Table 2.
Summary of audiometric results for the 19 IOPD patients at birth and after 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 |
T-ABR, threshold auditory brainstem evoked response; OAE, otoacoustic emission; Tym., tympanogram; HL, hearing loss; cond., conductive; SNHL, sensorineural hearing loss; bil., bilateral; ABG, air-bone conduction gap; + , present; -, absent; and NA, not applicable
aASHA definition of the degree of hearing loss (Clark 1981): The hearing loss degree was classified as normal (< -10 ≦ 15 dB HL), slight (> 15 ≦ 25 dB HL), mild (> 25 ≦ 40 dB HL), moderate (> 40 ≦ 55 dB HL), moderate severe (> 55 ≦ 70 dB HL), severe (> 70 ≦ 90 dB HL), or profound (> 90 dB HL) hearing loss
bConductive component in case of an air‐bone gap of ≥ 15 dB