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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:
November 01, 2007
Content source: National Center on Birth Defects and Developmental
Disabilities
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