Clinical analysis on patients with confirmed mutations in CIB2, a NGS meta-analysis of USH patients, and work on cib2 mouse models have recently raised doubts that CIB2 is a USH-causing gene, but rather a gene for non-syndromic deafness (DFNB48) (Michel et al., 2017; Booth et al., 2018; Jouret et al., 2019). Here, CIB2 is linked to Usher syndrome.