Individual | Age (ys.) | Hearing loss | Age of onset (ys.) | Course | OSBPL2 |
---|---|---|---|---|---|
II:1 | 36 (†) | no | no sample | ||
II:2 | 69 (†) | not noted by any of the five children, but nasal pronunciation | ? | ? | no sample |
II:5 | 93 (†) | no | wildtype | ||
II:7 | 78 (†) | no | no sample | ||
III:2 | 76 | no | wildtype | ||
III:4 | 71 | unilateral (untreated otitis media) | wildtype | ||
III:6 | 74 (†) | no | wildtype | ||
III:7 | 71 | yes | 12 | worse after birth of children, stress; CI around 50 ys. | p.Arg50Alafs*103 |
III:8 | 67 | no | wildtype | ||
III:9 | 61 | yes | 30 | CI at 50 ys. | p.Arg50Alafs*103 |
III:10 | 64 | only temporary, episodes of acute hearing loss | wildtype | ||
IV:6 | 58 | no | wildtype | ||
IV:7 | 50 | yes | 10 | CI at 36 ys. | p.Arg50Alafs*103 |
IV:8 | 50 | no | wildtype | ||
IV:9 | 49 | yes | 12 | CI at 39 ys. | p.Arg50Alafs*103 |
IV:10 | 45 | yes | 22 | hearing aids at 34 ys. | p.Arg50Alafs*103 |
IV:11 | 44 | no | wildtype | ||
IV:12 | 39 | no data from investigations; hearing worse under stress | ? | ? | p.Arg50Alafs*103 |
IV:13 | 35 | no; two healthy daughters | no sample | ||
V:2 | 28 | yes | 15 | worsening after birth of children; CI at 27 ys. | p.Arg50Alafs*103 |
V:3 | 26 | yes | 11 | hearing aids at 12 ys. | p.Arg50Alafs*103 |
V:4 | 15 | no | wildtype | ||
V:5 | 16 | no | wildtype | ||
V:6 | 20 | yes | 10 | hearing aids at 15 ys. | p.Arg50Alafs*103 |
V:7 | 17 | no | wildtype | ||
VI:1 | 3 | no (normal OAEs at 3 ys.) | wildtype | ||
VI:2 | 4 months | no |