Meniere’s disease variants and endolymphatic hydrops-related conditions: a systematic review of the literature

INTRODUCTION: Endolymphatic hydrops (EH) is associated par excellence with Meniere’s disease (MD); however, it is also related to other conditions including Lermoyez Syndrome, cochlear Meniere’s disease, vestibular Meniere’s disease, Tumarkin’s otolithic crisis, delayed endolymphatic hydrops and the secondary endolymphatic hydrops. These conditions produce symptoms similar to MD for which they go into differential diagnosis with it. Literature fragmentation and heterogeneity make it difficult to have a clear and up-to-date knowledge of MD variants and EH related pathologies, and differential diagnosis is often difficult. EVIDENCE ACQUISITION: We searched four international databases (PubMed, Embase, Scopus and Cochrane Library) for articles published up to March 2022 and in reference list from the relevant literature. We discussed eligible studies and data outcomes and summarized them in this article. EVIDENCE SYNTHESIS: We summarized the symptomatic, audiometric, vestibular, radiological, etiological, histopathological, and therapeutic evidence available in scientific literature of MD variants and EH related pathologies. CONCLUSIONS: While MD is extensively studied, little is known about its variants and EH related pathologies. Although when considered individually these diseases have a low incidence, together they represent a considerable portion of audiologic conditions. Differential diagnosis is of utmost importance for the correct treatment of these conditions.