Table 3.
Age at onset, progression, and severity of sensorineural deafness before CI in 10 MYH9 -RD patients
| Patient/ family | Age at onset 1 | Age at first use of hearing aids | Age at evolution toward PTA > 70 dB NHL | Age at CI | PTA before CI right/left 2 |
|---|---|---|---|---|---|
| 1/1 |
20 |
23 |
24 |
34 |
87/82 |
| 2/2 |
20 |
30 |
32 |
40 |
115/120 |
| 3/3 |
8 |
34 |
34 |
43 |
91/110 |
| 4/4 |
17 |
64 |
70 |
72 |
107/106 |
| 5/5 |
20 |
25 |
25 |
27 |
78/91 |
| 6/6 |
25 |
48 |
48 |
50 |
118/116 |
| 7/7 |
3 |
5 |
8 |
12 |
97/104 |
| 8/7 |
4 |
13 |
19 |
30 |
121/111 |
| 9/7 |
19 |
21 |
24 |
46 |
126/121 |
| 10/8 |
30 |
33 |
44 |
54 |
100/85 |
| Mean (SD) | 16.6 (9) | 29.6 (17) | 32.8 (17) | 40.8 (16) |
Notes: 1 = self-reported. 2 = the respective audiometric tracings are showed in Figure 1.