Audiometric thresholds in patients with Cornelia de Lange Syndrome
| Patient No. | Sex/Age (yr) | Severity of Clinical Features | PA Threshold/Air-Bone Gap (dB HL)* | BERA Threshold (dB nHL)† |
|---|---|---|---|---|
| 1 | F/7 months | Classic | R 60; L 75 | |
| 2 | F/8 months | Classic | R NR; L NR | |
| 3 | F/2 | Classic | R 75; L 85 | |
| 4 | M/3 | Classic | R 40; L 50 | |
| 5 | F/6 | Classic | R 65; L 65 | |
| 6 | M/17 months | Mild | R 30; L 40 | |
| 7 | F/3 | Mild | R 20; L 20 | |
| 8 | F/4 | Mild | R 15; L 15 | |
| 9 | M/7 | Mild | R 30/20; L 30/20 | |
| 10 | M/8 | Mild | R 20/5; L 20/5 |
Note:—PA indicates play audiometry; R, right ear; L, left ear; NR, no response.
* The normal range of hearing is considered to be below 25 dB in play audiometry.
† The normal range of hearing is considered to be below 40 dB nHL in BERA.