摘要Genetic defects are one of the most important etiologies of severe to profound sensorineural hearing loss and play an important role in determining cochlear implantation outcomes. While the pathogenic mutation types of a number of deafness genes have been cloned, the pathogenesis mechanisms and their relationship to the outcomes of cochlear implantation remain a hot research area. The auditory performance is considered to be affected by the etiology of hearing loss and the number of surviving spiral ganglion cells, as well as others. Current research advances in cochlear implantation for hereditary deafness, especially the relationship among clinic-types, genotypes and outcomes of cochlear implantation, will be discussed in this review.