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Published on: April 28, 2023
Meniere's disease
Tsutomu Nakashima1,2, Ilmari Pyykkö3, Megan A Arroll4
1Department of Rehabilitation, Ichinomiya Medical Treatment &Habilitation Center, 1679-2, Tomida-nagaresuji, Ichinomiya City, 494-0018, Japan.
Abstract:
Meniere's disease (MD) is a disorder of the inner ear that causes vertigo attacks, fluctuating hearing loss, tinnitus and aural fullness. The aetiology of MD is multifactorial. A characteristic sign of MD is endolymphatic hydrops (EH), a disorder in which excessive endolymph accumulates in the inner ear and causes damage to the ganglion cells. In most patients, the clinical symptoms of MD present after considerable accumulation of endolymph has occurred. However, some patients develop symptoms in the early stages of EH. The reason for the variability in the symptomatology is unknown and the relationship between EH and the clinical symptoms of MD requires further study. The diagnosis of MD is based on clinical symptoms but can be complemented with functional inner ear tests, including audiometry, vestibular-evoked myogenic potential testing, caloric testing, electrocochleography or head impulse tests. MRI has been optimized to directly visualize EH in the cochlea, vestibule and semicircular canals, and its use is shifting from the research setting to the clinic. The management of MD is mainly aimed at the relief of acute attacks of vertigo and the prevention of recurrent attacks. Therapeutic options are based on empirical evidence and include the management of risk factors and a conservative approach as the first line of treatment. When medical treatment is unable to suppress vertigo attacks, intratympanic gentamicin therapy or endolymphatic sac decompression surgery is usually considered. This Primer covers the pathophysiology, symptomatology, diagnosis, management, quality of life and prevention of MD.
Insights
Meniere's disease (MD) involves inner ear fluid buildup causing vertigo and hearing loss. Understanding the link between endolymphatic hydrops and symptom variability is key for better diagnosis and treatment.
Area of Science:
- Otolaryngology
- Neurology
- Inner Ear Disorders
Background:
- Meniere's disease (MD) is an inner ear disorder characterized by vertigo, fluctuating hearing loss, tinnitus, and aural fullness.
- A key pathological feature is endolymphatic hydrops (EH), an excess accumulation of endolymph leading to ganglion cell damage.
- The onset and severity of MD symptoms in relation to EH progression remain unclear, necessitating further investigation.
Purpose of the Study:
- To provide a comprehensive overview of Meniere's disease, covering its pathophysiology, clinical presentation, diagnostic methods, and management strategies.
- To highlight the role of endolymphatic hydrops (EH) in MD and the current understanding of its relationship with disease symptomatology.
- To discuss the evolving diagnostic tools, including advanced MRI techniques, and current therapeutic approaches for MD.
Main Methods:
- Review of existing literature on Meniere's disease pathophysiology, clinical features, and diagnostic modalities.
- Analysis of diagnostic criteria and functional tests used for MD, including audiometry, vestibular-evoked myogenic potential, caloric testing, electrocochleography, and head impulse tests.
- Evaluation of the application of optimized MRI for direct visualization of endolymphatic hydrops (EH) in clinical practice.
Main Results:
- Meniere's disease etiology is multifactorial, with endolymphatic hydrops (EH) being a characteristic sign.
- Diagnosis relies on clinical symptoms, supported by functional tests and increasingly by MRI visualization of EH.
- Management focuses on relieving acute vertigo and preventing recurrence, with conservative measures as first-line treatment, followed by intratympanic gentamicin or surgery if needed.
Conclusions:
- Further research is needed to elucidate the variable relationship between endolymphatic hydrops (EH) and Meniere's disease symptomatology.
- Optimized MRI offers direct visualization of EH, aiding in diagnosis and potentially guiding treatment strategies.
- A multi-faceted approach to management, from risk factor modification to surgical intervention, is essential for controlling Meniere's disease symptoms and improving quality of life.
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