The profound deafness observed in CIB2−/− mice, lacking a functional CIB2 protein for all four Cib2 splice isoforms (Fig 1A, see also Giese et al, 2017; Zou et al, 2017), is reminiscent of the prelingual severe‐to‐profound hearing loss observed in DFNB48‐affected patients (Riazuddin et al, 2012; Patel et al, 2015; Seco et al, 2016; and this study). Here, CIB2 is linked to deafness.