Guidance

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

Updated 19 July 2019

1. Overview

The aim of the NHS newborn hearing screening programme in England (NHSP) is the identification of permanent childhood hearing impairment (PCHI) in newborn babies.

PCHI is defined as a bilateral permanent hearing loss averaging >= 40dBnHL across 0.5 to 4kHz.

Even with a very sensitive newborn hearing screen, some children will develop a hearing loss later (Fortnum and others, 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
• who should do this

It covers surveillance, and referral and audiological monitoring - following newborn hearing screening.

It does not cover all of the conditions that require hearing assessment, which could occur in older children.

Training should be provided to primary care providers and other professionals, to ensure early referral of children who have a high risk of late-onset or acquired hearing loss.

Written care pathways should be developed at the local service level, to address these aspects.

1.1 Further testing following a NHSP screen

Babies whose newborn hearing screen shows clear responses in both ears, should not be subject to repeated screens, tests or a follow-up, unless they meet 1 or more of the specified criteria.

Parents are given appropriate checklists after their newborn screen, which they can refer to if there is a concern.

A child referred for audiological assessment should not be discharged, until testing clearly and definitively shows that they have satisfactory responses. In this case ‘clear and definitive’ is a matter for clinical judgement. Normally, it would mean ‘with ear-specific hearing thresholds within normal limits across the frequency range’.

1.2 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 actually be 2 to 8 weeks, and ‘8 months’ may actually be 7 to 9 months (and in exceptional circumstances, up to 12 months).

While auditory brainstem response (ABR) testing is technically possible at any age, in practice it becomes increasingly difficult over 12 weeks, as the required sleep state becomes less predictable.

Reliable behavioural assessment by visual reinforcement audiometry (VRA) is increasingly possible from 5 to 6 months of age, but developmental delay will affect the ability to test behaviourally, as will some serious medical conditions.

1.3 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).

Commissioners must 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), or the Improving Quality in Physiological Services (IQIPS), as set out by NHS England in 2018.

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.

1.4 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 the NHSP Operational Guidance.

In summary this includes audiological information for:

• all babies referred from the screen
• all babies referred for targeted follow-up
• any child (irrespective of screening outcome) who was eligible for newborn hearing screening in England with a later identified moderate-profound 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 national programme team, using the form Review of later identified PCHI which can be found using the link in section 3.3 of NHSP Operational Guidance Chapter 9.

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

2.1 Babies excluded from screen – refer to audiology for ABR

The 4 groups listed below should not undergo the newborn screen but must be referred directly to audiology for assessment using ABR. Detailed guidance on audiological assessment for these 4 groups is given by BSA (2013), or will be included in the next update.

Group 1. Microtia and 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 guidance 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 paediatrician or neonatologist) bacterial meningitis (any organism), or meningococcal septicaemia. This exclusion is made because the risk of sensorineural hearing loss (SNHL) is very high.

The urgency for post-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. 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.

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

This exclusion is due to 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 congenital cytomegalovirus (cCMV)

This exclusion is because the risk of hearing loss is very high and the window of opportunity for treatment is short. These babies should be referred by the paediatrician 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 to 3 should be seen either within 4 weeks of the decision that screening is not appropriate, recovery from the acute episode, or by 44 weeks gestational age.

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, screening teams should treat these babies as screen referrals and liaise closely with paediatric and audiology teams to ensure prompt referral to audiology.

The screening team should log them as referrals on the 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: paediatrician

Responsible for arranging appointment and follow-up: audiology (copy to NHSP screening manager)

2.2 Screen declined, incomplete or missed – no routine follow-up

Where the screen was:

• declined altogether
• missed
• not completed, despite invitations

Routine referral for targeted follow-up is no longer recommended.

Screening teams should make vigorous efforts to maximise newborn screening coverage by 3 months of age, particularly in hard to reach groups.

This should include:

• telephone, text and email reminders
• outreach clinics
• home visits
• liaison with trust antenatal and newborn screening coordinators
• liaison with midwifery and health visiting teams
• contact with paediatric wards and intensive care units - to identify readmitted and unscreened babies

Health visitors (HVs) and GPs must be informed of babies that have not completed screening.

Screening teams will need to ensure that parents are provided with information about how to seek assessment in the event of future concern. The HV or GP should discuss with parents the implications of not having (or not completing) the screen.

Parents and HVs (or GPs) should be made aware that they can request an audiological assessment at any time.

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

2.3 Moved into the country before 3 months of age – screen

A mechanism should be in place to identify babies who move into the area under 3 months of age who have not been screened. The local screening service must be informed of these babies by child health/HV/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.

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

Responsible for arranging newborn hearing screen appointment – NHSP screening manager

2.4 Moved into the country after the age of 3 months – 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 – HV/Child Health Department/other local arrangement

2.5 Referred on screen but missed audiology assessment – follow-up

The practice of offering 1 or 2 appointments in audiology and then making a referral for targeted follow-up if these are not attended is insufficient. These babies have a high risk of SNHL.

Audiology and screening teams 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 HV and GP to facilitate attendance.

In the event of inability to secure attendance, the HV and GP should be notified and advised 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

2.6 Passed screen or audiology follow up, but with specific neonatal risk factors – targeted follow-up

Babies must be referred for targeted follow-up (behavioural testing around 8 months) or sooner - if a local protocol is in place - if they have:

• syndromes associated with hearing loss (including Down’s syndrome)
• cranio-facial abnormalities, including cleft palate
• confirmed congenital infection (toxoplasmosis or rubella)
• been in a special care baby unit (SCBU) or neonatal intensive care unit (NICU) over 48 hours, with no clear response automated otoacoustic emissions (AOAE) test for both ears, but clear response automated auditory brainstem response (AABR) for both ears

Responsible for identifying child – screening team

Screening teams may not always be aware of these risk factors, particularly where screening is completed early.

It is assumed that other professionals involved with these families will be aware of the need for ongoing audiological assessment.

Responsible for arranging appointment – Audiology

3. Ototoxic drugs – Refer at the discretion of the paediatrician

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 the A1555G mitochondrial mutation, the baby should undergo newborn hearing screening and follow-up if required, in accordance with the standard screening protocol.

The responsibility for monitoring babies who are receiving ototoxic drugs, and appropriate referral for audiological assessment lies with the paediatrician and medical team.

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.

Consult the 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 the A1555G mitochondrial mutation 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 A1555G mitochondrial mutation may have a family history of sensorineural deafness from middle age, in the affected individuals and the transmission is maternally inherited. It is now possible to test for this mutation.

If there is such a history we urge paediatricians to test for this, be cautious, and consider alternative antibiotics, otherwise there is a risk of significant hearing damage (despite satisfactory blood levels).

Responsibility for making the referral and communication with family – Paediatrician

Responsibility for making appointment – Audiology

4. 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 clearly outside of the responsibility of NHSP teams, but this brief outline is included for completeness.

4.1 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.

4.2 Confirmed or strongly suspected bacterial meningitis, or meningococcal septicaemia, 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 and others 1993, Lee and others 1998, hyperbilirubinaemia – Boo and others 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.

5. 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.

6. Appendix A: changes in these guidelines

Some aspects of these guidelines have undergone revisions since the document was first issued in 2003.

6.1 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 in 10 (Fortnum 1992) compared to about 1 in 100 for other NICU or SCBU babies, and 1 in 1,000 for well babies.

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 a higher incidence of middle ear effusion following meningitis (Fortnum and Davis 1993). For this reason, a screen is not appropriate for this population, and a full assessment by ABR is essential.

This version, published in 2019, adds 2 further categories for exclusion from the screen.

These are babies with:

• PVP shunts in place
• cCMV

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 BSA 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.

Advice for audiology services who see babies with cCMV is available from the BSA or by emailing bsa@thebsa.org.uk.

6.2 Targeted follow-up

In 2012, following a major review of the evidence (Wood and others, 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 or hyperbilirubinaemia (exchange transfusion level)
• mechanical ventilation over 5 days, or who have undergone ECMO
• neuro-degenerative, or neuro-developmental disorders

6.3 Ototoxic drugs

In version 4, we amended our advice to the current position and clarified that it is the responsibility of the paediatrician to decide whether a 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 A1555G mitochondrial mutation who are abnormally susceptible to aminoglycoside ototoxicity, we advised immediate follow-up and audiological monitoring irrespective of screen outcome.

7. Appendix B: guidelines for audiological follow-up of babies diagnosed with bacterial meningitis and/or meningococcal septicaemia (June 2012)

7.1 Scope

These guidelines have been produced to help clinicians develop local protocols for hearing assessment in babies up to the age of 1 year who have been diagnosed with bacterial meningitis and/or meningococcal septicaemia.

7.2 Timing of assessment

Hearing assessment needs to be carried out after recovery and not within 48 hours of diagnosis, as 10% of cases may have a reversible SNHL in this time (Richardson and others 1997) but should be within 4 weeks of the child being well enough to be tested.

Urgent assessment is required to identify severe/profound hearing loss which may require cochlear implants 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, preferably using both high and low-frequency stimuli. If this is not possible, a diagnostic OAE test would be helpful but, in this case, further assessment (for ABR or behavioural testing) should be arranged.

Between 12 weeks and 7 months corrected age:

Options should be discussed by the audiologist with parents and include 1 or more of:

• ABR under natural sleep (especially if the baby is still quite young), preferably with both high and low-frequency stimuli
• diagnostic OAE test
• ABR under sedation (for older infants, if there is a considerable parental or 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 by 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.

7.3 Further follow up

After a review of evidence indicating a 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 and others (1999), against other studies showing no late-onset (Berlow and others 1980, Richardson and others 1997).

Therefore, no recommendation for further follow-up is made provided the hearing test is clearly normal.

8. References

Berlow SJ, Caldarelli DD, Matz GJ, Meyer DH, Harsch GG. (1980). Bacterial meningitis and sensorineural hearing loss: a prospective investigation. The 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 (2013) 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. Archives of Disease in Childhood, 67(9), 1128-33.

Fortnum HM & Davis AC (1993). Hearing impairment in children after bacterial meningitis: incidence and resource implications. British Journal of Audiology, 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. British Medical Journal, 323: 536–9.

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

National Patient Safety Agency (2010). Safer use of intravenous gentamicin for neonates. (Alert No.1085) DH Gateway reference 13534

NHS England. (2018) NHS Public Health functions agreement 2018-19. Service Specification No. 20 NHS Newborn Hearing Screening Programme

Richardson MP, Reid A, Tarlow MJ, Rudd PT (1997). Hearing loss during bacterial meningitis. Archives of Disease in Childhood. 76(2) 134-8

Shapiro SM (2003). Bilirubin toxicity in the developing nervous system. Review, Pediatric Neurology, Elsevier, 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. International Journal of Audiology, 52:394-399

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