s d r a d n a t S y t i l a u Q Bone anchored hearing aids for children and young people: Guidelines for professionals working with deaf children and young people Our vision is of a world without barriers for every deaf child With thanks to Cochlear Bone Anchored Solutions AB™ and Oticon Medical for providing some of the photographs and images for this publication and the families who gave us permission to use their photographs. Contents Summary 6 1. Introduction 8 1.1 A summary of the bone anchored hearing aid pathway 9 2. Hearing aids for conductive hearing loss 10 2.1 Aiding conductive hearing loss 10 2.2 The bone conduction (BC) hearing aid 11 2.3 The bone anchored hearing aid worn on a headband 11 2.4 The bone anchored hearing aid 12 2.5 The advantages and disadvantages of bone anchored hearing aids when compared to air conduction hearing aids (AC), bone conduction hearing aids (BC) and middle ear surgery (ME) 13 2.6 Bone anchored hearing aids for unilateral hearing loss (single-sided deafness) 16 3. Supporting the child, young person and family 17 3.1 Communication 17 3.2 Partnership and parental responsibility 17 3.3 Contact with other families 18 3.4 Children with additional/complex needs 18 3.5 Children with craniofacial abnormality 19 3.6 Children with otitis media with effusion (OME or glue ear) 19 3.7 Support groups and voluntary agencies 20 4. The role of local services 21 4.1 The local audiology service 21 4.2 The local education service 21 4.3 The local social care service 22 5. The bone anchored hearing aid service 23 5.1 The clinical scientist (audiology) or audiologist 23 5.2 The ENT surgeon 24 5.3 The audiovestibular physician 24 5.4 The speech and language therapist (SLT) 24 5.5 The ENT liaison nurse/advanced nurse practitioner 25 5.6 The administrator 25 5.7 The team coordinator 25 5.8 The key (link) worker 25 5.9 Additional support 25 5.10 Clinical facilities and accommodation 26 5.11 Training requirements 27 6. The bone anchored hearing aid equipment and features 28 6.1 Batteries 28 6.2 Colours and styles 28 6.3 Listening options 28 6.4 The equipment manufacturer/supplier 29 3 Bone anchored hearing aids for children and young people: Guidelines for professionals 7. The referral and selection procedure 30 7.1 Age of child 31 7.2 Attendance and commitment by the family and young person/child 31 7.3 Hygiene 31 8. The assessment process 32 8.1 Prior to the appointment at the clinic 32 8.2 The assessment 32 8.3 The audiological assessment 33 8.4 The speech, language and communication assessment 35 8.5 The medical assessment 35 8.6 Imaging 36 8.7 Ophthalmic assessment 36 8.8 Aetiological investigations 36 8.9 Multidisciplinary and multi-agency working 36 9. The outcome of the assessment 38 9.1 Consent 39 9.2 Establishing funding commitment for long-term NHS care 39 9.3 The treatment or management plan 39 9.4 Bilateral bone anchored hearing aids 40 10. Surgery and fitting the sound processor to children and young people 41 10.1 Nursing care prior to surgery, during surgery and post-operatively 42 10.2 First stage surgical procedure in children 43 10.3 Second stage surgical procedure in children 43 10.4 One-stage surgical procedure in young people 43 10.5 Seven to ten days post-operatively 44 10.6 Post-operative care and out of hours support 44 10.7 Discomfort, unexplained changes or swelling of the surgical site 44 10.8 Fitting the sound processor 44 10.9 Records of measurements 46 11. Ongoing evaluation and care 47 11.1 Regular inspection of the abutment 47 11.2 The surgeon’s responsibility for ongoing care 47 11.3 The clinical scientist (audiology)/audiologist and the speech and language therapist 47 11.4 Device failure 48 11.5 Loan equipment 48 11.6 Safety guidelines 49 11.7 Battery safety 49 11.8 Personal FM systems 49 11.9 Routine maintenance and monitoring of the bone anchored hearing aid and/or FM combination 50 11.10 Other assistive devices 51 12. Transition of care 52 12.1 Transition to an alternative paediatric bone anchored hearing aid service 52 12.2 Transition to adult care 52 4 Bone anchored hearing aids for children and young people: Guidelines for professionals 13. Service evaluation and audit 54 13.1 Complaints procedure 54 14. Appendices 55 Appendix 1: Glossary 55 Appendix 2a: Alphabetical listing of key professionals working with the child, family and the bone anchored hearing aid team 57 Appendix 2b: Alphabetical listing of professionals involved with the child and family who may need to contribute to the assessment and habilitation of a child with a bone anchored hearing aid (outside the immediate bone anchored hearing aid team) 57 Appendix 3: Sample patient profile 58 Appendix 4: Sample information leaflet 59 15. References 61 This document uses the words ‘parent’ and ‘family’ to include the child’s carer with parental responsibility. Throughout this document, the term ‘child’ is used to include babies and children, and ‘young person’ to include older children and young adults. The good practice and quality standards identified throughout the document are intended to be relevant to services throughout the UK. Some of the policy references highlighted in the text are not relevant to all countries of the UK. The abbreviation ‘QS’ is used throughout to define quality standards that must be implemented, monitored and audited. In this document NDCS uses the term ‘bone anchored hearing aid’ to mean any hearing system that uses bone conduction where contact with the skull is maintained by surgical implant and the clinical services that provide them. This document is intended to be applied to all such devices, available now or in the future, regardless of manufacturer. It is not intended to imply any manufacturer, supplier, or trade name. Currently: • Cochlear Bone Anchored Solutions AB™ manufactures and supplies a bone anchored hearing aid known as Baha®. For more information see www.cochlear.co.uk. • Oticon Medical manufactures and supplies a bone anchored hearing system known as Ponto. For more information see www.oticonmedical.com. 5 Bone anchored hearing aids for children and young people: Guidelines for professionals Summary More than two children a day are born in the UK with significant permanent hearing loss (Fortnum et al., 2001). Around 40% of deaf children will have additional needs, such as ophthalmic problems (Bamford et al., 2004, Guy et al., 2003, Fortnum and Davis, 1997) or developmental delay. Early identification and appropriate management lessens the impact of hearing loss on the child, the child’s family, and on society (Kuhl et al., 1992; Markides, 1986; Meadow-Orlans, 1987; Ramkalawan and Davis, 1992; Yoshinaga-Itano et al., 1998). The first bone anchored hearing aid devices were introduced to the UK more than 20 years ago and have been found to be an effective method of aiding some groups of children, such as those with chronic infection of the middle or outer ears, congenital abnormality of the ears or severe-profound unilateral deafness. There are more than 40,000 people worldwide using bone anchored hearing aids. Providing children with a bone anchored hearing aid requires a dedicated multidisciplinary team whose members understand the complex needs of each child and the impact any intervention will have. The paediatric bone anchored hearing aid team must be able to assess the individual needs of the child, be fully conversant with specialised bone anchored hearing aid equipment, and capable of providing long-term habilitative support to the child and their family, until transfer to an adult service. Essentially, the bone anchored hearing aid service must work closely with the child and their parents and involve them in every step of the procedure. The impact of such equipment will mean lifelong care and commitment by the NHS. NDCS believes that parents have the most important influence on their deaf child’s life. Optimal habilitation for the child can only occur when parents are considered and valued as equal members of a well-coordinated and accessible team. To ensure optimum benefit and support to the child, parents must be seen as partners. Equally, young people must be seen as partners when services are providing care to meet their needs. These Quality Standards are a revision of the NDCS Quality Standards in Bone Anchored Hearing Aids for Children and Young People (2003). NDCS has worked with bone anchored hearing aid services and consulted widely with local health and education services, families and young people in updating the quality standards and good practice guidance identified in this document. These quality 6 Bone anchored hearing aids for children and young people: Guidelines for professionals standards complement and should be used alongside national and country specific standards (where available) written for newborn hearing screening and audiology services in the UK. The National Deaf Children’s Society would like to thank the working party and all those who have taken part in the development and revision of this document. The document was available for public consultation on the NDCS website and invitations to respond were sent to NDCS membership and professional groups and organisations. All comments received have been considered as part of the editorial process. NDCS would like to pay particular thanks to the working party for the many hours spent on the development work and in helping to identify standards and good practice in bone anchored hearing aid provision for children and young people: Angela Deckett (Coordinator), Project Officer, NDCS Vicki Kirwin, Development Manager (Audiology and Health), NDCS Sheena Hartland, Birmingham bone anchored hearing aid programme Ken Higgins, The Ewing Foundation Members of Hearing and Balance UK (HABUK) Members of the British Society of Audiology’s Paediatric Audiology Interest Group (PAIG) Cochlear Bone Anchored Solutions AB™ Oticon Medical Further information or comments should be emailed to [email protected]. 7 Bone anchored hearing aids for children and young people: Guidelines for professionals 1. Introduction These quality standards and good practice guidelines have been written by NDCS in conjunction with professionals working in the clinical field. The purpose of this document is to ensure that deaf children and young people who can benefit from specialised bone anchored hearing aids receive the most appropriate service and long-term support to meet their individual needs. It is aimed at commissioners of services and professionals with an interest in providing services to deaf children, young people and their families. The objective is to provide a framework for audit with realistic and attainable standards for a bone anchored hearing aid service. Good practice guidance and quality standards are identified that will enable providers of health, education and social care services, as well as the voluntary sector, to deliver appropriate and effective support, from referral for a bone anchored hearing aid to transfer to an adult service. Services must be capable of adapting to change in technology and in other related services. Funding authorities have a duty to ensure that funding is made available for those children where a bone anchored hearing aid procedure is the most appropriate intervention method. ‘The bone anchored hearing aid service’ (see section 5) refers to the team responsible for the assessment of children, surgery, fitting and long-term maintenance of the hearing aid/s. ‘The local service’ (see section 4) refers to those services based in the child’s local hospital, education and social care services, which will liaise with the bone anchored hearing aid service to provide day-to-day support. Parents, children and young people must be fully involved in decisions about appropriate habilitation and implementation of procedures, as well as in monitoring the effectiveness and evaluating the success of any intervention. It is the responsibility of the service provider to ensure that they are well informed and fully aware of the potential implications their decision will have. Services must ensure that information is accessible and produced in a child- and family- friendly format. Young people must be fully involved in every step of their own care (Department of Health, 2002). Professionals from the bone anchored hearing aid programme should audit their service based on the quality standards identified in this document and on clinical governance. Service evaluation and continuous feedback, including the views of parents, families, users and professionals, are critical to the development of services. The best quality services will have a culture of learning continuously from families and children. Services must develop strategies to support all children with a hearing loss and their families. This will include children with additional needs, children from minority ethnic communities and children from ‘hard to reach’ families. 8 Bone anchored hearing aids for children and young people: Guidelines for professionals 1.1 A summary of the bone anchored hearing aid pathway Figure 1a – The local audiology service The local audiology service will: Identify the child’s/young person’s hearing loss Provide full audiological assessment, identifying any conductive component Trial bone conduction or air conduction aids if appropriate, and provide information on bone anchored hearing aids to the parents and/or young person Refer to the paediatric bone anchored hearing aid service, according to parental and young person’s wishes and local protocol Carry out ongoing liaison with the bone anchored hearing aid service Figure 1b – The bone anchored hearing aid service The bone anchored hearing aid team will: Carry out detailed multidisciplinary assessments with the child/young person Ensure the family and child/young person are fully involved in the process Liaise with local services Carry out bone anchored hearing aid surgery (this will be two stages for children, but is likely to be a combined single-stage procedure for young people) Arrange fitting of sound processor following osseointegration (three to six months after surgical procedure, depending on child’s bone thickness and quality) Follow up, evaluate and carry out long-term monitoring of outcomes with bone anchored hearing aid Carry out ongoing liaison, and training when required, with local services Transfer where appropriate to an adult bone anchored hearing aid programme 9 Bone anchored hearing aids for children and young people: Guidelines for professionals 2. Hearing aids for conductive hearing loss Congenital conductive hearing loss is often associated with atresia of the external auditory canal. Atresia is often associated with malformation of the middle ear and pinna. In most cases the cochlea is normal. The incidence of atresia is estimated to be 1 in 10,000 births. In about one quarter of all cases, atresia is bilateral (Declau et al., 1999). Congenital abnormalities can affect any or all of the outer and middle ear structures. The majority occur in isolation, but may be part of a syndrome such as Treacher Collins, Crouzons, Branchio-oto-renal (BOR) syndrome, Goldenhaar syndrome, etc. (Shprintzen, 1997). The severity of the hearing loss cannot be determined by the external examination of the outer ear. Other causes of long-term conductive hearing loss in children result from infection in the middle or outer ear. Chronic ear infection can cause a fluctuating conductive hearing loss that is dependent upon the severity of infection, inflammation and discharge present. Examples of such cases may be found in chronic suppurative otitis media, following mastoid surgery, or in children with Down’s syndrome (Miller et al., 1999; Roizen, 1997). 2.1 Aiding conductive hearing loss A child with a significant bilateral hearing loss must be provided with suitable amplification soon after diagnosis, and in accordance with published standards and guidance (Quality Standards in Paediatric Audiology, Volume IV, NDCS, 2000; Guidelines for the Fitting, Verification and Evaluation of Digital Signal Processing Hearing Aids within a Children’s Hearing Aid Service, MCHAS, revised 2005; Quality Standards in the NHS Newborn Hearing Screening Programme, NHSP, revised 2008; Quality Manual, Newborn Hearing Screening Wales, revised 2009; Quality Standards for Paediatric Audiology Services, Audiology Services Advisory Group, Scotland, 2009; Transforming Audiology Services for Children with Hearing Difficulties and their Families, Department of Health, 2008; Quality Enhancement Tool (QET) for audiology http://audiology.globalratingscale.com, Quality Standards in Paediatric Audiology (Wales), in development, 2009/10). Where the child has an infected ear, use of air conduction (AC) hearing aids may not be advisable as this can prevent adequate ventilation of the ear and may exacerbate the infection. The sound produced by an AC aid has to travel through debris and discharge and the sound quality may be lost. Bone conduction (BC) or bone anchored hearing aids can provide more consistent sound quality irrespective of the severity of infection and avoids ear occlusion. Therefore conventional BC hearing aids should be considered irrespective of whether a bone anchored hearing aid is a future consideration. With a congenital abnormality, sound from AC hearing aids is passed to the inner ear through structures that are malformed and therefore not able to conduct sound efficiently. An AC aid for this hearing loss may produce poorer than expected sound quality and elevated aided thresholds. In many cases, the fitting of an AC aid may not be possible due to the congenital abnormality of the external ear. Therefore a BC hearing aid should be fitted as soon as possible. QS01 Where a chronic conductive hearing loss is present, bone conduction hearing aids should always be considered, tried and evaluated. 10 Bone anchored hearing aids for children and young people: Guidelines for professionals