Deafness, Nonsyndromic Sensorineural, Mitochondrial, also known as mitochondrial non-syndromic sensorineural hearing loss, is related to rare mitochondrial non-syndromic sensorineural deafness and sensorineural hearing loss. An important gene associated with Deafness, Nonsyndromic Sensorineural, Mitochondrial is MT-CO1 (Mitochondrially Encoded Cytochrome C Oxidase I).