wDefinitions wImportance of detection wMethods of assessment wStatewide Infant Screening Programme- Hearing (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: wmay lead to significant educational and psychosocial delay wcan practically be detected in young children win the absence of an internationally agreed standard, is commonly used in research

shaded region shows the level and frequency of average speech Frequency in Hz Hearing level in dB

Frequency in Hz Hearing Level in dB

Frequency in Hz Hearing Level in dB

Frequency in Hz Hearing Level in dB

Frequency in Hz Hearing Level in dB

Estimated incidence in Australia is 20/10,000 live births Compare this with other currently screened disorders. DisorderIncidence/10,000 Galactosaemia0.3 PKU1.0 Hypothyroidism2.9 Cystic Fibrosis4.0

wcystic fibrosis 35 whypothyroidism 25 wPKU 1 wall others 14 wdeafness 174

wRisk 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 Aiding and early intervention within 2/12 Language & cognition assessed

Total language quotient in early compared to late treated groups * level of hearing loss (normal cognition) MCDI total language quotient * Yoshinaga-Itano

Discrepancy between cognitive quotient and language quotient by age of identification for children with normal cognition * * 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 * 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 wBy /36 birthing U.S. hospitals screening w25 matched pairs of children with hearing impairment born in screening or nonscreening hospitals wAssessed language outcome (quotient>80 vs<70) wIf born in a screening hospital have 2½ x chance of having the higher language score.

Other considerations Improved hearing usually results in: wIncreased academic achievement wDecreased costs of education and training wIncome proportional to language skills and wParent-child relationships improved if parents know about hearing impairment from the outset

wDistraction techniques wOtoacoustic Emissions (OAE) wAuditory Brainstem Response Audiometry (ABR) w combinations of the above

wVICS study child health nurses & distraction wMarked increase in earlier detection(<12m) wBUT still many late (3-4yrs) diagnoses wRaised community awareness wdearer than newborn screening(UK study)

wTests pathway to the level of the cochlea wCochlear hair cells emit sounds spontaneously, but usually tested in response to an input signal wNot of great value in the first 48 hours after birth due to ear canal debris

wProbe containing an earphone and microphone placed in the infant’s ear. wSounds measured in ear canal after click stimulus wQuiet room necessary wQuick and simple to perform wCauses of hearing loss beyond the cochlea are missed

wTests auditory pathways to brainstem wResponses elicitable by about 34 weeks gestation wCan be done immediately after birth

(Wave IV-V) Auditory Pathways in BAER External cochlear nerve (Wave I) (Wave I I) (Wave III) (end of wave V)

BAER waveform

wAABR (Automated ABR) is used wFalse positive very low wNeonatal high risk screens -sensitivity (100%) -specificity (94-100%) wAABR takes longer than OAE

AABR screening

*Finitzo, Albright & O’Neal, )Birth admission screen 2) Follow Up & diagnosis 3) Intervention services Breakdown at any stage jeopardizes the entire effort

wExpense wRepeat tests require extra time & resources from parents wParental anxiety wEarly discharge & rural births wResources for diagnosis and management wNon-compliance with screening wCultural concerns

wGeneral Public wAntenatal education wPrimary health providers wAudiologists

wHuge role for the family doctor wOngoing role once the diagnostic test has proven hearing impairment wInitial intensity of grieving may not be related to degree or type of hearing loss

wParents may experience depression, but wreport 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 passrefer 2nd screen pass refer Diagnostic testing - Sydney Children’s Hospital or Children’s at Westmead pass (false positive screen) Counselling, aids, intervention services, follow up and support