EN4.20 | Deaf Child — Summary & Reflection

KEY TAKEAWAYS

Childhood hearing loss affects 1–2 per 1000 live births with severe-to-profound SNHL:

• Aetiology: 50% genetic (connexin 26/GJB2 most common non-syndromic; syndromic: Waardenburg, Usher, Pendred, Alport) + 50% acquired (TORCH/CMV most common congenital viral; meningitis; ototoxicity; prematurity).
• CHL in children: glue ear (OME) is commonest — bilateral, ages 2–7, managed by watchful waiting or grommets.
• Neonatal screening: OAE (screen, 1 month) → ABR (confirm, 3 months) → hearing aid fitting (6 months) — the 1-3-6 rule.
• Critical period: primary auditory cortex most plastic in first 2 years; cochlear implantation before 18–24 months = best language outcomes.
• Cochlear implant: profound bilateral SNHL ≥90 dB, failed hearing aids, intact cochlear nerve on MRI; post-meningitic ossification = urgent CI before cochlea ossifies.
• Investigation: OAE + ABR (infants), VRA/play audiometry (older children), tympanometry, HRCT/MRI, CMV PCR, GJB2 mutation testing.

REFLECT

The 9-month-old in the opening scenario has already lost the most critical 9 months of auditory development. His mother responded appropriately to the neonatal screen but the system failed to follow up effectively. Reflect on the multiple levels at which the intervention system failed this child: the family was not adequately counselled about why follow-up was urgent; the health system did not have an active recall mechanism for screen-positive neonates; and the primary healthcare provider who saw the child at routine immunisation visits did not check hearing milestones. What single-point-of-care intervention — accessible even in a rural primary health centre — could have identified this child sooner? Consider also the ethical dimension: as this child's doctor, how do you manage the conversation with the parents about the developmental loss that has already occurred, while maintaining hope and focusing on the interventions still available?