We observed cases of hearing loss in which hearing deteriorated when steroid therapy was discontinued and improved upon re-administration. We identified these as cases of steroid-responsive sensorineural hearing loss (SNHL). The diagnostic criteria for determining steroid-responsive SNHL were stated, and 10 cases were examined. The 10 cases of steroid-responsive SNHL were divided into two groups: 3 cases associated with aortitis syndrome (systemic type) and 7 cases with no known systemic disease (localized type). Relatively satisfactory hearing was maintained in these cases by long-term oral administration of prednisolone (PSL). The combined use of Sairei-to made it possible to decrease the necessary maintenance dose of PSL. While the pathology of steroid-responsive SNHL is still unclear, it is suspected that immune complex disease or vasculitis may be involved.