There are increasing evidence of interactions between sex hormones and the structure and function of inner ear, especially in hearing impairment and balance disorders. In this chapter, we will discuss the mechanism of sex hormones on the inner ear, describe both clinical and basic research that has led us to our current understanding, and conclude with future perspectives on avenues of investigation that may lead to innovative treatments on the hearing loss, tinnitus, and dizziness resulted from the changes in estrogen and progesterone levels. The presence of estrogen receptors α and β has earlier been shown in the inner ear of mice. Expression of estrogen receptors (ER) correlates with the protection of auditory function. Estrogen may have certain protective effects on the hearing. Evidence for the treatment of sex hormone-induced symptoms is principally restricted to case reports and retrospective studies. Recognition and understanding of sex hormone-related inner ear problems will allow otologists to notice and manage these patients. Also, basic studies on the mechanism of how sex hormones act on inner ear provide the way to further prevent and treat on hearing impairment and balance disorders. High-quality evidence for their management is limited, with further research required.