ILDR1 and deafness: A review of all mutations reported up to date, the in-silico analysis of their consequences at the protein level (Table 1 and Fig 3) and the correlation with their associated phenotype (Table 2), suggested that the mutation in the extracellular domain, induces moderate deafness that is detected at low frequencies, but any mutation disturbing the intracellular domain of the ILDR1 protein will result in a severe deafness at low frequencies.