In a genetically-engineered mouse model carrying mutations in TMPRSS3, a serine protease in which mutations are associated with non-syndromic autosomal recessive deafness (DFNB8/10), inactivation of the protease shortly after birth led to severe and rapid degeneration of the organ of Corti and cochlear and saccular hair cells [30]. Here, TMPRSS3 is linked to hearing loss, autosomal recessive.