CIB2 is an important deafness gene whose mutations are associated with nonsyndromic deafness DFNB48 and syndromic deafness USH1J (Riazuddin et al., 2012; Patel et al., 2015; Seco et al., 2016), but its biological function and the mechanism through which CIB2 mutations cause hearing loss remain elusive. This evidence concerns the gene CIB2 and deafness.