Should%20Newborn%20Hearing%20Screening%20be%20the%20Standard%20of%20Care%20in%20the%20United%20States

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Newborn Hearing Screening Implications for Part C
IDEA Programs
Karl White, Ph.D. National Center for Hearing
Assessment and Management Utah State
University www.infanthearing.org
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3
Percentage of Newborns Screened for Hearing in
the United States (Dec 2001)
Percentage of Births Screened
.
90
51 - 90
21 - 50
1 - 20
5
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
6
National Universal Newborn Hearing Screening
Programs

• Austria
• United Kingdom
• Poland
• Flemish Belgium
• Singapore
• Canada (Ontario)

7
Early Hearing Detection and Intevention EHDI
8
Why are EHDI Programs Expanding?
Improved Screening
Techniques/Equipment
9
Early Hearing Detection and Interveniton (EHDI)
Programs
10
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

11
Why are EHDI Programs Expanding?
Improved Screening
Techniques/Equipment
Acceptance by
Increased Number of
Policy Makers
Successful Programs
Public
Awareness/Demand
12
Why is Early Identification of Hearing Loss so
Important?

• Hearing loss occurs more frequently than any
  other birth defect.

13
Rate Per 1,000 of Permanent Childhood Hearing
Loss in UNHS Programs

• Sample Prevalence
• Site Size Per 1000
• Rhode Island (3/93 - 6/94) 16,395 1.71
• Colorado (1/92 - 12/96) 41,976 2.56
• New York (1/95 - 12/97) 69,761 1.95
• Texas (1/94 - 6/97) 52,508 2.15
• Hawaii (1/96 - 12/96) 9,605 4.15
• New Jersey (1/93 - 12/95) 15,749 3.30

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Incidence per 10,000 of Congenital
Defects/Diseases
15
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.

16
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.
17
Effects of Unilateral Hearing Loss
Normal Hearing
Unilateral Hearing Loss
Math
Keller Bundy (1980)
(n 26 age 12 yrs)
Language
Math
Peterson (1981)
(n 48 age 7.5 yrs)
Language
Social
Bess Thorpe (1984)
(n 50 age 10 yrs)
Math
Blair, Peterson Viehweg (1985)
Language
(n 16 age 7.5 yrs)
Math
Culbertson Gilbert (1986)
Language
(n 50 age 10 yrs)
Social
Average Results
0th
10th
20th
30th
40th
50th
60th
Math 30th percentile
Percentile Rank
Language 25th percentile
Social 32nd percentile
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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
, 685-694.
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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.

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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.
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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
0
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
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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 lt6 mos (n 25)
5
Identified gt6 mos (n 104)
4
3
Language Age (yrs)
2
1
0
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
, moeller_at_boystown.org
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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.
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Is the Glass Half Empty or Half Full?

• 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

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Status of Early Hearing Detection and
Intervention (EHDI) in the United States
Medical Home
Data Management and Tracking
Program Evaluation and Quality Assurance
Family Support!!
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Status of EHDI Programs in the USUniversal
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

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Typical UNHS Screening Protocols (example for
1,000 newborns)
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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

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Purposes of an EHDI Data System
Research
Program Improvement and Quality Assurance
Screening
Diagnosis
Intervention
Medical, Audiological and
Educational
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Rate Per 1000 of Permanent Childhood Hearing Loss
in UNHS Programs

• Sample Prevalence of Refers
• Site Size Per 1000 with
  Diagnosis
• Rhode Island (3/93 - 6/94) 16,395 1.71
  42
• Colorado (1/92 - 12/96) 41,976 2.56
  48
• New York (1/96 - 12/96) 27,938 1.65
  67
• Utah (7/93 - 12/94) 4,012 2.99
  73
• Hawaii (1/96 - 12/96) 9,605 4.15
  98

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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

(1/93 - 12/96)

(99)

(10)

(85)

(1.3)

Hawaii
10,584
9,605

1,204

991

121
(1/96 - 12/96)

(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)


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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

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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

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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

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Efficiency of Early Hearing Detection and
Intervention in a Statewide Evaluation

• 1999 2000 2001(6
  mos.)
• (n43,547)
  (n46,771) (n23,307)
• Inpatient Refer Rates (state average)
  85.2 85.5 87.5
• 10 most effective hospitals
  92.8 93.4 93.7
• 10 least effective hospitals
  70.7 63.4 74.4
• Outpatient completion (state average)
  70.1 67.1 68.3
• 10 most effective hospitals
  94.5 95.9 94.7
• 10 least effective hospitals
  45.3 52.9 58.08
• Reported Completion of Diagnostic 133 of 357
  165 of 380 41 of 110
• Evaluations (state average)
  37.3 43.4 40
• of babies who complete Diagnostic 33 of 133
  65 of 165 12 of 41
• Eval have permanent hearing loss
  24.8 39.4 29.3
• Number of babies still in process 224
  215 69
• only 3 months worth of data

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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

37
State Coordinators Ratings of Obstacles to
Effective EHDI Programs


Serious or Extremely

Serious
Obstacle Shortage of qualified pediatric
audiologists 49 Physicians dont know enough
about newborn hearing screening, diagnosis, and
intervention 41 Unwillingness of
third-party payers to reimburse for hearing
screening 28
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Status of EHDI Programs in the USAudiological
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

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Confirmation of Permanent Hearing Loss
35
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)
0
10
20
30
40
50
60
70
Average Age in Months
40
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

41
Status of EHDI Programs in the USEarly
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

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Who is Eligible for Part C Services?

• Child has a profound, permanent sensorineural
  hearing loss in both ears (PTAgt100 dB)
• Child has a profound, permanent sensorineural
  hearing loss in one ear (PTAgt100dB), but normal
  hearing in the other ear
•  
• Child has a moderate, permanent sensorineural
  hearing loss in both ears (PTA55dB)
•  
• Child has a mild, permanent sensorineural
  hearing in both ears (PTA35dB)
•  
• Child has a mild, fluctuating conductive hearing
  loss (PTA35dB) in both ears due to otitis media
  (ear infections)
•  

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Hawai'i EHDI Progress
Age of Identification and Intervention
60
Identification
Intervention
50
40
Age in Months
30
20
10
0
pre
1992
1993
1994
1995
1996
1997
1998
Year
Data from HawaiI Zero to Three Project
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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

45
What Is a Medical Home?

• A primary care physician provides care which is
• Accessible
• Family-centered
• Comprehensive
• Continuous
• Coordinated
• Compassionate
• Culturally effective

46
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
47

• 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

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What Causes 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
Mitochondrial
1
49
Common Forms of Syndromic Hearing Loss
Syndrome Main Features (in addition to hearing loss) Main Features (in addition to hearing loss)
Alport Kidney problems
Branchio-oto-renal Neck cysts and kidney problems Neck cysts and kidney problems
Jervell and Lange-Nielsen Heart problems Heart problems
Neurofibromatosis Type 2 Nerve tumors near the ear Nerve tumors near the ear
Pendred Thyroid enlargement Thyroid enlargement
Stickler Unusual facial features, eye problems, arthritis Unusual facial features, eye problems, arthritis
Usher Progressive blindness Progressive blindness
Waardenburg Skin pigment changes Skin pigment changes
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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

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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

52
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.

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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

54
Emotions of Families with a Deaf orHard 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

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Communication Choices

• American Sign Language
• Total Communication
• Auditory Verbal
• Auditory-Oral
• Cued Speech

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Wanted
Received
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Parents 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
babys hearing 11
Confused about the results of screening
tests 10 Glad hearing
screening is done at this hospital 91 Confident
the hearing tests were correct 91
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 88

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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 babys
hearing 11 24
Confused about the results of screening
tests 10 24 Glad hearing
screening is done at this hospital 91 70 Conf
ident the hearing tests were correct 91 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 88 56

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EHDI Materials Available from State
Programs (n54) General Screening
Brochure 39
states What To Do If Your Baby Refers
35 states What To Do If
Your Baby has a Hearing Loss 41
states Guidelines for Audiologic Diagnostic
Evaluations 30 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 34 states

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Efforts by the Federal Government toPromote
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

61
National EHDI Technical Assistance System

• EHDI Network members located in each of ten
  geographic regions

62
National EHDI Assistance Network
Region VIII (91 currently born in UNHS
hospitals) Terry Foust
Region II (16 currently born in UNHS
hospitals) Beth Prieve
Region V (26 currently born in UNHS
hospitals) Karen Munoz
I
Puerto Rico Virgin Islands
Region I (38 currently born in UNHS
hospitals) Antonia MaxonB
VIII
II
X
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
63
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

64
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

65
National EHDI Technical Assistance System
(continued)

• EHDI Network members located in each of the MCHB
  regions
• Information dissemination and training

66
Support for Program Implementation

• Implementation Guide
• Booklets for AAP and March of Dimes
• Materials posted at www.infanthearing.org
• Video tape for parents
• Evaluation instruments and procedures

67
Sound Ideas Newsletter

• Topical articles, suggestions for program
  improvement
• Upcoming events
• Available online or mailed

68
National EHDI Technical Assistance System
(continued)

• EHDI Network members located in each of the MCHB
  regions
• Information dissemination and training
• Web site (www.infanthearing.org)

69
www.infanthearing.org
70
www.babyhearing.org
71
National EHDI Technical Assistance System
(continued)

• EHDI Network members located in each of the MCHB
  regions
• Information dissemination and training
• Web site (www.infanthearing.org)
• Collaboration with other groups and agencies

72
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
• NECTAS, Early Head Start, 0-3, Family Voices,
  NCCC, AMCHP, AHEC, March of Dimes, MCH Health
  Policy Center

73
Collaboration with AAP

• AAP News article
• Assisted with booklets for physicians and parents
  
• Collaborated on implementation of recently funded
  EHDI Initiative
• Chapter Champions
• Speakers Kit
• Bulletin Board
• Physician Guidelines
• Analysis of legislation
• National survey of physicians

74
Take Home Messages

• Deceptively simplethe devil is in the details
• EHDI is more than screening
• Medical Home is where the action is
• Thoughtful, ongoing, self appraisal
• Youre not alone

75
I am a great believer in luck, and I find that
the harder I work, the more I have of it.
---Thomas Jefferson
76
(No Transcript)