Research Objective: To investigate the correlation between clinical features and MRI-confirmed endolymphatic hydrops (EH) and blood-labyrinth barrier (BLB) impairment.Study Design: Retrospective... Show moreResearch Objective: To investigate the correlation between clinical features and MRI-confirmed endolymphatic hydrops (EH) and blood-labyrinth barrier (BLB) impairment.Study Design: Retrospective cross-sectional study.Setting: Vertigo referral center (Haga Teaching Hospital, The Hague, the Netherlands).Methods: We retrospectively analyzed all patients that underwent 4 h-delayed Gd-enhanced 3D FLAIR MRI at our institution from February 2017 to March 2019. Perilymphatic enhancement and the degree of cochlear and vestibular hydrops were assessed. The signal intensity ratio (SIR) was calculated by region of interest analysis. Correlations between MRI findings and clinical features were evaluated.Results: Two hundred and fifteen patients with MRI-proven endolymphatic hydrops (EH) were included (179 unilateral, 36 bilateral) with a mean age of 55.9 yrs and median disease duration of 4.3 yrs. Hydrops grade is significantly correlated with disease duration (P < 0.001), the severity of low- and high-frequency hearing loss (both P < 0.001), and the incidence of drop attacks (P = 0.001). Visually increased perilymphatic enhancement was present in 157 (87.7%) subjects with unilateral EH. SIR increases in correlation with hydrops grade (P < 0.001), but is not significantly correlated with the low or high Fletcher index (P = 0.344 and P = 0.178 respectively). No significant differences were found between the degree of EH or BLB impairment and vertigo, tinnitus or aural fullness.Conclusion: The degree of EH positively correlates with disease duration, hearing loss and the incidence of drop attacks. The BLB is impaired in association with EH grade, but without clear contribution to the severity of audiovestibular symptoms. Show less
Pont, L.M.H. de; Steekelenburg, J.M. van; Verbist, B.M.; Buchem, M.A. van; Blom, H.M.; Hammer, S. 2020
Purpose of ReviewMeniere's disease (MD) is a burdensome and not well understood inner ear disorder that has received increasing attention of scientists over the past decade. Until 2007, a certain... Show morePurpose of ReviewMeniere's disease (MD) is a burdensome and not well understood inner ear disorder that has received increasing attention of scientists over the past decade. Until 2007, a certain diagnosis of endolymphatic hydrops (EH) required post-mortem histology. Today, dedicated high-resolution magnetic resonance imaging (MRI) protocols enable detection of disease-related changes in the membranous labyrinth in vivo. In this review, we summarize the current status of MR imaging for MD.Recent FindingsThe mainstays of hydrops imaging are inversion recovery sequences using delayed acquisition after intravenous or intratympanic contrast administration. Based on these techniques, several methods have been developed to detect and classify EH. In addition, novel imaging features of MD, such as blood-labyrinth barrier impairment, have recently been observed.SummaryDelayed contrast enhanced MRI has emerged as a reliable technique to demonstrate EH in vivo, with promising application in the diagnosis and follow-up of MD patients. Therefore, familiarity with current techniques and diagnostic imaging criteria is increasingly important. Show less
Steekelenburg, J.M. van; Weijnen, A. van; Pont, L.M.H. de; Vijlbrief, O.D.; Bommelje, C.C.; Koopman, J.P.; ... ; Hammer, S. 2020
BACKGROUND AND PURPOSE:M?ni?re disease is characterized by endolymphatic hydrops, whereas perilymphatic enhancement on MR imaging has been suggested to be of additional value in diagnosing M?ni?re... Show moreBACKGROUND AND PURPOSE:M?ni?re disease is characterized by endolymphatic hydrops, whereas perilymphatic enhancement on MR imaging has been suggested to be of additional value in diagnosing M?ni?re disease. This study evaluates the presence of endolymphatic hydrops and perilymphatic enhancement in patients with M?ni?re disease and with other vertigo-associated inner ear pathology.MATERIALS AND METHODS:A 3D-FLAIR sequence 4?hours after intravenous gadolinium injection was performed to visualize the endolymph and perilymph in 220 patients suspected of having M?ni?re disease. Patients? ears were retrospectively categorized as having M?ni?re disease (probable or definite) or other vertigo-associated inner ear pathology not attributable to M?ni?re disease. Endolymphatic hydrops was evaluated using a visual classification system, and perilymphatic enhancement was scored both visually and quantitatively.RESULTS:Endolymphatic hydrops was present in 137 (91.9%) of the definite M?ni?re disease ears and in 9 (7.0%) of the ears with other vertigo-associated inner ear pathology (P Show less
