Guidance

Guidelines for surveillance and audiological referral for infants and children following newborn hearing screen

Updated 6 November 2024

1. Overview

The aim of the Newborn Hearing Screening Programme in England (NHSP) is the identification of permanent childhood hearing impairment [1](PCHI) in newborn babies.

Even with a very sensitive newborn hearing screen, some children will develop a hearing loss later (Fortnum et al 2001) or will have missed screening or follow up. Therefore, a wider system is needed to identify these children. This document identifies which babies should be followed up and monitored, how and when this should be done, and by whom. It covers surveillance, referral and audiological monitoring following newborn hearing screening. It does not cover all the conditions requiring hearing assessment which may occur in older children. Training should be provided to primary care and other professionals to ensure early referral of children who have a high risk of late-onset or acquired hearing loss, and written care pathways should be developed at local service level to cover these.

2. General comments

2.1 Further testing post NHSP screen

A general principle is that babies whose newborn hearing screen shows clear responses in both ears should not be subject to repeated screens, tests or follow-up unless they meet one or more of the criteria specified here. Parents are given appropriate checklists after their newborn’s screen to refer to in the first instance if there is concern.

A child referred for audiological assessment should not be discharged until testing meets the discharge criteria outlined in the BSA document “Guidelines for the early audiological assessment and management of babies referred from the newborn hearing screening programme”.

2.2 Age at testing

All ages in this document refer to corrected ages (from expected date of delivery) and are indicative; in practice ‘4 weeks’ may be 2 to 8 weeks, and ‘8 months’ may be 7 to 9 months (and in exceptional circumstances up to 12 months). While ABR testing is technically possible at any age, in practice it becomes increasingly difficult over 3 months (12 weeks) as the required sleep state becomes less predictable. Reliable behavioural assessment by Visual Reinforcement Audiometry (VRA) is increasingly possible from 6 months of age, but developmental delay will affect the ability to test behaviourally, as may some serious medical conditions.

2.3 Audiology teams and services

Audiological services that are responsible for the assessment of referrals from the newborn hearing screen must participate in a peer-review process of ABR as detailed by the British Society of Audiology (BSA). See Current Guidance - Principles of Peer Review

Commissioners are required to make sure that Audiology services participate in, and maintain accreditation to, defined quality standards operating under the umbrella of the United Kingdom Accreditation Schemes (UKAS) / Improving Quality in Physiological Services (IQIPS) and as set out by section 7a commissioning schedules 2 and 4, for the newborn hearing screening programme.   

Audiological testing should be carried out in accordance with national standards and guidance. Detailed clinical protocols are available from the BSA. For behavioural testing ear–specific testing should be routinely available.

2.4 Information and data

Audiology departments are responsible for the entry of audiological data onto the NHSP national IT system.  Requirements for audiological data are given in NHSP Operational Guidance Chapter 9 .

In summary this includes:

• Audiological information for all babies referred from the screen
• Audiological information for all babies referred for targeted follow up
• Audiological information for any child (irrespective of screening outcome)  with a later identified PCHI of any degree in either ear identified by 5 years of age. A subset of such cases (who may be ‘false negatives’ for the screen) also need to be notified to the newborn hearing screening programme using the “Review of case of PCHI not identified by the newborn hearing screen” guidance template and form held on the Resources tile of the NHSP national IT system.

3. Categories of babies and recommended follow-up related to the newborn hearing screen

The eligible population is all newborn babies born or resident in England and those babies under 3 months (12 weeks) of age or if born <40 weeks gestation, under 3 months (12 weeks) corrected age, who have moved into the area (and who are the responsibility of NHS England).

3.1 Babies excluded from screen – refer to audiology for ABR

The four groups listed below should not undergo the newborn screen but must be referred directly to audiology for assessment ABR, using the current BSA guidance.

Group 1. Microtia / external ear canal atresia

This exclusion is because these babies will always have a degree of hearing loss. Specific guidance on audiological assessment is given in the BSA document “Guidelines for the early audiological assessment and management of babies referred from the newborn hearing screening programme”.

Group 2. Neonatal bacterial meningitis or meningococcal septicaemia

Confirmed or strongly suspected (in the judgement of the medical team or neonatologist) bacterial meningitis[2] (any organism), or meningococcal septicaemia. This exclusion is made because the risk of sensorineural hearing loss (SNHL) is very high. The urgency for post-bacterial meningitis babies is related to the risk of ossification of the cochlea and that urgent cochlear implant referral may be required. Specific guidance on hearing assessment after meningitis is given in Appendix B.

Group 3. Programmable ventriculo-peritoneal (PVP) shunts in place

This exclusion is because of the risk of magnetic fields arising from audiological stimulus transducers affecting the shunt. Further guidance is available from the BSA. More information is available in Appendix A.

Group 4. Confirmed[3] congenital cytomegalovirus (cCMV)

This exclusion is because the chance of hearing loss is very high and the window of opportunity for treatment is short. These babies should be referred by the medical team to Audiology for an early hearing assessment. The urgency is related to the short window of opportunity for anti-viral treatment. More information is available in Appendix A.

Babies in groups 1-3 should be seen either within 4 weeks of the decision that screening is not appropriate/recovery from the acute episode or by less than 4 weeks (28 days) corrected age if born less than 40 weeks gestation.

Babies in group 4 may need to be seen sooner, within a time scale that is agreed with the paediatrician that permits early anti-viral treatment to start if needed.

Referral is the responsibility of the medical team caring for the baby. However, local screening services should treat these babies as screen referrals and liaise closely with medical and audiology teams to ensure prompt referral to audiology. The local screening service should set the record as a referral on the NHSP national IT system using the screening outcome of ‘incomplete-screening contraindicated’ and expedite and monitor their referral to audiology in conjunction with the medical team.

Responsible for identifying child and referral to Audiology – medical team caring for the child

Responsible for arranging appointment and follow-up - Audiology (copy to NHSP screening local manager)

3.2 Screen declined, incomplete or missed - no routine follow-up

Where the screen was declined, missed or not completed despite invitations, routine referral for targeted follow up is no longer recommended.

Local screening services should make vigorous efforts to maximise newborn screening coverage by 3 months (12 weeks) of age particularly in hard to reach groups. This should include use of the following: telephone / text / email reminders, outreach clinics, home visits, liaison with trust antenatal and newborn screening coordinators, midwifery and health visiting teams, contact with children’s wards and neonatal and paediatric intensive care units to identify readmitted unscreened babies.

Health Visitors (HVs) and GPs must be informed of babies that have not completed screening. Local screening services will need to ensure that parents are provided with information about how to seek assessment in the event of future concern. The HV/GP should discuss with parents the implications of not having (or not completing) the screen. Parents and HVs/GPs should be made aware that they can request an audiological assessment at any time.

Responsible for identifying child and notifying HV/GP – NHSP screening local manager

3.3 Moved into the country before 3 months of age (12 weeks) - screen

A mechanism should be in place to identify babies who move into the area/country under three months (12 weeks) of age who have not been screened. The local screening service must be informed of these babies by child health/health visitor/primary care teams. The local screening service is responsible for adding the baby’s details to the NHSP national IT system, arranging an appointment and undertaking the screen.

If a baby moves in under 3 months of age (12 weeks), but has been screened outside of England, hearing screening should be offered under England’s NHSP protocols. If the parents decline the offer of another screen the record does not need to be added to the national IT system.

Responsible for identifying child and notifying the newborn hearing screening service - HV/Child Health Department/other local arrangement

Responsible for arranging newborn hearing screen appointment - NHSP screening local manager

3.4 Moved into the country after the age of 3 months (12 weeks) – refer

These babies are not eligible for newborn screening. There should be local protocols in place to ensure that the need for audiological referral is identified and appropriate referrals are made.

Responsible for identifying child and making referral – GP/HV/Child Health Department/other local arrangement

3.5 Referred on screen but missed audiology assessment – follow up

When a baby has been referred for immediate follow up from the hearing screen, the practice of offering one or two appointments in audiology and then making a referral for targeted follow up if the baby is not brought is insufficient. These babies have a high risk of SNHL. Audiology and local screening services should liaise closely about these babies. Audiology must make strenuous efforts to secure early attendance of these babies for ABR including discussion with parents and liaison with the family health visitor and GP to facilitate attendance.  

In the event of inability to secure attendance local safeguarding policies must be followed and the HV and GP should be notified.  Advice should be provided about how to make a referral should the family indicate a willingness to attend in future. Completion of follow up for these children should be locally audited with responsibility for audit devolved to a named individual within screening or audiology.

Responsible for identifying child (as not having attended) – Audiology

Responsible for arranging further appointment – Audiology

3.6 Passed screen or audiology follow up, but with specific neonatal risk factors[4] – targeted follow up

Babies with the following specific risk factors must be referred for targeted follow up (behavioural testing around 8 months) or sooner if local protocol in place

• Syndromes associated with hearing loss (including Down’s syndrome)

• Cranio-facial abnormalities, including cleft palate, excluding cleft lip only, minor pits or minor ear tags

• Confirmed congenital infection (including toxoplasmosis, rubella)
• SCBU/NICU over 48 hours with no clear response AOAE both ears but clear response on AABR

Responsible for identifying child– NHSP local screening service

Responsible for arranging appointment– Audiology

4. Ototoxic drugs – Refer at discretion of medical team caring for the child

Various drugs are potentially ototoxic. The main group is aminoglycosides, and these are very commonly used prophylactically in babies. Unless a baby is suspected or known to have a change on the gene m.1555A>G (mitochondrial mutation) the baby should undergo newborn hearing screening and follow up if required as per standard screening protocol. It is now possible to test for this genetic change. If there is such a history, we urge medical teams caring for the child to test for this, be cautious and consider alternative antibiotics, otherwise there is a risk of significant hearing damage.

The responsibility for monitoring of babies receiving ototoxic drugs and appropriate referral for audiological assessment lies with the medical team caring for the child. In deciding whether to make a referral for follow up beyond the screen, one factor will be whether the monitored aminoglycoside levels have exceeded the therapeutic range: see also national guidance on use of gentamicin for neonates (NPSA 2010). In general hearing loss attributable to ototoxicity is likely to be mild and in the higher frequencies and this is more accurately assessed by frequency-specific behavioural testing around 8 months.

However, any baby that is suspected or known to have a change on the gene m.1555A>G and has received aminoglycosides (irrespective of whether blood levels are within the therapeutic range) should be referred for immediate follow-up and audiological monitoring irrespective of screen outcome. Babies with a change on the gene m.1555A>G may have a family history of sensorineural deafness from middle age in the affected individuals, changes in the gene m.1555A>G are usually inherited from a mother, however genetic changes can also occur for the first time in a pregnancy.  

Responsibility for making the referral and communication with family – medical team caring for the child

Responsibility for making appointment - Audiology

5. Specific risk factor or concern occurring later

This section includes a brief discussion about some of the issues that warrant immediate referral for audiological assessment irrespective of any earlier newborn hearing screening result. These are outside of the responsibility of local NHSP services, but this brief outline is included for completeness.

5.1 Parental or professional concern for any reason

Parental concern about an infant’s hearing, or development of auditory or vocal behaviour should always be taken seriously. All professionals who may be in contact with a child should always feel able to refer to Audiology if there is parental concern, or if they themselves are concerned.

5.2 Confirmed or strongly suspected bacterial meningitis, or meningococcal septicaemia, confirmed congenital cytomegalovirus (cCMV), temporal bone fracture, severe unconjugated hyperbilirubinaemia

These medical conditions can cause sensorineural hearing loss in a significant proportion of affected children (meningitis - Fortnum 1992, Fortnum & Davis 1993; temporal bone fracture - Zimmerman et al 1993, Lee et al 1998; hyperbilirubinaemia - Boo et al 1994, Shapiro 2003). If they occur at any point in infancy or childhood after the newborn hearing screen, then immediate referral should be made for audiological assessment on recovery and within 4 weeks of discharge from hospital.

Audiology services should refer to the BAA/BSA Paediatric Audiology Minimum Discharge Criteria (Aged 6 Months +) when assessing these children.

6. Authorship and acknowledgements

The guidance in this document was initially produced by the NHSP Clinical group. Many audiological and medical professionals shared their experience and expertise, and their contributions are acknowledged.

7. APPENDIX A: Historical changes

Some aspects of these guidelines have undergone revisions since the document was first issued in 2003; the main changes are detailed below.

Exclusions from the screen

In 2003 bacterial meningitis was added as a reason to exclude the baby from the newborn screen on the grounds that babies who have bacterial meningitis have a very high risk of having a permanent hearing loss around 1/10 (Fortnum 1992) compared to about 1/100 for other NICU/SCBU babies, and 1/1000 for well babies so that both the screen performance and positive predictive value are totally different from that for the general population. There would be a high risk of missing resulting mild/moderate or high frequency loss in a screen. Performing a screen might also lead to a high false positive rate due to higher incidence of middle ear effusion following meningitis (Fortnum & Davis 1993). Hence, we concluded a screen is not appropriate for this population, and that full assessment by ABR is essential.

The 2019 version added two further categories for exclusion from the screen. These are babies with programmable ventriculo-peritoneal (PVP) shunts in place and babies with confirmed congenital cytomegalovirus (cCMV).

Programmable ventriculo-peritoneal (PVP) shunts are implantable devices that can be fitted to young babies as a treatment for hydrocephalus to drain excess cerebrospinal fluid (CSF) from the brain to another part of the body.  PVP shunts have a magnetic valve, placed just under the skin behind the ear, for adjustment by an external control magnet.  This implanted magnetic valve can unintentionally be reprogrammed by other magnetic devices if they are placed near the ear, which can then lead to a change in CSF pressure, thereby putting the baby at risk.  Audiological equipment, including some screening devices, generate magnetic fields which can cause a potential risk. 

To reduce this risk from hearing screening devices all babies with PVP shunts should be referred directly to audiology for assessment instead of being offered the newborn hearing screen. Audiology services can use insert earphones which pose less risk as the magnetic field is further away from the PVP shunt.  The British Society of Audiology has guidance for audiology services on assessment for patients with PVP shunts. 

Congenital cytomegalovirus is a common virus which can be associated with hearing loss if contracted in utero (congenital CMV or cCMV).  It is the only cause of sensorineural hearing loss that can be treated, but the treatment is time dependent. Identification and treatment within 4 weeks of birth can help stop hearing loss deterioration. 

To speed the identification of hearing loss associated with cCMV it is now recommended that babies with confirmed cCMV before hearing screening has taken place should be referred immediately to audiology for hearing assessment instead of being offered the newborn hearing screen.  Audiology services should seek advice from their infectious diseases team to ensure the babies are followed up appropriately, (Pesch MH, 2021)

Targeted follow up

In 2012 following a major review of the evidence (Wood et al 2011) the following categories were removed from the list for targeted follow up:

• Family history of permanent SNHL from childhood (in parents or siblings)
• Severe jaundice / hyperbilirubinaemia (exchange transfusion level)
• Mechanical ventilation over 5 days, or who have undergone ECMO
• Neuro-degenerative or neuro-developmental disorders

Ototoxic drugs

In versions 4 and 7, we amended our advice to the current position and clarified that it is the responsibility of the medical team caring for the child to decide whether further audiological assessment is required beyond the newborn screen, either immediately or around 8 months and to make any referral to Audiology. For babies with the m.1555A>G mitochondrial genetic change who are abnormally susceptible to aminoglycoside ototoxicity we advised immediate follow-up and audiological monitoring irrespective of screen outcome.

8. APPENDIX B: Guidelines for audiological follow up of babies diagnosed with bacterial meningitis and/or meningococcal septicaemia. March 2024

Scope

NICE guidelines have been produced to help clinicians develop local protocols for hearing assessment in babies up to the age of one year who have been diagnosed with bacterial meningitis and/or meningococcal septicaemia.

Timing of assessment

Hearing assessment needs to be carried out after recovery and not within 48hours of diagnosis, as 10% of cases may have a reversible SNHL in this time (Richardson et al 1997) but should be within four weeks of the child being well enough to be tested. Urgent assessment is required to identify severe/profound hearing loss which may require cochlear implant(s) before any cochlear ossification takes place. The timing of tests needs to be practical and flexible. The aim should be to determine ear-specific and frequency-specific auditory thresholds as soon as possible, to identify hearing loss of any degree or configuration.

Less than 12 weeks corrected age:

Testing would normally be by ABR under natural sleep. The testing requirements for children referred with this risk factor are covered in the BSA Guidelines for the Early Audiological Assessment and Management of Babies Referred from the Newborn Hearing Screening Programme.

Between 12 weeks and 7 months corrected age:

Options should be discussed by the audiologist with parents and include one or more of:

• ABR under natural sleep (especially if the baby is still quite young) or ABR under sedation (for older infants), if there is considerable parental/professional concern or if it has not been possible to obtain a reliable test without sedation
• Waiting until behavioural testing around 7 months (if the baby is close to this age), bearing in mind the importance of urgent assessment as discussed above

Over 7 months corrected age:

Testing would normally be behavioural, for example Visual Reinforcement Audiology (VRA) using ear-specific and frequency specific stimuli. A significant hearing loss should be excluded. If ear- and frequency-specific information cannot be obtained for whatever reason, the child should be further reviewed to rule out any milder degrees of loss.

Further follow up

We reviewed the evidence that there might be late onset hearing loss requiring further follow up after a satisfactory hearing test. The only support found for this was a single case in 59 in Woolley et al (1999), against other studies showing no late onset (Berlow et al 1980, Richardson et al 1997). Therefore, no recommendation for further follow up is made provided the hearing test is clearly normal.

9. References

Berlow SJ, Caldarelli DD, Matz GJ, Meyer DH, Harsch GG. (1980).  Bacterial meningitis and sensorineural hearing loss: a prospective investigation. Laryngoscope. 1980 Sep; 90(9):1445-52.

Boo NY, Oakes M, Lye MS and Said H (1994). Risk Factors Associated with Hearing Loss in term neonates with hyperbilirubinaemia. Journal of Tropical Paediatrics, 40(4):194-7

BSA (2022) Guidelines for the Early Audiological Assessment and Management of Babies Referred from the Newborn Hearing Screening Programme

Fortnum HM (1992). Hearing impairment after bacterial meningitis: a review. Arch Dis Child 67(9), 1128-33.

Fortnum HM & Davis AC (1993). Hearing impairment in children after bacterial meningitis: incidence and resource implications. Brit J Audiol 27, 43-52

Fortnum HM, Summerfield AQ, Marshall DH, Davis AC, Bamford JM. (2001) Prevalence of permanent childhood hearing impairment in the United Kingdom and implications for universal neonatal hearing screening: questionnaire based ascertainment study. BMJ 323: 536–9.

Lee D, Honrado C, Har-El G, Goldsmith A. (1998). Pediatric temporal bone fractures. Laryngoscope. 108(6), 816-21

National Patient Safety Agency (2010). Safer use of intravenous gentamicin for neonates. (Alert No.1085) DH Gateway reference 13534. CAS - Search Alert (mhrs.go.uk) ref: NPSA/2010/PSA001

NHS England. NHS public health functions (‘Section 7A’) agreement

NICE (2024) Recommendations, Meningitis (bacterial) and meningococcal disease: recognition, diagnosis and management, Guidance, NICE

Pesch M H, Kuboushek K, McKee M M, Thorne M C, Weinberg J B. Congenital cytomegalovirus infection BMJ 2021; 373 :n1212 doi:10.1136/bmj.n1212

Richardson MP, Reid A, Tarlow MJ, Rudd PT (1997). Hearing loss during bacterial meningitis. Arch Dis Child. 76(2) 134-8

Shapiro SM (2003). Bilirubin toxicity in the developing nervous system. Review. Pediatr Neurol 29(5):410-21.

Wood SA, Davis AC, Sutton GJ. 2013. Effectiveness of targeted surveillance to identify moderate to profound permanent childhood hearing impairment in babies with risk factors who pass newborn screening. Int J Audiol 52:394-399

Zimmerman WD, Ganzel TM, Windmill IM, Nazar GB, Phillips M (1993). Peripheral hearing loss following head trauma in children. Laryngoscope.103, 87-91.

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[1] NHSP defines this as a permanent hearing loss averaging >= 40dBnHLacross 0.5 to 4kHz, in one of both ears

normal limits across the frequency range

[2] Note that viral meningitis is not considered to be a specific risk to hearing and where this is confirmed the screen can proceed as normal, with no need for extra follow up.

[3] Note: where CMV results are pending, the baby is eligible for the newborn hearing screen

[4] Note: Local screening services may not always be aware of these risk factors particularly where screening is completed early. It is assumed that other professionals, such as the medical team involved with these families will be aware of the need for ongoing audiological assessment and request audiological assessment.