Table 2 Summary of audiometric results for the 19 IOPD patients at birth and after enzyme replacement therapy
From: Hearing characteristics of infantile-onset Pompe disease after early enzyme-replacement therapy
Patient no. (n = 19) | Hearing screen (T-ABR) right/left (dB) | OAE | Age of last hearing exam (year) | Average hearing threshold (dB) (500, 1000, 2000 Hz) | Tym (right/left) | Post-ERT hearing loss typea, b | ||
|---|---|---|---|---|---|---|---|---|
Right | Left | Right | Left | |||||
1 | Pass | + | 7.5 | 21.7 | 21.7 | A | A | Normal |
2 | NA | + | 6.7 | 25.0 | 33.3 | A | A | Single ear SNHL, Mild |
3 | Pass | − | 6.8 | 41.7 | 45.0 | C | C | Bil. mixed HL, Moderate |
4 | Pass | + | 1.2 | 21.7 | 21.7 | A | A | Normal |
5 | Pass | + | 5.5 | 16.7 | 15.0 | A | A | Normal |
6 | Pass | + | 5.4 | 16.7 | 15.0 | A | A | Normal |
7 | Pass | + | 4.7 | 13.3 | 10.0 | A | A | Normal |
8 | Pass | + | 4.8 | 20.0 | 13.3 | A | A | Normal |
19 | Pass | + | 4.9 | 25.0 | 23.3 | A | A | Normal |
10 | Pass | NA | 4.3 | 26.7 | 28.3 | B | B | Bil. cond. HL, Mild |
11 | Pass | NA | 4.7 | 26.7 | 26.7 | A | A | Bil. SNHL, Mild, |
12 | Pass | + | 3.1 | 15.0 | 15.0 | A | A | Normal |
13 | Pass | + | 2.8 | 20.0 | 20.0 | A | A | Normal |
14 | Pass | + | 1.1 | 25.0 | 25.0 | A | A | Normal |
15 | Failed | − | 1.1 | 35.0 | 35.0 | A | A | Bil. SNHL, Mild |
16 | Pass | + | 1.8 | 20.0 | 20.0 | A | A | Normal |
17 | Pass | + | 1.5 | 20.0 | 20.0 | A | A | Normal |
18 | Pass | + | 0.8 | 20.0 | 20.0 | A | A | Normal |
19 | Failed | + | 0.3 | 40.0 | 40.0 | A | A | Bil. SNHL, Mild |