ANSD | Case 18 | Case 19 | Case 33 | Case 7 * |
---|---|---|---|---|
Gender | Male | Male | Male | Male |
Age (at diagnosis) | Congenital | Congenital | Congenital | Adult |
OAE | + | + | - | + |
ABR | absent | absent | absent | absent |
CM | - | - | + | - |
GJB2 Genotype | c.35delG/c.35delG | c.35delG/c.35delG | c.35delG/c.35delG | IVS1+G>A/wt |
Pure Tone Thresholds | 50-70 dB | 60-80 dB | 80-100 dB | 40-70 dB |
IRF | 68% | NA | 50% | 72% |
LRF | 65 dB | 70 dB | 60 dB | 40 dB |
Past History | None | None | Preterm baby (35 wks) NICU (5 d) | None |
CT/MNR | Normal | Normal | Normal | Normal |
Treatment | CI +HA + speech therapy | CI + HA + speech therapy | CI + HA + speech therapy | HA + speech therapy |
Ps. | detects plate and barrel in behavioral audiometry with hearing aids and / a / & / u / | Understanding speech difficulty; PTA oscillations; Some audiogram similar to conductive HL |