Visualization of endolymphatic hydrops and correlation with audio-vestibular functional testing in patients with definite Meniere's disease

doi:10.1016/j.anl.2012.07.009

Auris Nasus Larynx

Volume 40, Issue 2, April 2013, Pages 167-172

https://doi.org/10.1016/j.anl.2012.07.009 Get rights and content

Abstract

Objectives

Visulization of endolymphatic hydrops has been reported using magnetic resonance imaging (MRI) in patients with Meniere's disease. However, the relationship between the endolymphatic hydrops visualized by MRI and audio-vestibular functional tests have not been sufficiently investigated, such as pure tone audiometry (PTA), electrocochleography (EcoG), and vestibular evoked myogenic potential (VEMP).

Methods

Here we attempted to visualize endolymphatic hydrops in patients with Meniere's disease using 3 Tesla (3 T)-MRI following intratympanic gadolinium-diethylene-triamnie petaaetic acidbis (Gd-DTPA) injection and correlated the presence of hydrops with auditory testing. Following intratympanic Gd-DTPA contrast injections in 26 patients diagnosed as having definite Meniere's disease, 3D-fluid-attenuated inversion recovery (3D-FLAIR) sequence imagings were performed. The PTA, EcoG, and VEMP testing was done bilaterally. Patients had typical results of auditory testing for Meniere's disease.

Results

The 3D-FLAIR MRI clearly demonstrated endolymphatic hydrops with signal voids in the cochlea (81%) and saccule (69%) in the definite Meniere's disease group. Auditory tests verified that the visualized cochlear hydrops (basal turn) was correlated with a high tone hearing threshold in PTA and an abnormal EcoG. Though the absence of VEMP did not correlate with vestibular hydrops on MRI, large vestibular hydrops were detected by imaging. This study demonstrates that endolymphatic hydrops could be visualized by 3D-FLAIR MRI in Meniere's disease patients.

Conclusion

Introduction

In 1861, Prosper Meniere described the classic triadic symptomatology of disabling attacks of vertigo, fluctuating hearing loss and tinnitus, which he attributed to a labyrinthine disorder. Its cause has been believed to be related to endolymphatic hydrops, resulting from a malfunction of the endolymph volume regulation mechanisms [1]. According to the 1995 American Academy of Otolaryngology-Head and Neck Surgery (AAO-HNS) criteria for the diagnosis of Meniere's disease in [2], histopathologic confirmation of endolymphatic hydrops is necessary for a “certain” diagnosis of Meniere's disease; there are no useful objective examinations or methods for diagnosis of “definite” or “probable” Meniere's disease. These diagnoses are usually based on patients’ subjective symptoms, such as episodes of vertigo and hearing loss, or physical examination. There is a need for more objective confirmatory tests to diagnosis patients with possible Meniere's disease.

Recent advances in magnetic resonance imaging (MRI) techniques, with increasing magnetic field strength and technical developments of various imaging sequences, have enabled visualization of the delicate anatomy of the inner ear structures. Nakashima et al. [6] and Naganawa et al. [3] have reported that endolymphatic spaces can be visualized using the 3D-FLAIR sequence following intratympanic injection of gadolinium-diethylene-triamnie petaaetic acidbis (Gd-DTPA); enlarged endolymphatic spaces were identified in patients with heterogeneous and mixed Meniere's disease.

Hearing loss in pure tone audiometry (PTA) is a distinguishing test for Meniere's disease, which demonstrates a fluctuating pattern of hearing loss. The electrocochleography (EcoG) is known to be a test with a high sensitivity for denfinite Meniere's disease, especially in cochlear Meniere's disease. To evaluate vestibular lesions, Vestibular Evoked Myogenic Potential (VEMP) testing can be used. This is an inhibitory sacculocollic reflex recorded in the ipsilateral sternocleidomastoid muscle in response to acoustic stimulation of the saccule. Abnormal EcoG and VEMP testing may reflect cochlear and saccular hydrops, respectively, and may be a clinically valuable metric of disease severity or progression in patients with Meniere's disease [4], [5].

The purpose of the current study was to correlate positive and negative results in patients with known Meniere's disease using 3 Tesla (3 T)-MRI following intratympanic Gd-DTPA administration to evaluate for hydrops as well as various auditory vestibular tests (PTA, EcoG and VEMP) and to evaluate the reliability of visualized abnormalities (hydrops) using the auditory vestibular testing.

Section snippets

Subject selection and controls

Twenty-six patients with definite Meniere's disease, according to the diagnostic criteria proposed by the AAO-HNS, were evaluated by MR imaging and three auditory vestibular functional tests (PTA, EcoG, and VEMP). After reviewing histories and symptoms, such as vertigo, tinnitus, sensation of ear fullness, and fluctuating hearing loss (PTA), we selected the definite Meniere's disease group from patients treated for Meniere's disease in our hospital from 2006 to 2008. Of these, the study group

Visualization of endolymphatic hydrops

A total of 21/26 (81%) of the definite Meniere's disease patients had perilymphatic spaces enhanced and endolymphatic spaces demonstrating a signal void in the affected cochleas by MRI. Dilated endolymphatic spaces of the cochlea were seen wholly or partially in 21 patients (Fig. 1). A total of 18/26 (69%) patients had visualized endolymphatic hydrops of vestibular spaces (especially saccular lesions) on 3D-FLAIR imaging. 3 patients and 11 patients among these had severe dilated vestibular

Discussion

Meniere's disease was first described by Prosper Ménière in 1861. In 1938, post-mortem histological examination revealed endolymphatic hydrops in patients with Meniere's disease; this has been considered a major cause of Meniere's disease in recent years [10]. A large number of factors has been proposed as leading to the development of endolymphatic hydrops. The list includes excessive endolymph production, decreased endolymph absorption by the endolymphatic sac, ionic imbalance, genetic

Conclusion

Cochlea hydrops and vestibular (saccular) hydrops are readily visualized using these techniques. Hydrops, as visualized on MRI, may be a reliable means to diagnosis Meniere's disease; this is supported by appropriate correlations with auditory vestibular functional testing. We are sure that this method will reveal the pathophysiology of Meniere's disease as well as help to diagnosis the disease.

Conflict of interest

The authors have no funding, financial relationships, or conflicts of interest to disclose.

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Cited by (70)

Audio-vestibular and radiological analysis in Meniere's disease
2022, Brazilian Journal of Otorhinolaryngology
Citation Excerpt :
In studies evaluating patients with MD, abnormal responses in the cVEMP test vary in an extensive range.34,35 For example, De Waele et al. reported abnormal results in 54% of patients,36 while Seo et al. detected them at a rate of 96%.31 In the current study, there was an abnormal response in 81.3% (13/16) of the symptomatic ears.
Intravenous gadolinium-enhanced inner ear magnetic resonance imaging (IV Gd-enhanced inner ear MRI) is a new technique for diagnosing Meniere’s disease (MD). Vestibular tests have also long been used for MD, but which tests should be included in the oto-neurological test battery remains controversial. The evaluation method to be used to confirm the clinical diagnosis in MD is not clear. This study aimed to examine the results of vestibular tests and IV Gd-enhanced inner ear MRI in individuals diagnosed with unilateral definite Meniere's disease.
IV Gd-enhanced inner ear MRI (Endolymphatic Hydrops [EH] and Perilymphatic Enhancement [PE]), conventional audiometry (0.25–8 kHz), video Head Impulse Test (vHIT), cervical Vestibular Evoked Myogenic Potential (cVEMP), air caloric test, and dizziness handicap inventory were applied to 16 adult patients diagnosed with unilateral definite MD.
Among the patients with definite MD, EH (cochlear and/or vestibular) was identified in 93.7% and 68.7% of the symptomatic and the asymptomatic ears, respectively. There was a positive correlation between the hearing thresholds at 2, 4, 6 and 8 kHz and the degree of cochlear EH (p < 0.05). PE (cochlear and/or vestibular) was observed in 37.5% of the asymptomatic and symptomatic ears. The sensitivity of the vestibular test battery (vHIT, cVEMP, and caloric test) was 100% and its specificity was 50%, while the sensitivity of the IV Gd-enhanced inner ear MRI (EH and PE together) was 93.8% and the specificity was 81.3%.
MRI had higher sensitivity and specificity than the vestibular test battery. PE or vHIT alone was not considered to be reliable in the diagnosis of MD. In suspected MD, the clinical history, hearing tests, and IV Gd-enhanced inner ear MRI are sufficient for diagnosis. If MRI technique is not possible, vestibular tests (caloric test and cVEMP, not vHIT) can provide reliable results when evaluated together.
Correlation between magnetic resonance imaging classification of endolymphatic hydrops and clinical manifestations and audiovestibular test results in patients with definite Ménière's disease
2022, Auris Nasus Larynx
Citation Excerpt :
No significant differences were found between groups without EH and with mild EH in the cochlea. Seo et al. [13] divided patients with definite MD into two groups with visualized and non-visualized cochlear hydrops and found significant differences in TT-ECochG results between these subpopulations. In our study, vestibular EH was assessed with the Barath scale and, more precisely, using the four-grade Bernaerts scale.
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).
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.
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.
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.
Differences in responsiveness of intratympanic steroid injection for intractable vertigo in Meniere's disease
2021, American Journal of Otolaryngology - Head and Neck Medicine and Surgery
The efficacy of intratympanic steroid (ITS) injection for intractable Meniere's disease has been reported; however, its differences in responsiveness are not fully understood. This study investigated the clinical characteristics of patients who responded to ITS injection treatment.
This retrospective study included 32 patients with Meniere's disease who were unable to control frequent vertigo attacks despite conservative treatment for at least 3 months. They received an intratympanic injection of dexamethasone (3.3 mg/mL) in the affected side at least three times. We measured hearing threshold, subjective symptom scores, cervical and ocular vestibular evoked myogenic potential (cVEMP and oVEMP), and performed glycerol and bithermal caloric tests.
Satisfactory control of vertigo for 1 year after the first round of injection was found in 18 patients (56.3%; the response group). However, the injections failed to control vertigo in the other 14 patients (43.8%; the non-response group), and they were then treated with middle ear micropressure therapy. The response group showed improvement in low-frequency hearing, whereas hearing acuity did not change in the non-response group. Significantly reduced amplitude of cVEMP on the affected side was found in 62.5% of patients in the response group; however, no patients in the non-response group showed reduced amplitude of cVEMP.
ITS injection significantly improved the subjective symptoms for intractable Meniere's disease; however, the long-term effects were heterogeneous. Our results suggest that reduced amplitude in cVEMP is associated with the effectiveness of ITS injection treatment.
Different findings of morphological changes and functional decline in the vestibule and the semicircular canal in ipsilateral delayed endolymphatic hydrops
2020, Clinical Neurophysiology
To elucidate the pathophysiological process by analyzing the correlation between morphological and functional changes in patients with delayed endolymphatic hydrops (DEH).
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.
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.
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.
Our findings can provide a new perspective on the functional and morphological characteristics of patients with DEH.
Menière's disease patients improve specific posturographic parameters following diagnostic intratympanic injection
2020, American Journal of Otolaryngology - Head and Neck Medicine and Surgery
Citation Excerpt :
In particular, the MRI performed with the contrast medium, indicated the diffusion of Gd in the structures of the inner ear only in those 10 patients bearing the hydrops. The possibility of not observing the diffusion of the contrast medium has already been reported [24]. In all cases the clinical signs of hydrops were present, indeed, the remaining two patients that did not present the diffusion of the contrast agent reported to have suffered from otitis in childhood.
Evaluation of specific computerized posturographic parameters in patients with Menière's disease (MD) following the intratympanic injection of gadolinium, a contrast agent, used in radiological diagnosing.
We have observed 12 adult patients with unilateral Menière's Disease subjected to inner ear magnetic resonance imaging (MRI) examination after intratympanic gadolinium injection (ITG). The diagnoses have been performed according to the guidelines of the American Academy of otolaryngology. Before and after 24 h the ITG, all patients were subjected to the clinical evaluation and computerized posturography (CP), in 4 conditions depending on open/closed eyes and with/without foam cushion under feet.
After ITG, in the affected ear the MRI confirmed the endolymphatic hydrops revealing a thin or even disappeared perilymphatic space. The statokinesigram showed improvement of stability only with closed eyes on a foam cushion. The CP performed 24 h after the contrast intratympanic injection showed a significant reduction of Path Length and Confidence Ellipse Area, due to an improvement of vestibular function on static balance. This improvement could be directly dependent to intratympanic pressure modification mediated by volume of contrast liquid, by “columella effect”.
This study demonstrates the absence of vestibular damage in patients undergoing intratympanic gadolinium infiltration and confirms the relationship between intratympanic pressure and vestibular stability modifications providing positive evidences for an applicative use of CP as a functional assessment to better address diagnosis and follow-up in MD patients treated with intratympanic injections.
Endolymphatic hydrops imaging and correlation with clinical characteristics, audiovestibular function and mental impairment in patients with Meniere’s disease
2023, European Archives of Oto-Rhino-Laryngology

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Visualization of endolymphatic hydrops and correlation with audio-vestibular functional testing in patients with definite Meniere's disease

Abstract

Objectives

Methods

Results

Conclusion

Introduction

Section snippets

Subject selection and controls

Visualization of endolymphatic hydrops

Discussion

Conclusion

Conflict of interest

Lancet

Otolaryngol Head Neck Surg

Hear Res.

Am J Otolaryngol

Committee on Hearing and Equilibrium guidelines for the diagnosis and evaluation of therapy in Meniere's disease. American Academy of Otolaryngology-Head and Neck Foundation, Inc.

Otolaryngol Head Neck Surg

Imaging of endolymphatic and perilymphatic fluid at 3 T after intratympanic administration of gadolinium-diethylene-triamine pentaacetic acid

Am J Neuroradiol

Vestibular evoked myogenic potential (VEMP) in patients with Meniere's disease with drop attacks

Laryngoscope

Significance of some otologic function tests in dignosis of Meniere's disease

Lin Chuang Er Bi Yan Hou Ke Za Zhi

Visualization of endolymphatic hydrops in patients with Meniere's disease

Laryngoscope

Progression of endolymphatic hydrops in Ménière's disease as evaluated by magnetic resonance imaging

Otol Neurotol

In vivo visualization of endolyphatic hydrops in patients with Meniere's disease:correlation with audiovestibular function

Eur Arch Otorhinolaryngol

Diagnostic significance of transtympanic electrocochleography in Meniere's disease

Ann Otol Rhinol Laryngol