Meniere’s disease is a disorder of the inner ear characterized by intermittent episodic vertigo, fluctuating hearing loss, ear fullness and tinnitus. The pathophysiology is commonly explained by a distension of membranous labyrinth by the endolymph, equally called endolymphatic hydrops. Endolymphatic hydrops, found on post-mortem examination, is the histologic hallmark. The cause of Meniere’s disease remains unclear. Numerous factors play a role in the development of hydrops related cochleovestibular dysfunction. Currently, there is no universally accepted theory on the underlying pathophysiology of this disease. On the basis of hisopathologic studies, it is assumed that endolymphatic hydrops is the pathologic feature most descriptive of Meniere’s disease. This review highlights some of the recent advances in the understanding of the possible pathophysiology of endolymphatic hydrops and Meniere’s disease.