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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.