TMIE and deafness: In a cohort of 30 ARNSHL families the genetic defects were identified in 9 known deafness genes; GJB2, MYO15A, TMC1, BSND, TMPRSS3, MSRB3, HGF, SLC26A4 and TMIE. In the current panel recurrent as well as novel mutations were detected, the novel mutations were identified in GJB2, MYO15A, TMC1, BSND, TMPRSS3 and MSRB3.