WHRN and sensorineural hearing loss disorder: These findings are consistent with mapped pathogenic variants in human patients and their respective phenotypes: DFNB31 patients, who suffer from profound prelingual sensorineural hearing loss (Mustapha et al., 2002), have mutations within exons 10 and 11 of DFNB31 (Mburu et al., 2003, Mustapha et al., 2002, Tlili et al., 2005), which affect the PDZ3 domain of both WHRN-L and WHRN-S, likely resulting in truncated, non-functional proteins.