Abstract
The most frequent cause of recessive hearing loss are mutations in GJB2 gene (connexin26, Cx26). Among the individuals with hearing loss with mutations in this gene, 10% to 50% present a single recessive mutation in heterozygosis, indicating that haplo-insufficiency of GJB2 may interact with other types of mutations in the same gene, in the neighbor gene GJB6, or even in other genes. The …