Objectives Endolymphatic sac decompression surgery (ESDS) is one of the surgical methods for intractable Menière’s disease (MD), and it is known as a relatively safe treatment that does not cause hearing loss. However, the effectiveness and the degree of vertigo control rate of ESDS are still controversial. In this study, we aimed to evaluate the clinical outcomes of ESDS in intractable MD.
Methods We retrospectively reviewed 33 patients who underwent ESDS for intractable MD from January 2002 to March 2022. Clinical characteristics of patients, pure tone threshold, medical treatment method, and number of vertigo attacks before and after surgery were assessed. The improvement of hearing and vertigo was evaluated according to the 1995 American Academy of Otolaryngology-Head and Neck Surgery criteria.
Results Of the 33 patients, the average follow-up period was 21.2 months, with immediate follow-up within 2 months (28 patients), short term between 2 and 6 months (27 patients), and long term at 12 months or later (29 patients). In the immediate hearing threshold, both air conduction and bone conduction showed slight deterioration, but there was no significant change in the long-term hearing threshold. At long-term follow-up, 12 patients (41.4%) were able to live without medication, and 18 patients (62.1%) showed improvement in their vertigo symptoms. In addition, patients who showed improvement in hearing also showed improvement in vertigo at the same time.
Conclusions ESDS in intractable MD is a relatively safe and effective treatment method for reducing vertigo attack without worsening hearing threshold.
Vestibular migraine and Menière’s disease have similar clinical features which are recurrent dizziness or auditory symptoms, so it is challengeable to establish the correct diagnosis. Herein, a 31-year-old male and a 56-year-old female showed recurrent dizziness with auditory symptoms and suffered from vestibular migraine. They met the diagnostic criteria for vestibular migraine, but the Menière’s diagnostic criteria were not satisfied as there was no hearing loss. Delayed intravenous gadolinium enhanced magnetic resonance imaging of the inner ear was taken to find out of correlation of the inner ear and revealed endolymphatic hydrops. This case can improve the understanding of the pathophysiology of a vestibular migraine associated Menière’s disease.
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Functional and Molecular Markers for Hearing Loss and Vertigo Attacks in Meniere’s Disease Chao-Hui Yang, Ming-Yu Yang, Chung-Feng Hwang, Kuang-Hsu Lien International Journal of Molecular Sciences.2023; 24(3): 2504. CrossRef
Ménièreʼs disease is a poorly understood disorder of the inner ear characterized by intermittent episodic vertigo, fluctuating hearing loss, ear fullness and tinnitus. In this paper, we present a review of the histopathology, cytochemistry, gene, blood-labyrinthine barrier and imaging of Ménièreʼs disease. Histopathology is significant for neuroepithelial damage with hair cell loss, basement membrane thickening, perivascular damage and microvascular damage. Cytochemical alterations are significant for altered AQP4 and AQP6 expression in the supporting cell, and altered cochlin and mitochondrial protein expression. Since the discovery of aquaporin water channels (AQP1, AQP2, AQP3, AQP4, AQP5, AQP6, AQP7 and AQP9), it has become clear that these channels play a crucial role in inner ear fluid homeostasis. Several gene studies related to Ménièreʼs disease have been published, but there is no clear evidence that Ménièreʼs disease is associated with a special gene. Currently, imaging techniques to determine the extent and presence of endolymphatic hydrops are being studied, and further studies are needed to correlate the visualization of the endolymphatic hydrops with clinical symptoms.
Objective: There were few recent study concern about usefulness of cochlear hydrops analysis masking procedure (CHAMP) in progression of Ménièreʼs disease. The purpose of this study is to analyze changes in the CHAMP as advancement of Ménièreʼs disease.
Methods We studied 19 cases of ‘Definite’ group of Ménièreʼs disease. We assumed progression of Ménièreʼs disease would be checked by pure tone threshold by four tone average (4PTA), low tone average (LPTA). We also compared electrocochleography (ECoG), dizziness handicap inventory (DHI), tinnitus handicap inventory (THI) as parameters for progression of Ménièreʼs disease to CHAMP latency delay and amplitude ratio. Chi-square test was used as a statistical method.
Results In the group of patients had abnormal amplitude ratio, ECoG value were not improved, but there was not statistically significant (odds ratio [OR]=5.727, p>0.05). Better DHI and THI score were not necessarily construed as the improvement value in the CHAMP (p>0.05). In amplitude ratio abnormal group, 4PTA was aggravated, but not statistically significant (OR=1.5, p>0.05). In the group of patient had abnormality in both latency delay and amplitude ratio, LPTA was relatively aggravated (OR in latency delay: 2, OR in amplitude ratio: 10); however, reveals no statistically significance between them (p>0.05). Change of ECoG, hearing threshold including 4PTA and LPTA with progression of Ménièreʼs disease were not correlated significantly with latency delay or amplitude ratio of CHAMP.
Conclusion We conclude that CHAMP does not reflect clinical features with progression of Ménièreʼs disease.
Inner ear is composed of cochlea, vestibule, and endolymphatic sac which are enclosed by thin layer of epithelial cells. The enclosed space is filled with fluid named as endolymph where the [K+] is high and [Na+] is low. This unique ion composition is very important in maintaining normal hearing and balance function by providing K+ ions into sensory hair cells, which finally depolarize hair cells to facilitate the transport of sound and acceleration stimulation to central nervous system. The ion composition of inner ear is maintained by various ion transport through ion channels, transporters, and exchangers in the inner ear sensory and extra-sensory epithelium. The disruption of normal endolymphatic ion composition by the deterioration of the function of those ion channels can cause dysfunction of sensory epithelium, which consequently results in hearing and balance disorders. One of the possible pathology from the disruption of inner ear ion homeostasis is endolymphatic hydrops which is a phenomenon of excessive fluid accumulation of inner ear. The dysfunction of ion channels in inner ear epithelium can be an etiology of Ménièreʼs disease since endolymphatic hydrops is a main pathological finding of the disease. In this review, we discussed about the possible pathological mechanism of Ménièreʼs disease as a perspective of channelopathy as well as the role of various ion channels in the regulation of inner ear fluid volume based on the findings revealed by electrophysiological studies.
Endolymphatic hydrops is a representing pathologic finding of Ménière's disease. For the induction of endolymphatic hydrops in an animal model, surgical ablation of endolymphatic sac has been used. Although traditional model with the blockage of endolymphatic sac induced severe hydrops, it has several limitations for the study of pathophysiology of Ménière's disease. Recently, modified experimental models have been introduced, in which additional procedure was performed to induce the acute aggravation of hydrops after the surgical ablation. These new models could be helpful to elucidate the mechanism and develop a new treatment of Ménière's disease. In this review, we introduce the characteristics of animal models using surgical ablation of endolymphatic sac from the classical model to novel modified models.
It is known that endolymphatic hydrops is a pathophysiologic mechanism of Meniere’s disease, while the detailed role is not fully clarified. Study for endolymphatic hydrops mainly represents a pathologic histologic finding found only in the post-mortem examination of temporal bone, so endolymphatic hydrops animal models have played important role in terms of understanding how hydrops affects the function of the inner ear. Numerous challenges with diverse animals and techniques have been tried to reveal the possible pathophysiology of endolymphatic hydrops and develop an effective treatment. This review shows historical background and technical advances in experimental animal model for endolymphatic hydrops and Meniere’s disease.
Background and Objectives: Patients with Meniere’s disease often present with a great variety of symptoms, especially at the onset of the disease. However, there is no single test that is definitive for making the diagnosis of Meniere’s disease. Recently, a new diagnostic test, the cochlear hydrops analysis masking procedure (CHAMP) was introduced as a test for endolymphatic hydrops. The aim of this study is to evaluate the diagnostic value of CHAMP test in Meniere’s disease in comparison with that of electrocochleography.
Materials and Methods: The CHAMP test and electrocochleography were simultaneously performed in 14 cases of definite Meniere’s disease and 8 normal healthy volunteers (16 ears). Positive criteria were defined as being more than 0.35 in summating potential/action potential (SP/AP) ratio, 0.3 ms or less in latency delay and 0.95 or less in complex amplitude ratio. Measured parameters from each test were compared between the groups, and sensitivity and specificity of each test in diagnosing Meniere’s disease were calculated.
Results: There was no significant difference in the SP/AP ratio of electrocochleography between the Meniere’s disease and control groups. In CHAMPtest, the latency delay was shorter and the complex amplitude ratio was smaller (p<0.001). While taking positive results from both test parameters, the sensitivity and specificity of CHAMP test were 85.7% and 87.5%, respectively.
Conclusion: The CHAMP seems to be a clinically useful tool in diagnosing Meniere’s disease and might be better than electrocochleography to detect endolymphatic hydrops.
Lermoyez’s syndrome is usually regarded as a rare variant of Meniere’s disease which is characterized by the disappearance of aural symptoms after vertiginous attacks. It is recognized as an uncommon paroxysmal clinical syndrome caused by pathological endolymphatic change in the labyrinth. It is thought that the cause of Lermoyez’s syndrome is due to cochlea hydrops which result from endolymphatic blockage of the ductus reunions. With its distinct characteristic manifestations, the diagnosis is not difficult, but Lermoyez’s syndrome has been rarely reported universally. Otologists are in a whirl about its rare occurrence. We report a unique case of a 46-year-old woman, who showed typical clinical features of Lermoyez’s syndrome with review of literatures. We focused on the sequential clinical change during our observation.
Posterior fossa meningioma constitute approximately 10% of meningiomas. It can rarely involve the endolymphatic sac and produce symptoms of Meniere’s disease. We present the case of a 41-year-old male patient who was suffering from recurrent vertigo. The pure tone audiogram revealed low tone hearing loss on right side and temporal magnetic resonance image revealed a meningioma in posterior fossa. Posterior fossa meningioma should be considered in the differential diagnosis of patients with signs and symptoms of Meniere’s disease.
Endolymphatic hydrops is believed to be underlying pathophysiology of Meniere’s disease. The degree of endolymphatic hydrops observed by MRI (magnetic resonance imaging) in patients with Meniere’s disease offers a new method to study the progression of Meniere’s disease. There have been multiple attempts to diagnose Meniere’s disease with the help of radiologic tests including MRI in Korea. We describe a case report of a patient with Meniere’s who showed changes of endolymphatic sac in MRI after endolymphatic sac decompression.
Post-operative dizziness and vertigo have been variously reported from 30 to 60% after cochlear implantation. There have been reported that delayed vertigo is the most common type and characterized by similarities with dizziness in Meniere’s syndrome. Authors experienced a man who had delayed onset of vertigo, which developed six months after cochlear implantation on the left side. Postoperative left caloric response was decreased in both warm and cold stimulation. The direction of spontaneous nystagmus during spell was right side with torsional down beat and definite catch up saccades were observed during head thrust test on the plane of the left horizontal canal and posterior canal, which was resolved on the next day. These episodic and recurrent spells of vertigo might suggest that the hydropic changes of labyrinth corresponding to the pathology of Meniere’s disease occurred in this patient.
Key Words: Vertigo; Cochlear implantation; Endolymphatic hydrops
Background and Objectives: Endolymphatic hydrops are known as major causes of Meniere’s disease. MRI (Magnetic resonance imaging) with contrast tried recently makes it possible to visualize perilymphatic and endolymphatic space without invasive procedures. There are no tryouts in the interior of our country. We attempted MRI after injection of gadolinium-diethylen –triamine pentaacetic acid (Gd-DTPA) in normal adults and patients with Meniere’s disease to make sure 3D-FLAIR (fluid-attenuated inversion recovery) MRI parameters and to visualize endolymphatic spaces.
Materials and Methods: Five normal adults and Five patients with Meniere’s disease were included in this study. Twenty-four hours after Gd-DTPA intratympanic injection, we performed 3D-FLAIR and 3D-IR imaging at 3T. MRI region of interest signal intensity was used to determine the diffusion of Gd-DTPA into the perilymphatic fluid spaces over time.
Results: Five of five in normal group, using 3D-IR MRI after Gd injection, had enhanced imagings (perilymphatic spaces) of inner ears. Five of five in patients group, using 3D-IR after Gd injection, had enhanced perilymphatic spaces and non-enhanced endolymphatic hydrops.
Conclusions: Delayed contrast imaging of the inner ear with 3D-IR MRI after Gd-DTPA intratympanic injection revealed in vivo visualization of endolymphatic hydrops.
Key words: Endolymphatic hydrops, 3D-FLAIR MRI, Gd-DTPA, Intratympanic injection
Author divided the history of Meniere's disease into a three part based on two important historical affairs, the novel concept of Prosper Meniere which reported in 1861, and the discovery of temporal bone histopathology of endolymphatic hydrops by Hallpike and Yamakawa in 1938. This article traces the period since Prosper Meniere's report until discovery of endolymphatic hydrops.