Genes that are linked to hearing loss
| Gene | Protein | Mouse mutant | Usher syndrome subtype | Other forms of deafness in humans |
| MYO7A | Myosin VIIa | shaker 1; headbanger | USH1B | DFNB2, DFNA11 |
| USH1C | Harmonin | deaf circler; targeted mutation | USH1C | DFNB18 |
| CDH23 | Cadherin 23 | waltzer; salsa | USH1D | DFNB12 |
| PCDH15 | Protocadherin15 | Ames waltzer | USH1F | DFNB23 |
| USH1G | SANS | Jackson shaker | USH1G | – |
| USH2A | Usherin | targeted mutation | USH2A | – |
| GPR98 | VLGR1 | Gpr98del7TM; targeted mutation | USH2C | – |
| DFNB31 | Whirlin | whirler | USH2D | DFNB31 |
| ACTB | βcyto-actin | Not available | – | Syndromic hearing loss |
| ACTG1 | γcyto-actin | Targeted mutation | – | DFNA20/26 |
| ESPN | Espin | Jerker | – | DFNB36 |
| PTPRQ | PTPRQ | Ptprq−/− | – | – |
| MYO6 | Myosin VI | Snell’s waltzer; tailchaser | DFNA22 DFNB37 | |
| RDX | Radixin | Targeted mutation | – | DFNB24 |
| MYO3A | Myosin IIIa | Not available | – | DFNB30 |
| MYO15A | Myosin XV | Shaker 2 | DFNB3 | |
| SLC26A5 | Prestin | Targeted mutation | Nonsyndromic hearing loss |
| Gene | Protein | Mouse mutant | Usher syndrome subtype | Other forms of deafness in humans |
| Myosin VIIa | USH1B | DFNB2, DFNA11 | ||
| Harmonin | USH1C | DFNB18 | ||
| Cadherin 23 | USH1D | DFNB12 | ||
| Protocadherin15 | USH1F | DFNB23 | ||
| SANS | USH1G | – | ||
| Usherin | targeted mutation | USH2A | – | |
| VLGR1 | USH2C | – | ||
| Whirlin | USH2D | DFNB31 | ||
| βcyto-actin | Not available | – | Syndromic hearing loss | |
| γcyto-actin | Targeted mutation | – | DFNA20/26 | |
| Espin | – | DFNB36 | ||
| PTPRQ | – | – | ||
| Myosin VI | DFNA22 DFNB37 | |||
| Radixin | Targeted mutation | – | DFNB24 | |
| Myosin IIIa | Not available | – | DFNB30 | |
| Myosin XV | DFNB3 | |||
| Prestin | Targeted mutation | Nonsyndromic hearing loss |
Genes that are discussed in the text and linked to hearing loss. All genes are expressed in hair bundles and required for bundle development/function. DFNA, autosomal dominant mode of inheritance; DFNB, autosomal recessive. A more complete list of genes linked to hearing loss can be found at http://hereditaryhearingloss.org and http://hearingimpairment.jax.org/index.html.