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

4. Marion Downs “Grandmother of Newborn Hearing Screening”

5. 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.
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

6. 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

7. 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

8. 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

9. UNIVERSAL NEWBORN HEARING SCREENING: Prevalence of Infant Hearing Loss in the U.S.A.
Category Births Annually Prevalence Total Hearing Loss
Healthy 3,600,000 3/ ,800
At-risk 400, / ,000
Total 4,000, / ,800
Northern & Hayes, 1994

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

11. UNIVERSAL NEWBORN HEARING SCREENING: Recent Events in the U.S.A.
: 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.

12. 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

13. 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

14. Otoacoustic Emissions (OAEs)

18. NEWBORN HEARING SCREENING: OTOACOUSTIC EMISSIONS
Vohr et al. The Rhode Island Hearing Assessment Program: Experience with statewide hearing screening ( ). Journal of Pediatrics 133: , 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

19. Auditory Brainstem Response (ABR)

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

21. UNIVERSAL NEWBORN HEARING SCREENING WITH AUTOMATED AUDITORY BRAINSTEM RESPONSE (AABR): A MULTI-SITE INVESTIGATION J 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

22. NEWBORN HEARING SCREENING WITH AABR Test Performance and Outcome
SITE WBN ICN Refer % D/C Refer Lost F/U False Pos
Boulder 98% 2% % % 2%
Louisville >99% <1% % % .35%
Memphis >99% N= % % 2.5%
Huntsville 93% 7% % % .05%
Nashville 0% 100% % % 2%
N = 11, % % 0.9%
AAP < 4% < 5% < 2%

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

24. 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

25. 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.)

26. Year 2000 JCIH Position Statement: Protocol for Confirmation of Hearing Loss In 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

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

29. 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

30. FREQUENCY-SPECIFIC ABRs: Tone Burst Test Protocols

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

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

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

36. 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)

37. 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)

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

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

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

42. ASSR (Audera): Significant phase coherence

43. ASSR (Audera): Estimated Audiogram

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

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

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

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

50. Early Intervention for Infant Hearing Impairment: Amplification

51. Management of Infant Hearing Loss: Cochlear Implants

52. Newborn Hearing Screening and Assessment: Conclusions
Universal newborn hearing screening is feasible with automated OAE and ABR technology
Estimation of hearing thresholds (audiogram) is possible in infants with electrophysiologic techniques
ABRs evoked by tone burst stimuli
Auditory steady-state response (ASSR)
Intervention with hearing aids can be implemented early (before 6 months)
Cochlear implant candidacy can be determined during infancy
Early intervention leads to normal language development