Hereditary deafness is a common genetic disease. The incidence of the disease in newborns is 1‰ to 3‰. Patients carry the diseasecausing mutations throughout their lives, leading to irreversible deafness. There is no clinically available drug. In some animal models of several inherited deafness, gene therapy is a potentially effective treatment. Serial clinical trials of gene therapy for hereditary deafness have been carried out in the domestic and overseas. Among them, the clinical trials of genetic therapy for hereditary deafness, led by the Affiliated Zhongda Hospital of Southeast University and in collaboration with multiple domestic hospitals, have achieved significant therapeutic effects and accumulated rich experience. In order to standardize genetic deafness gene therapy and provide clinical research guidance, otologic experts from nationwide hospitals have discussed and formulated clinical guidelines for gene therapy on inherited deafness. The contents cover the ethics, diagnosis and enrollment criteria for genetic deafness, clinical preparation, drug administration, efficacy and safety evaluation criteria, and postoperative speech rehabilitation. This guideline will provide standardized theoretical and technical criteria for the clinical gene therapy for the deafness.