Abstract: Hydropic ear disease (HED) is a complex condition characterized by endolymphatic hydrops (ELH). Its primary clinical manifestations include hearing loss, vertigo, and tinnitus. This paper provides an overview of the research progress on the etiology and pathogenesis of HED, encompassing factors such as ELH, immune factors, genetic factors, inner ear structural abnormalities, inner ear ischemia, allergic reactions, viral and bacterial infections, water channel protein dysregulation, ion channel disorders, and abnormalities in water-salt metabolism hormones. By analyzing existing literature and integrating clinical practice, this study aims to provide new insights and methods for the diagnosis and treatment of HED.