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Table 2: 
Audiometric thresholds in patients with Cornelia de Lange Syndrome
Patient No.Sex/Age (yr)Severity of Clinical FeaturesPA Threshold/Air-Bone Gap (dB HL)*BERA Threshold (dB nHL)†
1F/7 monthsClassicR 60; L 75
2F/8 monthsClassicR NR; L NR
3F/2ClassicR 75; L 85
4M/3ClassicR 40; L 50
5F/6ClassicR 65; L 65
6M/17 monthsMildR 30; L 40
7F/3MildR 20; L 20
8F/4MildR 15; L 15
9M/7MildR 30/20; L 30/20
10M/8MildR 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.