Inner ear is composed of cochlea, vestibule, and endolymphatic sac which are enclosed by thin layer of epithelial cells. The enclosed space is filled with fluid named as endolymph where the [K⁺] is high and [Na⁺] is low. This unique ion composition is very important in maintaining normal hearing and balance function by providing K⁺ ions into sensory hair cells, which finally depolarize hair cells to facilitate the transport of sound and acceleration stimulation to central nervous system. The ion composition of inner ear is maintained by various ion transport through ion channels, transporters, and exchangers in the inner ear sensory and extra-sensory epithelium. The disruption of normal endolymphatic ion composition by the deterioration of the function of those ion channels can cause dysfunction of sensory epithelium, which consequently results in hearing and balance disorders. One of the possible pathology from the disruption of inner ear ion homeostasis is endolymphatic hydrops which is a phenomenon of excessive fluid accumulation of inner ear. The dysfunction of ion channels in inner ear epithelium can be an etiology of Ménière's disease since endolymphatic hydrops is a main pathological finding of the disease. In this review, we discussed about the possible pathological mechanism of Ménière's disease as a perspective of channelopathy as well as the role of various ion channels in the regulation of inner ear fluid volume based on the findings revealed by electrophysiological studies.