Download Should Newborn Hearing Screening be the Standard of Care in the

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
www.infanthearing.org
Early Hearing Detection and
Interveniton (EHDI) Programs
Hospitals with Universal Newborn Hearing
Screening Programs
4000
Number of Hospitals
3500
3000
2500
2000
1500
1000
500
0
1991 1992 1993 1994 1995 1996 1997 1998 1999 2000 2001
Year
Percentage of Newborns Screened for Hearing
Prior to Hosptial Discharge
100.0%
90.0%
80.0%
100.0%
70.0%
90.0%
60.0%
80.0%
70.0%
50.0%
60.0%
40.0%
50.0%
30.0%
40.0%
30.0%
20.0%
20.0%
10.0%
10.0%
Jan-02
Jan-01
Jan-00
Jan-99
Jan-98
Jan-97
Jan-96
Jan-95
Jan-94
Jan-93
0.0%
Percentage of Newborns Screened for Hearing
in the United States
(Dec 2001)
.
Percentage of Births
Screened
90%+
51 - 90%
21 - 50%
1 - 20%
3
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
National Universal Newborn
Hearing Screening Programs
•
•
•
•
•
•
Austria
United Kingdom
Poland
Flemish Belgium
Singapore
Canada (Ontario)
Why is Implementation of Newborn
Hearing Screening Accelerating?
Improved Screening
Techniques/Equipment
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
Why is Implementation of Newborn
Hearing Screening Accelerating?
Improved Screening
Techniques/Equipment
Acceptance by
Policy Makers
Increased Number of
Successful Programs
Public
Awareness/Demand
Why is Early Identification of
Hearing Loss so Important?
• Hearing loss occurs more frequently
than any other birth defect.
Rate Per 1,000 of Permanent
Childhood Hearing Loss in UNHS
Programs
Site
Sample
Size
Prevalence
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
Incidence per 10,000 of Congenital
Defects/Diseases
40
30
30
20
5
6
10
11
12
1
2
0
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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.
Grade Equivalents
Reading Comprehension Scores
of Hearing and Deaf Students
10.0
9.0
8.0
7.0
6.0
5.0
4.0
3.0
2.0
1.0
Deaf
Hearing
8
9
10
11
12
13
14
15
16
17
18
Age in Years
Schildroth, A. N., & Karchmer, M. A. (1986). Deaf children in America, San Diego: College Hill Press.
Effects of Unilateral Hearing Loss
Normal Hearing
Keller & Bundy (1980)
(n = 26; age = 12 yrs)
Math
Language
Peterson (1981)
(n = 48; age = 7.5 yrs)
Math
Language
Bess & Thorpe (1984)
(n = 50; age = 10 yrs)
Social
Blair, Peterson & Viehweg (1985)
(n = 16; age = 7.5 yrs)
Math
Language
Culbertson & Gilbert (1986)
(n = 50; age = 10 yrs)
Math
Language
Social
Average Results
Math = 30th percentile
Language = 25th percentile
Social = 32nd percentile
0th
10th
20th
Unilateral Hearing Loss
30th
40th
Percentile Rank
50th
60th
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.
Outcome Measure
WISC-R Full Scale
Metropolitan Achievement Test
Math
Reading
Goldman Fristoe Articulation
Effect Size for
Less vs. More OME
.62
.48
.37
.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.
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.
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.
Language Age in Months
Expressive Language Scores for Hearing Impaired
Children Identified Before and After 6 Months of Age
35
30
25
20
15
10
Identified BEFORE 6 Months
Identified AFTER 6 Months
5
0
13-18 mos
(n = 15/8)
19-24 mos
(n = 12/16)
25-30 mos
(n = 11/20)
Chronological Age in Months
31-36 mos
(n = 8/19)
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.
Language Age (yrs)
6
Identified <6 mos (n = 25)
Identified >6 mos (n = 104)
5
4
3
2
1
0
0.8
1.2
1.8
2.2
2.8
3.2
3.8
Age (yrs)
Moeller, M.P. (1997).Personal communication
, [email protected]
4.2
4.8
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.
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
Status of Early Hearing Detection and
Intervention (EHDI) in the United States
Diagnosis
before 3 months
Screening
Intervention
before 1 month
before 6 months
Medical Home
Data Management and Tracking
Program Evaluation and Quality Assurance
Family Support!!
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
Typical UNHS Screening Protocols
(example for 1,000 newborns)
Inpatient
Screening
1 Stage
OAE / AABR
Inpatient
Screening
Inpatient
Screening
Fail=80
Pass=920
Fail=40
Outpatient
Screening
n=80
Fail=10
Pass=70
Diagnosis
n=10
Diagnosis
n=40
Pass=960
Fail=20
Pass=980
Diagnosis
n=20
Hearing Loss=3
Normal Hearing=7
Hearing Loss=3
Normal Hearing=37
Hearing Loss=3
Normal Hearing=17
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
Purposes of an EHDI Data System
Research
Program Improvement
and Quality Assurance
Screening
Diagnosis
Intervention
Medical, Audiological and
Educational
Rate Per 1000 of Permanent Childhood Hearing Loss in
UNHS Programs
Site
Sample
Size
Prevalence
Per 1000
% of Refers
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%
Tracking "Refers" is a Major Challenge
(continued)
Initial
Refer
Rescreen
Rescreen
Refer
Births
Screened
Rhode Island
(1/93 - 12/96)
53,121
52,659
(99%)
5,397
(10%)
4,575
(85%)
677
(1.3%)
Hawaii
(1/96 - 12/96)
10,584
9,605
(91%)
1,204
(12%)
991
(82%)
121
(1.3%)
New York
(1/96-12/96)
28,951
27,938
(96.5%)
1,953
(7%)
1,040
(53%)
245
(0.8%)
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
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
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
Efficiency of Early Hearing Detection and Intervention
in a Statewide Evaluation
1999
(n=43,547)
2000
(n=46,771)
2001(6 mos.)
(n=23,307)
85.2%
92.8%
70.7%
85.5%
93.4%
63.4%
87.5%
93.7%
74.4%
Outpatient completion (state average) 70.1%
10 most effective hospitals
94.5%
10 least effective hospitals
45.3%
67.1%
95.9%
52.9%
68.3%
94.7%
58.08%
133 of 357
37.3%
165 of 380
43.4%
41 of 110*
40%
% of babies who complete Diagnostic 33 of 133
Eval & have permanent hearing loss
24.8%
65 of 165
39.4%
12 of 41*
29.3%
Inpatient Refer Rates (state average)
10 most effective hospitals
10 least effective hospitals
Reported Completion of Diagnostic
Evaluations (state average)
Number of babies still “in process”
*only 3 months worth of data
224
215
69
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
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
41%
Unwillingness of third-party payers
to reimburse for hearing screening
28%
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
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
Average Age in Months
60
70
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
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
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)
Hawai'i EHDI Progress
Age of Identification and Intervention
60
Identification
Intervention
Age in Months
50
40
30
20
10
0
pre
1992
1993
1994
1995
Year
Data from Hawai’I Zero to Three Project
1996
1997
1998
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
What Is a Medical Home?
•
A primary care physician
provides care which is:
•
Accessible
•
Family-centered
•
Comprehensive
•
Continuous
•
Coordinated
•
Compassionate
•
Culturally effective
EHDI and the Medical Home
Birthing
Hospital
Parent Groups
Audiology
Mental Health
Primary
Provider
3rd Party
Payers
ENT
Child/Family
Deaf
Community
Services for
Hearing Loss
Early
Intervention
Programs
Genetics
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
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
~50%
~30%
Non-syndromic
Autosomal dominant
Genetic
21%
Autosomal recessive
77%
~70%
~1%
X-Linked
~1%
Mitochondrial
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
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
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
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.
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
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
Communication Choices
• American
Sign Language
• Total Communication
• Auditory Verbal
• Auditory-Oral
• Cued Speech
Information
Information Wanted
Wanted vs.
vs. Received
Received by
by Parents
Parents
at Hearing Loss Confirmation
Degree of loss
Auditory system
Amplification
Educational options
Speech/Lang dev
Wanted
Received
Etiology
Home activities
*Written Information
*Financial Support
*Emotional Support
*Parent Contacts
*Referral Sources
0
20
40
60
80
Martin, George, O'Neal, & Daly (1987); *Sweetow & Barrager (1980)
100
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
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%
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?
Worried about my baby’s hearing
Strongly Agree
or Agree
total group
subgroup
11%
24%
Confused about the results of screening tests
10%
24%
Glad hearing screening is done at this hospital
91%
70%
Confident 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%
EHDI Materials Available from “State” Programs
(n=54)
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
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
National EHDI Technical
Assistance System
• EHDI Network members located in each
of ten geographic regions
National EHDI Assistance Network
Region VIII
(91% currently born
in UNHS hospitals)
Terry Foust
Region V
Region II
(26% currently born
in UNHS hospitals)
(16% currently born
in UNHS hospitals)
Karen Munoz
Beth Prieve
I
Puerto Rico
Virgin Islands
X
Region I
VIII
II
Antonia MaxonB
V
III
Region X
(21% currently born
in UNHS hospitals)
VII
Curt Whitcomb
Region III
(49% currently born
in UNHS hospitals)
IX
Sean Kastetter
VI
Region IX
(23% currently born
in UNHS hospitals)
Randi Winston
Yusnita Weirather
(38% currently born
in UNHS hospitals)
IV
-
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)
Region VII
(33% currently born
in UNHS hospitals)
Les Schmeltz
= indicates the locations of MCHB Regional Offices
Karen Ditty
Patti Martin
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
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
National EHDI Technical
Assistance System (continued)
• EHDI Network members located in
each of the MCHB regions
• Information dissemination and
training
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
Sound Ideas Newsletter
• Topical articles, suggestions
for program improvement
• Upcoming events
• Available online or mailed
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)
www.infanthearing.org
www.babyhearing.org
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
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
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
“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
“I am a great
believer in luck,
and I find that the
harder I work, the
more I have of it.”
---Thomas Jefferson