This probably reflected in the phenotype: in fact, in our sample, the homozygosis pattern was associated with an average PTA of 63.70 ± 32.09 dB, probably because of the prevalence of the GJB2 (35delG/35delG) mutation, which is known to be associated with severe-to-profound deafness [23]. Here, GJB2 is linked to deafness.