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Definitions Importance of detection Methods of assessment Statewide Infant Screening ProgrammeHearing (SWISH) A 3 frequency average hearing level of 40dB or worse in the better ear.* * Australian Working Party Report, G. Birtles et al. July 1998 Because this loss: may lead to significant educational and psychosocial delay can practically be detected in young children in the absence of an internationally agreed standard, is commonly used in research Frequency in Hz 125 250 500 1000 2000 4000 0 20 40 Hearing level in dB 60 80 100 130 shaded region shows the level and frequency of average speech 8000 Frequency in Hz -10 0 10 Hearing Level in dB 20 30 40 50 60 70 80 90 100 125 250 500 1000 2000 4000 8000 Frequency in Hz -10 125 250 500 0 10 20 30 40 50 60 70 80 90 100 1000 2000 4000 8000 Frequency in Hz -10 0 10 20 30 40 50 60 70 80 90 100 125 250 500 1000 2000 4000 8000 Frequency in Hz -10 125 250 500 1000 2000 4000 8000 0 10 20 30 40 50 60 70 80 90 100 Estimated incidence in Australia is 20/10,000 live births Compare this with other currently screened disorders. Disorder Galactosaemia PKU Hypothyroidism Cystic Fibrosis Incidence/10,000 0.3 1.0 2.9 4.0 cystic fibrosis hypothyroidism PKU all others 35 25 1 14 deafness 174 Risk factors for hearing impairment: low birthweight/ preterm positive family history craniofacial anomaly meningitis ototoxic medication use congenital infection BUT, 50% of hearing impaired do not have risk factors. Language of Early and Later identified Children with Hearing Loss Christine Yoshinaga-Itano, Colorado 150 deaf infants 72 identified <6/12 78 identified>6/12 Aiding and early intervention within 2/12 Language & cognition assessed Total language quotient in early compared to late treated groups* 100 90 94 93 91 91 90 84 77 80 MCDI total language 70 quotient 60 73 72 68 <6/12 >6/12 50 40 mild mod mod-severe severe profound level of hearing loss (normal cognition) *Yoshinaga-Itano Discrepancy between cognitive quotient and language quotient by age of identification for children with normal cognition* Mean difference score (CQ-LQ) 30 25 20 <6/12 >6/12 15 10 5 0 receptive expressive total Language scale *Yoshinaga-Itano Mean total language scores at 31-36months by age of identification of hearing loss* earlier identification/ normal cognition later identification/ normal cognition earlier identification/ low cognition later identification/ low cognition 20 30 40 50 60 70 80 90 100 110 mean language quotient * Yoshinaga-Itano Conclusion: from Yoshinaga-Itano There appears to be a critical time at around 6 months of age for identification and remediation of hearing impairment. 2nd C. Yoshinaga-Itano study J.Perinatol Dec2000 By 1997 26/36 birthing U.S. hospitals screening 25 matched pairs of children with hearing impairment born in screening or nonscreening hospitals Assessed language outcome (quotient>80 vs<70) If born in a screening hospital have 2½ x chance of having the higher language score. Other considerations Improved hearing usually results in: Increased academic achievement Decreased costs of education and training Income proportional to language skills and Parent-child relationships improved if parents know about hearing impairment from the outset Distraction techniques Otoacoustic Emissions (OAE) Auditory Brainstem Response Audiometry (ABR) combinations of the above VICS study child health nurses & distraction Marked increase in earlier detection(<12m) BUT still many late (3-4yrs) diagnoses Raised community awareness dearer than newborn screening(UK study) Tests pathway to the level of the cochlea Cochlear hair cells emit sounds spontaneously, but usually tested in response to an input signal Not of great value in the first 48 hours after birth due to ear canal debris Probe containing an earphone and microphone placed in the infant’s ear. Sounds measured in ear canal after click stimulus Quiet room necessary Quick and simple to perform Causes of hearing loss beyond the cochlea are missed Tests auditory pathways to brainstem Responses elicitable by about 34 weeks gestation Can be done immediately after birth Auditory (end of wave V) Pathways in BAER External cochlear nerve (Wave I) (Wave IV-V) (Wave III) (Wave I I) BAER waveform AABR (Automated ABR) is used False positive very low Neonatal high risk screens -sensitivity (100%) -specificity (94-100%) AABR takes longer than OAE AABR screening *Finitzo, Albright & O’Neal, 1998 1) Birth admission screen 2) Follow Up & diagnosis 3) Intervention services Breakdown at any stage jeopardizes the entire effort Expense Repeat tests require extra time & resources from parents Parental anxiety Early discharge & rural births Resources for diagnosis and management Non-compliance with screening Cultural concerns General Public Antenatal education Primary health providers Audiologists Huge role for the family doctor Ongoing role once the diagnostic test has proven hearing impairment Initial intensity of grieving may not be related to degree or type of hearing loss Parents may experience depression, but report that the benefit of early-identification is that they bond with their newborn as a child with a hearing loss and don’t have to change their mind about who their baby is. All babies born in public hospitals in NSW In CSAHS all babies either at RPAH or Canterbury Each area will have dedicated screeners (3 in CSAHS) Each area will have a co-ordinator All hospitals with >400 births per year Prior to discharge at the bedside Clinics on Monday morning at Canterbury and Tuesday at RPA if missed Automated Auditory Brainstem Responses (AABR) Birth admission screen pass refer 2nd screen refer pass pass (false positive screen) Diagnostic testing - Sydney Children’s Hospital or Children’s at Westmead Counselling, aids, intervention services, follow up and support