We confirmed with our data that the variants classified as “hypomorphic” in GJB2, such as c.101T>C: p.(Met34Thr) and c.109G>A:p.(Val37Ile) were in fact causative of the hearing loss, since no other relevant variants were detected, and the degree of hearing impairment is compatible with the occurrence of these variants. This evidence concerns the gene GJB2 and hearing loss disorder.