Table 3.
Distribution of audiological findings in FRDA patients
| Audiological presentation | No. of patients N (27) | Age (years old) | GAA1 | GAA2 |
p value GAA1 |
p value GAA2 |
|---|---|---|---|---|---|---|
| PTA (BSA average) | ||||||
|
Normal Abnormal |
15/27 (55%) 12/27 (45%) |
28.9 37.5 |
583 810 |
866 1026 |
0.02* | 0.17 |
| PTA (LF average) | ||||||
|
Normal Abnormal |
15/27 (55%) 12/27 (45%) |
28.5 36.3 |
601 750 |
865 1015 |
0.14 | 0.14 |
| OAEs | ||||||
|
Present Absent |
23/27 (85%) 4/27 (15%) |
31.1 42.5 |
661 818 |
922 1100 |
0.27 | 0.21 |
| ABR | ||||||
|
Normal Abnormal |
6/26 (23%) 20/26 (77%) |
34.3 31.1 |
289 802 |
720 1015 |
0.000* | 0.45 |
| SiQ | ||||||
|
Normal Abnormal |
5/22 (23%) 17/22 (77%) |
32.8 31.1 |
380 724 |
796 950 |
0.012* | 0.28 |
| SiB | ||||||
|
Normal Abnormal |
0/18 (0%) 18/18 (100%) |
27.6 | 687 | 925 | - | - |
| LiSN-S | ||||||
|
Normal/Mild SPD SPD |
3/20 (15%) 17/20 (85%) |
35.6 29.2 |
340 719 |
618 976 |
0.04* | 0.06 |
| GIN | ||||||
|
Normal Abnormal |
7/21 (33%) 14/21 (77%) |
32.3 29.6 |
369 722 |
738 1010 |
0.000* | 0.04* |
*denotes significance. LF, low frequency; BSA, British Society of Audiology; PTA, pure-tone audiometry; OAEs, otoacoustic emissions; ABR, auditory-evoked brainstem responses; SiQ, speech in quiet; SiB, speech in babble; LiSN-S, listening in spatialized noise sentences; GIN, gaps in noise test; Ab, abnormal