Long-term effects of middle ear pressure therapy with the EFET01 device in patients with Ménière's disease and delayed endolymphatic hydrops in Japan.

BACKGROUND: Long-term efficacy of middle ear pressure therapy (MEPT) with the EFET01 device in patients in Japan with definitive Ménière's disease (MD) and delayed endolymphatic hydrops (DEH) was evaluated. OBJECTIVE: To examine the effects of reducing vertigo attacks and improving hearing of MD and DEH patients by using MEPT with the EFET01 device for two years after treatment. MATERIAL AND METHODS: A retrospective study was conducted of 32 MD patients and 2 DEH patients treated by MEPT with the EFET01 device from December 2018 to April 2021. According to Japan Society for Equilibrium Research (JSER) guidelines, patients were investigated for the frequency of vertigo attacks and change in hearing levels during a period of 6 months before to 18-24 months after start of treatment. RESULTS: The frequency of vertigo attacks significantly decreased in both MD and DEH patients, and hearing level has remained stable in the majority of our patients after treatment. CONCLUSION: Our study clarified that MEPT with the EFET01 device was effective in controlling vertigo symptoms of MD and DEH. It should be considered a safe option for patients failing medical treatment. SIGNIFICANCE: The efficacy of MEPT with the EFET01 was shown over a 2-year follow-up period.

Ménière's disease and allergy: Epidemiology, pathogenesis, and therapy.

The etiology of Ménière's disease (MD) remains controversial. Allergies are potential extrinsic factors that, in conjunction with underlying intrinsic factors, may cause MD. The link between allergies and MD was first described in 1923. For nearly a century, studies have demonstrated a possible link between allergies and MD, even though a causal relationship has not been definitively determined. Previous reviews have mainly focused on clinical epidemiology studies of patients. In this review, we shed light on the association between allergies and MD not only in terms of its epidemiology, but also from an immunology, pathophysiology, and immunotherapy perspective in both patients and animal models. Patients with MD tend to have a high risk of comorbid allergies or an allergy history, showing positive allergy immunology characteristics. Other MD-related diseases, such as migraine, may also interact with allergies. Allergy mediators such as IgE may worsen the symptoms of MD. Deposits of IgE in the vestibular end organs indicate the ability of the inner ear to participate in immune reactions. Allergic challenges can induce vertigo in animals and humans. Anti-allergy therapy plays a positive role in patients with MD and animal models of endolymphatic hydrops.

Meniere's disease: Pathogenesis, treatments, and emerging approaches for an idiopathic bioenvironmental disorder.

Meniere's disease (MD) is a severe inner ear condition known by debilitating symptoms, including spontaneous vertigo, fluctuating and progressive hearing loss, tinnitus, and aural fullness or pressure within the affected ear. Prosper Meniere first described the origins of MD in the 1860s, but its underlying mechanisms remain largely elusive today. Nevertheless, researchers have identified a key histopathological feature called Endolymphatic Hydrops (ELH), which refers to the excessive buildup of endolymph fluid in the membranous labyrinth of the inner ear. The exact root of ELH is not fully understood. Still, it is believed to involve several biological and bioenvironmental etiological factors such as genetics, autoimmunity, infection, trauma, allergy, and new theories, such as saccular otoconia blocking the endolymphatic duct and sac. Regarding treatment, there are no reliable and definitive cures for MD. Most therapies focus on managing symptoms and improving the overall quality of patients' life. To make significant advancements in addressing MD, it is crucial to gain a fundamental understanding of the disease process, laying the groundwork for more effective therapeutic approaches. This paper provides a comprehensive review of the pathophysiology of MD with a focus on old and recent theories. Current treatment strategies and future translational approaches (with low-level evidence but promising results) related to MD are also discussed, including patents, drug delivery, and nanotechnology, that may provide future benefits to patients suffering from MD.

A bibliometric and visualization study of Meniere's disease: Current status and global hotspots and emerging trends.

BACKGROUND: Meniere's disease (MD) is a clinical condition characterized by endolymphatic hydrops. Persistent symptoms negatively affect patients mood, and the underlying etiology remains unclear. It is necessary to comprehensively understand the relevant publications, review the history and current status of research, and analyze hotspots and frontiers of research on MD. METHODS: We retrieved literature on Meniere's disease from 2003 to 2022 from the Web of Science database and extracted the data. Data visualization and analysis was conducted using Cite Space, VOS viewer, an online web tool, and Microsoft Office Power Point 2019. RESULTS: In total, 2847 publications were analyzed. The number of annual publications was relatively stable, with an accelerated upward trend over the past 5 years. The country with the most publications was USA (751, 26.38%), while the University of Munich contributed more publications than any other institution (117, 4.11%). The article titled "Diagnostic criteria for Meniere's disease" by Lopez-Escamez J et al in 2015 was the most cited and co-cited publication, and also had the top co-cited references with the strongest citation bursts. Naganawa S was the author with the most publications (85, 2.99%). The top 3 journals and co-cited journals were Otology Neurotology, Acta Oto Laryngologica, and Laryngoscope. Recently, the key theme words were "sensorineural hearing loss," "therapy," "intratympanic injection method," "vestibular-evoked myogenic potentials," "vestibular migraine," "magnetic resonance imaging," and "meniere's disease." CONCLUSIONS: The US has the largest number of publications and research institutions, many European countries have high-quality journals, and Japan has the highest number of scholars. The international opinion on Meniere's disease is relatively uniform. The stepped-therapy for MD is scientific and clear. Intratympanic injection of steroids and intratympanic injection of gentamicin are commonly used, but steroids are considered safer. Saccular dysfunction may be more common in patients with MD than in those with utricular dysfunctions. It is worth paying attention to study the relationship between MD and vestibular migraine through headache. Progress in magnetic resonance imaging technology is still required for the imaging diagnosis of MD.

Betahistine prevents development of endolymphatic hydrops in a mouse model of insulin resistance and diabetes.

BACKGROUND: Diabetes is associated with inner ear dysfunction. Furthermore, C57BL/6J mice fed high fat diet (HFD), a model for insulin resistance and diabetes, develop endolymphatic hydrops (EH). AIM: Evaluate if betahistine, spironolactone (aldosterone antagonist) and empagliflozin (sodium -glucose cotransporter2 inhibitor) can prevent EH induced by HFD and explore potential mechanisms. METHODS: C57BL/6J mice fed HFD were treated with respective drug. The size of the endolymphatic fluid compartment was measured using contrast enhanced MRI. Secondarily, mice treated with cilostamide, a phosphodiesterase3 inhibitor, to induce EH and HEI-OC1 auditory cells were used to study potential cellular mechanisms of betahistine. RESULTS: HFD-induced EH was prevented by betahistine but not by spironolactone and empagliflozin. Betahistine induced phosphorylation of protein kinaseA substrates but did not prevent cilostamide-induced EH. CONCLUSIONS: Betahistine prevents the development of EH in mice fed HFD, most likely not involving pathways downstream of phosphodiesterase3, an enzyme with implications for dysfunction in diabetes. The finding that spironolactone did not prevent HFD-induced EH suggests different mechanisms for EH induction/treatment since spironolactone prevents EH induced by vasopressin, as previously observed. SIGNIFICANCE: This further demonstrates that independent mechanisms can cause hydropic inner ear diseases which suggests different therapeutic approaches and emphazises the need for personalized medicine.

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.

Evolution of geriatric Meniere's disease during the past two decades.

BACKGROUND: This study compared the incidence of Meniere's disease (MD) in the elderly aged >65 years between the first (2001-2010) and second (2011-2020) decades to investigate the evolution of geriatric MD. METHODS: Totally, 1605 and 2550 patients with definite MD were experienced at the neurotological clinic during the first and the second decades, respectively. All patients were divided into three groups by 30-year age band, namely elderly (aged 65-94 years), adult (aged 35-64 years) and young (aged 5-34 years) groups, and underwent an inner ear test battery. Factors relating to the incidence of MD during the past two decades were analyzed. RESULTS: The elderly MD group comprised 198 (12.4%) of 1605 MD cases during the first decade, and 463 (18.2%) of 2550 MD cases during the second decade, showing a significantly increased incidence of elderly MD. Correlation between annual life expectancy (x) in Taiwan and annual prevalence (y) of the elderly MD in relation to total MD cases revealed y = 0.023x - 1.660 via linear regression analysis. In contrast, the adult MD group significantly differed in terms of age and gender ratio, but not incidence, between the two decades. Conversely, the young MD group exhibited significantly decreased incidence from the first decade (22.3%) to the second decade (13.8%). CONCLUSION: Evolution of geriatric MD during the past two decades reveals an increased incidence of the elderly MD patients, likely due to increased life expectancy coupled with altered life style.

Post-irradiation endolymphatic hydrops vs. post-irradiation sudden deafness.

BACKGROUND: Increasing numbers of acute sensorineural hearing loss (SNHL) are recently experienced in irradiated nasopharyngeal carcinoma (NPC) survivors. AIM: This study adopted MR imaging to differentiate between post-irradiation sudden deafness (PISD) and post-irradiation endolymphatic hydrops (PIEH) in long-term NPC survivors with acute SNHL. METHODS: From 2012 to 2021, consecutive 10 irradiated NPC survivors with acute SNHL were enrolled. All patients underwent an inner ear test battery and MR imaging using HYDROPS-Mi2 technique. Six patients (11 ears) with positive cochlear hydrops on MR images were diagnosed as PIEH, while another 4 patients (4 ears) without cochlear hydrops on MR images were referred to PISD. RESULTS: The interval from the onset of NPC to acute SNHL did not significantly differ between the PIEH (10 ± 6 years) and PISD (8 ± 2 years). No significant difference was found between the two disorders from any of the symptomatic, radiotherapeutic, audiological, or vestibular perspective. Interestingly, most (5/6) patients with PIEH had bilateral involvement, while all (4/4) patients with PISD showed unilateral affliction. A significantly declining sequence of abnormality rates in the inner ear test battery was noted in the PIEH patients, running from the audiometry (100%), cervical vestibular-evoked myogenic potential (VEMP) test (100%), ocular VEMP test (73%), to the caloric test (36%). However, such declining trend was not observed in patients with PISD. CONCLUSION: When facing an NPC survivor who had acute SNHL over a prolonged period after irradiation, MR imaging using HYDROPS-Mi2 technique should be performed to differentiate the PIEH from the PISD, since both disorders have various treatment modalities and hearing outcome.

Spironolactone Ameliorates Cochlear Implant Induced Endolymphatic Hydrops.

BACKGROUND: Endolymphatic hydrops (EH) has been observed in both animal and human cochleae following cochlear implant (CI) surgery. We tested whether EH could be eliminated by administration of mineralocorticoid steroid antagonist spironolactone and explored the electrophysiological consequences of this. METHODS: Sixty-four adult guinea pigs underwent cochlear implantation with a dummy electrode. Animals then survived either 2, 7, or 28 days. Auditory function was monitored by recording electrocochleography from the round window membrane preimplantation, and on the last day of the experiment. Spironolactone or control solution was added to animals' feed for 7 days (if they survived that long) beginning immediately prior to surgery. The presence of EH was determined using thin-sheet laser imaging microscopy. RESULTS: Treatment with spironolactone resulted in significant reduction in EH in the second cochlear turn 7 days postimplantation. In all animals, the compound action potential (CAP) threshold was elevated 2 days postimplantation, but for most frequencies had recovered substantially by 28 days. There was no treatment effect on CAP thresholds. SP/AP ratios were elevated at day 2. The amplitude growth of the CAP did not differ between test and control groups at any time after implantation. CONCLUSIONS: EH can be suppressed by antagonism of mineralocorticoid receptors in the week after cochlear implantation. Reduction in EH did not lead to any change in hearing, and there was no indication of synaptopathy signalled by reduced CAP amplitude at high sound intensities. We found no electrophysiological evidence that EH early after implantation impacts negatively upon preservation of residual hearing.

Pathogenesis and Etiology of Ménière Disease: A Scoping Review of a Century of Evidence.

IMPORTANCE: Ménière disease is a rare chronic benign disorder of the inner ear with a natural history of multiple clinical phenotypes of variable severity and a tendency to burnout with time. Although multiple treatment modalities have been shown to improve the disease process-some adversely affecting cochleovestibular function-it remains uncertain whether one, several separate, or a combination of pathophysiologic mechanisms affect the disease process. A scoping review of the evidence underlying proposed pathophysiologic mechanisms of Ménière disease is needed to determine which processes are most likely to be etiopathogenic factors. OBSERVATIONS: Of the 4602 relevant articles found through Embase, Ovid, and PubMed, 444 met inclusion criteria. The most common reported causes of Ménière disease were autoimmune or immune-mediated, genetic, or structural dysfunction of the inner ear. During the study period from inception to March 2021, etiologic theories shifted from structural dysfunction to autoimmune and genetic causes of Ménière disease. CONCLUSIONS AND RELEVANCE: This scoping review found that Ménière disease is a multifactorial disease with lifelong comorbidities and loss of quality-associated life-years whose most commonly reported causes were structural dysfunction, immunologic damage, and genetic susceptibility. Recent studies have examined how autoinflammatory processes and vestibular migraine may be associated with Ménière disease. Large heterogeneity among studies may be explained by historical differences in the clinical understanding of the disease, as well as evolving intervention methodologies and practitioner expertise. Ménière disease is a multifactorial disease with lifelong comorbidities and loss of quality-associated life-years; therefore, future studies of reliable biomarkers of endolymphatic hydrops and real-time imaging are warranted to improve understanding and treatment.

Post-traumatic peripheral vestibular disorders (excluding positional vertigo) in workers following head injury.

Benign paroxysmal positional vertigo has typically been reported to be the most common cause of post-traumatic dizziness. There is however paucity in the literature about other peripheral vestibular disorders post-head injury. This article provides an overview of other causes of non-positional dizziness post-head trauma from our large institutional experience. The UHN WSIB Neurotology database (n = 4291) between 1998 and 2018 was retrospectively studied for those head-injured workers presenting with non-positional peripheral vestibular disorders. All subjects had a detailed neurotological history and examination and vestibular testing including video nystagmography, video head impulse testing (or a magnetic scleral search coil study), vestibular-evoked myogenic potentials, and audiometry. Imaging studies included routine brain and high-resolution temporal bone CT scans and/or brain MRI. Based on a database of 4291 head-injured workers with dizziness, 244 were diagnosed with non-positional peripheral vertigo. Recurrent vestibulopathy (RV) was the most common cause of non-positional post-traumatic vertigo. The incidence of Meniere's disease in the post-traumatic setting did not appear greater than found in the general population. The clinical spectrum pertaining to recurrent vestibulopathy, Meniere's disease, delayed endolymphatic hydrops, drop attacks, superior semicircular canal dehiscence syndrome, and uncompensated peripheral vestibular loss are discussed.

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.

[Various types of vertigo and balance disorders in patients with Meniere's disease]. / Razlichnye formy golovokruzheniya i narusheniya ravnovesiya pri bolezni Men'era.

Vertigo and balance disorders in Meniere's disease (MD) may have various etiology. The aim of the review is discussing pathogenetic mechanisms of the typical vertiginous paroxysms in MD, resulting from endolymphatic hydrops as well as analysis of etiology, pathogenesis, clinical course and basic treatment of paroxysmal and permanent forms of vertigo and balance disorders, caused by other conditions, associated with MD. We discussed the course of MD complicated by vestibular migraine, benign positional paroxysmal vertigo, functional dizziness, bilateral vestibulopathy and vestibular drop-attacks.

The Correlation Between Endolymphatic Hydrops and blood-labyrinth barrier Permeability of Meniere Disease.

OBJECTIVES: This study was designed to assess the correlation between the grades of endolymphatic hydrops and the blood-labyrinth barrier permeability in the affected ear in Meniere's disease, following the administration of intravenous gadolinium contrast. STUDY DESIGN: Prospective study. METHODS: The quantitative values of endolymphatic hydrops were determined after intravenous injection of a double-dose of gadobutrol in 39 patients with unilateral definite Meniere's disease. Additionally, the signal intensity ratio of bilateral cochlear basal turns was evaluated and analyzed; The correlation between the grades of the endolymphatic hydrops and the signal intensity ratio of the cochlear basal turns in the affected ear was examined. RESULTS: The grades of the endolymphatic hydrops can be quantitatively evaluated using magnetic resonance imaging (MRI). The signal intensity ratio of the cochlear basal turns in the affected ear was significantly higher than in the unaffected ear (P = .001); there was a positive correlation between the signal intensity ratio of the cochlear basal turn and the grades of cochlear (r = 0.634, P = 0.000) and vestibular(r = 0.559, P = .000) hydrops in the affected ear. CONCLUSIONS: The increased permeability of the blood-labyrinth barrier may play a role in the process of endolymphatic hydrops in Meniere's disease.

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

OBJECTIVE: Like other vestibular schwannomas developing in the internal auditory canal, intralabyrinthine schwannomas (ILS) may present with similar symptoms as in endolymphatic hydrops. Two different studies have described MR saccular hydrops in ~ 30% of internal auditory canal vestibular schwannomas, but this association has never been studied in ILS before. The aim of this work is to study the prevalence of a saccular dilation in ILS, on a T2-weigthed sequence at 3 T, compared to a control group. MATERIAL AND METHODS: All patients presenting with typical ILS between January 2008 and October 2018 were included (n = 28, two patients with bilateral tumors) 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 47% of the cases (p = 0.0006 for the height, p = 0.0487 for the width). Bilateral saccular dilation was observed in 37% of the cases. There was a statistically significant correlation between the presence of a saccular hydrops and balance disorders (p = 0.02) as 50% of the patients with an intralabyrinthine schwannoma who presented with such symptoms had a saccular dilation. CONCLUSION: Forty-seven percent of ILS are associated with homolateral saccular dilation, which is an MR sign of endolymphatic hydrops (bilateral in 37%) and it appears related to the presence of balance disorders. This opens new therapeutic potentialities with the possible use of anti-vertiginous drugs, which could have a beneficial effect on their clinical symptomatology.

Assessment of the Progression of Vertical Semicircular Canal Dysfunction and Increased Vestibular Endolymphatic Hydrops in Patients With Early-Stage Ménière Disease.

Importance: Vertical semicircular canals and endolymphatic hydrops play important roles in the pathophysiological mechanisms of Ménière disease. However, their characteristics and associations with disease progression during medical treatment have not been determined. Objective: To examine the function of both the horizontal and vertical semicircular canals in patients with Ménière disease and to evaluate the change in endolymphatic hydrops volume during medical treatment, including treatment with diuretic therapy, over a 2-year period. Design, Setting, and Participants: This prospective longitudinal observational cohort study included 55 patients with definite unilateral Ménière disease and was performed in a tertiary care hospital in Japan. Participants were enrolled between April 1, 2017, and January 31, 2018, and those with vestibular migraine were excluded. All participants received education regarding diet and lifestyle modifications and treatment with betahistine mesylate (36 mg daily) and/or an osmotic diuretic (42-63 mg daily). Patients were followed up for vertigo and hearing evaluations at least once per month for more than 12 months and were instructed to record episodes of vertigo in a self-check diary. Audiometry was performed monthly, video head impulse testing and caloric testing were performed every 4 months, and magnetic resonance imaging was conducted annually. Data were analyzed from May 15, 2017, to January 31, 2020. Main Outcomes and Measures: Neurootological testing to evaluate vestibuloocular reflex gain over time, magnetic resonance imaging to evaluate the change in endolymphatic hydrops volume over time, and monthly vertigo and hearing evaluations for more than 12 months. Results: Among 55 participants with definite Ménière disease, 32 patients (58.2%) were female, and the mean (SD) age was 59.0 (15.1) years. The median disease duration was 2 years (interquartile range, 0-4 years), with 43 patients (78.2%) having an early stage (ie, disease duration ≤4 years) of Ménière disease. Over the 2-year study period, the vestibuloocular reflex gain decreased from 0.76 to 0.56 in the superior semicircular canals, for a difference of 0.20 (95% CI, 0.14-0.26) and from 0.68 to 0.50 in the posterior semicircular canals, for a difference of 0.18 (95% CI, 0.14-0.22). The maximum slow-phase velocity and vestibuloocular reflex gain in the horizontal semicircular canals were maintained. The volume ratio of vestibular endolymphatic hydrops increased from 19.7% to 23.3%, for a difference of 3.6% (95% CI, 1.4%-5.8%). The frequency of vertiginous episodes decreased, and the hearing level over the study period worsened from 40.9 dB to 44.5 dB, for a difference of 3.5 dB (95% CI, 0.7-6.4 dB). Conclusions and Relevance: In this study, during a 2-year period of medical treatment among patients with Ménière disease, vestibuloocular reflex gain decreased in the vertical semicircular canals but was maintained in the horizontal semicircular canals; the endolymphatic hydrops volume ratio increased, and the frequency of vertiginous episodes decreased. These findings describe the pathological progression of chronic Ménière disease and expand the understanding of its pathophysiological characteristics during the early stage of disease.

Live imaging and functional changes of the inner ear in an animal model of Meniere's disease.

The symptoms of Meniere's disease (MD) are generally considered to be related to endolymphatic hydrops (EH). There are many recent reports supporting the possibility that vasopressin (VP) is closely linked to the formation of EH in Meniere's disease. Based on this, we developed a clinically relevant animal model of Meniere's disease in which a VP type 2 receptor agonist was administered after electrocauterization of the endolymphatic sac. We report live imaging of the internal structure, and functional changes of the inner ear after electrocauterization of the endolymphatic sac and administration of a VP type 2 receptor agonist. In this model, the development of EH was visualized in vivo using optical coherence tomography, there was no rupture of Reissner's membrane, and low-tone hearing loss and vertiginous attacks were observed. This study suggested that acute attacks are caused by the abrupt development of EH. This is the first report of live imaging of the development of EH induced by the administration of a VP type 2 receptor agonist.

Endolymphatic Hydrops Secondary to Spontaneous Intracranial Hypotension.

BACKGROUND: Spontaneous intracranial hypotension may be associated with neuro-otological symptoms that might mimic Menière's disease. CASE PRESENTATION: We report the case of a 53-year-old male presenting bi-frontal headache with recurrent spells of vertigo, left fluctuating hearing loss, and tinnitus. Dedicated brain and inner ear Magnetic Resonance Imaging, including a post-contrast 4 hours delayed FLAIR sequence, revealed typical signs of spontaneous intracranial hypotension associated with endolymphatic hydrops involving the left saccule and cochlea. CONCLUSIONS: Audio vestibular manifestations mimicking Menière's disease in spontaneous intracranial hypotension could be explained by endolymphatic hydrops, which can be detected using dedicated magnetic resonance imaging sequences.

Gadolinium-enhanced MRI reveals dynamic development of endolymphatic hydrops in Ménières disease / Ressonância magnética com gadolínio revela o desenvolvimento dinâmico da hidropsia endolinfática na doença de Ménière

Abstract Introduction: Meniere's disease is associated with impaired hearing, tinnitus, vertigo, and aural fullness. Many anatomical studies have suggested idiopathic endolymphatic hydrops as the pathological basis of Meniere's disease, which now can be visualized by using gadolinium -enhanced magnetic resonance imaging of the inner ear. Objective: To investigate the development of endolymphatic hydrops in Meniere's disease by monitoring the vestibules and cochleae of affected patients. Methods: Inner ears of 178 patients with definite unilateral Meniere's disease diagnosis were visualized by 3-dimensional fluid-attenuated inversion recovery and three-dimensional real inversion recovery magnetic resonance imaging following bilateral gadolinium intratympanic injection. The scans were used to evaluate the presence and degree of endolymphatic hydrops in the vestibules and cochlear structures, including the cochlear apical turn, the cochlear middle turn, and the cochlear basal turn. The correlation of endolymphatic hydrops occurrence between the various parts of the inner ear was determined. Results: Symptomatic endolymphatic hydrops was detected on the affected side in all patients, whereas asymptomatic endolymphatic hydrops was detected on the unaffected contra-lateral side in 32 patients (18.0%). On the affected side, the cochlear apical turn and the cochlear middle turn demonstrated significantly higher rates of endolymphatic hydrops than the cochlear basal turn and the vestibule. The severity of endolymphatic hydrops gradually decreased from the cochlear apical turn to the cochlear basal turn. On the contra lateral side, the incidence and degree of the detected asymptomatic endolymphatic hydrops were significantly greater in the cochleae than in the vestibules (p < 0.05), with no significant difference detected between the cochlear turns. Conclusion: Progression of endolymphatic hydrops appears to be directional, initiated in the cochlea. The order of endolymphatic hydrops severity gradually decreases from the cochlear apical turn to the cochlear basal turn and then to the vestibule. Endolymphatic hydrops in the vestibule is associated with symptomatic Meniere's disease.

Resumo Introdução: A doença de Ménière está associada a deficiência auditiva, zumbido, vertigem e plenitude auricular. Muitos estudos anatômicos sugerem hidropsia endolinfática idiopática como a base patológica da doença, que agora pode ser visualizada através de estudo por imagem da orelha interna por ressonância magnética com gadolínio. Objetivo: Investigar o desenvolvimento da hidropsia endolinfática na doença de Ménière com monitoramento dos vestíbulos e das cócleas dos pacientes afetados. Métodos: Orelhas internas de 178 pacientes com diagnóstico definitivo de doença de Ménière unilateral foram visualizados através de imagem de recuperação de inversão atenuada por fluidos em ressonância magnética tridimensional, 3-D FLAIR, e por inversão real após injeção intratimpânica bilateral de gadolínio. Os exames foram usados para avaliar a presença e o grau de hidropsia endolinfática nos vestíbulos e nas estruturas cocleares, inclusive o giro coclear apical, o giro coclear médio e o giro coclear basal. A correlação da ocorrência de hidropsia endolinfática entre as várias partes da orelha interna foi determinada. Resultados: Hidropsia endolinfática sintomática foi detectada no lado afetado em todos os pacientes, enquanto hidropsia endolinfática assintomática foi detectada no lado contralateral não afetado em 32 pacientes (18,0%). No lado afetado, o giro apical da cóclea e o giro coclear médio demonstraram taxas significativamente mais altas de hidropsia endolinfática do que o giro basal e o vestíbulo. A gravidade da hidropsia endolinfática diminuiu gradualmente do giro apical da cóclea para o giro basal. No lado contralateral, a incidência e o grau da hidropsia endolinfática assintomática detectada foram significantemente maiores nas cócleas do que nos vestíbulos (p < 0,05), sem diferença significante entre os giros cocleares. Conclusões: A progressão da hidropsia endolinfática parece ser direcional, iniciando-se na cóclea. A sua ordem da gravidade diminui gradualmente do giro apical da cóclea para o giro basal e, em seguida, para o vestíbulo. A hidropsia endolinfática no vestíbulo está associada à doença de Ménière sintomática.