Among the genes encoding these proteins, mutated TRIC (tricellulin) and ILDR1 (angulin-2/ILDR1) have been reported to cause autosomal recessive nonsyndromic deafness DFNB49 (MIM 610153) [10–12] and DFNB42 (MIM 609646) [13,14], respectively. Here, ILDR1 is linked to hearing loss, autosomal recessive.