CIB2 in humans is associated with nonsyndromic deafness (DFNB48; Riazuddin et al., 2012) and is essential for hearing and auditory hair cell mechanotransduction in mice (Giese et al., 2017, Michel et al., 2017, Wang et al., 2017). The gene discussed is CIB2; the disease is nonsyndromic deafness.