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Early Hearing Detection & Intervention (EHDI) Program

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Research Projects

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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