Currently, up to 13 genes have been associated with Usher syndrome: MYO7A, USH1C, CDH23, PCDH15, USH1G, and CIB2 are responsible for USH1, although the role of CIB2 in the Usher syndrome has recently been put on doubt (Booth et al., 2018). Here, PCDH15 is linked to Usher syndrome.