Mitochondrial hearing loss is hearing loss caused by mitochondrial DNA (mtDNA) mutations, which are regulated by nuclear genes and interact with environmental factors. Mitochondrial dysfunction is associated not only with hereditary syndromic and nonsyndromic deafness but also with age-related hearing loss. In recent years, mitochondrial deafness has attracted more and more attention, and with the advancement of research methods, some progress has been made in the study of the mechanism and treatment of the disease. Therefore, this review introduces the Pathogenesis, research methods, and Intervention strategies of mitochondrial deafness, which is expected to provide a reference for future research.