Clinical Pathways for Assessment and Habilitation of babies following Newborn Hearing Screening in Wales



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Clinical Pathways for Assessment and Habilitation of babies following Newborn Hearing Screening in Wales Page 1

Process Map Screening Assessment Confirmation of Significant Hearing Loss Child & Family Support Paediatric & Developmental Assessment Early Years Support Team Early Language & Listening Programme Hearing Assessment Hearing Aid Fitting Multi-Agency Review and Evaluation and Planning Links to other Services Depending on Child and Family Needs Page 2

Introduction The aim of the newborn hearing screening programme in Wales is to identify babies with significant hearing impairment of sufficient severity to cause or potentially cause a disability without the introduction of habilitation in infancy. Significant hearing impairment is defined as a bilateral hearing loss of a level greater than 40dBHL in the better ear taken as an average over frequencies of 500, 1000, 2000 and 4000 Hz (1). The screening programme ends at the completion of the assessment process for each baby or at three months of age whichever is the sooner although audiological confirmation or certainty may not be complete at this stage. The screening programme has a role in ensuring that habilitation services are available for hearing impaired babies and their families up to two years of age. Minimum Standards for Habilitation of babies in Wales were produced at the time of introduction of the screening programme taking into account best practice guidelines, available resources, feasibility of improvements and realistic resource requirements (2). Clinical audit has been completed using standards in the (3, 4). Evaluation of implementation of key stages in the care pathway at Health Board sites has been a core component of Quality Assurance of Newborn Hearing Screening Wales (5). Quality Assurance of habilitation has identified the need for robust mechanisms of recording key events in the to facilitate clinical care and ongoing evaluation of services (5). The has therefore been reviewed and documentation developed for professionals to record key stages ( Appendix 1). Page 3

Clinical Pathways A clinical pathway allows a multi disciplinary team to co-ordinate care by setting out all the activities involved in the care of the patient with a defined condition. A pathway leads each patient towards a set of desired outcomes and ensures that specific interventions are delivered at the appropriate time, in the right way and by the right professional (6). The newborn hearing screening clinical pathway therefore provides a multi disciplinary template for a plan of care but takes into account the need for clinical and professional judgement in caring for individual babies. It must also be flexible to allow for variations reflecting individual baby s and family s needs. provides the opportunity to provide consistent management with the baby and family at the centre of the care cycle. Information on the clinical care pathway for each family and baby can encourage involvement. offers increased opportunities for collaboration between professionals and agencies and can promote a greater awareness of the role of each professional within a team. can be used to support clinical effectiveness, clinical audit and risk management. The implementation and use of a clinical care pathway for babies referred for assessment following newborn hearing screening should help to ensure delivery of Family Friendly Hearing Services (7). Page 4

Pathways of care for assessment and habilitation following newborn hearing screening. Pathways are provided for: babies referred for assessment; babies with significant permanent bilateral hearing loss > 40 dbehl; babies with permanent unilateral hearing loss; babies with permanent conductive hearing loss; babies with temporary conductive hearing loss. Each pathway lists: steps; actions to be taken; who should take the action; time allowed for the action; accommodation requirements; outcome measures; Guidelines and Standards. Professionals involved in the care pathway: audiologist/clinical scientist( Audiology) paediatrician/professional lead; teacher of the deaf; speech and language therapist; social worker; clerical support and administration. These professionals are members of the Early Years Support Team. Not all early years support teams have the benefit of specialist speech and language therapy or social worker. Other individuals and organisations who support children and families need to be aware of the pathway. Page 5

Use of the clinical care pathways These pathways provide a framework for use by local teams. Each local team can use the pathways to support clinical audit, clinical effectiveness and risk management. References: 1. Newborn Hearing Screening Wales Quality Manual ( Version 3,) Objectives and Standards 2008 http://www.wales.nhs.uk/sitesplus/documents/980/1_quality_system.pdf 2. Newborn Hearing Screening Wales Minimum Standards for Habilitation.( Internal Report 2002) 3. Newborn Hearing Screening Wales. Audit of audiology, medical and multidisciplinary clinical pathways for babies identified with permanent hearing loss following newborn screening (Internal Report 2010) 4. Newborn Hearing Screening Wales. An audit of aetiological investigation performed for babies identified with bilateral permanent hearing loss following newborn screening and assessment (Internal Report 2008) 5. Newborn Hearing Screening Wales Quality Assurance Report 2011. http://www.wales.nhs.uk/sitesplus/documents/980/microsoft%20word%20- %20NBHSW%20QA%20report%20finalc%20version.pdf 6. Assembly Government Clinical Pathways (September 2002) http://www.wales.gov.uk/subiihealth/content/keypubs/clinical/contents_e.htm 7. Baguley D, Davies A, Bamford J. 2000. Principles of family friendly hearing services for children, BSA News 29 35-39 Page 6

Guidelines and Standards Assessment and Management of Auditory Neuropathy /Auditory Dys-synchrony: A Recommended Protocol Version 1.1 May 2008 http://hearing.screening.nhs.uk/audiologyprotocols Baguely D, Davis A, Bamford J. Principles of family friendly hearing services for children, BSA News 29 35-39. 2000 British Association of Audiovestibular Physicians. British Association of Community Doctors in Audiology. Guidelines for Good Practice. Investigation of new cases of severe and profound hearing loss in children. 2002 http://www.baap.org.uk/docs/severe%20to%20profound%20permanent%20hearing%20loss%20in %20Children%20Aetiological%20Investigation%20BAAP%20BAPA%20Guideline%202008.pdf British Association of Audiovestibular Physicians. Medical Evaluation of children with permanent unilateral hearing loss. 2009 http://www.baap.org.uk/docs/unilateral%20permanent%20hearing%20loss%20in%20children%20 Medical%20Investigation%20BAAP%20BAPA%20Guideline%202009.pdf Competencies for investigating the cause of hearing impairment in babies identified through the Newborn Hearing Screening Programme. BACDA Newsletter April 2005 Counselling for Audiologists. The Ear Foundation 2008 Department for Education and Skills. Department of Health. Together from the Start- practical guidance on working with disabled children (birth to 2) and their families.2002 www.dfes.gov.uk/consultations Distraction Diagnostic Test Protocol. http://hearing.screening.nhs.uk/getdata.php?id=10759 Down s Syndrome Medical Interest Group. Surveillance Guidelines Hearing Impairment. 2007 www.dsmig.org.uk Facilities: Welsh Health Estates Health Building Note 12, Outpatient Department, Supplement 3,ENT and audiology clinics, hearing aid centres( 1994) Guidelines for aetiological Investigation of infants with congenital hearing loss identified through newborn hearing screening. NHSP Clinical Group. January 2009 http://hearing.screening.nhs.uk/getdata.php?id=16481 Guidelines for the Identification and Management of Infants and Young Children with Auditory Neuropathy Spectrum disorder. Guidelines Development Conference at NHS 2008, Como, Italy. Page 7

Guidelines for the fitting of Hearing Aids to Young Infants. December 2009 http://hearing.screening.nhs.uk/getdata.php?id=19254 Modernising Children s Hearing Aid Service (MCHAS) http://www.psych-sci.manchester.ac.uk/mchas/guidelines/ Guideline No 1. Ear Impressions and Earmoulds for Children http://www.psych-sci.manchester.ac.uk/mchas/guidelines/earmouldguidelines.doc Guideline No 2. (A) Notes on Testing DSP Aids in the Field (B) Guidelines for Testing DSP Hearing Aids in the Field http://www.psych-sci.manchester.ac.uk/mchas/guidelines/digitalsignalprocessinghearingaids.doc Guideline No 3. Hearing Aids for Children: Fitting, Verification and Evaluation Guidelines http://www.psych-sci.manchester.ac.uk/mchas/guidelines/fittingguidelines.doc Guideline No 6. Audiology Service Links between Health and Education Services for Childrens Hearing Management http://www.psych-sci.manchester.ac.uk/mchas/guidelines/audiologyandeducation.doc National Deaf Children s Society. Quality Standards s in Bone Anchored Hearing Aids for Children and Young People: Guidelines for Professionals Working with Deaf Children and Young People. May 2010. http://www.ndcs.org.uk/applications/site_search/search.rm?term=bone+anchored+hearing+aids&s earchreferer_id=2&submit.x=9&submit.y=7 National Deaf Children s Society. Quality Standards in Paediatric Audiology. Guidelines for the Early Identification and Audiological Management of Children with Hearing Loss. Volume IV. October 2000 National Deaf Children's Society. Quality Standards in the Early Years. Guidance on working with deaf children under 2 years old and their families.2002 National Deaf Children's Society/SENSE. Vision care for deaf children and young people Guidelines for professionals. 2009 Newborn Hearing Screening and Assessment. Guidance for Auditory Brainstem Response testing in Babies. Version 1.1 April 2010. Stevens, Lightfoot : Co editors http://www.wales.nhs.uk/sitesplus/980/opendoc/178960 Newborn Hearing Screening Wales. Quality Manual Operational Procedures 2008 http://www.wales.nhs.uk/sitesplus/980/page/55734 Page 8

NBHSW Process for Peer Review. Version 9. August 2011 Quality Standards in Paediatric Audiology.( Wales.) Welsh Assembly Government 2010 http://wales.gov.uk/docs/dhss/publications/100421qulaitystandardspaediatricaudiologyen.pdf Royal College of Surgeons. Steering Group on Cleft Lip and Palate 1996 The Royal National Institute for Deaf People. Effective early intervention for deaf children and their families. 2001 Scope. Right from the Start. Looking at diagnosis and disclosure-parents describe how they found out about their child's disability.1999 Surgical management of otitis media with effusion in children. Clinical Guidelines. National Institute of Clinical Excellence. February 2008. http://www.nice.org.uk/nicemedia/live/11928/48420/48420.pdf Visual Reinforcement Audiometry Testing of Infants. http://hearing.screening.nhs.uk/getdata.php?id=10763 Welsh Cleft lip and Palate Service. Managed Clinical Network 2000 Other references and resources: Early Support Programme http://www.earlysupportwales.org.uk/ Positive practices in Social Services for Deaf Children 2001 British Association of Audiovestibular Physicians and British Association of Paediatricians in Audiology. Aetiological Investigation into bilateral mild and moderate permanent hearing loss in children. 2009 http://www.baap.org.uk/docs/mild%20to%20moderate%20permanent%20hearing%20loss%20in% 20children%20Aetiological%20Investigation%20BAAP%20BAPA%20Guideline%202009.pdf Investigation of the child with permanent hearing impairment Breege Mac Ardle,Maria Bitner- Glindzicz Arch Dis Child Educ Pract Ed 2010;95:14-23 National Institute for Health and Clinical Excellence. Cochlear Implants for children and adults with severe to profound deafness. February 2011 http://www.nice.org.uk/_gs/link/?id=f97464a1-19b9-e0b5-d4ecba8c576746fd Page 9

Quality Standards for Paediatric Audiology: Cochlear Implants for children and young people. Guidelines for professionals working with deaf children and young people. British Cochlear Implant Group and National Deaf Childrens Society. March 2010 http://www.ndcs.org.uk/search_clicks.rm?id=6252&destinationtype=1&instanceid=281802 Voluntary Organisations British Deaf Association www.bda.org.uk/ National Children s Bureau www.ncb.org.uk/cpc/ National Deaf Children s Society www.ndcs.org.uk/ Royal National Institute for Deaf People www.rnid.org.uk/ Leaflets Contact a Family Fact Sheet: Fathers. www.cafamily.org Contact Family Fact Sheet: A Genetic Condition in the Family. www.cafamily.org Newborn Hearing Screening Wales. Your Baby s Visit to the Audiology Clinic. 2009 Newborn Hearing Screening Wales. Your Baby has a Hearing Loss. 2009 Newborn Hearing Screening Wales. Auditory Neuropathy Spectrum Disorder. 2009 Newborn Hearing Screening Wales. Your baby has glue ear. 2010 Newborn Hearing Screening Wales. Your baby has a hearing loss in one ear. 2010 National Deaf Children's Society. Unilateral hearing loss National Deaf Children's Society. Glue ear National Deaf Children's Society. Family information pack National Deaf Children Society. Genetic counselling National Deaf Childrens Society. Microtia National Deaf Childrens Society. Why does my child have a hearing loss? National Deaf Childrens Society. Downs Syndrome and Hearing loss. Hearing Aids: A Guide NDCS 2000 Tips on how to get your Child to wear their hearing aids NDCS 2002 Page 10

Pathway for assessment of referrals from the screening programme STEPS ACTIONS BY WHOM WHERE TIME OUTCOMES GUIDELINES/STANDARDS Assessment Ensure translator/health advocate availability Contact with parents Check parents/carer received information and understand assessment Explanation of assessment Consent verbal History taking Complete peer reviewed hearing assessment Audiology administration Audiologist/Clinical Scientist + Professional Lead for Advanced Assessment Assessment Clinic Appropriate sound treated or sound proofed room in child/baby friendly environment Prior to clinic Initial assessment within 4 weeks of screen result for well babies and within 8 weeks of screen result for NICU babies Advanced assessment within timescale agreed with parents and ensuring timely (<3months) completion of assessment Parental participation in assessment process informed understanding and consent to assessment Minimum number of babies lost to follow up Parental participation in assessment process Informed understanding and consent to assessment Ear specific thresholds established determining outcome of assessment 1. consideration for hearing aids 2. audiological follow up 3. targeted behavioural hearing test 4. no further follow up TBT for babies who DNA assessment Family friendly Hearing Services (Baguley et al, 2000) NDCS Quality Standards Volume IV. Oct. 2000 Counselling for Audiologists. The Ear Foundation 2008 Guidelines for the early audiological assessment and management of babies referred from the newborn hearing screening programme Version 5 (Welsh) March 2011 Guidelines for the assessment and management of ANSD in young infants. Version 2.1 October 2012 NBHSW Process for Peer Review Version 9 August 2011 NBHSW Quality Manual 2008 Provide information on outcome of assessment to parents. ( see pathway for confirmation Inform Professional Lead and NBHSW programme(dc)+/- PHCT and other involved professionals At time of assessment Within 1 working day Written report within 10 days Parents informed of hearing assessment result and follow up ( see pathway for confirmation) NBHSW programme and PHCT informed of assessment result and follow up. Page 11

Pathway: Confirmation of permanent bilateral hearing loss >40dBeHL or Auditory Neuropathy Spectrum Disorder STEPS ACTIONS BY WHOM WHERE TIME OUTCOMES GUIDELINES/STANDARDS Confirmation of sensorineural hearing loss or auditory neuropathy spectrum disorder Ensure translator/ health advocate availability Verbal explanation of results Written information on hearing loss Information on voluntary organisations and contact numbers Information on Early years support team and contact numbers Arrange appointment for hearing aid moulds or take impressions in consultation with parents (sensorineural hearing loss) Arrange appointment for hearing aid fitting if required Arrange audiological follow up for ANSD. Arrange for medical review/assessment Early years support teacher informed Professional lead informed ( if not present) Audiology administration Audiologist/Clinical Scientist Appropriate quiet room Prior to appointment Immediately following assessment and repeated as required by family As soon as possible Within 4 weeks of decision to offer hearing aids Within 9 months or at developmentally appropriate time. Within 5 days if Professional Lead not present at assessment. Within 24 hours Within 24 hours Parental participation in assessment process informed understanding and consent to assessment Parental access to clear information on hearing loss or ANSD. Support network established Timescales for moulds and hearing aid fitting or follow up behavioural test agreed with parents Medical follow up arranged Family friendly Hearing Services (Baguley et al, 2000) NDCS Quality Standards. Volume IV. October 2000 Disclosure of deafness. Peter Watkin. www.deafnessatbirth.org.uk Counselling for Audiologists. The Ear Foundation 2008 Guidelines for the early audiological assessment and management of babies referred from the newborn hearing screening programme Version 5 (Welsh) March 2011 Guidelines for the assessment and management of ANSD in young infants. Version 2.1 October 2012 Quality Standards in Paediatric Audiology (Wales) 2010. Written report for Professional Lead and Divisional Coordinator NBHSW Quality Manual 2008 Letter to GP/other professionals Page 12

Guidance notes for clinical pathways for babies with confirmed hearing loss. The pathway for babies with confirmed hearing loss will depend on: Audiological evaluation and certainty Family needs e.g. parental choice, social factors Baby needs e.g. other significant medical conditions Page 13

Pathway for Audiological Care of babies with permanent bilateral >40 dbehl : Decision to aid (1) STEPS ACTIONS BY WHOM WHERE TIME OUTCOMES GUIDELINES/STANDARDS Hearing aid fitting Ensure translator/ health advocate availability Ensure parental understanding of procedure and consent to fit. Fit appropriate hearing aids binaurally Undertake real ear measurements if appropriate Provide verbal and written information on hearing aid management Provide hearing aid care and maintenance pack (inc battery supply) Ensure parental understanding of access to Audiology Department and arrangements for provision of ear moulds. Appointment made if required Individual Management plan developed and distributed to Early support team and parents Inform early years support team/ professional/medical lead and Divisional Coordinator NBHSW. Arrange hearing aid review Ensure follow up in Multi-agency Planning meeting Audiology administration Audiologist/Clinical Scientist and audiology support staff Professional Lead Appropriate quiet room Prior to clinic appointment Within 4 weeks of confirmation of hearing loss At time of fitting Within 3 weeks Within 10 working days Within 10 working days Within 1 month Within 9 months Provision of appropriate amplification Optimal amplification Early habilitation and support Family Friendly Hearing Services (Baguley et al, 2000) NDCS Quality Standards. Volume IV. Oct. 2000 Facilities: Welsh Health Estates Health Building Note 12, MCHAS (Wales) Guideline No 1- Guideline No 6. 2005 Guidelines for the fitting of Hearing Aids to Young Infants. December 2009 Quality Standards in Paediatric Audiology ( Wales) 2010 Page 14

Pathway for Audiological Care of babies with permanent bilateral >40dBeHL : Decision to aid (2) STEPS ACTIONS BY WHOM WHERE TIME OUTCOMES GUIDELINES/STANDARDS Review of fitting and ongoing care Ensure translator/health advocate availability Liaison with family key worker Seek parental view of amplification New moulds Check aids in test box as appropriate Undertake real ear measurements and sound field verification, as indicated Provide verbal information to parents on fitting Audiology administration Audiologist/Clinical Scientist Appropriate quiet room Prior to every appointment At appointment or by telephone prior to appointment At appointment Parental participation in review Co-ordinated care/team working Parental involvement Maintenance of optimal amplification Family Friendly Hearing Services (Baguley et al, 2000) NDCS Quality Standards. Volume IV. Oct. 2000 Facilities: Welsh Health Estates Health Building Note 12, MCHAS(Wales) 2005 Guideline No 1. Guideline No 2. Guidelines No 3. Guidelines No 6. Quality Standards in Paediatric Audiology ( Wales) 2010 Develop Individual Management Plan to be shared with parents and Early Years team Replace hearing aids if not functioning/lost/ destroyed Provide batteries As necessary Page 15

Pathway for Audiological Care of babies with permanent bilateral >40 dbehl : Decision to aid and ANSD.(3) STEPS ACTIONS BY WHOM WHERE TIME OUTCOMES GUIDELINES/ STANDARDS Ongoing audiological assessment Ensure translator/health advocate availability Audiology administration Prior to every appointment Parental participation in review Liaison with family key worker Seek parental view of hearing responses Unaided behavioural test of hearing Middle ear impedance measures Provide verbal information to parents on hearing levels Develop Individual Management Plan to be shared with parents and Early Years Team Arrange review Audiologist/Clinical Scientist Sound proofed room with VRA facilities At appointment or by telephone prior to appointment At appointment Begin at 6 months developmental age At appointment As required to determine ear and frequency specific hearing levels as soon as possible Coordinated care/team working Parental involvement Verification of objective measures of hearing Establish minimum response levels Obtain ear specific and frequency specific response levels Determine amplification requirements include suitability for cochlear implant. Inform care plan and ongoing habilitation including medical/surgical management of OME if required Family Friendly Hearing Services (Baguley et al, 2000) NDCS Quality Standards. Volume IV. Oct. 2000 Distraction Diagnostic Test Protocol. Visual Reinforcement Audiometry Testing of Infants. A Recommended Test Protocol. MCHAS (Wales) Guidelines No 6. Audiology Service Links between Health and Education Services for Children s Hearing Aid Management Quality standards in Paediatric Audiology : (Wales ). 2010 Guidelines for the assessment and management of ANSD in young infants. Version 2.1 October 2012 Page 16

Pathway for Medical Care (1) STEPS ACTIONS BY WHOM WHERE TIME OUTCOMES GUIDELINES/ STANDARDS Medical support and assessment Ensure translator/health advocate availability for Audiology/Child Health Administration Prior to appointment NDCS Quality Standards Volume IV. Oct. 2000 Liaison with neonatologist /paediatrician (baby already identified as having significant medical condition) Ensure early support is in place with parents understanding the role of the key worker Ensure initial MASP developed and circulated to all professionals including parents Provide opportunity to discuss assessment results, proposed management plan and aetiology investigations as dictated by needs of parents Provision of verbal and written information on aetiological investigations Professional Lead Suitable consulting room or home visit Following confirmation of hearing loss/ansd Referral within 24 hours Within 3 months Within 5 days of confirmation of hearing loss Within 4-6 weeks of Confirmation of hearing loss OR as dictated by other medical conditions or parental/social factors Urine for CMV as soon as Parents informed and understand purpose of examination and investigations. Identifications of medical conditions to: Inform ongoing medical and audiological management including suitability for cochlear implant Quality standards in Paediatric Audiology : (Wales ). 2010 BACDA/BAPP Guidelines for Good Practice. Investigation of new cases of severe and profound hearing loss in children. 2002 Guidelines for aetiological investigation of infants with congenital hearing loss identified through newborn hearing screening. January 2009 Competencies for investigating the cause of hearing impairment in babies identified through the NBHSP. BACDA Newsletter April 2005 NBHSW Proforma Medical Assessment (2004) Identification and Management of Infants and Young Children with Auditory Neuropathy Spectrum Disorder. 2008 Page 17

possible. Other investigations as agreed with parents. Inform family Inform habilitation Guidelines for the assessment and management of ANSD in young infants. Version 2.1 October 2012 Arrange appointment for baby examination as determined by clinical need Improved epidemiological data Complete recommended medical examination and investigations agreed with parents Results of investigations discussed with parents and written summary provided Contribute to team planning relevant medical factors as agreed with parents Information to PHCT and Paediatric team(s) as appropriate Ensure Multi- Agency planning meeting scheduled Within 9 months Page 18

Pathway for Multi-Agency Involvement STEPS ACTIONS BY WHOM WHERE TIME OUTCOMES GUIDELINES/ STANDARDS Multi agency planning meeting Ensure interpreter/ health advocate present Meet with family members Audiology Administration Prior to appointment Parental participation in support Seek parental views and needs Summarise/review Audiology support Summarise/review Education early support Summarise/review other support provided Audiologist/Clinical Scientist Professional Lead Early Years Support Teacher and other team members. (Health Visitor, Speech and Language Therapist, Social Worker & other agencies) Family friendly environment At time agreed with parents and within 9 months of confirmation of hearing loss/ansd Shared information and understanding of roles of team member Written shared management plan between parents and early years support team. NDCS Quality Standards in the Early Years.2002 RNID Effective early intervention for deaf children and their families.2001 Department of Health. Together from the Start.2002 Quality Standards in Paediatric Audiology (Wales) 2010 Review role of key worker Develop agreed Family Support Plan /Multi Agency Support Plan Agreed follow up arrangements for Multi agency meeting. Arrange review Distribute Care Plan Designated Coordinator (Professional Lead) Audiology/Paediatric Administration Page 19

Pathway for babies with better ear <30dBeHL and worse ear > 40dBeHL-unilateral permanent hearing loss and ANSD STEPS ACTIONS BY WHOM WHERE TIME OUTCOMES GUIDELINES/ STANDARDS Confirmation of unilateral hearing loss Ensure translator/health advocate available Audiology administration Prior to appointment Parental participation in consultation Follow up Audiological assessment Verbal explanation of results Written information regarding unilateral loss Contact numbers provided Arrange audiological follow up Letter to GP, HV and Professional Lead and parents Consider medical review Ensure translator/health advocate available Audiologist/Clinical Scientist Professional Lead Audiology administration Clinical Assessment clinic Appropriate sound treated / Sound proofed room At time of assessment Prior to appointment Parental access to clear, unbiased information Parental understanding and implications of diagnosis Identify specific conditions Parental participation in consultation Guidance for Auditory Brainstem Response testing in Babies. Version1. 1 April 2010 Guidelines for the assessment and management of ANSD in young infants. Version 2.1 October 2012 Visual Reinforcement Audiometry Testing of Infants. A Recommended Test Protocol Seek parental view of hearing responses Behavioural test of hearing Middle ear impedance measures Provide parents with information on hearing responses and Individual Management Plan Arrange review Consider medical review/multi agency planning Information to PHCT and Professional Lead/other professionals as required. (Individual Management Plan) Scientist/Audiologist/Paediatric Audiology Service Clinical Scientist/Professional Lead Sound proofed room with VRA Within 9 months of initial assessment 6 monthly unless otherwise indicated. As appropriate Continued monitoring of better ear Ear specific hearing responses obtained OME management Coordinated care NDCS Quality Standards Volume IV. Oct 2000 Quality Standards in Paediatric Audiology (Wales) 2010 BAAP. Medical Evaluation of children with permanent unilateral hearing loss. 2009 Page 20

Guidance notes on pathway for babies with confirmed bilateral conductive hearing loss not associated with ear abnormalities and/or other conditions This pathway is for babies who, following assessment are found to have a bilateral conductive hearing loss the likely cause of which is middle ear effusions. These babies require monitoring of hearing response levels and middle ear function. Page 21

Pathway for babies with confirmed bilateral conductive hearing loss not associated with ear abnormalities or other conditions STEPS ACTIONS BY WHOM WHERE TIME OUTCOMES GUIDELINES/ STANDARDS Confirmation of bilateral conductive hearing loss Ensure translator/ health advocate available Audiology administration Assessment clinic Sound proofed room Prior to appointment Parental participation in consultation Guidance for Auditory Brainstem Response testing in Babies. Version1.1April 2010 Verbal explanation of results Provide written information Contact numbers provided Arrange follow up behavioural hearing test Letter to GP, HV/other professionals/parents Clinical Scientist/Audiologist Assessment clinic Appropriate Sound treated or sound proofed room At time of assessment Within 9 months or at developmentally appropriate time Parental access to clear, unbiased information Parental understanding of assessment results Follow up plan Distraction Diagnostic Test Protocol Visual Reinforcement Audiometry Testing of Infants. A Recommended Test Protocol Follow up audiological assessment Ensure translator/ health advocate available Seek parental view of hearing responses Behavioural test of hearing- Middle ear impedance measures Information for parents on hearing level (Individual Management Plan) IMP circulated to Primary Care team and other professionals as required Arrange hearing review Consider amplification or surgical opinion as indicated. Audiology administration Clinical Scientist/ Audiologist/Paediatric Audiology Service Appropriate sound proofed room Prior to appointment Within 9 months of initial assessment or at developmentally appropriate time 3 6 months unless otherwise indicated Parental participation in consultation Minimum response levels ( ear specific) and middle ear status established Management of glue ear. NDCS Quality Standards Volume IV. Oct. 2000 Quality Standards in Paediatric Audiology (Wales) 2010 NICE Guidelines on Glue Ear 2009. Page 22

Guidance notes on pathway for babies with confirmed bilateral conductive hearing loss associated with ear abnormalities or other conditions This pathway is for babies who, following assessment are found to have a bilateral or unilateral conductive hearing loss in association with ear abnormalities. These babies may have other medical conditions or syndromes. The audiological care and ongoing care of these babies will require liaison with a wide range of professionals e.g. Cleft Palate Team members, Consultant Otolaryngologist, Paediatrician. Babies with the conditions and syndromes listed below may follow this pathway: Down s Syndrome; Cleft Lip and Palate; Microtia; Goldenhar Syndrome; Moebius Syndrome Robin Syndrome; Treacher Collins Syndrome; Symphalangism; Osteogenesis Imperfecta; Klippel Feil Syndrome. Page 23

Pathway for babies with confirmed unilateral or bilateral conductive hearing loss associated with ear abnormalities or other conditions STEPS ACTIONS BY WHOM WHERE TIME OUTCOMES GUIDELINES/ STANDARDS Confirmation of unilateral or bilateral conductive hearing loss Follow up audiological assessment Ensure translator/ health advocate available Verbal explanation of results Provide written information Liaise with and provide written information for other professionals involved in care Consider amplification ( see Pathways for fitting and multiagency planning) Arrange follow up Audiological assessment Letter to GP, HV/other professionals and parents Consider medical review Ensure translator/ health advocate available Seek parental view of hearing responses Behavioural test of hearing Middle ear impedance measures Information for parents on hearing levels Arrange follow up behavioural tests Information to Professional Lead/ PHCT/parents Individual Management Plan Audiology administration Prior to appointment Parental access to clear, unbiased information Clinical Scientist/Audiologist and Professional Lead Assessment clinic Appropriate sound treated or Sound proofed room in baby friendly environment At time of assessment Within 9 months or developmentally appropriate time Parental understanding and implications of assessment results Coordinated care Early amplification Audiology administration Prior to appointment Parental participation in consultation Audiological Scientist / Audiology clinic minimum response Audiologist/ VRA facilities in sound levels( ear specific) and Paediatric proofed room middle ear status Audiology Service established Within 9 months of initial assessment or sooner if offered amplification 3-6 months Provision of amplification if appropriate Management OME Guidance for Auditory Brainstem Response testing in Babies. Version 1. 1 April 2010 Welsh Cleft Lip and Palate Service Guidelines 2000 DSMIG Surveillance for Guidelines Hearing Impairment. Version 6. 2000 www.dsmig.org.uk/ Distraction Diagnostic Test Protocol www.nhsp.info Visual Reinforcement Audiometry Testing of Infants www.nhsp.info Quality Standards in Paediatric Audiology (Wales) 2010 NDCS Quality Standards: Bone anchored hearing aids for children and young people: Guidelines for professionals working with children and young people. May 2010 NICE Guidelines on Glue Ear 2009. Page 24