Newborn Hearing Screening AAP Teleconference November 12, 2003

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Newborn Hearing ScreeningAAP TeleconferenceNovem
ber 12, 2003
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Review of Practice Implications from
Teleconference Part 1

• Moeller

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Review of Practice Implications

• 2-3 per 1000 newborns will have permanent
  childhood hearing loss (1/1000 WBN 10/1000 NICU)
• When a newborn does not pass screening Accurate
  diagnosis is essential to minimize parental
  stress and ensure timely treatment where
  indicated.
• Delayed identification of permanent childhood
  hearing loss, even of mild/moderate degrees,
  interferes with speech and language development.

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Review of Practice Implications

• Newborn hearing screening and diagnostic
  technologies allow for confirmation of hearing
  loss type and degree by 3 months of age. This
  supports the goal of intervention prior to 6
  months of age. Timely and appropriate
  interventions have lasting effects on outcomes.
• Appropriate medical referrals may include ENT,
  CT of temporal bones, Genetics, Ophthalmology,
  lab tests (CMV, EKG)

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Review of Practice Implications

• Several risk indicators are associated with late
  onset or progressive hearing loss in early
  childhood.
• This underscores the need for ongoing
  surveillance at well baby visits.
• In addition to JCIH risk factors, parent or
  caregiver expression of concern regarding
  hearing, speech, language or development should
  be considered a key risk factor.

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Review of Practice Implications

• Hearing aids should be fit within one month of
  confirmation, preferably lt 4 months and not gt 6
  months.
• Goal is to provide audibility of ambient speech
  through computerized prescriptive fitting
  methods.
• Audiology centers need appropriate equipment and
  experience to fit hearing aids in young children.
• Children with severe to profound, bilateral SNHL
  may be candidates for cochlear implants after 12
  to 18 months of age.

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Universal Newborn Hearing ScreeningGuidelines
for Pediatric Providers

• Mehl

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Universal Newborn Hearing ScreeningGuidelines
for Pediatric Providers

• Developed in 2002 by the AAP Committee for
  Improving the Effectiveness of Newborn Hearing
  Screening, Diagnosis, and Intervention
• Available on the web at www.medicalhomeinfo.org/s
  creening/hearing.html

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Birth Hospital-based Screening

• Physiologic testing (OAE, AABR, ABR)
• Re-screen before discharge whenever possible
• Hearing screen pass is not lifetime guarantee
• Identify risk factors for those who pass
• Commit to re-screening, any failure or incomplete
• Outpatient screening if missed screen, home birth
• Documentation if parents refuse screening
• Unilateral failure must be rescreened

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Before One Month of Age

• Outpatient re-screening for all failed, missed,
  or incomplete screenings
• Early re-screening allows earlier diagnosis
• Early re-screening is technically easier
• Early re-screening minimizes parental anxiety
• Unilateral failure must proceed to full audiology
  evaluation

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Before Three Months of AgePediatric Audiology
Evaluation

• Audiology evaluation by a professional with
  experience evaluating newborns
• Physiologic testing is required rather than
  behavioral response audiometry
• Earlier audiology evaluation is technically
  easier
• Earlier audiology evaluation is more likely to
  avoid requiring sedation
• Audiologists should report all results (both pass
  and fail) to state EDHI program.

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Before Three Months of AgeIf Hearing Loss is
Confirmed

• Report result to state EDHI program
• Early intervention through Part C
• Continuing medical evaluation including pediatric
  otologist evaluation and clearance for hearing
  aid fitting
• Hearing aid fitting by pediatric audiologist with
  experience amplifying hearing in babies.
• Parental information and choices concerning
  amplification and communication options

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Before Six Months of Age

• Continue early intervention
• Reinforce compliance with daytime amplification
• Genetics evaluation for every infant with
  confirmed congenital hearing loss
• Ophthalmology evaluation for every infant with
  confirmed hearing loss, repeat annually
• Other evaluations as indicated (developmental
  pediatrics, neurology, cardiology, nephrology)
• Continuing audiology management, transition to
  include behavioral response audiometry

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Early intervention practices Why and what?

• Moeller

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Why Intervene Early?
Early experiences have a decisive impact on the
architecture of the brain

• In the first year of life, neurons in the
  auditory brainstem are developing
• Billions of major neural connections are being
  formed(number of synapses increases 20 fold to
  1,000 trillion).
• Newborn brain is in a subcortical state Areas of
  cortex responsible for language are well
  developed by 12 months of age.

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Why Intervene Early?

• Animal studies suggest use it or lose it
  phenomenon
• When sensory input to the auditory system is
  interrupted, especially early in development, the
  morphology functional properties of neurons in
  the central auditory system can break down.
• These deleterious effects can be ameliorated by
  reintroduction of stimulation, but sensitive
  periods may exist for intervention (e.g.,
  Pre-implant stimulation is a predictor of
  post-implant outcomes)

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Foundations of Language

• In the first year of life-NH infants
  discriminate fine grained differences in speech
  sounds
• Werker Tess (1984) found that 6-8 month olds
  learning English discriminated Hindi contrasts,
  but 10-12 month olds could not

First words are just the tip of the iceberg
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Perceptual Foundations

• The loss of perceptual sensitivity to nonnative
  speech around 9 months reflects a shift to
  language-specific speech processes.
• Learning about the organization and
  characteristics of sounds in the ambient language
  helps infants discover how to segment continuous
  speech into word units. (Jusczyk, 1997).

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Perceptual Foundations

• 90 of English words have a strong/weak stress
  pattern Jusczyk, et al., (1993) found that 9 mo
  olds, but not 6 mo olds attended preferentially
  to this dominant pattern.
• Infants at 9 mos can use information about the
  sequencing of sounds within and between words to
  locate boundaries (Jusczyk, 2002).
• Early word-segmentation skills are important for
  eventually attaching meaning organizing sound
  patterns of words in memory.

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Perceptual Foundations

• In addition to ability to discriminate sound
  patterns, infants must selectively attend to
  sound patterns around them to become sensitive to
  how they are distributed.
• Auditory deprivation during early neural
  development (in utero and after birth) may
  interfere with sensitivity to native language
  organization, segmentation and word learning
  (Houston, 2000).

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Foundations in Production

• Canonical babble (well-formed syllables that
  sound speech-like) appears between 6-10 months
  in NH infants and infants with Down Syndrome
  (Oller Eilers, 1988).
• Many infants with significant SNHL are delayed in
  babble onset, variety of sounds, amount and
  complexity of babble.
• Such delays may influence word learning

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Early Vocabulary Learning
Mayne, et al, 2000
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Family-Centered Early Intervention Services
Services Coordination
Relationship-Focused Home Visits
Family Support Experiences
Ongoing Multi-Disciplinary Evaluation Process
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Relationship Focus
When joining families of newborns or young
infants, the specialist

• Keeps in perspective the adjustments the family
  system is making to the birth of a baby
• Observes, identifies strengths supports
  responsive parenting, attachment bonding

• Helps family fit stimulation into everyday
  routines in natural ways
• Supports the family in putting hearing loss in
  perspective and enjoying the infant

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Family-Centered Early Intervention

• Characteristics of Quality Program
• Gains comprehensive understanding of
  infant/family needs to develop IFSP
• Supports family in use of amplification and
  communication strategies Respects parental
  decision-making authority
• Guides family in stimulating infants language,
  auditory, speech communicative development
• Helps family understand and cope with HL

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Individual Family Services Plan

• Assessment of infants current achievements
• A list of family strengths
• Major goals or outcomes expected from program
• Specific services needed to achieve outcomes
• Timelines for achieving goals
• Team members, service coordinator
• Transition Steps

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Medical Home Task Force (RI)
N 95
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Family Centered Early Intervention

• Balanced partnerships are formed both parties
  contribute expertise
• Early interventionist provides support and
  coaching, rather than child-focused therapy
• Emphasis on parent-infant relationship
  developing parental confidence independence in
  implementing strategies
• Flexible responsive programming with ongoing
  evaluation of outcomes
• Services honor the culture values of the family

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Early Development Network Services Coordination

• Helps families -Find link to services to
  meet developmental, educational, financial,
  health care, child care, respite care other
  needs-Coordinate care of multiple providers
  know what to expect from community
  agencies-Become coordinators of services for
  their own children in the future
• This process avoids duplication of services
  develops resources where needed

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Premises

• Families should have access to information on the
  full range of options AND the knowledge that
  there is no single approach that is best for all
  infants who are deaf or hard of hearing
• Family involvement (especially quality of
  communication with the infant) early
  intervention positive outcomes

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Verbal Reasoning at Age 5
Age Enroll Family Involvement
High Verbal Reasoning lt13 months 4.4
Average Reasoning 21 months 3.6
Low Reasoning 27 months 2.6
N 80 (Moeller, 2000)
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Parent Wish List for EI

• We wish for choices unbiased information about
  options respect choices families make and their
  decision making authority
• We wish for information avoid absolutes in
  opinionsprovide a variety of resources and
  contacts
• Parent-Professional partnerships trust
  established through respect for parental goals,
  values and culture

www.handsandvoices.org
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Roles of the Medical Home

• Knowledgeable about the referral process to Early
  Intervention
• Assists family in linking to early intervention
  and family-support services
• Offers partnerships with families to develop a
  plan of health and habilitative care serves as
  member of IFSP team
• Provides ongoing surveillance (Is EI program
  effectively meeting needs?)

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Questions to Ask Family

• What have you been told about your babys hearing
  loss? How does that match your observations?
• How is early intervention working for your
  family? What are you learning about?
• Who do you go to for support?
• What changes are you seeing in your infant in
  response to what you are trying in EI?
• Do you have concerns? What would you like to
  know at this time? What should be our next plan
  of action?

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Case Example

• Although she had no risk factors for HL, Baby A
  did not pass a two stage screening in the
  birthing hospital Pediatrician referred to
  Audiology, ENT Genetics
• Diagnostic ABR at 1 month revealed moderate,
  bilateral sensorineural hearing loss
• Infant fitted for binaural hearing aids at 3
  months enrolled in auditory/oral education
  program
• By three and one-half years of age, A had speech
  and language skills in the high average range
• A was successfully mainstreamed in regular
  education setting

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Consequences of Late Identification for Families

• Guilt and frustration over missed diagnosis
• Pressure to catch up may influence interactions
• May be behavioral consequences related to childs
  communicative delays
• Increased time demands (extra appointments)
• Decreased confidence, independence in
  implementation of IFSP goals (Calderon, et al.,
  1998)

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Understanding Parental Issues Related to Newborn
Screening

• Mehl

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Understanding Parental Issues

• Anxiety after abnormal screen
• Need for information and prompt evaluation
• Range of reactions to diagnosis of hearing loss
• Emotional support, concurrent stresses of newborn
  in family
• Maternal feelings of guilt
• Cultural sensitivity and barriers
• Financial barriers

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Financial Issues for the Family

• Moeller

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Financial Issues

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Financial Issues

• Binaural (2) hearing aids -digital
  3600-5200-digitally-programmable
  2200-4800-conventional 1400 - 3200
• Most private insurance companies do NOT cover
  hearing aids
• In some cases, Medicaid or state agencies will
  assist with hearing aid coverage

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Financial Issues

• Some fraternal and charitable organizations
  provide financial assistance in obtaining new or
  reconditioned hearing aids
• Some Audiology clinics offer loaner hearing aids
  for a period of trial before purchase of hearing
  aids
• FDA recommends at least a 30 day trial period for
  new hearing aids
• Schools often provide FM systems, but there are
  few resources for home use.

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Costs of Cochlear Implants

• The combined costs for pre-implant evaluations,
  the implant device, surgery and post-surgical
  fittings ranges from 40,000 to 100,000
  depending on the CI center and the childs
  specific needs.
• CI teams typically assist families in
  investigating insurance coverage options.
• Companies often place limits on post-implant
  therapy coverage.

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Additional Costs

• Frequent replacement of earmolds
• In most states, EI costs are provided through
  Part Chowever, access can be an issue in some
  areas and situations
• Availability of skilled interpreters (foreign
  language sign language)
• Diagnostic services allocation of time

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Financial Issues Support from the Physician

• Ask families about these issues and provide
  support
• Provide contacts www. agbell.org
  www.listen-up .org for information on funding for
  devices
• Advocacy and education with insurance carriers
• Contact Services Coordinator for help

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Family Advocacy and Empowerment

• Mehl

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Family Advocacy, Empowerment

• IDEA (Individuals with Disabilities Education
  Act), Part C
• Medical insurance
• Parent support groups
• Hearing aids
• Communications options
• Cochlear implants

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State and National Resources

• Moeller

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Resources

• Early InterventionParent-to-Parent
• Physician support

• State Part C Coordinator State EHDI
  Coordinator public schools www nectac.org
• www handsandvoices.org www beginningssvcs.comwww
  babyhearing.org
• www aap.org
• www infanthearing.org
• AAP Pedialink Module (forthcoming)
• www nidcd.gov

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Resources

• Office of Ed Grant Marjorie D. Jung
  860-679-1500
• AlsoPediatric Resource Guide to Infant and
  Childhood Hearing Loss Jill Ellis 510-527-5544

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www.babyhearing.org
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www.infanthearing.org
www.infanthearing.org