Transcript Slide 1

DOES OAE/A-ABR HEARING
SCREENING MISS HEARING LOSS?
2005 EDHI Conference
Jean L. Johnson, DrPH
Center for Disability Studies
Director (Interim)
March 3, 2005
Atlanta, GA
International Conference on Newborn Hearing
Screening, Diagnosis and Intervention
Cernobbio, Italy - May 2004
Second Annual Conference of the CDC Centers
on Birth Defects and Developmental Disabilities
Washington, DC – July 2004
Asia Region Neonatal Screening Conference
Shanghai, China - September 2004
UH College of Education Research Symposium
Honolulu, HI - October 2004
American Speech-Language and Hearing Association
Philadelphia, PA – November 2004
CDC Ad Hoc Group – Teleconference
January 11, 2005
Research Team
Principal Investigator - Jean Johnson, DrPH
Research Coordinator - Karl R. White, PhD
Diagnostic Evaluation Coordinator - Judith E. Widen, PhD
Site Co-Principal Investigators
Judith Gravel, PhD
Michele James, AuD
Teresa Kennalley, MA
Antonia B. Maxon, PhD
Lynn Spivak, PhD
Maureen Sullivan-Mahoney, MA
Betty Vohr, MD
Yusnita Weirather, MA
Funded by the Centers for
Disease Control and Prevention
CDC Consultants:
June Holstrum, PhD
Roy Ing, MD, MPH
Brandt Culpepper, PhD
Krista Biernath, MD
Lee Ann Ramsey, BBA, GCPH
under a Cooperative Agreement with:
The Association of Teachers
of Preventive Medicine
with a sub-agreement to:
The University of Hawai`i
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.
• Dramatic benefits are associated with early
identification of hearing loss.
It is true for severe to profound loss.
Is it also true for milder hearing loss?
Background
 National Institutes of Health (NIH) Consensus Panel
recommended in March 1993 that:
“the preferred model for screening should begin with an evoked otoacoustic
emissions test and should be followed by an auditory brainstem response
test for all infants who fail the evoked otoacoustic emissions test.”
 Continuing improvement of ABR technology led to a number
of hospitals in the US implementing a variation of the NIH
recommendation that was based on automated ABR (AABR)
 Anecdotal reports to the Centers for Disease Control and
Prevention (CDC) in the mid to late 1990’s that the two-stage
OAE/AABR protocol was not identifying some infants with
permanent hearing loss.
 The CDC issued a competitive Request for Proposals in 2000
to investigate whether the OAE/AABR screening protocol was
not identifying babies with hearing loss.
Oto-Acoustic Emissions (OAE)
Screening
Auditory Brain Stem (ABR)
Screening
Research Question
Are infants with permanent hearing loss not
being identified when newborn hearing screening
is done with a two-stage OAE/A-ABR protocol in
which infants who fail OAE and pass AABR are
not followed?
Comparison Group
OAE Screening Prior to
Hospital Discharge
Pass
Fail
AABR
Screening
Pass
Fail
Comprehensive Hearing
Evaluation Before 3 Months
of Age
Study Sample
Comprehensive Audiological
Assessment at 8-12 months of
age
Discharge
Discharge
Criteria for Selecting Sites
•
2,000 or more births per year
•
Established newborn hearing screening program
with at least six month history of success
•
Historical refer rates of less than 10% for OAE
and 4% for ABR
•
Success in obtaining follow-up on 85% or more of
referrals
•
Ethnic and socio-economic distribution similar to
US population
Participating Sites
Name of Hospital
Location
Arnold Palmer Hospital
Orlando, Florida
Good Samaritan Hospital
Columbus, Ohio
Jacobi Medical Center
New York, New York
Kapi`olani Medical Center
Honolulu, Hawaii
Long Island Jewish Medical System
New York, New York
Via Christi Regional Medical Center
Kansas City, Kansas
Women & Infants Hospital
Providence, Rhode Island
Enrollment Process
•
Eligible babies (Failed OAE and Passed A-ABR) were
identified following newborn hearing screening.
•
Parents were contacted and research study
explained.
•
Consent was obtained from families.
•
Enrollment data was collected.
•
Contact was maintained with family at 2, 4, & 6
months of age via post cards.
•
Babies were seen for audiological diagnostic
evaluation at 8-12 months of adjusted age.
Date Collected for Each Participating Baby
Birthdate
Bronchio-pulmonary Dsplasia
Gender
Mechanical Ventilation >7 Days
Birth Weight
ECMO
Gestational Age
Number of Children in Home
APGAR Scores
Number of Adults in Home
Days in NICU
Total Household Income
Malformations of the Head and Neck
Child’s Race/Ethnicity
Syndrome Associated with Hearing Loss
Health Insurance
In-utero Infections
Family History of Hearing Loss
Study Sample

1,524 Infants Enrolled

973 (63.8%) Returned for Evaluation

1,432 Ears Evaluated
Enrollment of Study Participants
Enrollment
Period
Births
During
Enrollment
Site #1
May 1, 2001 to
Dec 31, 2002
16,608
6.3%
0.8%
WB/NICU
Site #2
June 1, 2001 to
Jan 31, 2003
9,393
4.5%
0.9%
WB/NICU
Site #3
May 1, 2001 to
Jan 31, 2003
24,032
2.4%
0.8%
WB/NICU
Site #4
Sep 20, 2001 to
Jan, 2003
4,509
8.0%
1.0%
WB
Site #5
May 15, 2001 to
Jan 31, 2003
9,252
3.1%
0.8%
WB
Site #6
May 1, 2001 to
Jan 31, 2003
16,623
5.3%
1.2%
WB/NICU
Site #7
May 1, 2001 to
Jan 31, 2003
6,217
9.6%
2.8%
WB/NICU
86,634
4.8%
1.0%
Total
Referral Rate
OAE
ABR
Recruitment
From
Enrollment of Study Participants
(continued)
Number of Babies:
Births During
Enrollment
Eligible for
Enrollment
Total Enrolled
Number Not
Recruited
Number of
Refusals
16,608
1,044
191
418
435
6.3%
18.3%
40.0%
41.7%
421
370
18
33
4.5%
87.9%
4.3%
7.8%
456
170
11
275
1.9%
37.3%
2.4%
60.3%
285
84
186
15
6.3%
29.5%
65.3%
5.3%
209
147
30
32
2.3%
70.3%
14.4%
15.3%
433
266
50
117
7.0%
61.4%
11.5%
27.0%
614
296
71
247
3.7%
48.2%
11.6%
40.2%
3,462
1,524
784
1,154
4.0%
44.0%
22.6%
33.3%
Site # 1
9,393
Site # 2
24,032
Site # 3
4,509
Site # 4
9,252
Site # 5
6,217
Site # 6
16,623
Site # 7
Total
86,634
Audiological Diagnostic
Evaluation
•
Visual reinforcement
audiometry (VRA)
•
Tympanometry
•
OAE - Either TOAE or DPOAE
VRA Protocol
• Protocol patterned after National institutes of
Health Study (Norton, Univ. of Washington)
• Responses at 500, 1K, 2K, 4K Hz
– Order of testing 2K, .5K, 4K, 1K
– Aiming for minimal response level of 15 dB HL
• Multiple visits often necessary to complete
testing
– 68% completed in 1 visit
– 24% required 2 visits
– 8% required 3 or more visits
Criteria for Categorizing Hearing Loss
Category
Criteria
Not Permanent Hearing
Loss
Using the ”best” results from all assessments, MRL thresholds of < 20dB at 1K, 2K,
and 4K.
Probable Not Permanent
Hearing Loss
Complete MRL data not available at 1K, 2K, and 4K, BUT
* All frequencies had MRLs < 20dB OR OAEs within normal limits* OR Tone burst
ABR data < 25dB.
Permanent Hearing Loss
(PHL)
Sensorineural
MRLs > 25dB at 1K, 2K, or 4K (tested with good confidence) OR ABR threshold >
30dB; AND if tested, OAEs below normal limits at the frequencies with elevated
MRLs; AND normal middle ear functioning based on tympanometry or bone
conduction.
Permanent Hearing Loss
(PHL) Conductive
MRLs > 25dB at 1K, 2K, or 4K (tested with good confidence); AND if tested, OAEs
below normal limits; AND bone conduction thresholds < 20dB with an Air/Bone gap >
15dB at frequencies with MRLs > 25dB.
Increased Suspicion of
PHL
High Suspicion
MRLs > 25dB at 1K, 2K, or 4K, BUT OAEs within normal limits for those frequencies
OR only fair confidence in VRA testing.
Some Suspicion
* MRLs > 30dB at 1 frequency or > 25dB at more than one frequency, BUT abnormal
tympanometry AND no bone conduction.
* Sound field thresholds > 25dB (with fair confidence) AND normal tympanometry
AND OAEs below normal limits.
Not Sufficient Data to Rule
Out PHL
* No MRLs or OAEs within normal limits for 1K, 2K, or 4K and none of the above
criteria for permanent hearing loss are met.
* OAEs within normal limits were defined as > 3-6dB at 1K and > 6dB at 2K and 4K.
Hearing Status of Study Ears from All Hospitals
# of Infants
with Dx Data
Percent
of Infants
w/ Dx
Data
Total
Ears
Not PHL
Permanent
Loss
Hearing
(PHL)
Increased
of
Suspicion
PHL:
SNHL
PC
High
Some
Probable
Not PHL
Not
Sufficient
Data
Site
#1
81
42.4%
148
131
0
0
0
0
6
11
Site
#2
299
80.8%
478
432
7
0
0
0
35
4
Site
#3
42
50.0%
59
40
0
0
0
2
6
11
Site
#4
109
74.1%
165
82
10
5
17
12
24
15
Site
#5
86
50.6%
111
58
2
0
0
5
16
30
Site
#6
184
69.2%
241
202
4
0
2
8
8
17
Site
#7
172
58.1%
230
195
2
0
0
1
5
27
Total
973
63.8%
1432
1140
100%
79.6%
25
1.7%
5
0.3%
19
1.3%
28
2.0%
100
7.0%
115
8.0%
PHL in Comparison Group Sites
(Fail OAE/Fail A-ABR)
All Comparison Group Sites
Site # 1
Site # 2
Site # 3
Site # 4
Site # 5
Site # 6
Site # 7
Total
Babies w/ PHL
Ears w/ PHL
Births during
enrollment
SN
PC
Total
SN
PC
Total
16,608
17
1
18
24
2
26
9,393
4,509
9,252
24,032
6,217
16,623
86,634
18
4
16
39
16
36
146
1
0
0
3
1
6
12
19
4
16
42
17
42
158
31
6
27
60
25
55
228
2
0
0
3
2
6
15
33
6
27
63
27
61
243
Prevalence
of PHL (per Referred for Dx Completed Dx
1000)
1.08
2.02
0.89
1.73
1.75
2.73
2.53
1.82
1.2%
82.4%
199
164
1.5%
95.7%
140
134
0.2%
88.9%
9
8
0.3%
96.4%
28
27
0.8%
87.6%
193
169
0.7%
65.9%
41
27
0.6%
79.8%
94
75
0.8%
85.8%
704
604
PHL in Ears of Study Infants that Passed Initial OAE
Ears of Study Infants that Passed Initial OAE
Total Ears
Not PHL
Permanent
Loss
Hearing
(PHL)
Increased
of
Suspicion
PHL:
SNHL
PC
High
Some
Probable
Not PHL
Not
Sufficient
Data
Site #1
13
11
0
0
0
0
0
2
Site #2
112
107
0
0
1
0
3
1
Site #3
25
3
0
0
1
0
15
6
Site #4
53
19
0
0
2
6
15
11
Site #5
53
30
0
0
1
2
5
15
Site #6
127
60
0
0
3
1
38
25
Site #7
113
30
0
0
0
0
22
61
496
100%
260
52.4%
0
0.0%
0
0.0%
8
1.6%
9
1.8%
98
19.8%
121
24.4%
Total
Degree of Hearing Loss in Study and
Comparison Group Babies
28.6%
Mild
Moderate
(25-40 dB) (41-70 dB)
Study Group
Comparison
Group
Total
Severe
through
Profound
(>70 dB)
Total
Infants
15
5
1
21
71.4%
23.8%
4.8%
100.0%
31
64
63
158
19.6%
40.5%
39.6%
100.0%
46
69
64
179
25.7%
38.5%
35./%
100.0%
As measured in the worse ear
80.3%
Comparability of Study and Comparison
Groups
What Percent of
“Referred” Babies Did
Sites Try to Follow?
What Percent of
“Followed” Babies
Were Diagnosed?
Study Group
(Fail OAE/ Pass A-ABR)
44%
64%
Comparison Group
(Fail OAE/ Fail A-ABR)
100%
87%
 Reasonable to adjust prevalence rates for those who were not recruited
 Adjusting prevalence rates for differences in the percent of diagnostics
completed is problematic
 Families who think their child has a hearing loss are more likely to return
 Families that are poor, single heads of household, transient , etc are less likely to
return and these variables may be correlated with the incidence of hearing loss
How Many Additional Babies with Permanent
Hearing Loss (PHL) were Identified?
Comparison Group
Study Group
(Fail OAE/ Fail A-ABR)
(Fail OAE/ Pass A-ABR)
Number of Babies
158
21
179
Prevalence per 1,000
1.82
.24
2.06
*Adjusted for proportion of OAE fails that enrolled
Represents 12% of
all babies with PHL
in birth cohort
Total
Is it important that 21 Babies (30 ears)
with PHL were found?
• How many does it add to what would have
been identified otherwise?
• How many ears with hearing loss were found
among those that passed the initial screen?
• How many babies would you have to follow
to find 21 babies PHL?
• Is this congenital or late-onset hearing loss?
How many babies must be
screened to find 21 with PHL?
The obvious answer is 973, but….
• This ignores that most screening programs
that use OAE also do second stage OAE
screen (usually following hospital discharge)
• Such outpatient screening is less expensive
than the diagnostic protocol used in this study
• Difficulty of getting babies to return for
outpatient screening must be considered
Screening Failures
Passed
Screening
Failed
Screening
True Positives
False Positives
Cost of Screening
• Direct cost
• Indirect cost
• Follow-up cost
Cost-Benefit of Screening
Benefit
Benefit
Benefit
COST
COST
COST
Good follow-up
Mediocre follow-up
Poor follow-up
Were any of these ears lateonset losses?
•
This study was not designed to answer that
question.
•
We do know that IF all of the ears with risk
factors had been followed and identified, 9 of
21 babies would still have been missed
•
Little is known about the incidence or what
predicts late-onset hearing loss
•
Most (>3/4ths) of the hearing losses
“missed” were mild as was expected
Different Criteria for Determining
Permanent Hearing Loss
Comparison
Group
Study
Group
Total
Based only on
those meeting
criteria for PHL
1.82
.24
2.06
Including those
categorized as high
suspicion of PHL
1.82
(21 babies)
.43
(33 babies)
2.49
Different Criteria for Determining
Permanent Hearing Loss
Comparison
Group
Study
Group
Total
Based only on
those meeting
criteria for PHL
1.82
.24
2.06
Including those
categorized as high
suspicion of PHL
1.82
X
(21 babies)
X
X X
.43
(33 babies)
2.49
Variation Among Sites
 The study design assumed that sites are
all equally well implemented
 To the degree that this isn’t true, data
from some sites may be a better estimate
of the number of babies being missed
Indicators of Implementation Quality at Each Site
Site # 1
Site # 2
Site # 3
Site # 4
Site # 5
Site # 6
Site # 7
# of Study
Group
Infants with
PHL
Average
“Rank” for
Implementa
tion Quality
of Site
Births
During
Enrollment
Period
% and N of
Eligible
Infants
Enrolled
% and N of
Refusals
During
Recruitment
%
Returning
for
Diagnostic
Evaluations
% with “Not
Sufficient
Data”
0
5.8
16,608
18.3%
41.7%
42.4%
7.4%
191
435
87.9%
7.8%
80.8%
0.8%
370
33
29.5%
5.3%
50.0%
18.6%
84
15
70.3%
15.3%
74.1%
9.1%
147
32
37.3%
60.3%
50.6%
27.0%
170
275
61.4%
27.0%
69.2%
7.1%
266
117
48.2%
40.2%
58.1%
11.7%
296
247
5
0
10
2
3
2
PHL=permanent hearing loss
1.3
4.8
2.8
6.0
3.0
4.5
9,393
4,509
9,252
24,032
6,217
16,623
Presumed Curve of Health Care
Bell-Shaped Curve of Health Care
Best Estimate of Amount of PHL Missed
by OAE/AABR protocol
12% of children with
23% of children with
PHL in birth cohort
Babies Who
Failed OAE /
Failed AABR
All Sites
Sites with Best
Implementation
1.82
2.27
PHL in birth cohort
Babies who failed OAE/ Passed AABR
Based on 44%
that participated
Adjusted for those
who did not participate
2.06
2.37
(.24 increase)
(.55 increase)
2.75
2.95
(.48 Increase)
(.68 increase)
17% of children with
23% of children with
PHL in birth cohort
PHL in birth cohort
What’s the Best Estimate of the Number
of Babies Missed by the OAE/AABR
Screening Protocol?
• Depends on the criteria used for
determining PHL
• Variation among sites
• Adjustments for Differences Between
Study and Comparison Groups
Conclusions
 The OAE/AABR protocol, as implemented at
these sites, failed to identify a substantial
number of infants with PHL.
 Best estimate is .55 per thousand or 24% of
all infants with PHL.
 Most were mild sensorineural hearing loss
 Impossible from this study to determine how
many are late-onset losses
 About 41% might be identified if all infants
with risk factors or opposite refer ears were
followed, but this is not likely.
Recommendations
 Screening for permanent hearing loss should extend
into early childhood (e.g. physician’s offices, early
childhood programs).
 Emphasize to families and physicians that passing
hospital-based hearing screening does not eliminate
the need to vigilantly monitor language development.
 Screening program administrators should ensure that
the stimulus levels of equipment used are consistent
with the degree of hearing loss they want to identify.
 The relative advantages and disadvantages of the twostage (OAE/A-ABR) protocol need to be carefully
considered for individual programs.
Further Research
Recommendations
 Prevalence and methods of identifying late-onset
hearing loss
 Identification and monitoring of progressive hearing
loss
 Ongoing investigation of sensitivity of various
screening protocols and equipment (including what
level of hearing loss is targeted)
 Practicality and cost-efficiency of alternative or
additional “continuous” screening and surveillance
techniques, especially in early childhood
Questions to Ponder
• What degree of hearing loss do you want to
identify?
• What cost will be required for that
identification?
• Can you assure that follow-up will occur?
• What interventions can you provide?
• How can you provide continuing surveillance
of late onset or progressive loss?
• How comfortable are you with the quality of
services being provided?
Key Large-Scale Newborn Hearing Screening Studies in the United States
Location/Dates
Cohort Size
Nurseries
Screening
Techniques
Refer Rates
Follow Up Rate
Prevalence Per
1000 of Hearing
Loss
RIHAP 3
(8/90 – 2/91)
1,850
NICU & WBN
OAE / ABR
26.9%
Colorado
(1/92-12/96
27,938
NICU & WBN
AABR
Rhode Island
(1/93 – 12/95)
53,121
NICU & WBN
OAE / ABR
14.7%
New Jersey
(1/93 – 12/95)
15,749
NICU & WBN
ABR
3%
Hawai‘i
(1/94 – 12/95)
9,605
WBN
OAE
89%
4.15
Texas
(1/94 – 6/97)
54,228
NICU & WBN
OAE & AABR
3.5%
82.3%
3.14
NIDCD
(1994-1997)
4,478
2,701
NICU & WBN
ABRTOAEDPOAE-
64.4%
(Research)
56.0
New York
(1/96 – 12/96)
69,761
NICU & WBN
OAE & AABR
72%
8.00 (NICU)
0.9 (WBN)
Washington, DC
(2/97 – 12/02)
39,437
NICU & WBR
TOAE
1.6%
82.7
2.3 overall
1.9 WBN
6.8 - NICU
ATPM/CDC
(5/2001 – 1/2003)
86,634
NICU & WBN
OAE: 4.8
AABR: 1.0
63.8(Research)
85.8
2.05
5.95
73%
2.56
74--88%a
2.00
3.3 overall
2.00 – WBN
13.0 NICU
Other Dissemination
• Article has been selected for publication in
Pediatrics
• Five articles prepared for publication in
American Journal of Audiology
• Presentation at Council on Exceptional
Children Conference in Baltimore in April
2005.
Additional Information on Newborn
Hearing Screening
www.infanthearing.org
www.babyhearing.org
[email protected]
Deafness separates people from people.
--- Helen Keller