X-Linked Nonsyndromic Hearing Loss
| Locus* (OMIM) | Gene (OMIM) | Key Locus References (PubMed) | Key Gene References (PubMed) | Evidence (ClinGen)  |
|---|---|---|---|---|
| DFNX1 | PRPS1 | Tyson et al., 1996 | Liu et al., 2010 | Definitive |
| DFNX2 | POU3F4 | de Kok et al., 1995 | de Kok et al., 1995 | Definitive |
| DFNX3 | unknown | Lalwani et al., 1994; Pfister et al., 1998 | n/a | n/a |
| DFNX4 | SMPX | del Castillo et al., 1996 | Huebner et al., 2011; Schraders et al., 2011 | Definitive |
| DFNX5 | AIFM1 | Wang et al., 2006 | Zong et al., 2015 | Definitive |
| DFNX6 | COL4A6 | Rost et al., 2014 | Rost et al., 2014 | Limited |
| DFNX7 | GPRASP2 | Xing et al., 2017 | Xing et al., 2017 | not reviewed |
n/a = not applicable
* Previous nomenclature designated X-linked loci as DFN but this has been changed to DFNX.
Y-Linked Nonsyndromic Hearing Loss
| Locus (OMIM) | Gene (OMIM) | Key Locus References (PubMed) | Key Gene References (PubMed) | Evidence (ClinGen) |
|---|---|---|---|---|
| DFNY1 | unknown | Wang et al., 2004 | n/a | n/a |
| DFNY2 | TBL1Y | Di Stazio et al., 2019 | Di Stazio et al., 2019 | not reviewed |
n/a = not applicable