Universal Newborn Hearing Screening the UK experience

Transcription

1 Universal Newborn Hearing Screening the UK experience Dr. Ghada Al-Malky, PhD Senior Lecturer Director of BSc & MSc Audiology Courses Ear Institute UCL History Descartes Sound amplitude- >neural reflex (time coding of freqs.) Helmoholtz Fourier Analysis in the cochlea ter Kuile Tone freq. determined from BM length activated Von Békésy Travelling wave theory Thomas Gold Active mechanical amplifier David Kemp Recorded OAEs reflecting the mechanical amplifier & travelling wave The first commercial OAE system available Georg Von Békésy David Kemp 1

2 Initiation of newborn screening in the US in 1999 endorsed by the Joint Committee on Infant Hearing (JCIH) Early Hearing Detection & Intervention (EHDI) programs: Advantages: Recordable at birth, Reliable, Quick, Non-invasive, Easily interpreted Cost effective, Objective, Specifically assesses cochlear function, Provides ear specific information, high sensitivity and specificity UK Newborn Hearing Screening Programmes North Wales - NBHSW : started in March 2003, and in October 2004 became the first fully implemented national newborn hearing screening programme in the UK. ( Scotland UNHSScotland: The roll out across the country was completed in December local programs (~60 000/annum). ( NHSP-England: introduced in a phased and nationally organized process between 2002 and fully implemented in March local programs covering all births in England (~ /annum). (Wood et al., 2015) Ireland -Newborn Hearing Screening Programme: rolled out in 19 hospitals 2

3 Definition of screening The practice of investigating apparently healthy individuals with the object of detecting unrecognized disease (hearing loss) and intervening in ways that will improve the prognosis 3

4 Prevalence of Permanent Childhood Hearing Impairment (PCHI) Hearing loss and deafness are global issues that affect at least 278 million people worldwide. Two-thirds of these people live in developing countries (Tucci et al., 2010) Estimated that 1 to 2/1000 babies in the UK are born with a permanent hearing loss in one or both ears 1/1000 in the well-baby nursery 1/100 who have spent at least 48 hour in the NICU/SCBU 840 children/ year with PCHI in the UK Basis for support of newborn hearing screening Early identification of hearing loss gives the baby a better life chance of developing speech and language skills needed for maximizing benefit of social and emotional interaction from an early age (Yoshinaga-Itano et al., 1998; Meoller, 2000) Neonatal screening had a significant role in the identification of congenital hearing impairment (Davis et al., 1997) 50-90% of babies with a hearing loss are born to families with no history of deafness (Barker et al., 2012) 4

5 +Overall Organization & Quality Standards The National Screening Committee for the UK (UKNSC) is responsible all screening programmes in the UK Setting and monitoring standards, developing protocols and procedures, specifying and procuring equipment and information systems, advising on incident management and quality assurance. All screening tests and outcomes that are part of NHSP are recorded in the national information system (e-screener Plus: esp). 5

6 The Local NHSP Provider Staffing: NHSP local Manager, Team Leader and enough trained Screeners Training: on-going educational programme for health professionals involved in hearing screening with CPD in line with best practice guidelines Audiology services: provide trained experienced staff to undertake post screen audiological assessments IT infrastructure: to support quick & reliable data transfer (esp) and equipment support Strong working relationship: the screening programme, esp system, audiology departments, maternity services, medical services (paediatric audiology, ENT, AVM, genetics), NICU/SCBU, child health departments, health visiting services and primary care professionals. What did the NHSP program aim to achieve? The parents of all babies born or resident in UK should be offered hearing screening for their baby within 4 to 5 weeks of birth. To identify permanent moderate, severe and profound (>40dBHL) bilateral hearing impairment (PCHI) of all newborn babies born or resident in UK within 4/5 weeks of birth Babies referred from the programme should be seen for a full audiological assessment within 4 weeks of screen completion To ensure the provision of safe, high quality age appropriate assessment by audiological services and provide ongoing support for the children with PCHI and their families. 6

7 Aims of NHSP continued There are clear care pathways for the screening and referral process for NHSP. Highlight the importance of continuous surveillance by parents and professionals to detect later onset hearing loss GPs, midwifery team and Health visitor are notified of screen referrals by letter Babies that miss screening should receive it as soon as possible, but not after 3 months of age. Some babies are not eligible for screening; e.g. having an already-known risk of hearing impairment or deafness from another condition (e.g. meningitis, ear canal atresia/microtia). Babies at less than gestational age 34 weeks or over 3 months of (corrected) age A local failsafe procedure needs to be in place to ensure that all babies who commence the screen complete it and ensure that all esp records are updated. Overview of screening structure There are different protocols for: Well babies Those who have spent over 48 hours in SCBU or NICU. The programme offers 2 types of test: automated Transient Evoked Otoacoustic Emissions (AOAE) automated auditory brainstem response (AABR) Local screening programmes should use approved equipment only. 7

8 NHS public health functions agreement Map of Medicine screening pathway with failsafe points NHS public health functions agreement Map of Medicine screening pathway with failsafe points 8

9 NHS public health functions agreement Map of Medicine screening pathway with failsafe points 9

10 AOAE: Well-baby protocol: Test twice only (max 2 attempts/screen session) If no clear response (NCR) - proceed to AABR If NCR - refer to audiological services AABR: NICU/SCBU >48 hrs protocol: Both AOAE & AABR are performed If no clear response in either tests refer to audiological services 10

11 PASS Criteria: 3 out of 5 frequency bands (e.g. 1,1.5, 2, 3, 4kHz) reach a signal-to-noise ratio (SNR) of at least 6dB Total TEOAE of 0dBspl (across all frequencies) OAE in each pass band of at least -5dBspl. If an ear appears to have no OAE then before deciding to terminate an OAE session and refer the baby for a second test session: Check the noise level is adequately low (quiet room, settled baby) Check the probe for contamination and change the filter if needed Refit the probe and retest Baby s ear may be filled with birth fluid Then repeat the test only two attempts can be made within each screening session The 2 nd test session can be made within the same day (4hrs later) or before discharge from hospital. 11

12 Printed outcome example AABR outcomes 12

13 Outcomes of the screen Clear Response (CR) both ears = PASS CR in both ears but the baby requires a targeted follow-up due to other risk factors, as per protocol. Response: REFER considered for audiological assessment at 8 months of corrected age No Clear Response (NCR) one ear confirmed = optional repeat screen in hospital outpatient clinic/ at 9 mths follow-up NCR both ears confirmed = REFER Checklist for Reaction to Sounds (given to parents) Shortly after birth - a baby Is startled by a sudden loud noise such as a hand clap or a door slamming. Blinks or opens eyes widely to such sounds or stops sucking or starts to cry. 1 month - a baby Starts to notice sudden prolonged sounds like the noise of a vacuum cleaner and may turn towards the noise. Pauses and listens to noises when they begin. 4 months - a baby Quietens or smiles to the sound of familiar voice even when unable to see speaker and turnes eyes or head towards the voice. Shows excitement at sounds, eg; voices, footsteps etc. 7 months - a baby Turns immediately to familiar voice across the room or to very quiet noises made on each side (if not too occupied with other things). 9 months - a baby Listens attentively to familiar everyday sounds and searches for very quiet sounds made out of sight. 12 months - a baby Shows some response to own name. May also respond to expressions like "no" and "bye bye" even when accompanying gesture cannot be seen. 13

14 Checklist for Making Sounds 4 months - a baby Makes soft sounds when awake. Gurgles and coos. 6 months - a baby Makes laughter-like sounds Starts to make sing-song vowel sounds eg; a-a, muh, goo, der, aroo, adah 9 months - a baby Makes sounds to communicate in friendliness or annoyance. Babbles (eg; da da da, ma ma ma, ba ba ba) Shows pleasure in babbling loudly and tunefully. Starts to imitate other sounds like coughing or smacking lips. 12 months - a baby Babbles loudly, often in a conversational-type rhythm May start to use one or two recognizable words. Diagnostic assessment session by Audiologist History & Otoscopy Threshold-ABR [AC & BC clicks, frequency specific tone pips and cochlear microphonic response] Tympanometry (High frequency) TEOAEs (Transient evoked otoacoustic emissions test) 14

15 AC Threshold estimation ABR- 1kHz TB AC Threshold estimation ABR 4kHz TB 15

16 BC Threshold estimation ABR 1& 4kHz TB Key Performance Targets All hearing screening providers must meet and report on key performance indicators (KPIs) set and reviewed by the national screening team (UKNSC). KPI 1 screen complete by 4/5 weeks of age = 95% KPI 2 - time from screen refer to first attended diagnostic appointment four weeks or less = 90% 90% of children with bilateral moderate to profound permanent childhood hearing impairment (PCHI) to have their hearing loss identified by eight weeks of age and 100% by 24 weeks of age. 16

17 NHSP KPIs KPI Description Data source Responsible for submission NH1 NH2 Newborn hearing screening coverage Newborn hearing timely assessment for screen referrals Newborn hearing coordinators via electronic submission from the national database Electronic submission from the programme centre based on data from local newborn hearing coordinators The NHSP submits KPI data electronically to the UK NSC three months after the end of each quarter. ( UKNSC_KPI_IndicatorSubmissionProcess_Year14-15_v1_4.pdf) Reporting periods for KPI data submission Quarter Data range Submission window Q1 1 April to 30 June 1 to 30 September Q2 1 July to 30 September 1 to 31 December Q3 1 October to 31 December 1 to 31 March Q4 1 January to 31 March 1 to 30 June 17

18 Performance and characteristics of the Newborn Hearing Screening Programme in England: The first seven years Sally A Wood, Graham J Sutton & Adrian C Davis 4, 645, 823 children born 1 April 2004 to 31 March 2013 Coverage: 97.5% of the eligible population complete screening by 4/5 weeks of age 98.9% complete screening by three months of age Timely referrals: screen positive (i.e. referred) babies commencing follow up by four weeks of age and six months of age is 82.5% and 95.8% respectively. yield of bilateral PCHL from the screen is around 1/1000 refer rate for the 12/13 birth cohort is 2.6% Children with bilateral PCHL referred from newborn screening, the median age (in days) at screening completion (9d in 12/13), first assessment (30), confirmation of PCHL (49), referral to early intervention services (50), and hearing-aid fitting (82) (n= >4000) (Wood et al., 2015) 18

19 Conclusion The UK Newborn Hearing Screening Programme continuously progressing successful national government programme Hierarchal structure, continuous audit and quality assurance assessment and monitoring support its success Funding is an essential requirement for it s maintenance & success screening is free and widely accepted Newborn screening in the UK videos NHS Choices newborn screening video: AIMS VIDEO\Newborn hearing screening.mp4 Newborn hearing screening in Derbyshire: Newborn Hearing screening in Irelandhttps:// 19

20 References Barker M.J., Hughes E.J. & Wake M NICU-only versus universal screening for newborn hearing loss: Population audit. Journal of Paediatrics and Child Health, 49, E74 E79. Davis A., Bamford J., Wilson I., Ramkalawan T., Forsaw M. et al A critical review of the role of neonatal hearing screening in the detection of congenital hearing impairment. Health Technol Assessment, 1 (10). Moeller M.P. Early intervention and language development in children who are deaf and hard of hearing. Paediatrics, 2000; 106(3): 1-9 Tucci D, Merson MH, Wilson BS. A summary of the literature on global hearing impairment: current status and priorities for action. Otol Neurotol Jan; 31(1): Wood S, Sutton G.J, & Davis A.C. Performance and characteristics of the Newborn Hearing Screening Programme in England: The first seven year. International Journal of Audiology 2015; 54: Yoshinaga-Itano C, Sedey A.L, Coulter B.A, Mehl A.L. Language of early and late-identified children with hearing loss. Paediatrics, 1998; 102: UKNSC_KPI_IndicatorSubmissionProcess_Year14-15_v1_4.pdf NHS public health functions agreement : aring_screening_programme_0.1.pdf Thank you for your attention Any??????? 20