In recent years, several pathogenic variants of CIB2 were identified after the discovery of USH1J, in families with the DFNB48 form of isolated deafness but no signs of retinitis pigmentosa or vestibular dysfunction (Patel et al. 2015; Seco et al. 2016; Michel et al. 2017; Booth et al. 2018; Souissi et al. 2021). Here, CIB2 is linked to retinitis pigmentosa.