Step 1 of 6
EV · HIGHUniversal Newborn Hearing Screening
Screen by 1 month — OAE or AABR, NICU infants get AABR
Every newborn is screened before discharge or by one month of age (the "1" of the JCIH 1-3-6 rule). Screening technology is risk-stratified: well-baby nursery infants can be screened with otoacoustic emissions (OAE) or automated auditory brainstem response (AABR); NICU infants and any baby with a JCIH high-risk factor are screened with AABR, because OAE misses auditory neuropathy spectrum disorder (ANSD) — and ANSD is over-represented in the NICU population.
- OAE — well-baby nursery
- AABR — NICU and any JCIH risk factor
- Two-stage screen permitted (OAE → AABR for fails)
- Document screen result before discharge
- Refer high-risk infants for periodic surveillance even if screen passes
Key statistics
EHDI 1-3-6 rule
1 mo · 3 mo · 6 mo
Screen by 1 month · diagnose by 3 months · intervene by 6 months — JCIH benchmark
★ High-yield pearls (chapter-wide)
- Screen by 1 month, diagnose by 3, intervene by 6 — the JCIH 1-3-6 rule is the binding operational metric.
- OAE-only screens miss ANSD — high-risk infants must be screened with AABR.
- A passed newborn screen does not rule out CHL — JCIH risk factors warrant ongoing audiological surveillance to age 5.
- Always send cCMV PCR (saliva or urine) before 3 weeks of age in any infant with congenital SNHL — the diagnostic window closes at 21 days.
- Comprehensive genetic testing yields a diagnosis in 40–65% of childhood SNHL — first-line in most modern centres.
- Profound bilateral congenital SNHL → ECG mandatory at diagnosis (Jervell & Lange-Nielsen syndrome).
- Preauricular pits + SNHL → renal ultrasound (branchio-oto-renal).
- Cochlear nerve deficiency on MRI changes the implant plan from CI to ABI — confirm before consenting for CI.
- Cochlear implantation before 12 months drives the best spoken-language outcomes — JCIH target.
- EVA progresses with head trauma — counsel against contact sports.
- Post-meningitic SNHL is a CI emergency — beat the cochlear ossification.
- Re-offer genetic testing every 3–5 years for previously idiopathic cases — panels keep improving.
Evidence base
5 sources- HIGH
Joint Committee on Infant Hearing · J Early Hear Detect Interv · 2019JCIHPMID 31633729
Foundational guideline for universal newborn hearing screening — defines 1-3-6 milestones, two-stage screening pathway, AABR requirement for NICU, and the risk-factor surveillance list. Vol 4(2):1-44.
- HIGH
Smith RJH, Bale JF Jr, White KR · Lancet · 2005Peer reviewPMID 15752533
Lancet seminar covering universal newborn hearing screening rationale, OAE vs AABR selection, and downstream diagnostic pathway. Vol 365(9462):879-890.
- HIGH
Morton CC & Nance WE · N Engl J Med · 2006Peer reviewPMID 16707752
Authoritative NEJM review of universal newborn hearing screening programmes, pickup rates, and the genetic-aetiology dominance of the screen-positive population. Vol 354(20):2151-2164.
- HIGH
Lieu JEC et al. · JAMA · 2020Peer reviewPMID 33258894
VERIFY — Contemporary JAMA narrative review framing the modern paediatric hearing-loss diagnostic and management approach — anchors the up-to-date practice context for Step 1. Vol 324(21):2195-2205.
- MOD
NHS England · 2026NHS NHSP / GOV.UK
UK national protocol for screen technology selection (AOAE first stage, AABR second stage, AABR-only for NICU), reporting timelines, referral pathways, and cCMV-pathway handling. GOV.UK page last updated 25 Feb 2026.