Newborn Hearing ScreeningAAP TeleconferenceNovember 12, 2003

Review of Practice Implications from Teleconference Part 1

Moeller

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.

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)

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.

Review of Practice Implications• Hearing aids should be fit within one month of

confirmation, preferably < 4 months and not > 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.

Universal Newborn Hearing ScreeningGuidelines for Pediatric Providers

Mehl

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/screening/hearing.html

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

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

Before Three Months of Age:Pediatric 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.

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

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

Early intervention practices: Why and what?

Moeller

Why Intervene Early?

• 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.

Early experiences have a decisive impact on the architecture of the brain

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)

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 notFirst words are just the tip of

the iceberg

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).

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.

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).

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

Early Vocabulary Learning

050

100150200250300350400

12 mos

14 mos

16 mos

18 mos

24 mos

Age

Nu

mb

er o

f W

ord

s

NH Boys

NH Girls

HH/ DeafInfants

90th% ileHH

Mayne, et al, 2000

Family-Centered Early Intervention Services

Relationship-Focused Home Visits

Family Support Experiences

Ongoing Multi-Disciplinary Evaluation Process

Services Coordination

Relationship Focus

• 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

When joining families of newborns or young infants, the specialist:

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 infant’s language, auditory, speech & communicative development

• Helps family understand and cope with HL

Individual Family Services Plan

• Assessment of infant’s 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

Medical Home Task Force (RI)Practice Participates in IFSP Process

0

10

20

30

40

Rating

Num

ber o

f Res

pons

es

N = 95

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

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

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

             

   

                                                      

Verbal Reasoning at Age 5

Age Enroll Family Involvement

High Verbal Reasoning

<13 months 4.4

Average Reasoning

21 months 3.6

Low Reasoning 27 months 2.6

N = 80 (Moeller, 2000)

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 opinions…provide a variety of resources and contacts

• Parent-Professional partnerships – trust established through respect for parental goals, values and culture

www.handsandvoices.org

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?)

Questions to Ask Family• What have you been told about your baby’s 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?

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

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 child’s

communicative delays• Increased time demands (extra appointments)• Decreased confidence, independence in

implementation of IFSP goals (Calderon, et al., 1998)

Understanding Parental Issues Related to Newborn Screening

Mehl

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

Financial Issues for the Family

Moeller

Financial Issues

• •

                                                                 

                                                          

                                                      

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

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.

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 child’s specific needs.

• CI teams typically assist families in investigating insurance coverage options.

• Companies often place limits on post-implant therapy coverage.

Additional Costs:

• Frequent replacement of earmolds

• In most states, EI costs are provided through Part C…however, access can be an issue in some areas and situations

• Availability of skilled interpreters (foreign language; sign language)

• Diagnostic services; allocation of time

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

Family Advocacy and Empowerment

Mehl

Family Advocacy, Empowerment

• IDEA (Individuals with Disabilities Education Act), Part C

• Medical insurance

• Parent support groups

• Hearing aids

• Communications options

• Cochlear implants

State and National Resources

Moeller

Resources:

• Early Intervention

Parent-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

Resources

• Office of Ed Grant – Marjorie D. Jung – 860-679-1500

• Also…Pediatric Resource Guide to Infant and Childhood Hearing Loss – Jill Ellis – 510-527-5544

www.babyhearing.org

www.infanthearing.org

www.infanthearing.org