Endolymphatic hydrops, a pathological feature of Ménière's disease, has been experimentally and clinically confirmed to be influenced by the blood circulation of vasopressin (VP). VP is a well-known hormonal regulator of water homeostasis. In addition, VP is influenced by various environmental changes, dehydration, fluctuation of atmospheric pressure, pregnancy, and other factors. Furthermore, VP is a key regulator of the hypothalamic-pituitary-adrenal (HPA) axis. The HPA axis is a major neuroendocrine system that controls reactions to emotional and physical stresses, as well as the sleep/wake cycle (circadian rhythm). Therefore, VP is susceptible to change via the HPA axis. This review considers possible mechanisms of the formation of endolymphatic hydrops from the perspective of the vasopressin-aquaporin 2 system.