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Title: Newborn Hearing Screening AAP Teleconference November 12, 2003


1
Newborn Hearing ScreeningAAP TeleconferenceNovem
ber 12, 2003
2
Review of Practice Implications from
Teleconference Part 1
  • Moeller

3
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.

4
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)

5
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.

6
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.

7
Universal Newborn Hearing ScreeningGuidelines
for Pediatric Providers
  • Mehl

8
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

9
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

10
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

11
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.

12
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

13
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

14
Early intervention practices Why and what?
  • Moeller

15
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.

16
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)

17
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
18
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).

19
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.

20
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).

21
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

22
Early Vocabulary Learning
Mayne, et al, 2000
23
Family-Centered Early Intervention Services
Services Coordination
Relationship-Focused Home Visits
Family Support Experiences
Ongoing Multi-Disciplinary Evaluation Process
24
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

25
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

26
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

27
Medical Home Task Force (RI)
N 95
28
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

29
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

30
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

31
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)
32
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
33
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?)

34
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?

35
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

36
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)

37
Understanding Parental Issues Related to Newborn
Screening
  • Mehl

38
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

39
Financial Issues for the Family
  • Moeller

40
Financial Issues

41
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

42
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.

43
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.

44
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

45
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

46
Family Advocacy and Empowerment
  • Mehl

47
Family Advocacy, Empowerment
  • IDEA (Individuals with Disabilities Education
    Act), Part C
  • Medical insurance
  • Parent support groups
  • Hearing aids
  • Communications options
  • Cochlear implants

48
State and National Resources
  • Moeller

49
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

50
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

51
www.babyhearing.org
52
www.infanthearing.org
www.infanthearing.org
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