Current Understanding and Clinical Management of Meniere's Disease

A Systematic Review

Patricia Perez-Carpena, MD; Jose A. Lopez-Escamez, MD, PhD

Disclosures

Semin Neurol. 2020;40(1):138-150. 

In This Article

Abstract and Introduction

Abstract

Meniere's disease (MD) is a set of uncommon disorders with core phenotype of tinnitus, episodic vertigo, and sensorineural hearing loss. MD shows a genetic predisposition and a family history is found in 10% cases, with an autosomal dominant inheritance pattern. It is a multifactorial condition whose onset and development are triggered by the combined effect of genetic and environmental factors. Histopathological studies have associated MD with the accumulation of endolymph in the cochlea and the vestibular organs. However, endolymphatic hydrops does not fully explain the persistence of tinnitus, hearing loss progression, or the frequency of vertigo attacks. There are several comorbidities associated with MD, such as migraine, anxiety, autoimmune, and autoinflammatory disorders, adding more complexity to the phenotype. This "extended phenotype" can make the diagnosis and clinical management more complex, but it could also lead to a better characterization, understanding, and treatment of MD patients.

We have conducted a systematic review on MD to update current knowledge, focusing on its mechanisms, diagnosis, comorbidities, and practical management.

Introduction

Meniere's disease (MD) is a syndrome caused by uncommon disorders affecting the inner ear and characterized by episodes of recurrent spontaneous vertigo, tinnitus, sensorineural hearing loss (SNHL), and aural fullness. It is a chronic, debilitating condition with an unpredictable course. Initially, it usually afflicts one ear (unilateral MD), but both ears can be involved (bilateral MD). Initially, the most disabling symptom is vertigo. Acute episodes usually occur repeatedly with a frequency of 5 to 10 crises per year, and they continue with remission stages of a variable duration of months or years.[1,2]

The condition is associated with endolymphatic hydrops (EH), which is caused by the accumulation of endolymph resulting in enlargement of the endolymphatic space in the inner ear. The increase of the cochlear duct pressure damages the organ of Corti and other inner ear membranes. However, EH itself does not explains the attacks of vertigo, since histopathological studies have reported EH in individuals without MD symptoms.[3]

MD is associated with genetic, autoimmune, and allergy factors. MD presents a strong familial aggregation, and familial MD have been reported in 10% of cases, with an autosomal dominant pattern of inheritance.[4]

Epidemiological evidence also supports a genetic etiology in MD. So, the finding of a higher prevalence in European population compared with others (Asian, Native American, sub-Saharan) and the description of multiplex families with MD and relatives with SNHL and episodic vertigo showing incomplete penetrance and variable expressivity support an additive model of common and rare variants.[5–8]

A higher prevalence of autoimmune disorders such as rheumatoid arthritis, systemic lupus erythematosus, autoimmune hypothyroidism, and psoriasis has been reported in MD.[9,10] This has supported the hypothesis of an innate immune dysfunction in certain patients with MD.[11] An allelic variant has been identified in 6p21.33 (leading signal rs4947296) associated with bilateral MD.[12] So, certain MD patients with this genetic marker should be considered as autoimmune MD.[13]

The relationship between MD and both respiratory and food allergies has been reported. Clinical improvement in MD patients with allergy has been observed after treatment of the allergy.[14]

Several studies have reported a higher prevalence of migraine in MD.[15] MD has also been related to migraine in several studies, and a possible common etiology has been suggested.[16,17]

This review focuses on the current knowledge and clinical management in MD. For this purpose, we have conducted a systematic review of the literature, including MD studies published in the past 5 years.

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