title: 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.
authors.affiliation: Department of Otorhinolaryngology Head and Neck Surgery, University of Munich, Marchioninistr. 15, 81377, Munich, Germany. rguerkov@med.uni-muenchen.de.
authors.forename: R
authors.identifier: http://orcid.org/0000-0002-4195-149X
authors.initials: R
authors.lastname: Gürkov
authors.affiliation: German Centre for Vertigo and Balance Disorder, University of Munich, Marchioninistr. 15, 81377, Munich, Germany.
authors.forename: I
authors.initials: I
authors.lastname: Pyykö
authors.affiliation: Hearing and Balance Research Unit, Otolaryngology, School of Medicine, University of Tampere, 33520, Tampere, Finland.
authors.forename: J
authors.initials: J
authors.lastname: Zou
authors.affiliation: Department of Otorhinolaryngology, Helsinki University Central Hospital, Haartmaninkatu 4E, 00290, Helsinki, Finland.
authors.forename: E
authors.initials: E
authors.lastname: Kentala
mesh_terms: D000818:Animals; D018159:Endolymphatic Hydrops / Q000175:diagnosis* / Q000209:etiology; D006801:Humans; D008279:Magnetic Resonance Imaging; D008575:Meniere Disease / Q000150:complications* / Q000175:diagnosis* / Q000453:epidemiology / Q000523:psychology; D011788:Quality of Life
keywords: Classification; Diagnosis; Endolymphatic hydrops; Magnetic resonance imaging; Menière’s disease