This includes a variant of unknown significance (VUS) in CDH23 (c.5653CT), previously reported as a VUS in a case with Usher Syndrome, [53] and a ClinVar reported pathogenic variant in NDUFAF3 (c.188dupA) [26], a gene associated with Mitochondrial complex I deficiency, nuclear type 18. This evidence concerns the gene NDUFAF3 and Usher syndrome.