Upload
sara-short
View
215
Download
0
Tags:
Embed Size (px)
Citation preview
Universal Newborn Hearing ScreeningWhat 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
0
500
1000
1500
2000
2500
3000
3500
4000
1991 1992 1993 1994 1995 1996 1997 1998 1999 2000 2001
Year
Num
ber
of H
ospi
tals
0.0%
10.0%
20.0%
30.0%
40.0%
50.0%
60.0%
70.0%
80.0%
90.0%
100.0%
Jan-
93
Jan-
94
Jan-
95
Jan-
96
Jan-
97
Jan-
98
Jan-
99
Jan-
00
Jan-
01
Percentage of Newborns Screened for Hearing Prior to Hosptial Discharge
0.0%
10.0%
20.0%
30.0%
40.0%
50.0%
60.0%
70.0%
80.0%
90.0%
100.0%
Jan-
93
Jan-
94
Jan-
95
Jan-
96
Jan-
97
Jan-
98
Jan-
99
Jan-
00
Jan-
01
Jan-
02
Percentage of Newborns Screened for Hearing in the United States
(Dec 2001)
.Percentage of Births
Screened
90%+
21 - 50%1 - 20%
3
51 - 90%
States with Legislative Mandates Related to Universal Newborn Hearing Screening
Status of UNHS Legislative Mandates
States with mandates
No mandate
No mandate, but statewide programs
National Universal Newborn Hearing Screening Programs
• Austria
• United Kingdom
• Poland
• Flemish Belgium
• Singapore
• Canada (Ontario)
Improved ScreeningTechniques/Equipment
Why is Implementation of Newborn Hearing Screening Accelerating?
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
Improved ScreeningTechniques/Equipment
Acceptance byPolicy Makers
Why is Implementation of Newborn Hearing Screening Accelerating?
Increased Number ofSuccessful Programs
PublicAwareness/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
ProgramsSample 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
Incidence per 10,000 of Congenital Defects/Diseases
30
12 11
6 52 1
0
10
20
30
40
Hearing Loss
Cleft lip or palate
Down Syndrome
Limb defects
Spina bifida
Sickle Cell Anemia
PKU
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.
Reading Comprehension Scores of Hearing and Deaf Students
1.0
2.0
3.0
4.0
5.0
6.0
7.0
8.0
9.0
10.0
8 9 10 11 12 13 14 15 16 17 18
Deaf
Hearing
Age in Years
Schildroth, A. N., & Karchmer, M. A. (1986). Deaf children in America, San Diego: College Hill Press.
Gra
de
Eq
uiv
alen
ts
Effects of Unilateral Hearing Loss
MathLanguage
MathLanguage
Social
MathLanguage
MathLanguage
Social
0th 10th 20th 30th 40th 50th 60th
Percentile Rank
Normal Hearing Unilateral Hearing Loss
Keller & Bundy (1980)(n = 26; age = 12 yrs)
Peterson (1981)(n = 48; age = 7.5 yrs)
Bess & Thorpe (1984)(n = 50; age = 10 yrs)
Blair, Peterson & Viehweg (1985) (n = 16; age = 7.5 yrs)
Culbertson & Gilbert (1986)(n = 50; age = 10 yrs)
Average ResultsMath = 30th percentile
Language = 25th percentileSocial = 32nd percentile
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 .62Metropolitan Achievement Test
Math .48Reading .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.
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.
13-18 mos(n = 15/8)
19-24 mos(n = 12/16)
25-30 mos(n = 11/20)
31-36 mos(n = 8/19)
0
5
10
15
20
25
30
35
Identified BEFORE 6 Months
Identified AFTER 6 Months
Expressive Language Scores for Hearing Impaired Children Identified Before and After 6 Months of Age
Chronological Age in Months
Lan
gu
age
Ag
e in
Mo
nth
s
0.8 1.2 1.8 2.2 2.8 3.2 3.8 4.2 4.80
1
2
3
4
5
6
Identified <6 mos (n = 25)
Identified >6 mos (n = 104)
Age (yrs)
Lan
gu
age
Ag
e (y
rs)
Boys Town National Research Hospital Study of Earlier vs. Later
Moeller, M.P. (1997). Personal communication, [email protected]
129 deaf and hard-of-hearing children assessed 2x each year.
Assessments done by trained diagnostician as normal part of early intervention program.
Good work,but I think we mightneed just a little more detail righthere.
Implementing Effective EHDI Programs
Then amiracleoccurs
out
Start
• 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
Is the Glass Half Empty or Half Full?
Status of Early Hearing Detection and Intervention (EHDI) in the United States
Screening before 1 month
Diagnosis before 3 months
Intervention 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)
Hearing Loss=3
Normal Hearing=37
Diagnosisn=40
InpatientScreening
Fail=40
Pass=9601 S
tag
eA
AB
R
Hearing Loss=3Normal Hearing=7
InpatientScreening Pass=920
Fail=80 OutpatientScreening
n=80
Diagnosisn=10
Pass=70
Fail=10
2 S
tag
eO
AE
Diagnosisn=20
InpatientScreening
Pass=980
Fail=20 Hearing Loss=3
Normal Hearing=17
1 S
tag
eO
AE
/ A
AB
R
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
Screening
Research
Diagnosis InterventionMedical, Audiological and
Educational
Program Improvementand Quality Assurance
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%
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%)
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 2000 2001(6 mos.)
(n=43,547) (n=46,771) (n=23,307)
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
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 newbornhearing screening, diagnosis, and intervention 41%
Unwillingness of third-party payersto 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
Average Age in Months
3
3
35
19
30
30
24
25
31
56
Coplan (1987)
Eissman et al. (1987)
Gustason (1987)
Meadow-Orlans (1987)
Yoshinago-Itano (1995)
Stein et al. (1990)
Mace et al. (1991)
O'Neil (1996)
Johnson et al. (1997)*
Vohr et al. (1998)*
0 10 20 30 40 50 60 70
Confirmation of Permanent Hearing Loss
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 ProgressAge of Identification and Intervention
Data from Hawai’I Zero to Three Project
pre 1992 1993 1994 1995 1996 1997 1998
Year
0
10
20
30
40
50
60
Ag
e in
Mon
ths
Identification Intervention
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
Parent Groups
Mental Health
Birthing Hospital
Audiology
Primary Provider
Child/Family
ENT
GeneticsEarly
Intervention Programs
3rd Party Payers
Deaf Community
Services for Hearing Loss
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
~50%
~50%
Environmental
Genetic
~70%
~30%
CMV meningitisrubella prematurityhead trauma asphyxiationototoxicity hyperbilirubinother infections Syndromic
Alport NorriePendred Usher
WaardenburgBranchio-oto-renal
Jervell and Lange-Nielsen
Non-syndromic
Autosomal dominant
Autosomal recessive
X-Linked
Mitochondrial
21%
77%
~1%
~1%
Congenital Hearing Loss
What Causes Hearing Loss?
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 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
Communication Choices
• American Sign Language
• Total Communication
• Auditory Verbal
• Auditory-Oral
• Cued Speech
Information Wanted vs. Received by Parents at Hearing Loss ConfirmationInformation Wanted vs. Received by Parents
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
0 20 40 60 80 100
Wanted
Received
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 screenAfter all hearing tests were completed, how did you feel?
Strongly Agree or Agree
total group subgroupWorried about my baby’s hearing 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 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
National EHDI Technical Assistance System
• EHDI Network members located in each of ten geographic regions
Region I(38% currently bornin UNHS hospitals)
Antonia MaxonB
Region IV(46% currently bornin UNHS hospitals)
Faye McCollister
Region III(49% currently bornin UNHS hospitals)
Sean Kastetter
Region VI(38% currently born in UNHS hospitals)
Karen DittyPatti Martin
Region VII(33% currently bornin UNHS hospitals)
Les Schmeltz
Region IX(23% currently bornin UNHS hospitals)
Randi WinstonYusnita Weirather
Region II(16% currently bornin UNHS hospitals)
Beth Prieve
Region V(26% currently bornin UNHS hospitals)
Karen Munoz
Region X(21% currently bornin UNHS hospitals)
Curt Whitcomb
Region VIII(91% currently bornin UNHS hospitals)
Terry Foust
National EHDI Assistance Network
-
VIII
IX
VII
VI
V
IV
III
II
I
X
Guam, American Samoa,Marshall Islands, Palau,
No. Mariana Islands,Fed. Micronesia
Puerto RicoVirgin Islands
= indicates the locations of MCHB Regional Offices
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)
• 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 Other Groups and Agencies
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