In the last 18 months, 102 patients affected by HHL and negative in GJB2, GJB6 and MT-RNR1 analyses were recruited and analysed through a multistep and integrative approach (Figure 1) that comprises: (1) a detailed clinical characterisation to distinguish NSHL patients from SHL ones; (2) the analysis of STRC-CATSPER2 and OTOA deletions in patients affected by NSHL; (3) STRC sequencing in patients carrying a heterozygous deletion of STRC and an audiometric pattern suggestive of Deafness, autosomal recessive 16; (4) WES analysis in patients negative to steps 2 and 3 and in SHL subjects. Here, GJB2 is linked to nodular sclerosis classical Hodgkin lymphoma.