Though most of the reported EYA4 mutations are truncating mutations leading to haploinsufficiency (Zhang et al., 2004), at least four EYA4 missense mutations have been reported to lead to non-syndromic deafness DFNA10(Tan et al., 2014; Liu et al., 2015; Sun et al., 2015; Cesca et al., 2018). Here, EYA4 is linked to deafness.