Table 2.
Locus | Gene | 1st | Last | Audioprofile* |
---|---|---|---|---|
DFNA2B | GJB3 | 73 | 65 | High frequency; progressive |
DFNA9 | COCH | 48 | 29 | High frequency; progressive |
DFNA2A | KCNQ4 | 6 | 7 | High frequency; progressive |
DFNA16 | unknown | 3 | 0 | - |
DFNA43 | unknown | 3 | 8 | - |
DFNA22 | MYO6 | 3 | 4 | High frequency; progressive |
DFNA57 | unknown | 2 | 5 | - |
DFNA15 | POU4F3 | 2 | 9 | High frequency; progressive |
DFNA16 | unknown | 0 | 3 | - |
DFNA25 | SCL17A8 | 1 | 2 | High frequency; progressive |
DFNA24 | unknown | 0 | 2 | - |
DFNA44 | CCDC50 | 0 | 2 | Low to mid frequencies; progressive |
DFNA8/12 | TECTA | 0 | 1 | Mid-frequency loss |
DFNA2 notAnotB | unknown | 0 | 1 | - |
DFNA13 | COL11A2 | 0 | 1 | Mid-frequency loss |
DFNA18 | unknown | 0 | 1 | - |
DFNA50 | MIRN96 | 0 | 1 | Flat; progressive |
*Gene and phenotype from: Shearer AE, Hildebrand MS, Smith RJH, 1993.15