| GENE | LOCUS or SYNDROME | AMINO ACID CHANGE | NUCLEOTIDE CHANGE | EXON | ETHNICITY (Number of families) | REFERENCES |
| COCH | DFNA9 | P51S | 207C>T | exon 4 | Belgian (1), Dutch (2) | Fransen 1999 |
| V66G | 253T>G | exon 4 | American (1) | Robertson 1998 | ||
| G88E | 319G>A | exon 5 | American (1) | Robertson 1998 | ||
| I109N | 382T>A | exon 5 | Australian (1) | Kamarinos 2001 | ||
| W117R | 405T>C | exon 5 | American (1) | Robertson 1998 |