Guidance

6. Patient journey from screen to referral

Updated 10 October 2024

Applies to England

The clinical care pathways for both well babies and babies cared for in a neonatal intensive care unit (NICU) describes the route that a parent or baby will take from their first contact with an NHSP team member to the completion of the screening process.

Operational guidance describes aspects of the NHSP patient journey for both pathways to help local programmes provide services which meet programme standards and service specification expectations. At the end of each chapter is a call to action box that will help you identify gaps that may need to be addressed. Useful resources and supporting documents have been hyperlinked throughout to support local services in their working practices.

Information in the antenatal period

During pregnancy all parents should be provided with written information on hearing screening. A booklet for parents to be, Screening tests for you and your baby, brings together all antenatal and newborn screening information and is available in other languages. This booklet, which should be supported by verbal advice, is usually given out by the midwife during the antenatal period.

You should ask yourself:

• do local audits show that the screening booklet is being given out?
• do the midwives providing the verbal information have regular training to enable them to answer questions appropriately?
• are NHS screening programme DVDs or videos and other resources being used?
• are there opportunities for parents to ask questions?
• do postcodes in your area map to your service on NHS.UK for finding newborn hearing services?

Further guidance on management of hearing screening services is available in local programme management tasks.

Accuracy of screening

No screen is 100% specific or sensitive (identifies all target cases and never refers those without). This is why parents are provided with a checklist after a baby has had clear responses following the hearing screen. This identifies sounds a baby should be making and their reaction to sound at different stages of their development. Parents are advised that hearing can change and children can acquire a hearing loss, such as glue ear, and they should speak to their GP or health visitor (HV) if they are concerned about their child’s hearing or speech in the future. A child’s hearing can be assessed at any age and parental concerns should always be treated seriously.

The screen is designed to pick up moderate or worse hearing loss. It will not necessarily pick up mild hearing losses and that is why it is important that parents continue to monitor their child’s reactions and making sounds. If at any stage a parent is concerned about their child’s hearing they should see their HV or GP.

Birth registration notification to the national IT system

Standard 1 – Identify the population and coverage

Local maternity services, or child health records departments (CHRD), are responsible for entering high quality, timely data into the NHS number registration patient demographic system (PDS) or manually via the birth notification application (BNA). This enables electronic identification of babies eligible for screening within the national IT system. This should ideally be completed as soon as possible after birth and prior to any newborn screening being performed.

NHS numbers

The NHS number is used to match test results, monitor quality of care, improve neonatal research and help provide clinicians with the information they need to implement best practice. The fact that newborn babies now have an NHS number is vital for newborn hearing screening to ensure that all babies are offered the screen.

The national NHSP IT system has an interface with PDS and BNA to receive electronic birth notifications for all live births, within seconds of an NHS number being generated. Any change made to the demographic information in the NHSP national IT system will be duplicated in the other screening programmes national IT systems that use this common data. So, care should be taken when editing demographic data.

Non-live births should automatically be excluded from the national IT system. However, a robust mechanism for notification of deceased babies is vital to ensure the national IT system is up to date and NHSP services do not offer hearing screening to a deceased baby in error (incident report).

If live births are registered as a stillbirth or neonatal death in error, these records will not reach the national IT system and the NHSP IT system helpdesk should be informed. If, following an investigation, the birth was incorrectly marked as stillbirth or deceased, the incident should be reported and appropriate action taken to add the record manually.

You should ask yourself:

• is the midwifery team aware of the PDS or BNA, its interface to NHSP and other newborn screening programmes and its importance?
• is a NHS number and demographics data for each baby generated quickly and accurately?
• do you know what to do if a birth notification record does not reach the national IT system?
• is there a clear escalation route when there is a problem identified eg a lead midwife who deals with PDS/BNA issues?
• are there failsafe systems in place when the national IT system is unavailable?
• what is the local process when a baby is ‘discovered’ that is not on the national IT system?

Further guidance on management of NHSP services and their data can be found in the operational guidance on the national IT system.

Failsafe processes describe how NHSP providers can ensure incidents are avoided where possible. NHSP programme standards advise NHSP services of key performance indicators and programme standard expectations.

Offering the newborn hearing screen

Standard 1 – Identify the population and coverage

All babies under 3 months of age, born or resident in England, should be offered a newborn hearing screen. All babies are eligible for screening.

Babies are excluded (contra-indicated) if they are less than 34 weeks gestational age, or over 3 months of (corrected) age.

They are also excluded if they are diagnosed with:

• confirmed congenital cytomegalovirus (cCMV)
• microtia or external ear canal atresia
• neonatal bacterial meningitis or meningococcal septicaemia
• programmable ventriculo-peritoneal (PVP) shunts in place

A referral is the responsibility of the medical team caring for the baby. Further guidance on these exclusions is available in the Eligibility section below.

Screening teams should treat these babies as screen referrals, and continue to log them as such on the national IT system using the screening outcome of ‘incomplete-screening contraindicated’.

Screening teams should then expedite and monitor their referral to audiology, in conjunction with the medical team as described in the NHSP Surveillance and audiological referral guidelines.

Systems need to be in place to assist in identifying all eligible babies for both hospital and community provider services. This includes notification of deceased babies throughout the screening and referral pathway, both within acute trusts and CHRDs.

It is important to note that some babies less than 3 months of age are not registered as ‘new births’ by maternity but as ‘new registrations’ by the local CHRD system, for example babies born abroad, and these records will not appear in the national IT system. Local NHSP programmes need a procedure in place with their CHRD to ensure that they are made aware of these new registrations so the screen can be offered.

Where and when does screening take place?

Hospital-based screening service (well babies)

Screening is undertaken by NHSP trained staff. Ideally, the screen should be completed prior to discharge from hospital. If the initial screening process cannot be completed as an inpatient, an outpatient or home visit appointment will need to be arranged by the responsible NHSP service provider so that the screen can be completed within four weeks. In a hospital model the majority of babies will be screened by 10 days of age. If no clear response is obtained on completion of the screen, the baby is referred to diagnostic services provided by audiology.

You should ask yourself the following questions.

1. Are all babies appearing in the national IT system?
2. Are other babies listed on ward lists or birth registers?
3. Is baby on NICU?
4. Are procedures in place to ensure a mother of a deceased baby is not approached or contacted regarding the screen?
5. Is the mother being discharged to a temporary address from maternity?
6. Is every effort made to ensure the screen is offered and completed prior to discharge?
7. Do midwives know the importance of completing the screen before discharge and how to contact your service if needed?
8. Are screeners on a rota that maximises coverage and is 7-day service provided?
9. Is there an understanding of ‘hard to reach sectors’ locally?

Community-based screening service (well babies)

Screening is carried out by NHSP trained health visitors or other NHSP trained screeners. The first screening should take place at the primary health visitor birth visit at approximately 10 days of age.

Any subsequent screening required should be completed by 5 weeks of age in the home or community clinic. In a community model screening will not usually be commenced until after 10 days of age. If no clear response is obtained on completion of the screen, the baby is referred to diagnostic services provided by audiology.

You should ask yourself the following questions.

1. Are all babies appearing in the national IT system?
2. Does CHRD system have other babies listed?
3. Are HVs notified of births in a timely manner?
4. Are HVs made aware of all transfers in?
5. Are HVs informed of any change of address?
6. Are HVs made aware of which babies have been screened in NICU?
7. Are procedures in place to ensure a mother of a deceased baby is not approached or contacted regarding the screen?
8. Is there an understanding of ‘hard to reach sectors’ locally?

NICU babies – hospital and community providers

Screening is carried out by NHSP-trained screeners. For community providers this is usually NHSP trained NICU nurses. The baby should be well and not less than 34 weeks gestational age. The entire screening process should be completed while the baby is still in hospital but as close to discharge as possible. If the screening process is not completed, an outpatient or home visit appointment should be arranged to complete the screen.

Regardless of the screening protocol or service delivery model, all babies with a screen positive outcome should be referred for audiological assessment and seen for the initial appointment within four weeks of screen completion or by 44 weeks gestational age.

Further guidance on management of NHSP services and their data is available from guidance notes on non-availability of national IT system and missing birth notifications and operational guidance on the national IT system.

Failsafe processes describe how NHSP providers can ensure incidents are avoided where possible. NHSP programme standards advise NHSP services of key performance indicators and programme standard expectations.

Eligibility

Although rare, there are some circumstances where babies eligible for screening are not screened.

Babies too poorly to screen

Screening should normally be completed by 3 months corrected age. However, there is room for flexibility, and screening can be carried out at up to 6 months of age if the baby is well enough.

If not, set the national IT system screening outcome as ‘Incomplete – screening contraindicated’, deactivate the record selecting ‘Incomplete – other’, and enter a case note that explains the circumstances.

Write to the medical consultant (including a copy to the GP and HV), discharging the baby from the screening programme, and suggesting they refer to audiology should this be appropriate in the future.

We suggest this course of action rather than scheduling the baby for routine targeted follow-up to avoid any possibility of contacting a family of a deceased baby and because the behavioural assessment used in targeted follow-up clinics is unlikely to be appropriate.

Urgent audiological assessment

There are 4 groups of babies that require immediate audiological assessment.

Group 1. Microtia or external ear canal atresia

This exclusion is put in place because these babies will always have a degree of hearing loss.

Group 2. Neonatal bacterial meningitis or meningococcal septicaemia

In cases where it is confirmed, or strongly suspected, in the judgement of the paediatrician, or the neonatologist in 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.

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 shunts in place

This exclusion is due to the risk of magnetic fields arising from audiological stimulus transducers affecting the shunt.

Programmable ventriculo-peritoneal 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 (BSA) has guidance for audiology services on assessment for patients with PVP shunts.

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.

Congenital cytomegalovirus is a common virus, which can be associated with hearing loss if contracted in utero (congenital cytomegalovirus or cCMV). It is the only cause of SNHL 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

Audiology and paediatric teams will need to agree referral mechanisms, time scales and implications for audiological testing of babies that have not reached term. The regional lead for paediatric infectious diseases and/or local paediatric audio-vestibular physician should be contacted by the paediatrician for further advice if required.

Referral timeframes

Babies in groups 1 to 3 should be seen either within 4 weeks of the decision that screening is not appropriate for recovery from the acute episode, or by 44 weeks gestational age.

Babies in group 4 may need to be seen sooner, within a timescale 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. When making the referral, audiology colleagues must be made aware of the reason.

Screening teams should treat these babies as screen referrals, and continue to log them as such 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. The NHSP national IT system can be used to monitor that the baby has been appointed in audiology, in the same way as for babies who are referred to audiology for other screening contra-indicated conditions, such as confirmed meningitis as described in the NHSP Surveillance and audiological referral guidelines.

Guidance for audiology teams for the assessment and management of these groups of babies is available from the BSA within its Guidelines for the early audiological assessment and management of babies referred from the newborn hearing screening programme Contact the BSA for details.

Data reporting

For standards and KPI reporting, ‘complete screens’ (numerator) is the total number of eligible babies for whom a decision about referral or discharge from the screening programmes, is made within an effective timeframe.

This includes:

• babies for whom a conclusive screening result was available by 4 weeks or 5 weeks corrected age
• babies referred to from an audiology department because a newborn hearing screening encounter was inconclusive or contra-indicated

If babies have already had an auditory brainstem response (ABR), audiological assessment following an urgent referral by the medical team, prior to the screen being offered, then the steps required are:

• set the national IT system screening outcome as “Incomplete – screening contraindicated”
• add a case note detailing the circumstances
• make sure that the audiological data has been entered in the national IT system

Skin conditions

Babies who require an AABR screen but with epidermolysis bullosa or a similar skin condition where it is not possible to attach sensors. These babies must be referred to audiology for assessment.

Set the screening outcome as ‘Incomplete – screening contraindicated’, write a case note detailing the circumstances and an immediate referral made to audiology.

It is the responsibility of the audiologist to liaise with the parents, and agree a suitable timeframe for the assessment. This may need to be deferred until a behavioural assessment can be obtained but this is a decision for audiology.

Lack of screening by 6 months corrected age

Set the screening outcome as “incomplete-lack of capacity” with a case note to explain the circumstances and make an immediate referral to audiology. Report this to the national programme team and locally as an incident.

Very early screening

The screen can be carried out as soon as baby is born but with very young babies there may be fluid or debris in the ear canal which will affect the ability to record an AOAE so it may be best to wait until as close to discharge from hospital as possible.

Responsibility

NHSP providers are responsible for ensuring screen completion and audiological follow-up of all the babies registered with a GP that maps to them as the responsible provider.

Hospital providers should try to complete screening for all babies (well and NICU) born in their maternity units, regardless of where they live. Most screening will be completed before discharge, but for those that are not, outpatient appointments should be offered. If the parents live outside the providers area then they may prefer to complete the screen where it was started, or if they live a long way away it may be easier for them to have the screen completed by their local NHSP team. In the latter case, the birth provider should liaise with the responsible provider to ensure timely screen completion, family friendliness and parental choice.

Community providers are responsible for screening all the NICU babies resident in their units, and those well babies whose GP maps to them as the responsible provider. This applies, regardless of whether the patient’s postcode is geographically outside their Local Authority area, however liaison with neighbouring NHSP providers is vital to ensure no babies miss their hearing screen.

NICU babies

It is advisable to defer screening until babies are not receiving treatment and ready for discharge. Warmer cots may cause electrical interference which would affect the screening equipment.

Informed consent

Prior to screening, informed consent must be gained from an individual with parental responsibility for the baby. In order to make an informed decision and therefore give valid consent parents (including those with parental responsibility where they are not the biological parent) should:

• be provided with information that gives the risks and benefits of screening
• have been provided with written information on hearing loss within the Screening tests for you and your baby booklet, during the mother’s antenatal care
• be provided with verbal information about the screen, at an appropriate time by an NHSP trained screener, before being offered the screen
• understand that verbal consent to screen covers the entire screening process including the audiological assessment if it is required immediately or as a targeted follow-up
• understand consent may be withdrawn at any time
• understand how the data is stored and used must also be explained, as without the data being stored the screen cannot be carried out
• be given the information sheet for parents to ensure that the consent giver has been informed about and understands the facts listed there. It may be helpful to provide parents with a copy of the information sheet as part of the discussion
• if they decline, be directed to the 2 checklists ‘reactions to sounds’ and ‘making sounds’ in the personal child health record PCHR (Red Book) together with an explanation about why these lists are important

The GP and HV should be notified of the decline, withdrawal of consent, or non-attendance at appointments.

You should ask yourself the following questions.

1. Are all mothers receiving written and verbal information in a format/language they understand, enabling them to make an informed decision?
2. How do the primary care team (GPs and HVs) receive notification of screens declined or not completed due to non-attendance to outpatient or withdrawal of consent?
3. Is there a mechanism for parents that decline, not attend appointments or withdraw consent to access hearing screening later?

When the verbal explanation is compromised, for example there is no opportunity to discuss the screen prior to attending an outpatient appointment, the Screening tests for you and your baby booklet should only be handed out again if requested. Otherwise reference to the booklet or NHS Choices should be suggested in the appointment letter.

Screening coverage can be improved and incomplete screening outcomes, caused by missed appointments, withdrawn consent or decline, may be avoided by making vigorous efforts to maximise coverage by 3 months of age, including:

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

If an incomplete outcome is not avoidable:

• referral for targeted follow-up is not required
• screening teams need to ensure parents are provided with information about how to seek assessment in the event of future concern
• HVs and GPs must be informed of babies that have not completed screening and are responsible for ongoing surveillance

Further guidance on management of hearing screening services is available in guidance on local programme management tasks.

Who can give consent?

Consent must be given by a person with parental rights and responsibilities for the child. This is defined by law and described in The Children Act 1989; amended Dec. 2003.

This means:

• a mother always has parental responsibility even if she is under 16 years of age unless she is deemed not competent; parental responsibility is then decided by a court order
• a father will only have automatic parental responsibility if he is married to the mother

An unmarried father will only have parental responsibility if he:

• has registered the child’s birth jointly with the mother
• has obtained a parental responsibility order from the court
• has registered a parental responsibility agreement with the court

If in doubt postpone the screen and seek advice locally. Maternity staff and HV’s should be able to advise about parental responsibility and how they have obtained consent in difficult circumstances like mother is in ITU and unable to consent. Safeguarding teams and medical records teams may also be able to help.

You should ask yourself:

• do you know how to contact local social care services for guardian or parental consent?

Adopted babies

Follow your local information governance procedures regarding adoption but if these are not available the following action is advised:

If there are no child protection issues and a retrievable audit trail is not an issue, update the demographic details in the original record.

If there is a need to protect the identity of the child, create a new record and enter the new demographic details. Do not re-assign the screening test results but set the appropriate screening outcome. De-activate the original record selecting ‘other’ and write a case note stating a new record has been created.

Recording consent status

The person who takes verbal consent must ensure that the information is recorded on the screening equipment if the screening offer is accepted then set consent to “full”.

However, if it’s a decline or consent is withdrawn, providers must set consent to “not given” on the national IT system.

When recording the name of the person who gives consent:

• the birth mother’s details are shown in the national IT system as the primary contact and she would normally be the consent giver
• and consent was given by someone other than the mother their details should be added to the national IT system as a secondary contact and the consent giver

Alternatively in community sites it may be acceptable to your Caldicott Guardian to record the details of the person who gives consent in the Health Visiting notes (for well babies) or the hospital notes (for NICU babies) rather than in the national IT system.

When recording the name of the person who takes consent this is generally the screener that carries out the first screening test. This information is uploaded by SEDQ (screening equipment data quality) and recorded in the national IT system (via a system case note) as the user that set the consent status; the default assumption is that this person took consent.

If this is not the case, record the name of the person who took consent in a case note. Where no screening tests are carried out (as in screen decline) the name of the person who discussed consent or decline should be recorded in a case note.

Consent need only be obtained once; however any screener carrying out a screening test has a responsibility to assure themselves that appropriate consent has been obtained. When there is a time lag between taking consent and carrying out the screen (as sometimes occurs in NICU) there will need to be a local process to ensure that the consent is recorded in the national IT system.

Further guidance on management of NHSP services and their data is available in the national IT system chapter.

Use of interpreters

The hearing screening team is responsible for determining if an interpreter is required. The Screening tests for you and your baby booklet is available in 12 other languages.

The availability of interpreting services is the responsibility of the provider organisation.

You should ask yourself the following questions.

1. Do you record the use of interpreters on the national IT system?
2. Do local audits show that interpreters are always available when required?
3. Is there a written local policy on the use of interpreters/language line?
4. Do you have an awareness of the ethnic and cultural diversity of your local population?
5. Locally is the need for interpretation shared amongst health professionals and NHSP stakeholders such as paediatrics, maternity, audiology or early intervention?

Further guidance on management of hearing screening services is available in guidance on local programme management tasks and operational guidance on the national IT system.

Starting the screen

Before starting the screen, the correct screening protocol needs to be determined and any risk factors identified.

There are 2 screening protocols.

• the NICU and SCBU protocol – for babies who have been in a neonatal intensive care unit (NICU) or special care baby unit (SCBU) for more than 48 hours continuously.
• the well baby protocol – for all other babies.

The term NICU includes SCBU, paediatric intensive care (PICU) or children’s intensive care unit (ICU). It does not include transitional care.

The baby should be screened as close to discharge as possible while still in hospital. Wherever possible the baby should be well, not less than 34 weeks gestational age and with any major medical or drug treatment completed. If the screen cannot be completed in hospital an outpatient appointment or home visit should be offered to complete the process.

Risk factors

It is important to obtain risk factors prior to screening because this can influence what pathway a baby may need to follow from a screening, audiological and ongoing surveillance perspective. Some conditions or factors can mean babies have a higher risk of hearing loss. These may need to be referred immediately or for targeted follow-up and parents need to understand the implications of these risk factors.

There are 3 ways to categorise risk factors within NHSP. They are conditions or treatments:

• which are known to have a risk of hearing loss
• that mean a baby can develop a hearing loss that needs targeted follow-up
• where screening is contraindicated and should be referred immediately or for targeted follow-up

The presence or absence of these risk factors must be recorded for all babies in the national IT system.

Risk factor or contraindication	Definition	Immediate or targeted referral to audiology?
Bacterial meningitis	Confirmed or strongly suspected bacterial meningitis (any organism) or meningococcal septicaemia	Immediate
Confirmed congenital cytomegalovirus	Confirmed congenital infection	Immediate
Microtia / external ear canal atresia	Malformation of the external part of the ear / Absence of an ear canal in one or both ears	Immediate
Programmable ventriculo-peritoneal shunt	A baby with a surgically implanted programmable ventriculo-peritoneal shunt used as a treatment for hydrocephalus	Immediate
Congenital infection	Confirmed congenital infection such as toxoplasmosis or rubella	Targeted
Craniofacial anomalies	A (noticeable) cranio-facial anomaly such as cleft palate (excluding cleft lip only, minor pits or ear tags)	Targeted
NICU baby protocol with NCR/NCR on AOAE and CR/CR on AABR	Resident on NICU over 48 hours with no clear response (NCR) on AOAE both ears but clear response(CR) on AABR both ears	Targeted
Syndrome (please add syndrome name)	Confirmed syndrome related to hearing loss	Targeted
Family History of hearing loss (broad family)	Hearing loss (unilateral or bilateral) in baby’s parents, grandparents, uncles and aunts (if blood relatives), cousins and siblings; should be permanent (not glue ear) and present from childhood, irrespective of degree of loss	Not required
Family history of hearing loss (parents/siblings only)	Permanent, uni or bi-lateral hearing loss of any degree, from childhood, in baby’s immediate blood relative (not glue ear)	Not required
IPPV > 5 days or ECMO	SCBU/NICU baby who had IPPV > 5 days or who underwent Extra-Corporeal Membrane Oxygenation (ECMO)	Not required
Jaundice at exchange transfusion level	Jaundice where bilirubin (normally unconjugated) reached a level indicating the need for exchange transfusion, taking into consideration other factors such as hypoxia, acidaemia and prematurity	Not required
Neurodegenerative or neurodevelopmental disorder	Developmental delay associated with a neurological disorder	Not required
NICU > 48 hours	Baby resident in a SCBU or NICU for a continuous period of 2 days (48 hours) or more	Not required

Obtaining information about risk factors

Family history for both well and NICU protocol babies should be ascertained by screeners questioning of the birth mother and father. An ascertaining family history questionnaire and family history flow diagram should be used to assist screeners.

Well baby protocol with risk factors

Generally these babies are nursed on wards and therefore:

• hospital screeners should seek confirmation from the paediatric or appropriate maternity staff
• community screeners would ascertain this from the discharge summary

An example would be craniofacial anomalies such as cleft palate.

NICU baby protocol with risk factors

A member of the paediatric or NICU or SCBU team should supply this information and a mechanism for obtaining this must be established locally. It is not appropriate for screeners to try and ascertain this information from medical notes. A risk factor recording sheet should be used to assist screeners.

Ototoxic drugs

Administration of ototoxic drugs is not an indication for targeted follow-up. Where a baby is receiving ototoxic drugs and the levels have risen outside the therapeutic range it is the responsibility of the paediatric team to make decisions about referral for audiological assessment based on the information in the NHSP Surveillance and audiological referral guidelines.

Unknown congenital infection status

Unless a blood test has been performed to confirm yes or no, this risk factor is unknown.

You should ask yourself the following questions.

1. Do screeners screen without knowing whether risk factors are present?
2. Are there locally agreed procedures for ascertaining risk factors?
3. Do screeners understand the importance of recording all risk factors on national IT system, regardless of need for follow-up?
4. Do you have liaison with or access to the paediatric and neonatal teams to ascertain risk factor information?

Further guidance on management of NHSP services is available in local programme management tasks.

Infection control

Agreed local infection control policies must be followed at all times, ensuring:

• screening equipment is cleaned following the procedures defined in the manufacturer’s equipment protocols
• local hand hygiene procedures must be followed before and after every baby screened
• consumables such as ear tips, muffs and sensors are disposed of in an appropriate manner after each screen has been completed
• consumables, which are single use items, are never reused

You should ask yourself the following questions.

1. Do you have a local infection control policy?
2. Are screeners assessed for their compliance?

Screening test

Standard 2 – test performance – Well babies who do not show a clear response in both ears at automated otoacoustic emission 1 (AOAE1)

Well baby protocol

Automated Otoacoustic Emission (AOAE)

A soft clicking sound is played into the baby’s outer ear. When the cochlea in the inner ear receives sound, it responds and this is collected by the microphone in the screening equipment. Usually the initial test, unless considered inappropriate due to the presence of excess debris or fluid in one or both ears and the baby’s imminent discharge. If this is the case the automated auditory brainstem response (AABR) may be used as the initial test.

AOAE 1 – both ears are tested. If the result is a clear response in both ears the screen is complete. If the result is a no clear response in one or both ears the next protocol stage of the screen is AOAE 2. Occasionally AOAE 2 may be considered inappropriate due to:

• lack of suitable time interval (minimum of 5 hours) between AOAE 1 and AOAE 2 due to the discharge of the baby
• technical difficulty with the test, such as persistent noisy breathing causing high artefact (noise) levels

In these circumstances, the AABR would be the next protocol stage of the screen.

AOAE 2 – carried out on ears that have not shown a clear response or not been attempted at AOAE 1, allowing an interval of greater than 5 hours and that is as long as possible between AOAE 1 and AOAE 2 to give the birth fluid or debris chance to disperse. If the result of the AOAE 2 is a clear response in the ear(s) that required re-testing the screen is complete. If the result is a no clear response or not complete in one or both ears the next protocol stage of the screen is the AABR.

Automated Auditory Brainstem Response (AABR)

Soft clicking sounds played into the baby’s outer ear via soft baby ear muffs, trigger electrical impulses, which are collected into the screening equipment by 3 small sensors placed on the baby’s high forehead, back of shoulder and nape of the neck. AABR is usually carried out as the final protocol stage of the screen on both ears, irrespective of the results obtained at AOAE 1 and/or AOAE 2. Once a result has been obtained in both ears the AABR is complete. If the result is clear response in both ears the screen is complete. If the result is a no clear response in one or both ears an immediate referral for audiological assessment is required. An AABR should never be repeated on an ear where a no clear response result has already been recorded.

NICU baby protocol

The AOAE and AABR are performed on both ears – the tests can be completed in any order, but both must be completed.

Only one AOAE test is carried under the NICU protocol, and once a result is obtained from both ears, regardless of the outcome, the AOAE is complete.

The AABR is carried out on both ears irrespective of the results obtained at AOAE. If the AABR result shows no clear response in one or both ears, then a referral for audiological assessment is required.

However, if after completion the results show ‘AOAE no clear response’ and ‘AABR clear response’, you must offer a targeted follow-up, as explained in NHSP Surveillance and audiological referral guidelines.

The baby should be screened as close to discharge as possible while still in hospital.

Wherever possible, the baby should be no less than 34 weeks gestational age, and in good health, with any major medical or drug treatment completed.

If the screen cannot be completed in hospital, then an outpatient appointment or home visit is required to complete the process.

In exceptional circumstances where the baby is not well enough by 3 months corrected age, the screening may be carried out between 3 and 6 months corrected age. If the baby is not well enough at 6 months of age, the baby should be discharged from the screening programme to the paediatrician, and to the GP.

A recommendation for a referral to audiology should be made at an appropriate time.

Change from well baby to NICU protocol

There are 3 scenarios for babies moving from ‘well baby’ to NICU baby protocol.

Transfer to NICU before discharge

If a baby completes the screen with a clear response (at AOAE or AABR) under the well baby protocol, but before discharge is transferred to an intensive care unit for more than 48 hours, then they should be rescreened under the NICU protocol.

This is the only circumstance in which repeat screening – after a screening outcome has already been set – can be permitted.

Admission to NICU after discharge

If a baby has not started or not completed screening, discharges from hospital to community, and is then readmitted to an intensive care unit for more than 48 hours, then screening should be completed under the NICU protocol.

If screening was started, you will need to unset any screening outcome on the national IT system and change the baby’s screening protocol to NICU.

Any screening test results obtained under the well baby protocol will be removed from the grid on the demographics section, will not be used to suggest a screening outcome, but will be retained in the list of screening test results.

You should also ensure to:

• carry out AOAE and AABR as close to discharge as possible
• upload the new results to the national IT system – and set the screening outcome accordingly
• enter a case note to explain the circumstances

Re-admission to hospital

If a baby completes the screen, discharges home, but is subsequently admitted to hospital, and the medical staff feel the baby’s condition or treatment may affect the baby’s hearing, the baby should not be rescreened.

The medical team caring for the baby should refer the baby to the audiology department.

Optimising screening test conditions

Establishing good test conditions before starting a screen is important, and will avoid the need to pause or stop during a screen and restart. Guidance on how to use NHSP screening equipment is provided in equipment supplier manuals, and other supporting documents.

Screeners should receive training and competency assessment for clinical skills, including how to manage equipment and difficult screening test conditions.

AOAE: fitting the earpiece

Good earpiece fit is crucial for a successful AOAE test, because it keeps noise out of the ear and maximises the microphone’s ability to record a response from the ear if one is present.

1. Examine the ear by pulling the pinna gently up and back so that you can assess the size of the canal.
2. Choose an ear tip that is large enough to provide a secure fit. Do not be tempted to use a small earpiece because it’s easier to fit.
3. To fit the earpiece gently pull the pinna up and back to open the ear canal, insert the earpiece turning it gently but firmly to secure it in place, release the pinna and hold the earpiece briefly in place until the baby settles. A baby is often slightly restless when the earpiece is fitted The earpiece should be secure enough to stay in place and should not be held.
4. The video clip Fitting the earpiece in screener e-learning shows you how to achieve this. If the fit is not good either reinsert the earpiece and/or select a larger ear tip. When reinserting an earpiece always check for blockages.
5. Ensure the lead is not lying across the baby’s body or face.

Use the information provided by the screening equipment to assess the fit of the earpiece. With some equipment you can do this before pressing the start button. However, with other equipment you may need to press the start button before information is provided. Either way you should allow the baby to settle and wait for good test conditions before you press the start button.

AOAE: test conditions and monitoring the test

The equipment will provide information that enables the screener to monitor the progress of the test and the test conditions. The screener should monitor the earpiece fit and the state of the baby visually throughout the test as well as monitoring the information provided by the equipment during the test. Pause or stop the test as necessary, for example, if the baby becomes restless, the earpiece falls out or the environmental noise becomes high.

AABR: sensor positions

Use the positions given in the manufacturer’s instruction manual (except for the Accuscreen where the black sensor is placed on the back of the shoulder and not the cheek).

ABBR: skin preparation

Good preparation is essential to ensure low and balanced impedances. This optimises the chance of recording a response, if one is present, and minimises test time.

When using a dry prep pad hold the skin taut and give 3 to 5 firm but gentle wipes with the dry prep pad. Then wipe the area with gauze dampened with water. This removes the layer of dead skin cells and ensures good contact with the sensor.

Some manufacturers may recommend other methods. Ensure that any other substance you use has been approved for use locally.

The video clip Skin preparation for AABR in screener e-Learning shows you how to achieve this.

ABBR: earphone placement

Earphones are usually colour coded red=right ear and blue=left ear. Ensure the earphones are sealed and making good contact with the skin around the whole circumference and that the loudspeaker is not blocked by the pinna.

Keep the sensor leads parallel with each other. Ensure that the earphone leads are well separated from the sensor leads.

ABBR: test conditions and monitoring the test

The baby must be sleeping or well settled. Testing unsettled or crying babies is counterproductive. Do not start a test until the baby is settled and test conditions are good.

It is preferable, and easiest, to screen the baby in a cot. If this is not possible and baby is in mother’s arms, for example, ensure there is no contact between her arm and the nape sensor.

Reduce sources of external acoustic noise by switching off TVs, closing doors, tactfully asking other people present to keep noise to a minimum.

Reduce electrical noise by keeping the equipment at least one metre away from other electrical equipment, large metal objects or power outlets. Use the length of the leads to keep as a great a distance as possible between the baby and the screening machine.

Monitor the state of the baby, and the attachment of the sensors and earphones visually, and using the information on the screening equipment before the test is started (and during the test).

Screening devices present information differently. Some allow a screener to establish impedance and interference levels, including myogenic noise to optimise test conditions before starting the screen.

Other screening devices allow the same checks, but if a screener stops the screen during the time allowed to improve test conditions, these will be stored as a screen attempt.

Most will show some sort of progress indicator and some indication of myogenic (muscle) or electrical interference.

If test conditions deteriorate, pause the test and resettle the baby. Use the pause function when necessary during the test - machines allow different pause times.

However, as a general rule, it is best not to prolong a test beyond about 20 minutes, especially if the parent is becoming anxious.

Errors and issues

Repeating tests

Babies whose newborn hearing screen shows clear responses in both ears should not be subject to repeated screens and screening tests with a result of no clear response must not be repeated. Repeating tests may compromise the ability of the screen to detect hearing loss.

A test may be repeated if the result was incomplete because poor test conditions developed during the screen. In this circumstance the screener must take steps to improve the test conditions before repeating the test or may need to return to screen at another time if test conditions cannot be improved.

For AOAE tests a maximum of 3 test attempts per ear per protocol stage is permitted.

For AABR tests a further test is permitted following an incomplete result in one or both ears if the incomplete result was due to poor test conditions. However, if the AABR outcome is a no clear result in one ear with an incomplete result in the other a referral to audiology is needed. In this circumstance there is nothing to be gained by repeating the incomplete test.

Local managers must monitor the number of test attempts to ensure that tests are only stopped by the user in appropriate circumstances; such as poor test conditions and not in an attempt to avoid a no clear response result. To review the stop reasons local managers will need to download the screening test results from the national IT system and review the results in the intermediate software.

Saving results to the incorrect ear

Occasionally a screener will screen both ears but inadvertently save both results against the same ear. This can be managed as follows:

• if possible test the ear that has not got a result recorded. In the national IT system, set the unwanted duplicate result to ‘not required’
• if not possible and the screener and local manager are confident both ears have been screened, manage this in the national IT system by manually adding the missing ear result and setting the duplicate ear result to not required
• if you are not confident that both ears were tested recall the baby to be tested

Always write a case note to explain the circumstances.

Tests started in error

If an ear tip is not placed in the baby’s ear or the sensor leads are not connected to a baby and a test is started in error and stopped, it is acceptable to set these results to ‘not required’ in the national IT system with a case note to explain the reason why.

Time lapse between AOAE 1 and AOAE 2

The national IT system allocates AOAE results to the AOAE 1 or AOAE 2 stage according to predefined rules. AOAE 1 is regarded as complete when a ‘clear response’ or ‘no clear response’ result is obtained or when 5 hours have elapsed from the first AOAE 1 test attempt.

Thus, if a screener records a ‘not complete’ result in one ear , does not complete the screen because the baby becomes unsettled and completes the screen the next day, then these results will be classed as AOAE 2 because more than 5 hours has elapsed since the initial test attempt.

AOAE 2

At AOAE 2 it is only required to retest the ear(s) that did not show a ‘clear response’ result at AOAE 1. However, if there is any doubt about which ear showed a ‘clear response’ at AOAE 1 then AOAE 2 should be carried out on both ears.

AOAE 3 in error

If on uploading screening results to the national IT system the screener realises they have completed a further AOAE when the protocol stage should have been AABR the following course of action is indicated.

1. Contact the parent and explain the mistake.
2. Offer the AABR
3. If the parents accept the AABR carry out the test and enter the results on the national IT system.
4. Set the screening outcome and document the actions in a case note on the national IT system.
5. If the parents do not accept the AABR, document the offer in the national IT system and set the screening outcome according to the results you have.

AABR

Both ears are tested on AABR irrespective of the AOAE results. Where the screening equipment permits both ears should be tested simultaneously. Where it does not the screener must take great care to ensure that both ears are tested. This is best achieved by attaching the ear couplers to both ears initially and switching ears as soon as one is completed. In the event that only one ear requires testing it is essential that the screener is absolutely certain which ear is still to be tested. If there is any doubt both ears should be tested.

Uploading electronic results

Use the screening equipment data quality (SEDQ) interface to upload electronic results to the patient’s record in the national IT system.

SEDQ data should be uploaded in a timely way (within 3 working days of the test being carried out), where it is not automatically uploaded. If data does not reach the national IT system, providers can find guidance in SEDQ missing data process.

Information about SEDQ and its interface can be found in Chapter 5 Equipment.

You should ask yourself the following questions.

1. Are screening protocols followed and audited?
2. Is equipment calibrated as required?
3. Can you evidence that routine QA checks are undertaken?
4. Are screeners having their competency assessed as recommended?
5. Is screening data uploaded via SEDQ?
6. Do you know what action to take when *SEDQ data does not reach the national IT system?
7. Is manually entered data logged and checked?

Further guidance on management of babies within NHSP services and their data is available through NHS screening programmes care pathway (NHSP – Well and NICU baby), in guidance notes on local programme management tasks and operational guidance on national IT system, equipment and data and reports. Failsafe processes describe how NHSP providers can ensure incidents are avoided where possible.

Screens completed

Standard 4 – Intervention time from screening outcome to offered appointment for diagnostic audiological assessment

When the hearing screen is complete:

• explain the results as described in the NHSP e-learning screener dialogue
• provide written results by recording them on the hearing screening page within the PCHR (‘Red Book’), or if this is not available give the NHSP ‘Clear Response’ letter
• direct the parents to the 2 checklists ‘reactions to sounds’ and ‘making sounds’ in the PCHR and explain why these are important for monitoring their babies hearing and speech development as they grow up

If the completed screen outcome is a clear response in both ears discharge the baby to be monitored by its parents as it gets older.

If the completed screen outcome is a clear response in both ears but baby requires a targeted follow-up due to risk factors:

• inform the parents of the likely appointment location for audiological follow-up and ask for their preferred location if there is a choice
• give the parent the appropriate NHSP targeted follow-up screen outcome letter

If the completed screen outcome is a no clear response in one or both ears:

• refer baby for an audiological assessment having negotiated the best time with the parents and following agreed local referral processes
• give the parent the appropriate NHSP screen referral outcome letter
• ensure parents have local contact details, site maps and transport methods plus car parking facilities
• provide parents with ‘Your baby’s visit to the Audiology Clinic’ guidance
• advise parents to take someone with them to the appointment
• ensure the midwifery and HV teams are aware of the screen outcome
• write to the families GP and Health visitor to inform them of them screen outcome and referral

You should ask yourself the following questions.

1. Is the number of incomplete screens monitored?
2. Have outpatient clinic locations/times been selected to meet patient needs?
3. Are did not attend (DNA) and declines or withdrew consent rates monitored and is a reduction strategy in place?
4. Has a clear, documented referral mechanism been agreed between the screening team and paediatric audiology team(s)?
5. Is the referral rate from each stage of the screen monitored?
6. How are midwifery informed when a hospital screened baby refers on the screen?
7. How do the primary care team (GPs and HVs) receive notification that a baby has referred on the screen?
8. Is there a strategy in place for reducing the number of referrals to audiology if higher than expected?
9. Do the GPs and HVs locally want to know about the babies with clear responses and those requiring a targeted follow-up?
10. How do you ensure unscreened or incomplete screen transfers are followed-up?
11. How do the primary care team (GPs and HVs) receive notification of withdrawn consent after screening has started?

To assist providers further guidance on management of babies within NHSP services and their data is available through NHS screening programmes care pathway (NHSP – Well and NICU baby), in guidance notes local programme management tasks and operational guidance on national IT system and data and reports.

Failsafe processes describe how NHSP providers can ensure incidents are avoided where possible.

Parental concerns

If a baby was screened at birth and had clear responses but parents now have concerns whether baby can hear normally, you should advise them to contact their local GP or HV who will be able to arrange for baby/child to have a hearing assessment if necessary as per NHSP surveillance and audiological referral guidelines.

Babies resident in Wales

If you have a baby born in your hospital that is resident in Wales you should use the ‘Wales’ NHSP provider on the national IT system which will permit the transfer of records between both programmes. Transfer the record and inform the appropriate personnel in Wales if any further action is required. There is a list of Welsh contacts on the NHSP contacts database and NBHSW and NHSP interaction guidance advises providers how to manage records with Wales

Screening outcome set

When all screening data has been uploaded, the screening outcome should be set on the national IT system as soon as possible.

The logic in the national IT system suggests an outcome based on the baby’s protocol its risk factors, the test protocol and result, including targeting a baby whose protocol was NICU, with no clear response on AOAE, and clear response on AABR. There are a few occasions when a screener may need to override an outcome suggested by the national IT system.

These permitted overrides include:

• referral for targeted follow-up for professional concern, where a reason for overriding the suggested outcome must be given
• referral for targeted follow-up for the presence of a local risk factor

The local manager must monitor the use of status override to ensure this is not being used incorrectly.

If a follow-up has been offered for a risk factor other than those recommended nationally it is the provider responsibility to offer targeted follow-up in the event of transfer to a different provider that follows the national recommendations within NHSP surveillance and audiological referral guidelines.

You should ask yourself the following questions.

1. Are there robust systems in place to ensure screening results are uploaded and the outcome set in a timely manner?
2. Is there a system in place to check outcomes have been set correctly?
3. Is the use of ‘outcome override’ monitored closely?
4. Are transfers and shares between providers and services on national hearing screening IT system monitored regularly, actioned appropriately and dealt with in a timely way?

To assist providers further guidance on management of babies within NHSP services and their data is available through NHS screening programmes care pathway (NHSP – Well and NICU baby), in guidance notes local programme management tasks and operational guidance on national IT system and data and reports.

Failsafe processes describe how NHSP providers can ensure incidents are avoided where possible.

Screening results from private hospitals

If a parent phones to say baby has been screened and all is ok, you may still wish to offer an NHSP screen; this is a matter for local policy. If you do, upload results as usual. If not or the parents do not respond to your offer set the screening outcome as incomplete-out of coverage, making a record of the screen results in a case note.

Referral for immediate and targeted follow-up

Standard 4 – Intervention time from screening outcome to offered appointment for diagnostic audiological assessment.

Babies with a no clear response result in one or both ears or other result who require an immediate onward referral for audiological assessment, must be referred for diagnostic audiological assessment in a timely manner. The required timescale is either within 4 weeks of screen completion or by 44 weeks GA.

All parents should be provided with details of where and when the appointment will be taking place and details of who to contact should they need to cancel and rearrange the appointment. The audiology appointment for an immediate referral should be agreed and arranged with the parent at the time of screen completion.

Referral or targeted follow-up from screen would normally take place at the patients associated Audiology service, however arrangements for offering referral to out of area babies must be in place. Ensure all parties are made aware of the referral and the family know exactly who to contact and where their appointment will be.

To ensure attendance at audiology make sure parents realise the importance of the assessment and are not over reassured that the fluid is the reason for the hearing screening results. Families, where their baby was diagnosed with permanent childhood hearing impairment (PCHI), have said that they went to the appointment thinking the baby would be discharged and it was just the fluid that caused the no clear response, rather than the hearing loss.

Attendance can be improved by reminder text or phone calls to families just prior to their appointment due date, this will improve your attended assessment by 4 weeks or 44 weeks GA rates.

You should ask yourself the following questions.

1. Is there a written policy or protocol for dealing with immediate and targeted referrals locally?
2. Are immediate referral appointments made at the bedside or at the family home in conjunction with the parents?
3. Do parents receive verbal and written confirmation of the referral appointment and additional information such as directions, car parking etc?
4. Is there a failsafe in place that ensures audiology receive the referral?
5. Do parents receive reminders about attending the audiology appointment?
6. Are weekly audits carried out to ensure referred babies have been appointed or attend audiology?
7. Is the uptake of appointments and babies lost to follow-up being monitored closely?

Further guidance for providers and audiologists can be found within audiology operational guidance and from the BSA.

To assist providers further guidance on management of babies within NHSP services and their data is available through NHS screening programmes care pathway (NHSP – Well and NICU baby), in guidance notes local programme management tasks, operational guidance on national IT system and data and reports.

Failsafe processes describe how NHSP providers can ensure incidents are avoided where possible.

Hearing loss in one ear

If a baby is referred and the outcome of the audiological assessment is a hearing impairment in one ear it depends on the cause and severity of the loss as to what treatment or intervention is given. The Audiologist will explain the results at the time and discuss what is best for the baby with its parents.

Complete hearing satisfactory outcome following assessment

When audiology discharge babies from the assessment pathway, they direct the parent to the 2 checklists, ‘reaction to sounds’ and ‘making sounds’, in the PCHR and explain why these lists are important. If a follow-up is required for risk factors audiology will target the record on the national IT system and arrange this appointment at an age appropriate time.

Providers and their associated audiology services must enter assessment data and complete follow-up outcomes on the national IT system. There is further operational audiology guidance to support audiologists with this process.