Paeds · ent-hearing-and-oral-health
Hearing assessment and childhood hearing loss
Also known as Hearing assessment and childhood hearing loss · Paediatric hearing loss · Newborn hearing screening · Permanent childhood hearing loss · Glue ear and conductive hearing loss
Fellowship guide to hearing assessment and childhood hearing loss: how universal newborn hearing screening (AABR and OAE) and the 1-3-6 milestones catch permanent congenital hearing loss, the conductive-versus-sensorineural framework with otitis media with effusion as the commonest acquired cause, the aetiology (genetic including GJB2, congenital CMV as the commonest infective cause, auditory neuropathy spectrum disorder), age-appropriate audiometry and tympanometry, grommet criteria with three months of watchful waiting and the Paradise developmental evidence, hearing aids and cochlear implantation, and the principle that the open critical period makes early identification non-negotiable.
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- A baby who refers on newborn hearing screening and does not return for diagnostic testing is the single most preventable cause of lifelong language delay — every refer must be tracked to a confirmed diagnosis by three months and enrolment in early intervention by six months, because the brain's critical period for auditory development is already open
- Sensorineural hearing loss after bacterial meningitis is an audiological emergency — the cochlea can ossify within weeks, closing the window for cochlear implantation, so every child with bacterial meningitis must have hearing assessed urgently before discharge and again in the weeks that follow
- An otoacoustic emissions screen can be normal in auditory neuropathy spectrum disorder, because it tests outer hair cells and not the nerve — NICU graduates, who carry the highest risk of this entity, should be screened with automated auditory brainstem response
- A child who passes the newborn screen is not guaranteed normal hearing for life — congenital CMV, enlarged vestibular aqueduct and GJB2-related loss can be delayed-onset or progressive, so any parental concern, speech delay or regression in responding must trigger re-assessment
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- Hearing assessment and childhood hearing loss
- Childhood hearing loss: screening, conductive versus sensorineural and stepwise management
- Newborn hearing screening and childhood hearing loss: the 1-3-6 milestones and grommet criteria
- Long case: the child with permanent childhood hearing loss
- Short case: the child with delayed speech and suspected hearing loss
- Childhood hearing loss: screening, classification and management
- Communication: breaking the news of a failed newborn hearing screen
- Ear, nose and throat: hearing assessment and childhood hearing loss
- Patient Care: newborn hearing screening and childhood hearing loss
- Medical Expert: hearing assessment and childhood hearing loss
Overview & Definition
A baby who does not startle to a hand-clap at six months is the picture most clinicians carry of childhood deafness, yet the whole point of modern paediatric hearing care is that this picture should never be the presenting one. Universal newborn hearing screening finds permanent congenital hearing loss in the first weeks of life, before any parent or clinician could notice it, because the brain's window for learning to process sound and language opens in infancy and narrows with every passing month. Childhood hearing loss is therefore a screening-driven, time-critical diagnosis in which the paediatrician's job is to keep every child moving along a audited pathway, not to wait for symptoms. [1] [6]
Hearing loss in a child is any reduction in the sensitivity or the neural processing of sound that interferes with the detection of speech and the development of spoken language, and it is conventionally separated by where the fault lies. A conductive loss sits in the outer or middle ear, where sound is mechanically impeded before it reaches the cochlea; a sensorineural loss sits in the cochlea or the auditory nerve; and a mixed loss has elements of both. The distinction matters because the causes, the tests and the treatments diverge completely, and because the single commonest acquired childhood hearing loss, otitis media with effusion, is conductive and largely reversible, whereas permanent childhood hearing loss is usually sensorineural and irreversible. [1]
The number that frames the whole topic is roughly one to two babies in every thousand born with permanent hearing loss, with a larger group accumulating by school age as delayed-onset and progressive losses declare themselves. The aim of universal screening, codified as the one-three-six milestones, is to identify these children early enough that the brain still has time to build normal auditory pathways. This topic covers the screening programme, conductive-versus-sensorineural framework, aetiology and risk factors, the age-appropriate audiometric tests, the grommet decision for glue ear, and the definitive rehabilitation of permanent loss with hearing aids and cochlear implantation. [2] [1]
References12ShowHide
- [1]Korver AMH; Smith RJH; Van Camp G; Schleiss MR; Bitner-Glindzicz MA; Lustig LR; Usami S; Boudewyns AN Congenital hearing loss. Nat Rev Dis Primers, 2017.PMID 28079113
- [2]Butcher E; Dezateux C; Cortina-Borja M; Knowles RL Prevalence of permanent childhood hearing loss detected at the universal newborn hearing screen: Systematic review and meta-analysis. PLoS One, 2019.PMID 31295316
- [3]Liddle K; Beswick R; Fitzgibbons EJ; Driscoll C Aetiology of permanent childhood hearing loss at a population level. J Paediatr Child Health, 2022.PMID 34546616
- [4]Kraft CT; Malhotra S; Boerst A; Thorne MC Risk indicators for congenital and delayed-onset hearing loss. Otol Neurotol, 2014.PMID 25251299
- [5]Sininger YS; Cone-Wesson B; Folsom RC; Gorga MP; Vohr BR; Widen JE; Eilers E; Norton SJ Identification of neonatal hearing impairment: auditory brain stem responses in the perinatal period. Ear Hear, 2000.PMID 11059700
- [6]Watkin PM; Baldwin M Identifying deafness in early childhood: requirements after the newborn hearing screen. Arch Dis Child, 2011.PMID 21047829
- [7]Vos B; Noll D; Whittingham J; Pigeon M Cytomegalovirus - A risk factor for childhood hearing loss: A systematic review. Ear Hear, 2021.PMID 33928914
- [8]Fowler KB; Boppana SB Congenital cytomegalovirus infection. Semin Perinatol, 2018.PMID 29503048
- [9]Goderis J; Keymeulen A; Smets K; Van Hoecke H; De Leenheer E; Boudewyns A; Desloovere C; Kuhweide R; Dhooge I; Deggouj N Hearing in children with congenital cytomegalovirus infection: results of a longitudinal study. J Pediatr, 2016.PMID 26858192
- [10]Rosenfeld RM; Shin JJ; Schwartz SR; Coggins R; Gagnon L; Hackell JM; Hoelting D; Hunter LL; Kummer AW; Payne SC; Moore DS Clinical Practice Guideline: Otitis Media with Effusion (Update). Otolaryngol Head Neck Surg, 2016.PMID 26832942
- [11]MacKeith S; Mulvaney CA; Galbraith K; Webster KE; Rovers MM; Schilder AGM; Brenner MJ; Russell RT; Blackmore H; Daniel M; Williamson IG; Burton MJ Ventilation tubes (grommets) for otitis media with effusion (OME) in children. Cochrane Database Syst Rev, 2023.PMID 37965944
- [12]Paradise JL; Feldman HM; Campbell TF; Dollaghan CA; Colborn DK; Bernard BS; Rockette HE; Janosky JE; Pitcairn DL; Sabo DL; Kurs-Lasky M; Smith CG Effect of early or delayed insertion of tympanostomy tubes for persistent otitis media on developmental outcomes at the age of three years. N Engl J Med, 2001.PMID 11309632