Mutations in LRTOMT2 are responsible for DFNB63, a non-syndromic, autosomal recessive form of human deafness that is characterized by severe to profound neurosensory hearing loss that can be congenital or prelingual (Ahmed et al., 2008; Du et al., 2008; Kalay et al., 2007; Khan et al., 2007; Tlili et al., 2007). Here, LRTOMT is linked to deafness.