A1258150338doJDZ

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NEWBORN HEARING SCREENING
EMERGING STANDARD OF CARE
Deborah Hayes, Ph.D. University of Colorado The
Childrens Hospital - Denver
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AIMS OF THIS PRESENTATION

• Review the justification for universal newborn
  hearing screening (UNHS)
• Prevalence of childhood hearing loss
• Effects of hearing loss on child development
• Availability of diagnosis and early intervention
• Screening techniques
• Cost-effectiveness of UNHS
• Summarize current status of UNHS in the United
  States
• Recognize the benefits and challenges of
  large-scale implementation of UNHS

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JUSTIFICATION FOR UNHS

• Significant hearing loss is one of the most
  common major anomaly present at birth
• Undetected hearing loss adversely impacts child
  development
• Early diagnosis intervention are available
• Easy-to-use screening tests with acceptable
  sensitivity and specificity are available
• Screening is cost effective
• AAP Task Force on Newborn and Infant Hearing,
  1999 Pediatrics

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PREVALANCE OF CONGENITAL HEARING LOSS (per
1000)

• Study WBN NICU
• Barsky-Firsker et al 2.1 13.2
• Vohr et al 2.1 9.8
• Mason Herrmann 0.9 5.0
• Finitzo et al 3.1
• Wessex Trial 1.0
• Prieve et al 0.9 8.0
• Colorado UNHS 1.9

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AUDITORY DEVELOPMENT

• Peripheral auditory system is fully functional in
  newborns
• Infants engage in active language-learning in the
  earliest months of life
• By 6 months, normally-hearing infants make
  language-specific phonemic distinctions
• By 30 months, normally-hearing children have
  receptive vocabulary exceeding 300 words

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EFFECTS OF HEARING LOSS

• Hearing loss in children results in significant
  language and speech delay
• On the average, children with hearing loss have a
  20 - 40 point discrepancy between verbal and
  performance IQ
• Reading and academic achievement levels in deaf
  children typically plateau at age equivalent 9-10
  year old level

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DETECTION OF CHILDHOOD HEARING LOSS

• In the absence of UNHS, detection of childhood
  hearing loss depends, in part, on the degree of
  hearing loss
• Degree Average age of detection
• Profound 12 months
• Severe 18 months
• Moderate 36 months
• Mild 60 months

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EARLY vs LATER INTERVENTION

• Infants with hearing loss and normal cognition
  who receive intervention before age 6 months
• achieve language quotients equal to their
  cognitive quotients
• Yoshinaga-Itano, Pediatrics, 1999
• maintain age-appropriate expressive language
  skills through the preschool years
• Yoshinaga-Itano, J Perinatology, 2000

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Yoshinaga-Itano, Pediatrics, 1999
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DIAGNOSIS OF CHILDHOOD HEARING LOSS

• Valid and reliable, non-behavioral measures of
  hearing sensitivity can be applied to infants
  referred from screening
• Non-invasive, low-risk, relatively low-cost
  physiologic measures
• Accurately provide ear-specific estimates of
  hearing sensitivity in the frequency region
  important for speech understanding
• Permit initiation of intervention by 3 months of
  age

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INTERVENTIONS FOR CHILDHOOD HEARING LOSS

• Medical/surgical management when needed
• Amplification, including hearing aids and
  cochlear implants
• Family-centered home-based education
• Infant-centered early intervention
• Family/parent support groups
• Community-based preschool language groups

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PROPERTIES OF APPROPRIATE UNHS SCREENING
TECHNIQUES

• Physiologic technique with well-understood
  response characteristics
• Reliably present in newborn/pre-term infants
• Predictably affected by non-auditory factors
  (e.g., sleep, sedation, maturation)
• Well-correlated with hearing sensitivity
• Rapid, non-invasive, very low risk
• May be applied by non-professional staff (e.g.,
  automated response detection)

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UNHS SCREENING TECHNIQUES

• Auditory brainstem response (ABR or BAER) an
  auditory evoked potential electrical response of
  the nervous system to auditory stimuli extracted
  from the on-going EEG by non-invasive
  signal-averaging techniques.
• Robust response, present in fetuses at about 28
  weeks gestation unaffected by sleep or sedation
  predictable maturational course.

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ABR FROM A NEWBORN

a moderately-loud acoustic signal
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UNHS SCREENING TECHNIQUES

• Otoacoustic emissions (OAEs) acoustic signals
  generated in the cochlea by movement of the outer
  hair cells in response to sound part of the
  normal hearing process detected by a miniature
  microphone sealed in the external ear canal.
• Robust response present in newborns sensitive
  to hearing loss in the middle and inner ear.

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OAE FROM A NEWBORN
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STATUS OF UNHS IN THE U.S.

• 38 states require hospitals/birthing facilities
  to screen the hearing of newborn babies prior to
  discharge approximately 85 of the 4 million
  babies born in the U.S. each year receive hearing
  screening
• Federal legislation provides additional monies
  for research, implementation, and tracking in
  UNHS programs

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STATE MANDATES FOR UNHS
Colorado
Mandated screening
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2003 SCREENING RESULTSCOLORADO

• Births 69,781
• Screened 67,586 (96.8)
• Referred for follow-up 2,350 (3.5)
• Follow-up screenings reported 2,203 (93.7 of
  referred infants)
• Number infants identified 129
• 1.9 per 1000

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2003 SCREENING RESULTSCOLORADO

• Average age of identification 3 months
• Number risk factors 49 (37.9)
• Number bilateral loss 68 (52.7)
• Number cochlear loss 73 (56.6)

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UNHS PRACTICAL ISSUES

• Are there alternatives to UNHS?
• What infrastructure is needed to support
  effective UNHS?
• What are the costs of UNHS?
• What are the long-term benefits of UNHS?
• How might UNHS be implemented?

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ALTERNATIVES TO UNHS

• Risk-registry targeted screening
• Identify infants at risk due to pre- or
  peri-natal factors (in utero infection perinatal
  trauma care in a NICU craniofacial anomaly)
  screen only those infants
• Higher yield based on prevalence
• Identifies 45 60 infants with hearing loss
• - Fails to identify infants with recessive
  genetic hearing loss
• - Costly to detect all infants with risk factors
• - Not effective in lowering mean age of ID

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INFRASTRUCTURE NEED TO SUPPORT UNHS

• Country, state, or health-district system of care
  
• Screening
• Follow-up and tracking
• Rapid diagnosis audiological and medical
• Intervention services medical/surgical, hearing
  aids, auditory stimulation and language
  development
• Family education, counseling, and support

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COSTS OF UNHS vs.SPECIAL EDUCATION

• Cost per screen (UK or USA)
• 17.00 to 40.00 USD
• Cost per identification (UK or USA)
• 3,000 to 17,000 USD
• Cost of education of deaf children (USA)
• Regular education 3,500/year
• Special education 10,000/year
• School for the Deaf 42,000/year

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LONG-TERM BENEFITS OF UNHS

• Opportunity to improve child health and to
  prevent childhood disability
• Opportunity to collect relevant epidemiologic
  data to support prevention efforts
• Opportunity for parent education, support, and
  decision-making before disability is manifest
• Opportunity to develop medical, audiological, and
  educational services to benefit all citizens

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OPPORTUNITIES FOR IMPLEMENTING UNHS

• Hospital-based screening of well-babies and NICU
  infants
• Immunization clinics at 1 - 3 months of age
• Home healthcare visits by health workers for
  parent support and education
• Public school-based screening of newborns and
  young infants

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SUMMARY

• UNHS can be justified
• UNHS is feasible
• Effective early identification programs reflect
  mature systems of care to insure screening,
  follow-up and tracking, rapid diagnosis, and
  prompt intervention
• UNHS benefits not only the child and family, but
  also the community
• Multiple sites can serve as opportunities for
  screening