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Early Hearing Detection & Intervention (EHDI) Program |
Integrating Early Hearing Detection and
Intervention
and Newborn Screening Programs
State: Colorado
Contact: Bill Letson,
Bill.Letson@state.co.us
Background:
Newborn screening (NBS), which is used to identify underlying conditions at
birth that can cause or contribute to disease, disabilities, and death,
has been an essential preventive public health program in the United
States since the 1960s. Each year approximately 4 million newborns are
screened for an increasing number of inherited metabolic, hematologic,
and endocrine disorders. Much of the data collected by individual NBS
programs has been used to evaluate the screening and short‑term
follow-up components that essentially take the infant from the heel
stick to the referral for clinical care after definitive diagnosis.
Public health agencies need to evaluate additional outcome measures to
ensure that the potential benefits of early identification are realized
for individual infants and to confirm that the public has attained the
broad goals of reducing morbidity, mortality, and disability.
Purpose:
The purpose of this project is to develop ways of
integrating Early Hearing Detection and Intervention (EHDI) with the NBS
program and to do prospective follow‑up of cohorts of newborns who are
deaf or hard of hearing, hemoglobinopathies, phenylketonuria, and other
disorders identified through NBS. This project plans to provide
aggregate data relating to surveillance, short-term management, and
long-term follow-up of affected infants identified through NBS, as well
as to provide information for health care providers on their clients.
The intent is to track
affected children identified through NBS and collect data relating to a
variety of long‑term outcomes.
Design:
Centers for Disease Control and Prevention will
work with officials in the Colorado Department of Public Health and
Environment (CDPHE) to develop ways of integrating EHDI with the NBS
program and to do prospective follow-up of cohorts of newborns with
hearing loss, hemoglobinopathies, phenylketonuria, and other disorders
identified through NBS. Funding, which is planned for a period of 5
years, began in October 2000.
Current Status: A study protocol was developed
and exempted from full institutional review board review by the CDPHE.
Electronic birth certificates, which include
hearing screening status, are now being sent to the CDPHE and an electronic
database has also been developed for use by sickle cell and inherited
metabolic disease clinics.
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Etiology of Hearing Loss
States:
Contacts:
Utah
John Carey;
John.Carey@hsc.utah.edu
Hawaii
Beppie Shapiro; Beppie@hawaii.edu
Rhode Island
Betty Vohr; bvohr@wihri.org
CDC/Georgia
Aileen Kenneson; akenneson@cdc.gov
Background:
Approximately
1 to 3 in every 1,000 newborns have a moderate to severe congenital
hearing loss in both ears that can affect language development. Advances
in the study of genetics suggest that 50% of all hearing loss cases are
caused by genetic factors. About 70% of the hearing loss cases caused by
genetic factors are nonsyndromic, which means the infant does not have
any other health problems. Research indicates that up to 50% of
nonsyndromic hearing loss cases in some populations result from
mutations in a single gene, GJB2. The GJB2 gene contains the
instructions for a protein called connexin 26, which plays an important
role in the functioning of the cochlea (the part of the ear that changes
sound into nerve signals that travel to the brain). Many different
variations in the GJB2 gene can cause hearing loss. Determining the
genetic causes of infant hearing loss could help in the development of
effective treatment options.
Purpose:
The purpose of this study is to
investigate genetic and other causes of “prelingual” hearing loss. The
primary objective of this study is to evaluate all infants with a
unilateral or bilateral hearing loss of any degree (mild to profound) in
an attempt to determine the causes.
Design:
Infants with hearing loss will be identified through the
universal newborn hearing screening programs. The parents will be
offered a test for their child that identifies mutations in the GJB2
(connexin 26) gene and other genes found to be involved in the etiology
of hearing loss. The information gained from this study will increase
understanding of the influence of genetics and other factors on hearing,
with potential impact on the development of treatment options for people
with hearing loss.
Current Status:
Utah
developed a study protocol
which Rhode Island, Georgia, and Hawaii are adopting. Utah, Rhode
Island, and Georgia have received all necessary IRB approvals and are
now enrolling patients. Hawaii is still awaiting local IRB approval.
In Georgia, CDC is leading the investigation and is enrolling patients
from the Lawrenceville and Macon health districts. The research sites
are using common data collection tools so that the data can be combined
to facilitate data analysis.
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Cost
of Early Hearing Detection and Intervention Programs
State: Utah
Contact: Linda Goetze;
lgoetze@cpd2.usu.edu
Background: Recent efforts associated with newborn
hearing screening have been directed towards establishing programs that
will identify the approximately 12,000 infants with hearing loss born
each year in the United States. Because attention has focused on the
implementation of newborn hearing screening, few studies have been
undertaken to determine the financial costs of Early Hearing Detection
and Intervention (EHDI) programs . Determining the costs of EHDI
programs will assist public health agencies in deciding how to best
implement a newborn hearing screening program.
Purpose:
The purpose of
this study is to determine the economic costs associated with EHDI
programs and to assess the impact of screening and diagnostic procedures
on parents and families.
Design:
The Centers for Disease Control and
Prevention EHDI team will work with officials from the Utah Department
of Health to conduct a complete and state‑of‑the‑art economic analysis
of the screening, follow‑up, and diagnostic components of eight Utah
hospital‑based EHDI programs. Funding for this intended 5-year project
started in October 2000.
Current Status:
Institutional review board (IRB) approval
for the collection of data has recently been obtained and the
investigators at Utah State University are in the process of collecting
cost data from participating hospitals and contacting parents to begin
collecting survey data.
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Efficacy
of Otoacoustic Emissions/Automated Auditory Brainstem Response Protocol
in Identifying Hearing Loss
State: Hawaii
Contact: Jean Johnson;
jeanj@hawaii.edu
Background:
Hospital‑based screening of newborns for hearing loss is rapidly becoming
the standard of care in the United States. The National Institutes of
Health (NIH) in their 1993 Consensus Development Conference Statement,
recommended a two‑stage screening protocol: Otoacoustic Emissions (OAE)
followed by Automated Auditory Brainstem Response (AABR) for those
infants who do not pass the OAE. However, some researchers and
practitioners have suggested that this particular application of the
two‑stage screening protocol might miss some newborns with mild hearing
loss.
Purpose:
The purpose of this study
is to investigate the efficacy of the OAE/AABR screening protocol in
identifying hearing loss.
Design:
The project
will be conducted from October 1, 2000, through September 30, 2002, in
12 birthing hospitals located in different parts of the United States.
Participating hospitals have a total annual birth census of
approximately 60,000 and have been conducting universal newborn hearing
screening programs using a two‑stage OAE/AABR protocol prior to
discharge for at least 6 months. Mothers of babies who fail the OAE but
pass the AABR will be asked to participate in the study. Any mother who
chooses to enroll will be asked to sign an informed consent, complete a
general information form, and sign a consent to review her infant's
medical record. Each mother will be asked to return for an assessment of
her infant's hearing status (including routinely used physiologic and
behavioral tests) when the baby is 7 months of age. The results of the
assessment will be used to determine if the two‑stage OAE/AABR newborn
hearing screening protocol is missing some babies with mild hearing
loss. If so, additional analyses will determine the type and severity of
hearing loss and whether this is correlated with demographic or medical
variables.
Current Status: The
12 participating hospitals are enrolling infants (over 1,200 have been
enrolled) who qualify for the study based on their hearing screening
results. Two-thirds of the infants enrolled in the study so far
have reached the age when follow-up testing can be scheduled (i.e.,
audiometric assessment), and about one-third of those scheduled
have completed the hearing assessments. Data will continue to be
collected, then analyzed and disseminated upon completion.
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Efficacy of
Early Identification and Intervention for Congenital Hearing Loss
State: Hawaii
Contact: Beppie Shapiro;
Beppie@hawaii.edu
Background:
As noted by the U.S. Preventive Services Task Force, few studies present
evidence on the effectiveness of Early Hearing Detection and
Intervention (EHDI) in improving long-term outcomes for children who are
deaf and hard of hearing in the major areas of receptive and expressive
language, reading, and behavioral or social skills. The goal of the
project is to demonstrate a methodology to determine the effectiveness
of universal newborn hearing screening as the first component into the
EHDI system towards improving children's long-term outcomes in the areas
of language, academic, and social achievement. The study will also
investigate the impact of early intervention initiated before the 6
months of age, age at identification of hearing loss, type and duration
of early intervention, and other variables that may affect outcome
measures for children who are deaf and hard of hearing.
Purpose:
The purpose of this study is to investigate the effectiveness of early
identification and intervention of infants and children with hearing
loss in terms of their long-term communication, cognitive, and social
outcomes.
Design:
This study will be
conducted from 2003 through 2005. Children eligible for the study
include those (1) born during the period January 1, 1992, through
December 31, 1998; (2) who have a congenital hearing loss; and (3)
currently living in Hawaii.
A
packet, including a brochure and an invitation to join the study, will
be sent to the parents of all eligible children. Information needed as a
basis to compare the outcomes between children (such as the type and
degree of hearing loss, mode of communication, family demographics, and
age at first intervention) will be gathered first. Outcomes will be
assessed by collecting information about each child’s communication,
language, and academic levels; cognitive status; and behavioral and
social or emotional functioning. Information will be coded to ensure
identifying information about each participating child is eliminated
before any analysis is done.
Current Status:
A
protocol is currently being developed for this project. Once completed,
it will be submitted to the institutional review board (IRB) for review
and approval. If IRB approval is obtained, children will be enrolled in
the study.
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Psychological
and Family Issues
State:
Rhode Island
Contact: Betty Vohr; bvohr@wihri.org
Background:
Studies of other types of medical newborn screening have reported that
false positive results produced increased levels of parental anxiety,
and that there might be residual effects 12 to 24 months after
screening, even after the family is informed the child is well.
Purpose:
The purpose of this study is to assess the psychoemotional impact of false
positive hearing screening results on families, including the potential
for vulnerable child syndrome.
Design:
Families who are given false positive first screen results will be given
the opportunity to participate in the study and will be asked to
complete surveys and questions during an interview when their children
reach 6, 18, and 30 months of age.
Current Status:
The initial study design has been expanded to include
families of children who passed the initial hearing screen. A study
protocol has been developed and submitted to the Centers for Disease
Control and Prevention Institutional Review Board (IRB) for review and
approval.
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Assessment
of Loss to Follow-up
Coordinator:
Research Triangle Institute; James Hersey;
hersey@rti.org
States:
Contacts:
Michigan
Ann Jarrett;
jarretta@state.mi.us
Massachusetts
Janet Farrell;
janet.farrell@state.ma.us
Virginia
Pat Dewey;
pdewey@vdh.state.va.us
Utah John Eichwald;
jeichwal@doh.state.ut.us
Arkansas Millie Sanford;
msanford@HealthyArkansas.com
Background:
States and hospitals are well on the way to meeting the objective for
completing hearing screenings for all infants. However, national
data indicate that nearly half of all newborns who do not pass hearing
screening tests do not return for follow-up services (a hearing
rescreening, audiometric evaluation, and medical diagnosis). The Early
Hearing Detection and Intervention Program at Centers for Disease
Control and Prevention is currently funding 29 states and 1 territory to
assist in the development of data surveillance and tracking systems to
reduce loss to follow-up. However, there is no mechanism in place
to determine why newborns do not return for rescreening, evaluation, and
diagnosis. The purpose of this application is to hire a contractor
to conduct a multistate study with key stakeholders to identify the
barriers, identify procedures that work, and recommend solutions to
improve follow-up rates.
Purpose:
The objectives of the multistate evaluations are (1) to identify
factors or barriers responsible for loss to follow-up in EHDI programs
and (2) to develop successful and innovative strategies that will reduce
loss to follow-up.
Design:
Five states with varying rates of follow-up have agreed to participate in
the proposed evaluation. Research Triangle Institute (RTI), an outside
contractor, will work with key stakeholders in each state (such as the
advisory committee, hospitals, parents, audiologists, physicians, and
state EHDI and early intervention/Part C programs) to identify study
questions. Potential questions will focus on parent issues,
hospital policy, insurance and payment issues, audiologist involvement,
physician role and support, nonhearing-specific barriers
(transportation, child care, and mobility), and follow-up techniques and
components. One critical issue is an assessment of cultural barriers and
the adequacy of cultural competency by individuals involved in the
follow-up process.
Current Status:
RTI has been contracted to work with CDC and the
five selected states to conduct this study.
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Champion
Project
Coordinator: Michelle
Esquivel;
mzesquivel1@yahoo.com
States: All U.S. states and
territories
Background:
Physicians are an important part of the Early Hearing
Detection and Intervention (EHDI) process, but they are not always aware
of the status of EHDI programs in their states. To increase the
awareness and involvement of physicians with EHDI programs, the Centers
for Disease Control (CDC) and Prevention and the Health Resources and
Services Administration (HRSA) awarded funding to the American Academy
of Pediatrics (AAP) to implement the Improving the Effectiveness of
Newborn Hearing Screening, Diagnosis and Intervention through the
Medical Home program.
As part of this program, AAP chapters will appoint an EHDI chapter champion
to serve as the point person on EHDI-related issues at the state and
local levels.
Purpose:
Goals of this project include (1) increasing the awareness and involvement
of physicians with EHDI programs, (2) strengthening state policy related
to EHDI, and 3) engaging other medical organizations in improving EHDI
programs at the national and state levels.
Design: A
pediatrician from each AAP chapter will be identified as an EHDI chapter
champion. Champions will be encouraged to coordinate efforts between
pediatricians, health care professionals, state health departments,
hospitals, and others in an effort to help implement EHDI programs. The
AAP will also institute a Task Force on Improving the Effectiveness of
Newborn Hearing Screening, Diagnosis and Intervention that will serve as
the oversight group for this project. Members of the task force
will work with champions to ensure that state-level issues are
understood and that information about policies and programs are
communicated.
Current Status:
Nearly every AAP chapter has appointed an EHDI
chapter champion. The majority of these champions participated in the
February 2002 National Early Hearing Detection and Intervention meeting.
Champions have also begun to collaborate with state EHDI coordinators to
provide pediatric expertise. To find the name of the chapter champion in
your state, visit the AAP website at:
http://www.medicalhomeinfo.org/screening/hearing.html
(scroll down this page to download the chapter champion listing in a PDF
format).
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Sorry, we can't give you
medical advice. Please talk with your doctor for questions about
yourself or your family. For other information, please contact
ehdi@cdc.gov
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Date:
March 19, 2009
Content source: National Center on Birth Defects and Developmental
Disabilities
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