What is Menière’s disease? A contemporary re-evaluation of endolymphatic hydrops

Abstract

Menière’s disease is a chronic condition with a prevalence of 200–500 per 100,000 and characterized by episodic attacks of vertigo, fluctuating hearing loss, tinnitus, aural pressure and a progressive loss of audiovestibular functions. Over 150 years ago, Prosper Menière was the first to recognize the inner ear as the site of lesion for this clinical syndrome. Over 75 years ago, endolymphatic hydrops was discovered as the pathologic correlate of Menière’s disease. However, this pathologic finding could be ascertained only in post-mortem histologic studies. Due to this diagnostic dilemma and the variable manifestation of the various audiovestibular symptoms, diagnostic classification systems based on clinical findings have been repeatedly modified and have not been uniformly used in scientific publications on Menière’s disease. Furthermore, the higher level measures of impact on quality of life such as vitality and social participation have been neglected hitherto. Recent developments of high-resolution MR imaging of the inner ear have now enabled us to visualize in vivo endolymphatic hydrops in patients with suspected Menière’s disease. In this review, we summarize the existing knowledge from temporal bone histologic studies and from the emerging evidence on imaging-based evaluation of patients with suspected Menière’s disease. These indicate that endolymphatic hydrops is responsible not only for the full-blown clinical triad of simultaneous attacks of auditory and vestibular dysfunction, but also for other clinical presentations such as “vestibular” and “cochlear Menière’s disease”. As a consequence, we propose a new terminology which is based on symptomatic and imaging characteristics of these clinical entities to clarify and simplify their diagnostic classification.

Caffeine and Meniere’s Disease

“…It is believed that cafeine afected the peripheral and central auditory pathways [45]. As the inner ear is the site of lesion for this clinical syndrome, we direct our eforts in presenting the efects of cafeine on the peripheral auditory system [46].…”

Ménière’s Disease and Tinnitus

“…His initial description of the disease including hearing loss, vertigo and tinnitus was accurate, but the pathophysiology of the condition was only described 75 years later. The endolymphatic hydrops is the main inding, initially ascertained only in post-mortem exams and nowadays detectable in high resolution magnetic resonance imaging (MRI) [2].…”

“…Classical symptoms include luctuating hearing loss, aural fullness and periodic vertigo and tinnitus [2]. The diagnosis criteria from the American Academy of Otolaryngology and Head and Neck Surgery (AAO-HNS) and the Bárány Society do not recognize an eventual 'cochlear' Ménière disease [4], but recent imaging studies (gadolinium contrasted 3T MRI) are more sympathetic with this possibility [5].…”

MRI Sequences in Head & Neck Radiology – State of the Art

“…3 D fluid-attenuated inversion recovery (3D-FLAIR) sequences produce high-resolution 3 D images with a suppressed fluid signal. 3D-FLAIR allows separate visualization of endo-and perilymphatic components and has become a new diagnostic tool for the visualization of endolymphatic hydrops in Menière's disease [23]. Gadolinium chelate-enhanced images are typically obtained 24 h after microscopically controlled intratympanic application (i.t) (8-times diluted), or 4 h after intravenous (i. v.) application [24].…”