We looked at the control of nuclear gene mutations on the clinical expression of m.3243A > G and found patient P2 harbor variants in two genes namely LOXHD1 (p.Leu1564Phe, p.Arg685His) and NEUROD1 (p.Glu59Gln) which is reported to be associated with non-syndromic hearing loss and maturity-onset diabetes, respectively. Here, NEUROD1 is linked to hearing loss disorder.