The Cldn11 gene, which encodes Claudin-11, has not, thus far, been identified as a deafness gene while mutations in the genes encoding Claudin-14 (DFNB29) and Claudin-9 cause autosomal recessive deafness in humans and mice (Wilcox et al., 2001; Nakano et al., 2009). Here, CLDN11 is linked to deafness.