Although a large study remains to be performed to investigate the possible influences of mtDNA haplogroups on clinical phenotypes of OPA1-related ADOA (ie age of onset, progressivity of the disease, additional neurological symptoms such as neurosensorial deafness), our result indicates the absence of major influence of mtDNA haplogoups in the basic penetrance of the disease. This evidence concerns the gene OPA1 and autosomal dominant optic atrophy.