Hydropic Ear Disease: Correlation Between Audiovestibular Symptoms, Endolymphatic Hydrops and Blood-Labyrinth Barrier Impairment.

Thijs O Verhagen,Maartje Houben,Claire C Bommeljé,Henk M Blom,Jelle J Goeman,Lisa M H De Pont,Mark A Van Buchem,Sebastiaan Hammer,Josephine M Van Steekelenburg,Berit M Verbist

doi:10.3389/fsurg.2021.758947

Thijs O Verhagen, Maartje Houben + Show 8 more

Open Access

https://doi.org/10.3389/fsurg.2021.758947

Copy DOI

Abstract

Research 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.

Highlights

Menière’s disease (MD) is a refractory otologic disorder that predominantly manifests in adults between 40 and 60 years of age [1, 2]
The degree of endolymphatic hydrops (EH) positively correlates with disease duration, hearing loss and the incidence of drop attacks
The blood-labyrinth barrier (BLB) is impaired in association with EH grade, but without clear contribution to the severity of audiovestibular symptoms

Summary

Introduction

Menière’s disease (MD) is a refractory otologic disorder that predominantly manifests in adults between 40 and 60 years of age [1, 2]. Its typical features are recurrent vertigo spells associated with fluctuating cochlear symptoms in the affected ear [3,4,5,6]. The disease may affect one ear, albeit bilaterality has been reported in 2–73% of cases [7]. The most striking features of MD are rotatory vertigo and hearing loss, which show great variability among patients with respect to onset, duration and severity [10, 11]. In initial stages of the disease, hearing loss is typically reversible after each vertigo attack, but profound and permanent hearing loss develops [13]. Patients may experience a myriad of symptoms consisting of (but not limited to) tinnitus, aural fullness, or a sudden fall without loss of consciousness known as drop attacks [14,15,16]. Migraine and autoimmune diseases are common comorbidities, reported in up to 16 and 11% of patients respectively [17,18,19,20,21]

Objectives

Methods

Results

Discussion

Conclusion