Mitochondrial disease and sensorineural hearing loss have a certain correlation.With the understand-ing and research of mitochondrial sensorineural hearing loss,mitochondrial sensorineural hearing loss has become a treatable disease,wearing hearing aids and cochlear implantation is the mainstream treatment.However,there are few reports on cochlear implantation in patients with mitochondrial sensorineural hearing loss.This article summarizes and analyzes the current domestic and foreign literature reports related to cochlear implantation in patients with syndromic mitochondrial sensorineural hearing loss,and reviews the effect after cochlear implantation,providing help for further clinical diagnosis,treatment,and research.