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Newborn Hearing Screening AAP Teleconference November 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 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 Screening Guidelines for Pediatric Providers Mehl
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Universal Newborn Hearing Screening Guidelines 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
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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 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.
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Before Three Months of Age If 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? 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
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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 Relationship- Focused Home Visits Family Support Experiences Ongoing Multi- Disciplinary Evaluation Process Services Coordination
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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:
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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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I ndividual F amily S ervices P lan 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 EnrollFamily Involvement High Verbal Reasoning <13 months4.4 Average Reasoning 21 months3.6 Low Reasoning27 months2.6 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 opinions…provide 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 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
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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 Intervention Parent-to-Parent Physician support State Part C Coordinator; State EHDI Coordinator; public schools; www nectac.org www handsandvoices.org www beginningssvcs.com www 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 Also…Pediatric 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
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