DFNB31 mutations were initially reported for the mouse ortholog in the whirler mouse (Whrn) in autosomal recessive nonsyndromic profound sensoryneural deafness (Deafness, autosomal recessive 31) [20-22] and subsequently in one German family with Usher type 2 syndrome [19]. This evidence concerns the gene WHRN and Usher syndrome type 2.