Mitochondrial deafness is a hearing loss of mitochondrial dysfunction, which is mainly caused by mitochondrial DNA (mtDNA) mutations. Mitochondrial deafness is regulated by nuclear genes and affected by the environmental factors. Mitochondrial dysfunction is associated with not only hereditary syndromic and nonsyndromic deafness,but also with age-related deafness. In recent years, mitochondrial deafness has attracted more and more attention. Some progress has been made in the study of mitochondrial deafness. Therefore, this paper reviews the research models, pathogenic mechanisms, research methods, and intervention strategies of mitochondrial deafness.The purpose of the paper is to provide reference for the further study of the disease in the future.