Association between the Putative Meningeal Lymphatics at the Posterior Wall of the Sigmoid Sinus and Delayed Contrast-agent Elimination from the Cerebrospinal Fluid.

PURPOSE: To investigate the characteristics of the putative meningeal lymphatics located at the posterior wall of the sigmoid sinus (PML-PSS) in human subjects imaged before and after intravenous administration (IV) of a gadolinium-based contrast agent (GBCA). The appearance of the PML-PSS and the enhancement of the perivascular space of the basal ganglia (PVS-BG) were analyzed for an association with gender, age, and clearance of the GBCA from the cerebrospinal fluid (CSF). METHODS: Forty-two patients with suspected endolymphatic hydrops were included. Heavily T2-weighted 3D-fluid attenuated inversion recovery (hT2w-3D-FLAIR) and 3D-real inversion recovery (IR) images were obtained at pre-administration, immediately post-administration, and at 4 and 24 hours after IV-GBCA. The appearance of the PML-PSS and the presence of enhancement in the PVS-BG were analyzed for a relationship with age, gender, contrast enhancement of the CSF at 4 hours after IV-GBCA, and the washout ratio of the GBCA in the CSF from 4 to 24 hours after IV-GBCA. RESULTS: The PML-PSS and PVS-BG were seen in 23 of 42 and 21 of 42 cases, respectively, at 4 hours after IV-GBCA. In all PML-PSS positive cases, hT2w-3D-FLAIR signal enhancement was highest at 4 hours after IV-GBCA. A multivariate analysis between gender, age, CSF signal elevation at 4 hours, and washout ratio indicated that only the washout ratio was independently associated with the enhancement of the PML-PSS or PVS-BG. The odds ratios (95% CIs; P value) were 4.09 × 10-5 (2.39 × 10-8 - 0.07; 0.0078) for the PML-PSS and 1.7 × 10-4 (1.66 × 10-7 - 0.174; 0.014) for the PVS-BG. CONCLUSION: The PML-PSS had the highest signal enhancement at 4 hours after IV-GBCA. When the PML-PSS was seen, there was also often enhancement of the PVS-BG at 4 hours after IV-GBCA. Both observed enhancements were associated with delayed GBCA excretion from the CSF.

Volume ratio and distribution rate in patients with orthostatic vertigo/dizziness using MR imaging: a comparison with vertiginous diseases.

BACKGROUND: Orthostatic dizziness/vertigo (ODV) is characterized by lightheadedness owing to postural changes. AIMS/OBJECTIVES: To measure the endolymphatic space (ELS)/total fluid space (TFS) volume ratio and the distribution rate of endolymphatic fluid (ELF) of patients with ODV and compare them with those of control subjects (CS). MATERIALS AND METHODS: This study included 22 patients (44 ears) with ODV and 52 controls (104 ears, CS). The ELS/TFS volume ratio (%) and distribution rate (%) of the inner ear components were measured using 3-dimensional magnetic resonance imaging. RESULTS: In the ODV group, the mean ELS/TFS volume ratios of the cochlea, vestibule, and semi-circular canals (SCCs) were 12.1%, 18.6%, and 18.1%, respectively; the mean ELS distribution rates for the cochlea, vestibule, and SCCs were 27.3%, 26.2%, and 46.6%, respectively. The ELS distribution rate of the vestibule was significantly lower (p < .01) and the ELS distribution rate of the SCCs was significantly higher in the ODV than in the CS group (p < .01). CONCLUSIONS AND SIGNIFICANCE: The ELS distribution rate in the vestibule + SCCs among patients with ODV did not differ from that in the CS; ELF in the vestibule moved to the SCCs, and a large amount of ELF was distributed only in the SCCs.

Differential Volume Increase of Endolymphatic Compartments in Ménière's Disease Is Inversely Associated With Membrane Thickness.

OBJECTIVES: Our aim in this study was to characterize the morphology of the endolymphatic compartment on histopathology in individuals with Ménière's disease (MD) and to determine why hydrops of the saccule is more pronounced than that of other compartments of the inner ear in MD. METHODS: Temporal bones from 9 patients with idiopathic MD and from 10 individuals without MD/endolymphatic hydrops were examined. The inner ear fluid compartments in normal ears, and ears with MD were three-dimensionally reconstructed and their volume was calculated. The thickness of the membranes of the labyrinth was measured, and both ruptures of the membranes and patency of the utriculoendolymphatic (UEV; Bast's) valve were assessed. RESULTS: In ears with MD, the saccule and the cochlear duct were most frequently hydropic; the utricle was involved approximately half as frequently. In ears without MD, the Reissner's membrane and the membranous wall of the saccule were thinner than that of the utricle and of the lateral semicircular canal ( p < 0.01). The lateral semicircular canal did not show signs of hydrops. In all ears with MD in which the utricle exceeded the average volume of normals (6 of 12), the UEV was open or there was a rupture in the utricle. CONCLUSION: Increases in endolymphatic pressure may cause a primary swelling of the apical cochlear duct and saccule, both of which have relatively thin membranes. Hydrops in the utricle may occur less frequently because of a thicker wall, because of a functioning UEV, and when the saccule has already occupied most of the vestibular perilymphatic space.

Magnetic resonance imaging of endolymphatic hydrops: a comparison of methods with and without gadolinium-based contrast agent administration.

In the evaluation of endolymphatic hydrops (EH) using magnetic resonance (MR) imaging, hybrid of reversed image of positive endolymph signal and native image of perilymph signal multiplied with heavily T2-weighted MR cisternography (HYDROPS-Mi2) imaging with the intravenous administration of a gadolinium-based contrast agent (IV-GBCA) has been utilized. Recently, MR cisternography (MRC) without GBCA has been proposed as a potential alternative method. However, the feasibility of EH evaluation by MRC without GBCA has not been established. The present study aimed to compare HYDROPS-Mi2 imaging with IV-GBCA to MRC without IV-GBCA for the evaluation of EH. In 40 ears of 20 patients with clinically suspected EH, MRC at pre-IV-GBCA and HYDROPS-Mi2 images from 4 h post-IV-GBCA were analyzed. The saccular height on the MRC (SH-MRC) was measured. The percentage of the volume of the endolymphatic space within the whole lymphatic space of the vestibule on the HYDROPS-Mi2 image (%ELvolume-HYD) was measured. The correlation between the SH-MRC and %ELvolume-HYD was calculated. The receiver operating characteristic (ROC) of the SH-MRC and %ELvolume-HYD for the clinical diagnosis of EH was evaluated. The Spearman's rank correlation coefficient between the SH-MRC and %ELvolume-HYD was 0.102. The areas under the ROC curve were 0.570 for the SH-MRC, and 0.926 for the %ELvolume-HYD. In conclusion, there was no significant correlation between the MRC without IV-GBCA and the HYDOROPS-Mi2 with IV-GBCA in the evaluation of EH.

Aldosterone inhibits Dot1l expression in guinea pig cochlea.

BACKGROUND: Aldosterone relieves transcriptional repression of epithelial sodium channel (ENaC) by inhibiting Dot1a and Af9 expression and their interaction with ENaC promoter in various tissues. Expressions of ENaC and Af9 in inner ear have been identified. However, it is not known how Dot1l is regulated by aldosterone in inner ear. METHODS: Twenty-eight adult guinea pigs were randomly divided into the control group and treatment group. Aldosterone 1 mg/kg/d was injected intraperitoneally in the treatment group and saline in the control group for 7 days. Animals were killed 1 month later following auditory brainstem response examination. Histomorphology of cochlea was detected with hematoxylin-eosin staining, and Dot1l expression was examined with immunohistochemistry and Western blot. RESULTS: There was no significant difference in ABR thresholds before and after injection of aldosterone or saline in either group. Endolymphatic hydrops was found in 75% of animals in the treatment group. Dot1l was found in both groups in the stria vascularis, Reissner's membrane, spiral limbus, organ of Corti and spiral ligament. Dot1l expression in the treatment group was decreased by aldosterone. CONCLUSIONS: Dot1l in guinea pig cochlea is inhibited by aldosterone with induction of endolymphatic hydrops. Dot1l may be closely related to endolymph regulation by aldosterone and to pathogenesis of Meniere's disease.

Temporal changes in endolymphatic hydrops on MRI with or without intervention: A systematic review.

OBJECTIVE: The pathophysiology of Meniere's Disease (MD) involves endolymphatic hydrops (ELH) of the inner ear. Magnetic Resonance Imaging (MRI) has been shown to detect ELH, but changes in ELH have been poorly described using this modality. Our objective was to review MRI-measured changes in ELH over time and after medical and/or surgical intervention in patients with MD. We secondarily aim to associate changes in ELH with changes in MD symptomatology. DATABASES REVIEWED: Medline, Web of Science, and Embase databases. METHODS: A systematic review of articles was performed to identify studies utilizing MRI to measure ELH changes over time, and after medical or surgical treatment. Articles on non-human subjects and without direct measurement of ELH were excluded. RESULTS: Of 532 studies identified, 12 were included, involving 170 patients (mean age 56.3 years). Ten studies were prospective; two were retrospective. Five studies strictly utilized medical means of intervention, four utilized surgical treatments, one utilized both, and two observed temporal changes without treatment. Across all interventions, 72.1 % of patients exhibited the same or worsening ELH on imaging. In studies reporting vertigo outcomes, 95.9 % of patients exhibited improvement after the treatment period. CONCLUSION: Medical and surgical interventions often yield symptomatic relief of vertigo in MD patients despite stable or increasing ELH volume. MRI may have greater clinical utility in diagnosing ELH as opposed to assessing treatment response.

Pitfalls of Using T2-contrast Enhancement Techniques in 3D-FLAIR to Detect Endolymphatic Hydrops.

PURPOSE: To determine whether T2-contrast enhancement techniques can be used to diagnose endolymphatic hydrops, we compared fluid signal artifacts with and without T2-contrast enhancement techniques in 3D fluid-attenuated inversion recovery (3D-FLAIR). METHODS: We prepared a custom-made phantom consisting of eight tubes half-filled with saline. Images were obtained using four 3D-FLAIR: without T2-contrast enhancement (Normal), with non-selective T2-inversion recovery (T2-IR), and two with non-selective T2 preparation IR (T2-prep). Scans were performed with and without rice covering the phantom to simulate minimal and severe B0-inhomogeneity conditions. The average signal intensity (SI) values of eight saline tubes were compared between the four sequences and between each other. Comparisons were performed for all measurement slices and the central 10 slices. The images using T2-contrast enhancement technique were obtained from a volunteer and a patient suspected of Meniere's disease. RESULTS: The Normal sequence SI for all slices was significantly lower than that for the other sequences, with smaller standard deviation (SD) and no outliers. Several outliers were detected in the other sequences. The SDs and outliers were larger without rice than with rice. When the central 10 slices with rice, the T2-IR had a significantly higher SI with more outliers compared with the Normal sequence. The T2-prep had no outliers and SIs that were comparable to those of the Normal sequence. However, without rice, the T2-IR and T2-prep sequences had significantly higher SIs with outliers and larger SDs compared to the Normal sequence. In the corresponding images, the Normal sequence achieved excellent fluid suppression, whereas the T2-IR and T2-prep sequences showed high-signal artifacts. Imperfect fluid suppressions were observed in the volunteer image and the endolymphatic hydrops on the post-gadolinium image differed in size and shape in the non-injected T2-IR in the patient image. CONCLUSION: T2-contrast enhancement techniques should be used with caution in 3D-FLAIR for diagnosing endolymphatic hydrops.

Pathological significance and classification of endolymphatic hydrops in otological disorders.

We summarize the presence of endolymphatic hydrops (EH) in otological disorders evaluated with magnetic resonance imaging (MRI) of temporal bones, and propose a classification of EH based on its pathological significance. A search of the literature published in English-language journals was performed using electronic databases, especially focusing on EH-related otological disorders. Clinical articles that contained the terms EH and contrast-MRI published from 2007 to the present, with relevant human temporal bone studies, were included. The following three main points are discussed based on the results in the relevant articles: i) otological disorders that present EH, ii) current grading for evaluation of EH on MRI, and iii) a proposed classification of EH based on its pathological significance. MRI evaluation revealed that EH exists extensively not only in ears with typical Meniere's disease (MD), but also in those with various other otological disorders. The etiological classification of EH helps to summarize ideas for determining the pathophysiology of otological disorders, while a therapeutic classification provides clues to their management. MRI evaluations of EH have led to breakthroughs in investigations of EH in otological disorders. Precise grading for evaluation and clarification of EH on MRI based on its pathological significance could provide keys to elucidating the pathophysiology of EH-related otological disorders.

Space-Occupying Lesions of the Inner Ear Are Easily Misdiagnosed as Endolymphatic Hydrops in a Perilymph-Enhanced Sequence Without the Assistance of a Heavily T2-Weighted Sequence.

OBJECTIVE: The aim of the study was to explore the value of T2-sampling perfection with application-optimized contrasts by using different flip angle evolutions (T2-SPACE) in identifying space-occupying lesions of the inner ear. METHODS: We collected the T2-SPACE and 3-dimensional inversion-recovery sequence with real reconstruction (3D-real IR) images of 220 patients with inner ear symptoms, including 15 patients with inner ear space-occupying lesions. With T2-SPACE images hidden, a senior and junior radiologist made a diagnosis for all patients using only the 3D-real IR images. After 4 weeks the images were shuffled, and T2-SPACE images were made available to the 2 radiologists in addition to 3D-real IR to reconsider the diagnosis for all patients. RESULTS: With the SPACE images hidden, the correct diagnosis rate of the space-occupying lesions was 8/15 (53.3%) for the senior radiologist, whereas it was only 2/15 (13.3%) for the junior radiologist. Without the SPACE images hidden, the correct diagnosis rate of the space-occupying lesions was 15/15 (100.0%) for the senior radiologist, whereas it was 13/15 (86.7%) for the junior radiologist. Of the 15 patients, 7 had only vestibular space-occupying lesions, 2 had only cochlear space-occupying lesions, and 6 had both. No semicircular canal space-occupying lesion was observed. CONCLUSIONS: T2-SPACE can help identify space-occupying lesions of the inner ear that tend to be misdiagnosed as endolymphatic hydrops on 3D-real IR. The senior radiologist had a higher rate for the identification of space-occupying lesions than the junior radiologist when using only 3D-real IR, although the senior radiologist detection rate was still only 53.3%. With the addition of T2-SPACE, both the junior and senior radiologist achieved a high detection rate, which increased to 86.7% and 100%, respectively.

Low-salt diet increases mRNA expression of aldosterone-regulated transporters in the intermediate portion of the endolymphatic sac.

The endolymphatic sac is a small sac-shaped organ at the end of the membranous labyrinth of the inner ear. The endolymphatic sac absorbs the endolymph, in which the ion balance is crucial for inner ear homeostasis. Of the three sections of the endolymphatic sac, the intermediate portion is the center of endolymph absorption, particularly sodium transport, and is thought to be regulated by aldosterone. Disorders of the endolymphatic sac may cause an excess of endolymph (endolymphatic hydrops), a histological observation in Meniere's disease. A low-salt diet is an effective treatment for Meniere's disease, and is based on the assumption that the absorption of endolymph in the endolymphatic sac abates endolymphatic hydrops through a physiological increase in aldosterone level. However, the molecular basis of endolymph absorption in each portion of the endolymphatic sac is largely unknown because of difficulties in gene expression analysis, resulting from its small size and intricate structure. The present study combined reverse transcription-quantitative polymerase chain reaction and laser capture microdissection techniques to analyze the difference of gene expression of the aldosterone-controlled epithelial Na+ channel, thiazide-sensitive Na+-Cl- cotransporter, and Na+, K+-ATPase genes in the three individual portions of the endolymphatic sac in a rat model. A low-salt diet increased the expression of aldosterone-controlled ion transporters, particularly in the intermediate portion of the endolymphatic sac. Our findings will contribute to the understanding of the physiological function of the endolymphatic sac and the pathophysiology of Meniere's disease.

Endolymphatic Hydrops Magnetic Resonance Imaging in Menire's Disease Patients on a Vertigo Attack.

OBJECTIVE: To investigate the characteristics of endolymphatic hydrops (EH) in Menire's disease (MD) patient on a vertigo attack. STUDY DESIGN: Prospective study. SETTING: Tertiary referral center. PATIENTS: Thirty-six MD patients underwent the enhanced magnetic resonance imaging (MRI) examinations of the inner ear on a vertigo attack were enrolled. MAIN OUTCOME MEASURES: All patients met the diagnostic criteria for MD and underwent intravenous gadolinium injection 4 hours before the MRI examinations. The MRI examinations were performed in MD patients on a vertigo attack. RESULTS: Various degrees of vestibular EH appeared in almost all affected ears (2 ears had no EH, 11 ears had mild EH, 26 ears had significant EH). The positive rate of vestibular EH was 37/39 (94.9%). Cochlear EH occurred in 29 ears among 39 affected ears (17 ears had mild EH, 12 ears had significant EH). CONCLUSION: MRI with intravenous gadolinium injection can provide a better assessment of EH in MD patient on a vertigo attack. Vestibular EH seems to be closely related with the vertigo attacks in MD patients, which needs further study.

MR Imaging of Endolymphatic Hydrops in Five Minutes.

In this study, we present images acquired by a fast-imaging method for the evaluation of endolymphatic hydrops after intravenous administration of a single dose of gadolinium-based contrast agent. We utilized the hybrid of reversed image of MR cisternography and a positive perilymph signal by heavily T2- weighted 3D-fluid attenuated inversion recovery-multiplied by T2 (HYDROPS2-Mi2) method combined with deep learning reconstruction denoising. The scan time for the fast protocol was approximately 5 mins, which is far shorter than previously reported scan times. The fast acquisition provides similar image quality and less motion artifacts compared to the longer method.

Cross-sectional Area of the Superior Petrosal Sinus is Reduced in Patients with Significant Endolymphatic Hydrops.

PURPOSE: To evaluate the relationship between the size of the venous structures related to the inner ear and the degree of endolymphatic hydrops (EH). METHODS: Thirty-four patients with a suspicion of EH underwent whole brain MR imaging including the inner ear. Images were obtained pre- and post-administration, and at 4 and 24 hours after the intravenous administration of a gadolinium-based contrast agent (IV-GBCA). The cross-sectional areas (CSA) of the internal jugular vein (IJV), superior petrosal sinus (SPS), and inferior petrosal sinus (IPS) were measured on the magnetization prepared rapid acquisition of gradient echo (MPRAGE) images obtained immediately after the IV-GBCA. The grade of EH was determined on the hybrid of reversed image of positive endolymph signal and native image of positive perilymph signal (HYDROPS) images obtained at 4 hours after IV-GBCA as no, mild, and significant EH according to the previously proposed grading system for the cochlea and vestibule, respectively. The ipsilateral CSA was compared between groups with each level of EH grade. P < 0.05 was considered statistically significant. RESULTS: There were no statistically significant differences between EH grades for the CSA of the IJV or that of the IPS in either the cochlea or the vestibule. The CSA of the SPS in the groups with significant EH was significantly smaller than that in the group with no EH, for both the cochlea (P < 0.01) and the vestibule (P < 0.05). In an ROC analysis to predict significant EH, the cut-off CSA value in the SPS was 3.905 mm2 for the cochlea (AUC: 0.8762, 95% confidence interval [CI]: 0.7952‒0.9572) and 3.805 mm2 for the vestibule (AUC: 0.7727, 95% CI: 0.6539‒0.8916). CONCLUSION: In the ears with significant EH in the cochlea or vestibule, the CSA of the ipsilateral SPS was smaller than in the ears without EH.

Validation of inner ear MRI in patients with Ménière's disease by comparing endolymphatic hydrops from histopathologic specimens.

Intravenous 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.

Magnetic resonance imaging of endolymphatic hydrops in patients with unilateral Meniere's disease: a comparison between with and without herniation into the posterior and lateral semi-circular canals.

BACKGROUND: The presence of endolymphatic hydrops (ELH) in patients with Meniere's disease (MD) is considered a pathological hallmark. AIMS/OBJECTIVES: We aimed to conduct a quantitative volumetric measurement of inner ear ELH in patients with unilateral MD (uMD). The values of uMD with and without herniation into the posterior semi-circular canal (h-PSC) and the lateral semi-circular canal (h-LSC) were compared using 3 D magnetic resonance imaging. MATERIAL AND METHODS: This study included 130 individuals (47 controls and 83 patients with uMD). We measured the total fluid space (TFS) and endolymphatic space (ELS) volumes. We also evaluated the ELS/TFS volume ratios (%). RESULTS: The ELS/TFS volume ratios in the inner ear, cochlea, and vestibule were significantly different between the affected and contralateral sides in patients with h-PSC. Moreover, the ELS/TFS volume ratios of the inner ear, vestibule, and semi-circular canals in the affected ear were significantly higher in patients with h-PSC than in those without h-PSC. The vestibular ELS/TFS volume ratio in the affected ear was significantly higher in patients with h-LSC than in those without h-LSC. CONCLUSIONS AND SIGNIFICANCE: H-LSC is present in extended vestibular ELH. However, this is a result of ELH progression in the inner ear.

Long-term efficacy of triple semicircular canal plugging in the treatment of patients with ipsilateral delayed endolymphatic hydrops.

This study aims to explore the long-term efficacy of triple semicircular canal plugging (TSCP) in the treatment of intractable ipsilateral delayed endolymphatic hydrops (DEH), so as to provide an alternative therapy for this disease. Forty-eight patients diagnosed with ipsilateral DEH referred to vertigo clinic of our hospital between Dec. 2010 and Dec. 2017, were included in this study for retrospective analysis. All patients were followed up for 2 years. Vertigo control and auditory functions were measured and analyzed. Pure tone audiometry, caloric test, and vestibular evoked myogenic potential (VEMP) were performed in two-year follow-up. Forty-five patients who accepted intratympanic gentamicin (26.7 mg/mL) twice given one week apart were selected as a control group. The total control rate of vertigo in TSCP group was 97.9% (47/48) in the two-year follow-up, with complete control rate of 83.3% (40/48) and substantial control rate of 14.6% (7/48). The rate of hearing loss was 22.9% (11/48). The total control rate of vertigo in intratympanic gentamicin group was 80.0% (36/45), with complete control rate of 57.8% (26/45) and substantial control rate of 22.2% (10/45), and the rate of hearing loss was 20.0% (9/45). The vertigo control rate of TSCP was significantly higher than that of intratympanic gentamicin (χ2 = 6.01, p < 0.05). There was no significant difference of hearing loss rate between two groups. (χ2 = 0.12, p > 0.05). TSCP, which can reduce vertiginous symptoms in patients with intractable ipsilateral DEH, represents an effective therapy for this disorder.

Different findings of morphological changes and functional decline in the vestibule and the semicircular canal in ipsilateral delayed endolymphatic hydrops.

OBJECTIVE: To elucidate the pathophysiological process by analyzing the correlation between morphological and functional changes in patients with delayed endolymphatic hydrops (DEH). METHODS: Twenty-nine patients diagnosed with DEH were enrolled in this retrospective study. All patients were assessed using the caloric test, cervical and ocular vestibular evoked myogenic potentials, and gadolinium-enhanced magnetic resonance imaging (MRI) of the inner ear. RESULTS: According to the MRI, the hydrops localization was categorized as hydrops in the vestibule (saccule and utricle) (14%), hydrops in the vestibule and cochlea (72%), and hydrops in the vestibule, cochlea, and lateral semicircular canal (LSCC) (14%). Vestibular hydrops could definitely be observed as function declined; however, a dysfunction of both the saccule and utricle was not always present when vestibular hydrops was detected with MRI. In the LSCC, a decline in functional tests was not necessarily accompanied by morphological abnormalities. However, dysfunction could definitely be detected when LSCC hydrops was observed with MRI. CONCLUSIONS: Hydrops can be found mainly in the vestibule as shown by MRI. In the vestibule, abnormalities are commonly morphologic rather than functional, whereas in the LSCC a functional deterioration can be detected more frequently than morphological changes. SIGNIFICANCE: Our findings can provide a new perspective on the functional and morphological characteristics of patients with DEH.

Early Detection of Endolymphatic Hydrops using the Auditory Nerve Overlapped Waveform (ANOW).

Endolymphatic hydrops is associated with low-frequency sensorineural hearing loss, with a large body of research dedicated to examining its putative causal role in low-frequency hearing loss. Investigations have been thwarted by the fact that hearing loss is measured in intact ears, but gold standard assessments of endolymphatic hydrops are made postmortem only; and that no objective low-frequency hearing measure has existed. Yet the association of endolymphatic hydrops with low-frequency hearing loss is so strong that it has been established as one of the important defining features for Ménière's disease, rendering it critical to detect endolymphatic hydrops early, regardless of whether it serves a causal role or is the result of other disease mechanisms. We surgically induced endolymphatic hydrops in guinea pigs and employed our recently developed objective neural measure of low-frequency hearing, the Auditory Nerve Overlapped Waveform (ANOW). Hearing loss and endolymphatic hydrops were assessed at various time points after surgery. The ANOW detected low-frequency hearing loss as early as the first day after surgery, well before endolymphatic hydrops was found histologically. The ANOW detected low-frequency hearing loss with perfect sensitivity and specificity in all ears after endolymphatic hydrops developed, where there was a strong linear relationship between degree of endolymphatic hydrops and severity of low-frequency hearing loss. Further, histological data demonstrated that endolymphatic hydrops is seen first in the high-frequency cochlear base, though the ANOW demonstrated that dysfunction begins in the low-frequency apical cochlear half. The results lay the groundwork for future investigations of the causal role of endolymphatic hydrops in low-frequency hearing loss.

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.

MRI of endolymphatic hydrops in patients with vestibular schwannomas: a case-controlled study using non-enhanced T2-weighted images at 3 Teslas.

OBJECTIVE: Vestibular schwannomas (VS) may present with similar symptoms endolymphatic hydrops. Association between hydrops and internal auditory canal VS has been described by Naganawa et al. (Neuroradiology 53:1009-1015, 2011), but has never been confirmed since. The aim of this work was to study the prevalence of a saccular dilation on a T2-weighted sequence at 3 T MRI in VS compared to a control group. MATERIALS AND METHODS: All patients presenting with typical VS between May 2009 and July 2018 were included (n = 183) and compared to a control group (n = 53). All underwent a high-resolution T2-weighted 3D sequence (FIESTA-C). The height and width of the saccule were measured on a coronal plane by two radiologists. RESULTS: The saccule was dilated on the side of the schwannoma in 28% of the cases (p = 2.81 × 10- 5), with 15.7% of bilateral dilation. Saccular dilation was correlated to sensorineural hearing loss (OR 3.26, p = 0.02). There was also a significant correlation between saccular hydrops on the normal contralateral side of patients with VS and vertigo (p = 0.049), and between saccular hydrops on the side of the tumour and tinnitus (p = 0.006). CONCLUSION: A third (29%) of VS are associated with a saccular dilation on the side of the tumour, which is an MR sign of endolymphatic hydrops (bilateral in 15.7% of the cases) and it appears related to sensorineural hearing loss and tinnitus, as well as vertigo if a contralateral dilation is present. This opens new therapeutic potentialities with the use of anti-vertiginous drugs, which could have a beneficial effect on the clinical symptoms.