Consistently, knock-in mice harboring a nonsense mutation of Tric encoding a truncated tricellulin (TricR497X/R497X mice), which mimics one of the mutations observed in the DFNB49 pedigrees, exhibited congenital profound deafness associated with degeneration of hair cell in the cochlea of the inner ear [15]. This evidence concerns the gene MARVELD2 and deafness.