1. Universal Newborn Hearing Screening
What It Is and How It Happens
Karl White, Ph.D.
National Center for Hearing Assessment and Management
Utah State University

2. Early Hearing Detection and Interveniton (EHDI) Programs

5. Percentage of Newborns Screened for Hearing3
Percentage of Newborns Screened for Hearing
in the United States
(Dec 2001)
Percentage of Births
Screened .
90%+ % %
1 - 20%

6. States with Legislative Mandates
Related to Universal Newborn Hearing Screening
Status of UNHS Legislative Mandates
States with mandates
No mandate, but statewide
programs
No mandate

7. National Universal Newborn Hearing Screening Programs
Austria
United Kingdom
Poland
Flemish Belgium
Singapore
Canada (Ontario)

8. Why is Implementation of Newborn Hearing Screening Accelerating?
Improved Screening
Techniques/Equipment

9. Acceptance By Policy Makers
National Institutes of Health
American Academy of Pediatrics
Maternal and Child Health Bureau
Centers for Disease Control & Prevention
Joint Committee on Infant Hearing
American Academy of Audiology
American Speech-Language-Hearing Association
National Association of the Deaf

10. Why is Implementation of Newborn Hearing Screening Accelerating?
Improved Screening
Techniques/Equipment
Acceptance by
Increased Number of
Policy Makers
Successful Programs
Public
Awareness/Demand

11. Why is Early Identification of Hearing Loss so Important?
Hearing loss occurs more frequently than any other birth defect.

12. Rate Per 1,000 of Permanent Childhood Hearing Loss in UNHS Programs
Sample Prevalence
Site Size Per 1000
Rhode Island (3/93 - 6/94) 16,
Colorado (1/ /96) 41,
New York (1/ /97) 69,
Texas (1/94 - 6/97) ,
Hawaii (1/ /96) 9,
New Jersey (1/ /95) 15,

13. Incidence per 10,000 of Congenital Defects/Diseases

14. Why is Early Identification of Hearing Loss so Important?
Hearing occurs more frequently than any other birth defect.
Undetected hearing loss has serious negative consequences.

15. Reading Comprehension Scores of Hearing and Deaf Students
Grade Equivalents
Age in Years
Schildroth, A. N., & Karchmer, M. A. (1986). Deaf children in America, San Diego: College Hill Press.

16. Effects of Unilateral Hearing Loss
Normal Hearing
Unilateral Hearing Loss
Keller & Bundy (1980)
Math
(n = 26; age = 12 yrs)
Language
Peterson (1981)
Math
(n = 48; age = 7.5 yrs)
Language
Bess & Thorpe (1984)
Social
(n = 50; age = 10 yrs)
Math
Blair, Peterson & Viehweg (1985)
(n = 16; age = 7.5 yrs)
Language
Math
Culbertson & Gilbert (1986)
(n = 50; age = 10 yrs)
Language
Social
Average Results
0th
10th
20th
30th
40th
50th
60th
Math = 30th percentile
Percentile Rank
Language = 25th percentile
Social = 32nd percentile

17. Effects of Mild Fluctuating Conductive Hearing Loss
Teele, et al., 1990
194 children followed prospectively from 0-7 years.
Days child had otitis media between 0-3 years assessed during normal visits to physician.
Data on intellectual ability, school achievement, and language competency individually
measured at 7 years by "blind" diagnosticians.
Results for children with less than 30 days OME were compared to children with more than
130 days adjusted for confounding variables.
Effect Size for
Outcome Measure Less vs. More OME
WISC-R Full Scale
.62
Metropolitan Achievement Test
Math
.48
Reading
.37
Goldman Fristoe Articulation
.43
Teele, D.W., Klein, J.O., Chase, C., Menyuk, P., Rosner, B.A., and the Greater Boston Otitis media Study Group (1990).
Otitis media in infancy and intellectual ability, school achievement, speech, and language at age 7 years.
The Journal
of Infectious Diseases ,
162 ,

18. Why is Early Identification of Hearing Loss so Important?
Hearing loss occurs more frequently than any other birth defect.
Undetected hearing loss has serious negative consequences.
There are dramatic benefits associated with early identification of hearing loss.

19. Yoshinaga-Itano, et al., 1996
Compared language abilities of hearing-impaired children identified
before 6 months of age (n = 46) with similar children identified after 6
months of age (n = 63).
All children had bilateral hearing loss ranging from mild to profound,
and normally-hearing parents.
Language abilities measured by parent report using the Minnesota
Child Development Inventory (expressive and comprehension scales)
and the MacArthur Communicative Developmental Inventories
(vocabulary).
Cross-sectional assessment with children categorized in 4 different
age groups.
Yoshinaga-Itano, C., Sedey, A., Apuzzo, M., Carey, A., Day, D., & Coulter, D. (July 1996).
The effect of early
identification on the development of deaf and hard-of-hearing infants and toddlers
. Paper presented at the
Joint Committee on Infant Hearing Meeting, Austin, TX.

20. Expressive Language Scores for Hearing Impaired
Children Identified Before and After 6 Months of Age 35 30 25
Language Age in Months 20 15 10
Identified BEFORE 6 Months 5
Identified AFTER 6 Months
13-18 mos
19-24 mos
25-30 mos
31-36 mos
(n = 15/8)
(n = 12/16)
(n = 11/20)
(n = 8/19)
Chronological Age in Months

21. Boys Town National Research Hospital Study of Earlier vs. Later
129 deaf and hard-of-hearing children assessed 2x each year.
Assessments done by trained diagnostician as normal part of early intervention program. 6
Identified <6 mos (n = 25) 5
Identified >6 mos (n = 104) 4
Language Age (yrs) 3 2 1
0.8
1.2
1.8
2.2
2.8
3.2
3.8
4.2
4.8
Age (yrs)
Moeller, M.P. (1997).
Personal communication ,

22. Good work, but I think we might need just a little more detail right
Implementing Effective EHDI Programs
out
Then a
miracle
occurs
Start
Good work,
but I think we might
need just a little
more detail right
here.

23. Is the Glass Half Empty or Half Full?
More than 2.5 million babies are screened every year prior to discharge
Less than 30 hospitals with UNHS in 1993; compared with almost 2500 today
37 states have passed legislation related to newborn hearing screening
Or half empty?
1,500 hospitals are not yet screening for hearing loss
Almost 1.5 million babies are NOT screened every year prior to discharge
Existing legislation is of variable quality
Follow-up rates are often alarmingly low
Some hospitals have unacceptably high referral rates

24. Status of Early Hearing Detection and Intervention (EHDI) in the United States
Diagnosis before 3 months
Screening before 1 month
Intervention before 6 months
Medical Home
Data Management and Tracking
Program Evaluation and Quality Assurance
Family Support!!

25. Status of EHDI Programs in the US: Universal Newborn Hearing Screening
With over half of all babies are screened prior to discharge, has newborn hearing screening become the standard of care?
There are hundreds of excellent programs regardless of the type of equipment or protocol used
Many programs are still struggling with high refer rates and poor follow-up

26. Typical UNHS Screening Protocols (example for 1,000 newborns)
Hearing Loss=3
Normal Hearing=7
Inpatient
Screening
Pass=920
Fail=80
Outpatient
n=80
Diagnosis
n=10
Pass=70
Fail=10
2 Stage
OAE
Hearing Loss=3
Normal Hearing=37
Diagnosis
n=40
Inpatient
Screening
Fail=40
Pass=960
1 Stage
AABR
Diagnosis
n=20
Inpatient
Screening
Pass=980
Fail=20
Hearing Loss=3
Normal Hearing=17
OAE / AABR
1 Stage

27. Status of EHDI Programs in the United States
Universal Newborn Hearing Screening
Effective Tracking and Follow-up as a part of the Public Health System

28. Purposes of an EHDI Data System
Research
Program Improvement
and Quality Assurance
Screening
Diagnosis
Intervention
Medical, Audiological and
Educational

29. Rate Per 1000 of Permanent Childhood Hearing Loss in UNHS Programs
Sample Prevalence % of Refers
Site Size Per with Diagnosis
Rhode Island (3/93 - 6/94) 16, %
Colorado (1/ /96) 41, %
New York (1/ /96) 27, %
Utah (7/ /94) , %
Hawaii (1/ /96) 9, %

30. Tracking "Refers" is a Major Challenge
(continued)
Initial Rescreen
Births Screened Refer Rescreen Refer
Rhode Island
53,121
52,659
5,397
4,575
677
(1/ /96)
(99%)
(10%)
(85%)
(1.3%)
Hawaii
10,584
9,605
1,204
991
121
(1/ /96)
(91%)
(12%)
(82%)
(1.3%)
New York
28,951
27,938
1,953
1,040
245
(1/96-12/96)
(96.5%)
(7%)
(53%)
(0.8%)

31. Options for Developing an EHDI Patient/Data Management System
Develop your own
Modify an existing system, for example
electronic birth certificate, or
“heelstick” data management system
Purchase an existing system

32. Can EHDI Data Management be Combined with Heelstick?
Both do initial screening of babies in the nursery prior to hospital discharge
Both do outpatient screening for many babies
Poor follow-up is biggest challenge for hearing screening
Heelstick programs extremely successful with follow-up
Infrastructure for Heelstick follow-up already exists
EHDI information management looks deceptively simple at first glance.

33. Issues to be Resolved Before Combining EHDI with Heelstick Follow-up Systems
Recording results of EHDI on heelstick form is only the beginning
Timing and procedures of data collection/entry are quite different for EHDI
Electronic transfer of data from screening equipment to data form
Availability of staff with expertise in EHDI issues to do follow-up
Hospital staff need timely access to data
Costs of modifying data entry/ reporting systems and duplicate data entry
EHDI information management looks deceptively simple at first glance.

34. Efficiency of Early Hearing Detection and Intervention in a Statewide Evaluation
(6 mos.)
(n=43,547) (n=46,771) (n=23,307)
Inpatient Refer Rates (state average) % 85.5% 87.5%
10 most effective hospitals % 93.4% 93.7%
10 least effective hospitals % 63.4% 74.4%
Outpatient completion (state average) % 67.1% 68.3%
10 most effective hospitals % 95.9% 94.7%
10 least effective hospitals % 52.9% %
Reported Completion of Diagnostic 133 of of of 110*
Evaluations (state average) % 43.4% 40%
% of babies who complete Diagnostic of of of 41*
Eval & have permanent hearing loss % 39.4% 29.3%
Number of babies still “in process”
*only 3 months worth of data

35. Status of EHDI Programs in the United States
Universal Newborn Hearing Screening
Effective Tracking and Follow-up as a part of the Public Health System
Appropriate and Timely Diagnosis of the Hearing Loss

36. State Coordinator’s Ratings of Obstacles to Effective EHDI Programs
Serious or Extremely
Serious Obstacle
Shortage of qualified pediatric audiologists 49%
Physicians don’t know enough about newborn
hearing screening, diagnosis, and intervention %
Unwillingness of third-party payers
to reimburse for hearing screening %

37. Status of EHDI Programs in the US: Audiological Diagnosis
Equipment and techniques for diagnosis of hearing loss in infants continues to improve
Severe shortages in experienced pediatric audiologists delays confirmation of hearing loss
State coordinators estimate only 56.1% “receive diagnostic evaluations by 3 months of age

38. Confirmation of Permanent Hearing Loss35
Coplan (1987) 19
Eissman et al. (1987) 30
Gustason (1987) 30
Meadow-Orlans (1987) 24
Yoshinago-Itano (1995) 25
Stein et al. (1990) 31
Mace et al. (1991) 56
O'Neil (1996) 3
Johnson et al. (1997)* 3
Vohr et al. (1998)* 10 20 30 40 50 60 70
Average Age in Months

39. Status of EHDI Programs in the United States
Universal Newborn Hearing Screening
Effective Tracking and Follow-up as a part of the Public Health System
Appropriate and Timely Diagnosis of the Hearing Loss
Prompt Enrollment in Appropriate Early Intervention

40. Status of EHDI Programs in the US: Early Intervention
Current system designed to serve infants with bilateral severe/profound losses---but, majority of those identified have mild, moderate, and unilateral losses
Part C of IDEA is severely under utilized
State Coordinators estimate:
Only 53% of infants with hearing loss are enrolled in EI programs before 6 months of age
Only 31% of states have adequate range of choices for EI programs

41. Who is Eligible for Part C Services?
Child has a profound, permanent sensorineural hearing loss in both ears (PTA>100 dB)
Child has a profound, permanent sensorineural hearing loss in one ear (PTA>100dB), but normal hearing in the other ear
Child has a moderate, permanent sensorineural hearing loss in both ears (PTA=55dB)
Child has a mild, permanent sensorineural hearing in both ears (PTA=35dB)
Child has a mild, fluctuating conductive hearing loss (PTA=35dB) in both ears due to otitis media (ear infections)

42. Hawai'i EHDI Progress Age of Identification and Intervention 60 50 40
Age in Months 30 20 10
pre
1992
1993
1994
1995
1996
1997
1998
Year
Data from Hawai’I Zero to Three Project

43. Status of EHDI Programs in the United States
Universal Newborn Hearing Screening
Effective Tracking and Follow-up as a part of the Public Health System
Appropriate and Timely Diagnosis of the Hearing Loss
Prompt Enrollment in Appropriate Early Intervention
A Medical Home for all Newborns

44. What Is a Medical Home?
A primary care physician provides care which is:
Accessible
Family-centered
Comprehensive
Continuous
Coordinated
Compassionate
Culturally effective

45. EHDI and the Medical Home
Birthing Hospital
Audiology
Parent Groups
Mental Health
Primary Provider
Child/Family
ENT
3rd Party Payers
Deaf Community
Early Intervention Programs
Genetics
Services for Hearing Loss

46. Types of Hearing Loss
Sensorineural versus Conductive versus Mixed
Congenital versus Acquired (prelingual or post lingual)
Progressive versus non-progressive
Syndromic versus non-syndromic
Familial versus sporadic

47. What Causes Hearing Loss? Congenital Hearing Loss
Environmental
CMV meningitis
rubella prematurity
head trauma asphyxiation
ototoxicity hyperbilirubin
other infections
Syndromic
Alport Norrie
Pendred Usher
Waardenburg
Branchio-oto-renal
Jervell and Lange-Nielsen
~50%
Congenital Hearing Loss
~30%
~50%
Non-syndromic
Autosomal dominant
21%
Genetic
Autosomal recessive
77%
X-Linked
~70%
~1%
~1%
Mitochondrial

48. Common Forms of Syndromic Hearing Loss
Syndrome
Main Features (in addition to hearing loss)
Alport
Kidney problems
Branchio-oto-renal
Neck cysts and kidney problems
Jervell and Lange-Nielsen
Heart problems
Neurofibromatosis Type 2
Nerve tumors near the ear
Pendred
Thyroid enlargement
Stickler
Unusual facial features, eye problems, arthritis
Usher
Progressive blindness
Waardenburg
Skin pigment changes

49. Benefits of Genetic Testing for Hearing Loss
Determine the chances of hearing loss in subsequent children
Avoid unecessary (and often costly) tests such as electroretinograms, temporal bone imaging, and electrocardigrams
Anticipate potential health problems
Monitoring for myopia and early retinal detachment for Stickler syndrome
Renal examinations can identify kidney problems in BOR
Vitamin A therapy may be beneficial in slowing retinal degeneration in child with Usher syndrome
Treatment of children with Jervell and Lange-Nielsen syndrome can minimize cardiac complications
Dispel misinformation and offer emotional support by allaying parental guilt

50. Connexin 26
A protein responsible for intracellular communication (transfer of ions between the hair cells in the cochlea and their support cells)
Responsible for 20-30% of all congenital hearing loss
Several different mutations
35delG is found in 2-3% of all Caucasians of European descent
167delT is found in 5% of Ashkenazi Jewish population
Usually recessive, occasionally dominant
Almost always results in hearing loss that is:
Congenital
Severe-profound
Non-progressive
Non-syndromic

51. Susceptibility to Aminoglycoside Ototoxicity
mitochondrial mutation A1555G in the rRNA gene in combination with exposure to aminoglycoside antibiotics results in rapid onset of hearing loss
prevalent in Chinese and other oriental ethnic groups but has also been found in Caucasians, Greeks, etc.

52. Status of EHDI Programs in the United States
Universal Newborn Hearing Screening
Effective Tracking and Follow-up as a part of the Public Health System
Appropriate and Timely Diagnosis of the Hearing Loss
Prompt Enrollment in Appropriate Early Intervention
A Medical Home for all Newborns
Culturally Competent Family Support

53. Emotions of Families with a Deaf or Hard of Hearing Baby
(grief) Reactions to Unexpected Diagnosis
(pressure) Urgency of Communication Decisions Search
(confusion) Search for Experienced Professionals
(isolation) Availability of Services and Support

54. Communication Choices
American Sign Language
Total Communication
Auditory Verbal
Auditory-Oral
Cued Speech

55. Information Wanted vs. Received by Parents
at Hearing Loss Confirmation
Martin, George, O'Neal, & Daly (1987); *Sweetow & Barrager (1980)
Degree of loss
Auditory system
Amplification
Educational options
Speech/Lang dev
Etiology
Home activities
*Written Information
*Financial Support
*Emotional Support
*Parent Contacts
*Referral Sources 20 40 60 80
100
Wanted
Received

56. Parent’s Attitudes About Newborn Hearing Screening
(Results of a Statewide Evaluation)
After all hearing tests were completed, how did you feel?
Strongly Agree
or Agree
Worried about my baby’s hearing %
Confused about the results of screening tests %
Glad hearing screening is done at this hospital 91%
Confident the hearing tests were correct %
Frustrated by how long it took to get results 13%
Happy with the professional way screening was done 86%
Confident about what I needed to do next %

57. After all hearing tests were completed, how did you feel?
If the analysis is limited to those whose babies did not pass the inpatient or outpatient screen
After all hearing tests were completed, how did you feel?
Strongly Agree
or Agree
total group subgroup
Worried about my baby’s hearing % 24%
Confused about the results of screening tests % 24%
Glad hearing screening is done at this hospital 91% 70%
Confident the hearing tests were correct % 70%
Frustrated by how long it took to get results 13% 28%
Happy with the professional way screening was done 86% 76%
Confident about what I needed to do next % 56%

58. EHDI Materials Available from “State” Programs
(n=54)
General Screening Brochure states
What To Do If Your Baby Refers states
What To Do If Your Baby has a Hearing Loss 41 states
Guidelines for Audiologic Diagnostic Evaluations states
List of Qualified Pediatric Audiologists 39 states
Brochure about Genetics of Hearing Loss 7 states
Fair or Excellent Availability of Materials in
other Languages states

59. Efforts by the Federal Government to Promote Early Identification of Hearing Loss
Federal funding for research and program development
NIH Consensus Development Conference in 1993
Consortium for Universal Newborn Hearing Screening funded in 1993
Marion Downs National Center for Infant Hearing Established in 1996
National EHDI Technical Assistance System Established in 2000
NIH and Dept of Educ Projects at Boys Town and University of North Carolina

60. National EHDI Technical Assistance System
EHDI Network members located in each of ten geographic regions

61. National EHDI Assistance Network
Region VIII
(91% currently born
in UNHS hospitals)
Terry Foust
Region V
(26% currently born
in UNHS hospitals)
Karen Munoz
Region II
(16% currently born
in UNHS hospitals)
Beth Prieve I
Puerto Rico
Virgin Islands
Region I
(38% currently born
in UNHS hospitals)
Antonia MaxonB
VIII X II V
Region X
(21% currently born
in UNHS hospitals)
Curt Whitcomb
III
VII
Region III
(49% currently born
in UNHS hospitals)
Sean Kastetter IX IV VI -
Region IX
(23% currently born
in UNHS hospitals)
Randi Winston
Yusnita Weirather
Guam, American Samoa,
Marshall Islands, Palau,
No. Mariana Islands,
Fed. Micronesia
Region IV
(46% currently born
in UNHS hospitals)
Faye McCollister
Region VI
(38% currently born in UNHS hospitals)
Karen Ditty
Patti Martin
Region VII
(33% currently born
in UNHS hospitals)
Les Schmeltz
= indicates the locations of MCHB Regional Offices

62. Examples of Network Activities
State-wide EHDI meetings
Individualized TA with state EHDI programs
Telephone Conference calls with State EDHI Coordinators
Assist with development of state plans and grant applications
Regional workshops on Diagnostic ABR
6 weeks of on-line preparation
2 day face-to-face workshop
3 month follow-up practicum

63. National EDHI Meetings
Next meeting: February 24-26, 2002 (Atlanta)
Speakers, panels, and round tables
State displays
Product exhibits (commercial and non-profit)
Networking opportunities

64. National EHDI Technical Assistance System (continued)
EHDI Network members located in each of the MCHB regions
Information dissemination and training

65. Support for Program Implementation
Implementation Guide
Booklets for AAP and March of Dimes
Materials posted at
Video tape for parents
Evaluation instruments and procedures

66. Sound Ideas Newsletter
Topical articles, suggestions for program improvement
Upcoming events
Available online or mailed

67. National EHDI Technical Assistance System (continued)
EHDI Network members located in each of the MCHB regions
Information dissemination and training
Web site (

70. National EHDI Technical Assistance System (continued)
EHDI Network members located in each of the MCHB regions
Information dissemination and training
Web site (
Collaboration with other groups and agencies

71. National EHDI Technical Assistance System (continued)
Collaboration with Other Groups and Agencies
Groups actively promoting and assisting with EHDI activities
AG Bell, NCHH, ASHA, AAA, JCIH, AAP, SKI-HI, ASDC, Boys Town, DSHPSHWA
Relevant groups whose main focus has been elsewhere
NEC*TAS, Early Head Start, 0-3, Family Voices, NCCC, AMCHP, AHEC, March of Dimes, MCH Health Policy Center

72. Collaboration with AAP
AAP News article
Assisted with booklets for physicians and parents
Collaborated on implementation of recently funded EHDI Initiative
Chapter Champions
Speaker’s Kit
Bulletin Board
Physician Guidelines
Analysis of legislation
National survey of physicians

73. “Take Home” Messages Deceptively simple—the devil is in the details
EHDI is more than screening
Medical Home is where the action is
Thoughtful, ongoing, self appraisal
You’re not alone

74. “I am a great believer in luck, and I find that the harder I work, the more I have of it.”
---Thomas Jefferson