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Table 5 Results of speech discrimination tests administered 6 or 12 months after the switch-on of the device (short-term outcome) and at the last follow-up evaluation (long-term outcome) in 10 MYH9 -RD patients

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

Patient/family Short-term outcome Long-term outcome
Discrimination of words (-HA)1[%] Discrimination of sentences (-HA)1[%] Time after CI (years) Discrimination of words (-HA)1[%] Discrimination of sentences (-HA)1[%]
1/1 90 (88) 95 (95) 5 100 (95) 100 (100)
2/2 90 (nd) 90 (nd) 4.5 92 (nd) 90 (nd)
3/3 95 (90) 95 (95) 4.5 98 (97) 100 (100)
4/4 80 (nd) 100 (nd) 5 90 (nd) 100 (nd)
5/5 90 (nd) 98 (nd) 25 nd nd
6/6 0 (0) 0 (0) 17 0 (0) 0 (0)
7/7 97 (88) 100 (nd) 3 100 (85) 100 (nd)
8/7 90 (75) 99 (nd) 9 nd (85)2 nd (100)2
9/7 4 (nd) 24 (nd) 19 nd (42)2 nd (65)2
10/8 95 (nd) 82 (nd) 1 100 (nd) 82 (nd)
  1. Notes: 1 = whenever recorded, results obtained without the contralateral external hearing aid (HA) are reported in parentheses (-HA). 2 = At the time of their last follow-up evaluations, patients 8 and 9 did not usually utilize a hearing aid: therefore, they were tested with CI alone. Abbreviations: CI = cochlear implant, nd = not determined.
  2. The speech perception scores are intended without the use of visual support.