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NEWBORN INFANT SCREENING AND ASSESSMENT: Emerging Technologies and Protocols

NEWBORN INFANT SCREENING AND ASSESSMENT: Emerging Technologies and Protocols. James W. Hall III, Ph.D. Clinical Professor and Chair Department of Communicative Disorders College of Health Professions University of Florida Gainesville, Florida 32610-0174 Jhall@hp.ufl.edu.

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NEWBORN INFANT SCREENING AND ASSESSMENT: Emerging Technologies and Protocols

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  1. NEWBORN INFANT SCREENING AND ASSESSMENT: Emerging Technologies and Protocols James W. Hall III, Ph.D. Clinical Professor and Chair Department of Communicative Disorders College of Health Professions University of Florida Gainesville, Florida 32610-0174 Jhall@hp.ufl.edu

  2. Marion Downs“Grandmother of Newborn Hearing Screening”

  3. Year 2000 JCIH Position Statement: Principles and Guidelines for Early Hearing Detection and Intervention Programs • JCIH = Joint Committee on Infant Hearing • Published in: • Audiology Today(Special Issue): August 2000, pp. 6-27 • American Journal of Audiology 9: 9-29, 2000. • www.audiology.org • Member organizations: • American Academy of Audiology & ASHA • American Academy of Otolaryngology-Head & Neck Surgery • American Academy of Pediatrics • Council on Education of the Deaf • Directors of Speech and Hearing Programs in State and Welfare Agencies

  4. JCIH Risk Indicators for Hearing Impairment:Birth to 29 Days • Illness or condition requiring admission of > 48 hours to an NICU. • Stigmata or other findings associated with a syndrome known to include a sensorineural or conductive hearing loss. • Family history of permanent childhood sensorineural hearing loss • Craniofacial anomalies, including those with morphological abnormalities of pinna and ear canal • In-utero infection such as cytomegalovirus, herpes, toxoplasmosis, or rubella

  5. JCIH Risk Indicators for Hearing Impairment:29 Days to 2 Years (1) • Parental or caregiver concern regarding hearing, speech, language, and or developmental delay • Family history of permanent childhood hearing loss • Stigmata or other findings associated with a syndrome known to include a sensorineural or conductive hearing loss. • Postnatal infections associated with sensorineural hearing loss including bacterial meningitis • In-utero infection such as cytomegalovirus, herpes, toxoplasmosis, rubella, syphilis

  6. JCIH Risk Indicators for Hearing Impairment:29 Days to 2 Years (2) • Neonatal indicators, specifically hyperbilirubinemia at a serum level requiring exchange transfusion, persistent pulmonary hypertension of the newborn associated with mechanical ventilation, and conditions requiring extracorporeal membrane oxygenation • Syndromes associated with progressive hearing loss, such as neurofibromatosis, osteopetrosis, and Usher’s syndrome • Neurodegenerative disorders, such as Hunter syndrome, or sensory motor neuropathies, such as Friedreich’s ataxia • Head trauma • Recurrent or persistent otitis media with effusion for at least 3 months

  7. UNIVERSAL NEWBORN HEARING SCREENING: Prevalence of Infant Hearing Loss in the U.S.A. Category Births Annually Prevalence Total Hearing Loss Healthy3,600,000 3/1000 10,800 At-risk 400,000 30/1000 12,000 Total 4,000,000 5.7/1000 22,800 Northern & Hayes, 1994

  8. Universal Newborn Hearing Screening:Turning Point in the United States of America

  9. UNIVERSAL NEWBORN HEARING SCREENING:Recent Events in the U.S.A. • 1975-1985: Hearing screening of at risk infants with ABR • 1993: National Institutes of Health Consensus Conference on Early Identification of Hearing Impairment in Children • 1994: Joint Committee on Infant Hearing (JCIH) Position Statement (recommending ABR and OAE techniques) • 1998: Yoshinaga-Itano et al. Language of early and later identified children with hearing loss. Pediatrics 102. • 1999: American Academy of Pediatrics Task Force on Newborn and Infant Hearing Screening: Diagnosis and intervention. Pediatrics 103. • 2000: JCIH Position Statement. Principles and Guidelines for Early Hearing Detection & Intervention Programs.

  10. UNIVERSAL NEWBORN HEARING SCREENING:Converging Trends in 1990s • Automated OAE and ABR devices manufactured for newborn hearing screening • Evidence of low “refer” rates (< 4%) • Evidence of low false-positive rates (< 2%) • Documentation of benefits of early intervention (before 6 months) • Successful implementation of UNHS in selected states

  11. EARLY IDENTIFICATION OF AND INTERVENTION FOR HEARING IMPAIRMENT IN CHILDREN • "Hearing loss of 30dB HL and greater in the frequency region important for speech recognition will interfere with the normal development of speech and language. • "Techniques used to assess hearing of infants must be capable of detecting hearing loss of this degree in infants by age three months and younger. • Of the various approaches to newborn hearing assessment currently available, two physiologic measures...auditory brainstem response (ABR) and otoacoustic emissions (OAE)...show good promise for achieving this goal"Joint Committee on Infant Hearing 1994 Position Statement

  12. Otoacoustic Emissions (OAEs)

  13. NEWBORN HEARING SCREENING:OTOACOUSTIC EMISSIONS • Vohr et al. The Rhode Island Hearing Assessment Program: Experience with statewide hearing screening (1993-1996). Journal of Pediatrics 133: 353-357, 1998 • 53,121 babies underwent screening (NICU =5130) • average initial failure rate = 10% • failure rate for rescreens at 2 to 6 weeks = 14.7% • over failure (refer) rate = 1.2% • 111 infants identified with permanent hearing loss • average age of intervention (amplification) = 5.7 months

  14. Auditory Brainstem Response(ABR)

  15. PEDIATRIC AUDIOLOGY: Auditory brainstem response (ABR) 0.5 uV V I III stimulus click @ 35 dB 8 ms electrodes Auditory Evoked Response System (computer) 1974 - present

  16. UNIVERSAL NEWBORN HEARING SCREENING WITH AUTOMATED AUDITORY BRAINSTEM RESPONSE (AABR): A MULTI-SITE INVESTIGATIONJ Perinatology 20 ((8): S128, December 2000. James W. Hall III, Ph.D. Dan Stewart, M.D. University of Florida Kosair Children’s Hospital Gainesville, Florida, U.S.A. Louisville, Kentucky Albert Mehl, M.D. Mark Carroll, M.S. Boulder Community Hospital E.N.T. Associates Boulder, Colorado Huntsville, Alabama Vicki Thomson, M.A. James Hamlett, M.D. Boulder Community Hospital Baptist Memorial Hospital East Boulder, Colorado Memphis, Tennessee

  17. NEWBORN HEARING SCREENING WITH AABRTest Performance and Outcome SITE WBN ICN Refer % D/C Refer Lost F/U False Pos Boulder 98% 2% 2% 16% 2% Louisville >99% <1% 1% 45% .35% Memphis >99% N=1 3% 13% 2.5% Huntsville 93% 7% 1% 21% .05% Nashville 0% 100% 6% 32% 2% N = 11,711 2% 28% 0.9% AAP < 4% < 5% < 2%

  18. Combination Device for Newborn Hearing Screening and Diagnosis with OAEs and ABR: AudioScreener by Grason Stadler

  19. Rationale for Combined OAE/ABR Screening • In ear calibration of signal intensity (OAE and ABR) • Lower refer (< 2%) and false-positive rates (< 0. 2%) • Minimal parental anxiety • Fewer diagnostic follow-ups with lower costs • Less hearing impaired infants lost to follow-up • Differentiation of conductive vs. sensory vs. neural auditory dysfunction • Quicker and more appropriate management • Identification of auditory neuropathy • Earlier identification of hearing impairment

  20. Early Identification and Intervention for Hearing Impairment in Children: Important Steps Screening with AABR or OAE before hospital discharge Pass? Fail? Secondary screening within 3 months (optional) Parent Info Diagnostic audiometry to define hearing loss Hearing loss? Progressive factor? Hearing aid fitting and habilitation (by 6 mos.)

  21. Year 2000 JCIH Position Statement: Protocol for Confirmation of Hearing LossIn Infants and Toddlers (0 to 6 months) • Child and family history • Otoacoustic emissions • ABR during initial evaluation to confirm type, degree & configuration of hearing loss (ASSR?) • Acoustic immittance measures (including acoustic reflexes) • Behavioral response audiometry (if feasible) • Visual reinforcement audiometry or • Conditioned play audiometry • Speech detection and recognition • Parental report of auditory & visual behaviors • Screening of infant’s communication milestones

  22. Estimation of Frequency-Specific Auditory Thresholds with Tone Burst ABRs: Initial Data Points for DSL

  23. Auditory brainstem response (ABR): 0.5 uV V I III Tone-burst signals 8 ms electrodes Auditory Evoked Response System (computer) ABR mature by 18 months

  24. FREQUENCY-SPECIFIC ABRs:Tone Burst Test Protocols

  25. Click versus Tone Burst ABRs I III V click I III V 50 1000 Hz V 500 Hz 0 15 msec Stimulus Analysis Time

  26. FREQUENCY-SPECIFIC AUDITORY BRAINSTEM RESPONSE (ABR): Relation to Audiogram(Oates & Stapells, 1998)

  27. ELECTROPHYSIOLOGIC ASSESSMENT OF AUDITORY FUNCTION IN INFANTS CASE REPORT: ABR Estimated Audiogram

  28. Auditory Steady-State Response (ASSR): General Principles • An electrophysiologic response, similar to ABR. • Instrumentation includes: • Insert earphones • Surface electrodes • Averaging computer • Stimuli are pure tones (frequency specific, steady state signals) activating cochlea and CNS • ASSR is generated by rapid modulation of “carrier” pure tone amplitude (AM) or frequency (FM). • Signal intensity can be as high as 120 dB HL • ASSR phase or frequency is detected automatically (vs. visual detection)

  29. Auditory Steady State Response (ASSR):Clinical Devices • GSI VIASYS • Audera • Descendant of Melbourne Australia system Field (Rickards, Gary Rance, Barbara Cone-Wesson, et al) • Bio-Logic Systems Inc. • MASTER • Descendent of Canadian system (Terry Picton et al)

  30. ASSR:2000 Hz tone modulated at rate of 100 Hz

  31. ASSR:Response imbedded within EEG 0.7 0.6 0.5 0.4 0.3 0.2 0.1 0.0 0 10 20 30 40 50 60 70 80 90 100 ms

  32. ASSR: Graphic display in vector plot of EEG samples at modulation frequency Vector length (c’) = magnitude of activity Vector angle (a’) = phase lag between stimulus MF and EEG at MF B c’ b’ a’ C A

  33. ASSR (Audera):Significant phase coherence

  34. ASSR (Audera):Estimated Audiogram

  35. Pure Tones vs. ASSR: Case 1 (child) 8K 6K 8K .50 1K 2K 3K 4K 6K .50 1K 2K 3K 4K dBHL 20 40 60 80 100 Frequency in Hz Frequency in Hz PT ABR ASSR

  36. ABR vs. ASSR: Case 4 (infant) 8K 6K 8K .50 1K 2K 3K 4K 6K .50 1K 2K 3K 4K dBHL 20 40 60 80 100 Frequency in Hz Frequency in Hz PT ABR ASSR

  37. Limitation of Tone Burst ABR in Severe-to-Profound Hearing Loss 8K 6K 8K .50 1K 2K 3K 4K 6K .50 1K 2K 3K 4K dB HL 20 40 60 80 100 No ABR > 80 dB HL No ASSR > 120 dB HL AC BC Frequency in Hz Frequency in Hz

  38. Estimation of Frequency-Specific Auditory Thresholds with Auditory Electrophysiology: DSL Hearing Aid Fitting

  39. Early Intervention for Infant Hearing Impairment: Amplification

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