Another tight junction protein, ILDR1 (immunoglobulin‐like domain containing receptor 1), does not recruit tricellulin to the cochlear tTJ but rather plays an important role in maintaining the position of tricellulin, and its defects can lead to autosomal recessive hearing loss DFNB42, which further demonstrates the important role of tricellulin in maintaining normal hearing (Morozko et al., 2015). Here, MARVELD2 is linked to autosomal dominant nonsyndromic hearing loss.