benign positional paroxysmal vertigo, functional dizziness, bilateral vestibulopathy and vestibular drop-attacks.

The round window sign: a sensitive sign to detect perilymphatic fistulae on delayed postcontrast 3D-FLAIR sequence.

OBJECTIVES: The aim of this study is to assess the diagnostic performance of a new MR sign, named the round window sign (RWS), to diagnose perilymphatic fistula (PLF) in a population of patients with chronic cochleo-vestibular symptoms, classified as definite or probable Menière's disease (MD). METHODS: A total of 164 patients (mean age 52 ± 35 years) with chronic cochleo-vestibular symptoms underwent MRI, between 4 and 5 h after intravenous gadoteric acid injection (Dotarem®, 0.1 mmol/kg). MRI exploration was carried out on a 3-T Achieva® TX scanner. We analyzed the presence of the RWS, defined as a nodular FLAIR high signal in the round window (RW) and the presence of associated saccular hydrops. When this RWS was present, a temporal bone CT scan was performed and the RW was analyzed. RESULTS: Of the 164 patients with definite MD (85 patients) or probable MD (79 patients), we found the RWS in 18 (11%), and 17/18 were classified into the group of probable MD. All these 18 patients showed other MR sequences considered as normal, including heavily weighted T2 imaging. Among these 18 patients, the temporal bone CT examination presented a filling of the RW in 13 patients (72%) and no filling of the RW in 5 patients (28%). Seven patients were surgically managed confirming in vivo the PLF diagnosis. The RWS was associated with the presence of a saccular hydrops in 4 cases. CONCLUSION: Delayed postcontrast 3D-FLAIR may reveal perilymphatic fistulae in patients with probable Menière's disease using the round window sign. KEY POINTS: • MRI with delayed acquisition can detect perilymphatic fistulae with perfect sensitivity, based on the presence of the round window sign. • This visual sign is only visible on a 3D-FLAIR sequence. • 3D-FLAIR sequence with delayed acquisition is more sensitive than temporal bone CT scan examination in detecting PLF.

Endolymphatic Hydrops Secondary to Spontaneous Intracranial Hypotension.

BACKGROUND: Spontaneous intracranial hypotension may be associated with neuro-otological symptoms that might mimic Menière's disease. CASE PRESENTATION: We report the case of a 53-year-old male presenting bi-frontal headache with recurrent spells of vertigo, left fluctuating hearing loss, and tinnitus. Dedicated brain and inner ear Magnetic Resonance Imaging, including a post-contrast 4 hours delayed FLAIR sequence, revealed typical signs of spontaneous intracranial hypotension associated with endolymphatic hydrops involving the left saccule and cochlea. CONCLUSIONS: Audio vestibular manifestations mimicking Menière's disease in spontaneous intracranial hypotension could be explained by endolymphatic hydrops, which can be detected using dedicated magnetic resonance imaging sequences.

Relationship Between Audio-Vestibular Functional Tests and Inner Ear MRI in Meniere's Disease.

OBJECTIVES: Meniere's disease is an inner ear disorder generally attributed to an endolymphatic hydrops. Different electrophysiological tests and imaging techniques have been developed to improve endolymphatic hydrops diagnosis. The goal of our study was to compare the sensitivity and the specificity of delayed inner ear magnetic resonance imaging (MRI) after intravenous injection of gadolinium with extratympanic clicks electrocochleography (EcochG), phase shift of distortion product otoacoustic emissions (shift-DPOAEs), and cervical vestibular-evoked myogenic potentials (cVEMP) for the diagnosis of Meniere's disease. DESIGN: Forty-one patients, with a total of 50 affected ears, were included prospectively from April 2015 to April 2016 in our institution. Patients included had definite or possible Meniere's disease based on the latest American Academy of Otolaryngology-Head and Neck Surgery guidelines revised in 2015. All patients went through delayed inner ear MRI after intravenous injection of gadolinium (three dimension-fluid attenuated inversion recovery sequences), pure-tone audiometry, extratympanic clicks EcochG, shift-DPOAEs, and cVEMP on the same day. Endolymphatic hydrops was graded on MRI using the saccule to utricle ratio inversion defined as when the saccule appeared equal or larger than the utricle. RESULTS: Abnormal EcochG and shift-DPOAEs in patients with definite Meniere's disease (DMD) were found in 68 and 64.5%, respectively. The two methods were significantly associated in DMD group. In DMD group, 25.7% had a positive MRI. The correlation between MRI versus EcochG and MRI versus shift-DPOAEs was not significant. MRI hydrops detection was correlated with hearing loss. Finally, 22.9% of DMD group had positive cVEMP. CONCLUSIONS: EcochG and shift-DPOAEs were both well correlated with clinical criteria of Meniere's disease. Inner ear MRI showed hydrops when hearing loss was higher than 35 dB. The shift-DPOAEs presented the advantage of a rapid and easy measurement if DPOAEs could be recorded (i.e., hearing threshold <60dB). In contrast, EcochG can be performed regardless of hearing loss. In combination with shift-DPOAEs, it enhances the chances to confirm the diagnosis with a better confidence.

3D-real IR MRI of Meniere's disease with partial endolymphatic hydrops.

OBJECTIVES: A three-dimensional inversion-recovery sequence with real reconstruction (3D-real IR) sequence 4 h after intravenous gadolinium injection (IV) has been used to visualize the endolymphatic hydrops (ELH) in Meniere's disease (MD). This study was designed to explore the pathology of MD with partial ELH. METHODS: We collected 338 patients with definite MD, all of whom underwent the IV method. Patients who were found to have partial ELH (vestibular or cochlear) were enrolled. The hearing thresholds of the enrolled patients were analyzed, the regions of interest of the cochlear perilymph and the cerebellum white matter were determined, and the signal intensity ratio in the former to the latter (CC ratio) for both sides in the patients was subsequently evaluated. RESULTS: Of the 338 collected patients with definite MD, 19 patients (5.6%) had unilateral vestibular ELH (N = 18) or cochlear ELH (N = 1), and 4 patients (1.2%) with bilateral ELH had contralateral cochlear ELH. The CC ratio of the affected side (1.44 ±â€¯0.46) was higher than that of the unaffected side (1.15 ±â€¯0.33, P < 0.05) in the 19 patients with unilateral ELH. Conversely, there was no difference between the ratio of the contralateral side (1.18 ±â€¯0.16) and the unaffected side (P > 0.05) in the 4 patients with bilateral ELH. CONCLUSIONS: Partial vestibular ELH was more common than partial cochlear ELH in MD. Moreover, vestibular ELH, rather than cochlear ELH, may correlate with the elevated contrast effect in the affected side, which may better reflect the pathologic mechanism of MD.

Clinical manifestations of hydropic ear disease (Menière's).

INTRODUCTION: Hydropic ear disease, initially described by and named after Prosper Menière, is one of the most frequent vertigo disorders and one of the most frequent inner ear disorders. It is the syndrome of endolymphatic hydrops which until 2007 could be diagnostically confirmed only by post-mortem histology. In the past, various attempts to formulate clinical diagnostic criteria have been undertaken but were hampered by the inability to ascertain the diagnosis in living patients. With the milestone achievement of endolymphatic hydrops imaging, today the pathology can be ascertained. In this study, we have performed a detailed analysis of the clinical features of hydropic ear disease for the first time by examining a large cohort of patients with morphologically confirmed endolymphatic hydrops using a detailed physician-administered neurotologic face-to-face interview. RESULTS: During a hydropic vertigo attack, the patients report nausea, vomiting, sweating, urge to defecate, urge to urinate, phosphenes, headache, photophobia, phonophobia and even transient loss of consciousness. A third of the patients does not experience auditory symptoms during the vertigo attacks. Vertigo attacks last less than 20 min in more than one-fourth of the patients. Audiometric hearing loss has its greatest diagnostic value at the frequencies of 1 kHz and below. Cochleovestibular symptom onset simultaneity is associated with a high frequency of drop-attacks. Migraine and autoimmune disorders are not associated with hydropic ear disease. CONCLUSION: This study marks the beginning of the clinical characterization of hydropic ear disease. The findings have important implications for the future formulation of clinical diagnostic criteria.

Quantitative evaluation of endolymphatic hydrops with MRI through intravenous gadolinium administration and VEMP in unilateral definite Meniere's disease.

PURPOSE: To help clinicians to further understand the significance of vestibular-evoked myogenic potential (VEMP) examinations to diagnose MD and the quantitative relationship between VEMP and MRI in assessing the location and degree of endolymphatic hydrops (EH) in definite Meniere's disease (MD) patients. METHODS: Fifty-six patients with unilateral definite MD participated in this study, which used MRIs through intravenous gadolinium administration (IV-Gd), audiometry, caloric tests and VEMP tests. The VEMP results of 26 healthy volunteers were used as a normal reference value. RESULTS: The participants were found through MRI to have differing degrees of vestibular and cochlear EH. Quantitative comparison of MRI and VEMP results found that the response rates of oVEMP decreased with cochlear EH increasing; the asymmetry ratio (AR) of oVEMP can be used to find whether cochlear EH or not, and the P1-N1 amplitude was lower in the extreme cochlear EH group (P < 0.01). The AR of cVEMP was larger in severe vestibular EH group than that of the mild or no vestibular EH group (P < 0.01). The correlation between the degree of cochlear EH and the mean PTA threshold was statistically significant (P < 0.05). The duration of MD correlated positively with vestibular EH (P < 0.05). The abnormal rate of caloric tests was higher in severe vestibular EH group than that of the mild or no vestibular EH group (P < 0.05). CONCLUSIONS: The advantages of MRIs by IV-Gd administration were obvious in assessing the location and degree of EH. oVEMP and PTA can be indirectly used to evaluate the extent of cochlear EH, cVEMP and caloric tests can be used to assess the extent of vestibular EH on the condition of absent MRIs.

A Comparison of Distortion Product Otoacoustic Emission Properties in Ménière's Disease Patients and Normal-Hearing Participants.

OBJECTIVES: Postmortem examination of temporal bones of Ménière's disease patients consistently show dilated endolymphatic spaces of the inner ear, for which the term endolymphatic hydrops has been coined. During the past decade, magnetic resonance imaging techniques for the inner ear appeared, advancing the diagnosis of Ménière's disease. They require, however, a field-strength of at least 3 T, are costly and not universally available. Alternative, noninvasive, cost-effective tests with high sensitivity and specifity for endolymphatic hydrops are desirable. In this study, we test the suitability of distortion product otoacoustic emissions (DPOAEs) for endolymphatic hydrops detection. Previous measurements of the commonly recorded cubic DPOAEs mainly register cochlear hearing loss and are not specific for Ménière's disease. Simultaneous recordings of cubic and quadratic DPOAEs might be more suitable to detect endolymphatic hydrops, because both DPOAE orders react differently to changes of the cochlear operating point as they might occur in Ménière's disease patients. DESIGN: Cubic and quadratic DPOAEs were recorded in normal-hearing participants (N = 45) and in the affected and unaffected ears of patients with a diagnosis of definite Ménière's disease (N = 32). First, to assess the integrity of DPOAE-generating mechanisms, cubic DPOAE-grams were obtained with primary tone frequencies f2 between 1 and 8 kHz with primary tone levels l1 = 60 dB SPL and l2 = 50 dB SPL, and a fixed primary tone frequency ratio of 1.22. Then, cubic and quadratic DPOAEs were simultaneously recorded with primary tone levels l1 = l2 = 65 dB SPL and at primary tone frequencies f2 = 4 and 5 kHz, where f1 was successively varied such that the ratio f2/f1 ranged between 1.1 and 1.6 in 0.04 steps while quadratic and cubic DPOAE levels were extracted from the same recording. RESULTS: Cubic DPOAEs were significantly reduced in the affected ears of Ménière's disease patients, and slightly reduced in the unaffected ears of Ménière's disease patients, relative to the ears of normal-hearing participants. In contrast, no significant changes could be seen in quadratic DPOAEs across the ears of normal-hearing participants and Ménière's disease patients. CONCLUSIONS: We could identify a relatively good preservation of quadratic DPOAE levels in relation to a reduction of cubic DPOAE levels as a potential noninvasive diagnostic approach in the early stage of suspected Ménière's disease. Future studies validating the differential diagnostic power of this parameter in control groups with nonhydropic forms of hearing loss are warranted.