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Audiology & Neuro-otology 2019Several otologic conditions can present with fluctuating sensorineural hearing loss, including Ménière's disease, autoimmune inner ear disease, and enlarged vestibular... (Review)
Review
BACKGROUND
Several otologic conditions can present with fluctuating sensorineural hearing loss, including Ménière's disease, autoimmune inner ear disease, and enlarged vestibular aqueduct. Although these 3 etiologies vary greatly, distinguishing between these conditions at initial presentation can be challenging. Furthermore, initial treatment of these conditions is often similar. In this review, we discuss historical and current perspectives on diagnosis and treatment of these conditions.
SUMMARY
A literature search was performed regarding fluctuating hearing loss, and current treatment of these etiologies of fluctuating hearing loss was summarized. Immediate measures at the onset of acute hearing loss include corticosteroid therapy, while preventative and chronic therapies, which can limit disease severity and frequency, vary based on the specific condition treated. Key Messages: Fluctuating hearing loss can represent a range of pathologies, but the precise etiology may not be clear at initial presentation. Timely treatment and long-term follow-up, along with appropriate diagnostics, are necessary to optimize long-term hearing.
Topics: Endolymphatic Hydrops; Hearing Loss, Sensorineural; Hearing Tests; Humans; Meniere Disease
PubMed: 31315108
DOI: 10.1159/000500658 -
Current Opinion in Neurology Feb 2024This review discusses the recent developments on the understanding of epidemiology and genetics of Meniere's disease. (Review)
Review
PURPOSE OF REVIEW
This review discusses the recent developments on the understanding of epidemiology and genetics of Meniere's disease.
RECENT FINDINGS
Meniere's disease has been shown to be associated with several comorbidities, such as migraine, anxiety, allergy and immune disorders. Recent studies have investigated the relationship between environmental factors and Meniere's disease such as air pollution, allergy, asthma, osteoporosis or atmospheric pressure, reporting specific comorbidities in East Asian population. The application of exome sequencing has enabled the identification of genes sharing rare missense variants in multiple families with Meniere's disease, including OTOG and TECTA and suggesting digenic inheritance in MYO7A . Moreover, knockdown of DTNA gene orthologue in Drosophila resulted in defective proprioception and auditory function. DTNA and FAM136A knockout mice have been studied as potential mouse models for Meniere's disease.
SUMMARY
While it has attracted emerging attention in recent years, the study of Meniere's disease genetics is still at its early stage. More geographically and ethnically based human genome studies, and the development of cellular and animal models of Meniere's disease may help shed light on the molecular mechanisms of Meniere's disease and provide the potential for gene-specific therapies.
Topics: Mice; Animals; Humans; Meniere Disease; Hypersensitivity; Comorbidity; Migraine Disorders; Proprioception
PubMed: 37865853
DOI: 10.1097/WCO.0000000000001227 -
BMJ (Clinical Research Ed.) Jan 2016What is the long term efficacy of betahistine dihydrochloride on the incidence of vertigo attacks in patients with Meniere's disease, compared with placebo? (Randomized Controlled Trial)
Randomized Controlled Trial
Efficacy and safety of betahistine treatment in patients with Meniere's disease: primary results of a long term, multicentre, double blind, randomised, placebo controlled, dose defining trial (BEMED trial).
STUDY QUESTION
What is the long term efficacy of betahistine dihydrochloride on the incidence of vertigo attacks in patients with Meniere's disease, compared with placebo?
METHODS
The BEMED trial is a multicentre, double blind, randomised, placebo controlled, three arm, parallel group, phase III, dose defining superiority trial conducted in 14 German tertiary referral centres (for neurology or ear, nose, and throat). Adults aged 21-80 years (mean age 56 years) with definite unilateral or bilateral Meniere's disease were recruited from March 2008 to November 2012. Participants received placebo (n=74), low dose betahistine (2 × 24 mg daily, (n=73)), or high dose betahistine (3 × 48 mg daily, (n=74)) over nine months. The primary outcome was the number of attacks per 30 days, based on patients' diaries during a three month assessment period at months seven to nine. An internet based randomisation schedule performed a concealed 1:1:1 allocation, stratified by study site. Secondary outcomes included the duration and severity of attacks, change in quality of life scores, and several observer-reported parameters to assess changes in audiological and vestibular function.
STUDY ANSWER AND LIMITATIONS
Incidence of attacks related to Meniere's disease did not differ between the three treatment groups (P=0.759). Compared with placebo, attack rate ratios were 1.036 (95% confidence interval 0.942 to 1.140) and 1.012 (0.919 to 1.114) for low dose and high dose betahistine, respectively. The overall monthly attack rate fell significantly by the factor 0.758 (0.705 to 0.816; P<0.001). The population based, mean monthly incidence averaged over the assessment period was 2.722 (1.304 to 6.309), 3.204 (1.345 to 7.929), and 3.258 (1.685 to 7.266) for the placebo, low dose betahistine, and high dose betahistine groups, respectively. Results were consistent for all secondary outcomes. Treatment was well tolerated with no unexpected safety findings. Without a control group of patients who did not receive any intervention to follow the natural course of the disease, the placebo effect could not be accurately assessed and differentiated from spontaneous remission and fluctuation of symptoms.
WHAT THIS STUDY ADDS
Current evidence is limited as to whether betahistine prevents vertigo attacks caused by Meniere's disease, compared with placebo. The trial provides information on symptom relief on placebo intervention which is relevant for the design of future studies on potential disease modifying treatments in patients with Meniere's disease.
FUNDING, COMPETING INTERESTS, DATA SHARING
Support from the German Federal Ministry of Education and Research (BMBF support code 01KG0708). Potential competing interests have been reported in full at the end of the paper on thebmj.com. Data are available from the corresponding author ([email protected]) or biostatistician ([email protected]). Study registration EudraCT no 2005-000752-32; ISRCTN no ISRCTN44359668.
Topics: Adult; Aged; Aged, 80 and over; Betahistine; Dose-Response Relationship, Drug; Double-Blind Method; Female; Germany; Histamine Agonists; Humans; Incidence; Male; Meniere Disease; Middle Aged; Quality of Life; Vertigo; Young Adult
PubMed: 26797774
DOI: 10.1136/bmj.h6816 -
Clinical and Experimental Medicine Nov 2023The etiology of Ménière's disease (MD) remains controversial. Allergies are potential extrinsic factors that, in conjunction with underlying intrinsic factors, may... (Review)
Review
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.
Topics: Animals; Humans; Meniere Disease; Endolymphatic Hydrops; Hypersensitivity; Immunotherapy; Immunoglobulin E
PubMed: 37743423
DOI: 10.1007/s10238-023-01192-0 -
Medwave Oct 2017Menieres disease is an inner ear disorder characterized by episodes of spontaneous vertigo, fluctuating hearing loss and tinnitus. Betahistine has been used to reduce... (Meta-Analysis)
Meta-Analysis Review
PROBLEM
Menieres disease is an inner ear disorder characterized by episodes of spontaneous vertigo, fluctuating hearing loss and tinnitus. Betahistine has been used to reduce intensity and frecuency of vertigo attacks, but there is controversy regarding its effectiveness.
METHODS
To answer this question we used Epistemonikos, the largest database of systematic reviews in health, which is maintained by screening multiple information sources, including MEDLINE, EMBASE, Cochrane, among others. We extracted data from the systematic reviews, reanalyzed data of primary studies, conducted a meta-analysis and generated a summary of findings table using the GRADE approach.
RESULTS AND CONCLUSIONS
We identified four systematic reviews including 12 trials overall. We concluded betahistine might reduce the number of attacks, vertigo intensity and lead to a symptomatic improvement according to global judgement in patients with Menieres disease, but the certainty of evidence is low. On the other hand, it probably does not have significant adverse effects.
Topics: Betahistine; Humans; Meniere Disease; Treatment Outcome; Vasodilator Agents; Vertigo
PubMed: 29099816
DOI: 10.5867/medwave.2017.08.7068 -
Medwave Mar 2017Ménières disease affects the inner ear and its main symptoms are vertigo, hearing loss and fluctuating aural symptoms. Nowadays, there are many therapeutic... (Meta-Analysis)
Meta-Analysis Review
Ménières disease affects the inner ear and its main symptoms are vertigo, hearing loss and fluctuating aural symptoms. Nowadays, there are many therapeutic alternatives, being the use of intratympanic corticosteroids one that has become popular. To answer this question, we searched in Epistemonikos database, which is maintained by screening multiple databases. We identified four systematic reviews including 15 studies overall, of which seven were randomized trials. We extracted data and generated a summary of findings table using the GRADE approach. We concluded intratympanic corticosteroids probably do not decrease tinnitus, and might not decrease vertigo, hearing loss or aural fullness sensation in Ménières disease. Intratympanic corticosteroids probably do not cause important adverse effects.
Topics: Glucocorticoids; Hearing Loss; Humans; Injection, Intratympanic; Meniere Disease; Randomized Controlled Trials as Topic; Tinnitus; Treatment Outcome; Vertigo
PubMed: 28306710
DOI: 10.5867/medwave.2017.6863 -
Handbook of Clinical Neurology 2016Menière's disease (MD) is a chronic multifactorial disorder of the inner ear characterized by episodic vestibular symptoms associated with sensorineural hearing loss,... (Review)
Review
Menière's disease (MD) is a chronic multifactorial disorder of the inner ear characterized by episodic vestibular symptoms associated with sensorineural hearing loss, tinnitus, and aural pressure. Epidemiologic and genomic evidence supports a genetic susceptibility with multiple biochemical pathways involved, including the endocrine system, innate immune response, and autonomic nervous system. Allergens, infectious agents, vascular events, or genetic factors could modify inner-ear homeostasis and trigger MD. The diagnosis of MD is based on clinical criteria and requires the observation of an episodic vertigo syndrome associated with low- to medium-frequency sensorineural hearing loss and fluctuating aural symptoms (hearing loss, tinnitus, and/or fullness) in the affected ear. Headache is also found during the attacks and bilateral involvement is found in 25-40% of cases. Audiologic and vestibular assessment is recommended to monitor the clinical course. The treatment of MD is symptomatic to obtain relief of vestibular episodes and preventive to limit hearing loss progression. Treatment options include sodium restriction, betahistine, intratympanic gentamicin, or steroids and eventually surgery, such as cochlear implantation.
Topics: Audiometry; Autonomic Nervous System; Humans; Meniere Disease
PubMed: 27638077
DOI: 10.1016/B978-0-444-63437-5.00019-4 -
Neuroimaging Clinics of North America Feb 2019Visualization of the morphologic substrate of Ménière disease, the endolymphatic hydrops, can be performed using noncontrast or contrast-enhanced MR imaging... (Review)
Review
Visualization of the morphologic substrate of Ménière disease, the endolymphatic hydrops, can be performed using noncontrast or contrast-enhanced MR imaging techniques. Noncontrast MR imaging uses a heavily T2-weighted sequence; however, its reproducibility remains to be confirmed. Contrast-enhanced MR imaging techniques mainly use a 3-dimensional fluid-attenuated inversion recovery sequence after intratympanic gadolinium administration or after a 4-hour delayed intravenous gadolinium administration. The latter technique is most frequently used and is able to detect and grade Ménière disease. It is a reliable technique with a high diagnostic accuracy, enabling visualization of endolymphatic hydrops.
Topics: Contrast Media; Ear, Inner; Humans; Image Enhancement; Imaging, Three-Dimensional; Magnetic Resonance Imaging; Meniere Disease
PubMed: 30466641
DOI: 10.1016/j.nic.2018.09.002 -
Otolaryngologic Clinics of North America Oct 2021Initial diagnosis of peripheral vestibulopathy requires a detailed history, physical examination, and, in some cases, audiovestibular testing, radiographic imaging, or... (Review)
Review
Initial diagnosis of peripheral vestibulopathy requires a detailed history, physical examination, and, in some cases, audiovestibular testing, radiographic imaging, or serology. Differentiation of a peripheral vestibulopathy as progressive or degenerative is often nuanced and influenced by a characterization of a patient's symptoms or natural history over time. A diverse group of vestibular pathology may fit into this category, including Ménière's disease, autoimmune conditions, congenital pathologies, ototoxic medications, radiation therapy, and perilymphatic fistula. Differentiation among these entities may be guided by initial or subsequent symptomatology, with various combinations of audiovestibular testing, serology, and imaging. Treatment options are disparate and disease-specific, ranging from observation to medical management or surgical intervention, underscoring the need for astute investigation and diagnosis.
Topics: Autoimmune Diseases; Humans; Meniere Disease; Radiography
PubMed: 34301401
DOI: 10.1016/j.otc.2021.05.015 -
European Annals of Otorhinolaryngology,... Apr 2015Endolymphatic pressure measurement is of interest both to researchers in the physiology and pathophysiology of hearing and ENT physicians dealing with Menière's disease... (Review)
Review
Endolymphatic pressure measurement is of interest both to researchers in the physiology and pathophysiology of hearing and ENT physicians dealing with Menière's disease or similar conditions. It is generally agreed that endolymphatic hydrops is associated with Menière's disease and is accompanied by increased hydrostatic pressure. Endolymphatic pressure, however, cannot be measured precisely without endangering hearing, making the association between hydrops and increased endolymphatic pressure difficult to demonstrate. Several integrated in vivo models have been developed since the 1960s, but only a few allow measurement of endolymphatic hydrostatic pressure. Models associating measurement of hydrostatic pressure and endolymphatic potential and assessment of cochlear function are of value to elucidate the pathophysiology of endolymphatic hydrops. The present article presents the main types of models and discusses their respective interest.
Topics: Ear, Inner; Endolymph; Endolymphatic Hydrops; Evidence-Based Medicine; Hearing Loss; Humans; Hydrostatic Pressure; Meniere Disease; Vestibular Function Tests
PubMed: 25467202
DOI: 10.1016/j.anorl.2014.05.004