JOURNAL CLUB · ARCHIVE
Late-onset, progressive sensorineural hearing loss in the paediatric population: a systematic review
Corazzi V, Fordington S, Holland Brown T, et al. European Archives of Oto-Rhino-Laryngology. 2024;281(7):3397-3421. A systematic review.
DOES NOT RULE OUT
LATER HEARING LOSS
Congenital CMV is the leading link
21 of 37 studies associated late-onset SNHL with congenital CMV. Age at hearing-loss diagnosis ranged 0.75-204 months (mean ~46).
Perinatal and NICU factors
16 studies linked it with NICU stay, prematurity, neonatal respiratory failure, mechanical ventilation, ECMO support and hypocapnia.
A pass is not a guarantee
Every child had passed the newborn screen, yet hearing declined later - screening is a snapshot, not lifelong cover.
Surveillance matters
At-risk children (cCMV, NICU graduates) need ongoing audiological follow-up, with a low threshold to re-test for any speech or language concern.
WHY THIS MATTERS
- Newborn screening catches congenital hearing loss, but some SNHL appears or progresses later
- A "pass" can create false reassurance and delay diagnosis
- Late-onset SNHL in early childhood harms speech, language and development if missed
- Congenital CMV is a common, often silent cause - and one with surveillance and treatment pathways
- NICU graduates carry several overlapping risk factors
STRENGTHS
- PRISMA-compliant search across four databases
- Focused on children who PASSED the newborn screen - a clinically important blind spot
- 37 studies synthesised
- Clarifies cCMV as the dominant association
- Highlights actionable perinatal risk factors
LIMITATIONS
- Observational studies only - association, not causation
- Heterogeneous definitions of "late-onset" and "progressive", and varied audiology methods
- Very wide range in age at diagnosis (0.75-204 months)
- Publication and ascertainment bias are likely
- No pooled meta-analysis or quantitative risk estimates
Practice implications
In NSW, newborns are screened through SWISH (Statewide Infant Screening - Hearing). This review is a reminder that a pass is not a lifelong guarantee. Babies with congenital CMV or significant perinatal risk (NICU stay, prematurity, ventilation, ECMO) warrant ongoing audiological surveillance and a low threshold to re-test if speech or language concerns arise. Targeted CMV testing in babies who refer on screening, and structured follow-up of NICU graduates, can catch progressive loss early enough to help.
Which of your NICU graduates would you flag for ongoing hearing surveillance, and how?
How does congenital CMV cause late-onset and progressive hearing loss, and does early antiviral treatment change it?
What do you tell parents about the meaning - and the limits - of a "pass" on the newborn hearing screen?
How would you act on a parental concern about hearing or speech in a child who passed screening?
Eur Arch ORL 2024 SWISH (NSW)
Newborn hearing screen CONGENITAL CMV
Information & surveillance JCIH 2019
Infant hearing guidance
Take-home message: Passing the newborn hearing screen does not rule out hearing loss for life. In children up to 16 who had passed screening, this systematic review found late-onset, progressive sensorineural hearing loss most strongly linked to congenital CMV, and also to perinatal factors such as NICU stay, prematurity, ventilation and ECMO. The neonatal lesson: identify at-risk babies and arrange ongoing audiological surveillance, with a low threshold to re-test for speech or language concerns.
For educational purposes only. Journal club appraisal - figures paraphrased from the published review; read the full article for complete data. Always align practice to current local guidelines.