STRC deletions are reported in approximately 1% of mixed deafness populations (Francey et al., 2012; Hoppman et al., 2013), making it as a major contributor to congenital hearing impairment, and can cause autosomal recessive NSHL (Verpy et al., 2001) or Deafness-infertility syndrome (DIS) in males if the adjacent CATSPER2 gene is also involved in the deletion (Zhang et al., 2009). Here, CATSPER2 is linked to deafness.