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Table 2 Mean pure-tone averages (PTAs) and average air conduction thresholds from 125 Hz to 8 kHz as a function of age for affected individuals.

From: Patients affected with Fabry disease have an increased incidence of progressive hearing loss and sudden deafness: an investigation of twenty-two hemizygous male patients

Age group (years)

21–30

31–40

41–50

51–60

61–70

Mean ± SD (years)

25.1 ± 2.6

36.6 ± 3

50 ± 0

58.3 ± 0.5

62.5 ± 1.7

Number of ears

(n = 14)

(n = 16)

(n = 4)

(n = 6)

(n = 4)

PTAs, (dBHL)

     

right ear

15.9 (11.9)

19.6 (13.8)

45.8 (10.6)

100.6 (33.7)

23.3 (14.1)

left ear

19 (19)

34 (37)

51.7 (2.3)

45.5 (9.2)

41.7 (30.6)

Air conduction thresholds

     

125 Hz

17.1 (16.1)

27.8 (29.8)

33.7 (4.7)

60 (47)

30 (20.4)

250 Hz

15.3 (15.1)

25.3 (28.4)

36.2 (8.5)

64.2 (43.6)

26.3 (22.5)

500 Hz

17.5 (17.5)

25.9 (28.1)

47.5 (5)

73.3 (36.8)

30 (23.8)

1 kHz

16.1 (13.7)

25.6 (28.5)

46.2 (7.5)

74.2 (36.1)

33.7 (21.7)

2 kHz

18.9 (21.7)

28.8 (30.1)

52.5 (11.9)

71.7 (39.3)

33.7 (22.8)

4 kHz

24.6 (19.8)

49.1 (30.1)

62.5 (6.4)

78.3 (32.6)

50 (10.8)

8 kHz

32.1 (31.8)

60.6 (27.6)

67.5 (8.7)

89.2 (25.4)

66.3 (20.2)

  1. Data are given as mean (SD). DBHL = decibels hearing level.