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Table 3 Age at onset, progression, and severity of sensorineural deafness before CI in 10 MYH9 -RD patients

From: Cochlear implantation is safe and effective in patients with MYH9-related disease

Patient/ family Age at onset1 Age at first use of hearing aids Age at evolution toward PTA > 70 dB NHL Age at CI PTA before CI right/left2
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)  
  1. Notes: 1 = self-reported. 2 = the respective audiometric tracings are showed in Figure 1.