This study presents a pathogenic variant p.Arg1746Gln in CDH23 gene associated with congenital hearing loss with mild pigmentary changes in the retina, indicative of Usher syndrome, and an incidental finding of a missense variant (ARSB:p.Arg159Cys) reported as likely pathogenic for MPS VI in an Emirati patient. Here, ARSB is linked to Usher syndrome.