Table 2.
Severity of Hearing Losses at Baseline by Genotype Group
| PEX1 - G843D/Null (4 patients; 8 ears) | PEX1 - G843D/G843D (11 patients; 22 ears) | Other PEX -Intermediate & Mild (13 patients; 26 ears) | DBPD (3 patients; 6 ears) | |
|---|---|---|---|---|
|
| ||||
| Ear-specific a | ||||
| Right | 76.0 (16.1) | 76.1 (13.0) | 68.1 (24.2) | 67.9 (14.6) |
| Left | 79.5 (16.1) | 73.3 (10.8) | 67.9 (23.4) | 63.8 (10.7) |
| Severity of Hearing Loss b | ||||
| Normal (<20 dB) | - | - | 2 (7.7%) | - |
| Mild (21–40 dB) | - | - | 2 (7.7%) | - |
| Moderate (41–55 dB) | - | - | 0 (0%) | 2 (33.3%) |
| Mod. Severe (56–70 dB) | 4 (50.0%) | 7 (31.8%) | 4 (15.4%) | 2 (33.3%) |
| Severe (71–90 dB) | 1 (12.5%) | 12 (54.6%) | 15 (57.7%) | 2 (33.3%) |
| Profound (>90 dB) | 3 (37.5%) | 3 (13.6%) | 3 (11.5%) | - |
a
Mean (SD) of 4F-PTA of baseline audiograms. 4F-PTAs were first calculated for each individual using baseline audiograms. Individual baseline 4F-PTAs were then averaged to determine group baseline 4F-PTAs.
b
Degree of hearing loss at baseline shown as number (percentage) of ears.