Centers for Disease Control & Prevention EHDI Grants (2000):
Washington

GRANT ABSTRACT
• Grant Narrative

GRANT NARRATIVE

A. Understanding the Problem and Current Status

Hearing impairment is one of the most common major abnormalities present at birth. Nationally, 1 to 3 out of every 1000 children will be born with a hearing loss. ^1,2,3,4 For Washington, that translates into approximately 80-239 children born each year with significant hearing loss. Without newborn hearing screening, several studies have reported that the median age of identification of hearing loss is between 12-25 months.^5,6,7 Children identified late with hearing loss experience developmental delays that place them at risk for life-long disadvantage. According to the Washington State Department of Social and Health Services, 65% of the deaf population in Washington lives at or below the federal poverty level. In addition, the unemployment rate for deaf high school graduates is twice that of their hearing peers.

It has been clearly demonstrated that when hearing loss is detected early and infants receive appropriate and timely intervention, they can develop age-appropriate language and developmental skills, similar to those children with full hearing capabilities. The effectiveness of early intervention, coupled with recent technologic advances that have made screening for congenital hearing loss effective, reliable, and affordable, has resulted in numerous organizations recommending early hearing screening followed by appropriate diagnostic and intervention services. Specifically, Healthy People 2010 includes an objective to "increase the proportion of newborns who are screened for hearing loss by age 1 month, have audiologic evaluation by age 3 months, and are enrolled in appropriate intervention services by age 6 months." In Washington, this process of screening newborns for hearing loss, providing diagnostic confirmation for those newborns with suspected hearing loss, and initiating early intervention for those newborns with confirmed hearing loss is collectively referred to as early hearing-loss detection, diagnosis, and intervention (EHDDI).

Current Status: EHDDI Efforts in Washington State

In March 1998, a Resolution was passed in the Washington State House of Representatives recognizing the importance of hearing screening for all children within the first three months of life. Prior to the passage of this Resolution, the Department of Health (DOH) commissioned a statewide needs assessment on EHDDI. The needs assessment revealed that as many as 76% of children identified with congenital hearing-loss are not identified before two years of age and/or are not receiving early intervention services. (Refer to Appendix B for Needs Assessment)

Following the needs assessment report and the passage of the Resolution, DOH convened an EHDDI Task Force comprised of thirty representatives from professional organizations, the Washington State Hospital Association, other state and local education, health, and social service agencies, audiologists, and advocacy groups. The EHDDI Task Force generated a report to the legislature in which numerous recommendations were made. These included that "every baby born in Washington State be screened for hearing-loss by the age of three months using objective technology," that further studies be done to identify existing services and/or gaps in services for children with hearing loss, and to provide necessary training, education, and coordination to ensure that children with hearing loss have access to appropriate intervention services. (Refer to Appendix C for Task Force Report)

In 1999, the Washington State Legislature included a budget proviso of $100,000 for DOH to further promote EHDDI statewide. To this end, DOH has undertaken the following activities:

• Listserve: A listserve was established to enhance communication about EHDDI throughout Washington State. Over 125 members have been added to this discussion group, including local representatives from the Infant Toddler Early Intervention Program (the IDEA, Part C program) and the Children with Special Health Care Needs (CSHCN) program, members of the former EHDDI Task Force, physicians, audiologists, and other hospital staff. Additional members can be added on an ongoing basis. (Refer to Appendix D for examples of the types of communications shared through the listserve)
• Educational materials: DOH and the Washington State Hospital Association mailed educational materials developed by the National Center for Hearing Assessment and Management about setting up EHDDI programs to the appropriate staff members at all birthing hospitals in the state.
• Reimbursement: Discussions are underway with the Washington State Health Officer, Dr. Maxine Hayes, regarding how to educate public and private health care payers about the importance of newborn hearing screening and follow-up services for children identified with congenital hearing-loss and to explore possible reimbursement options.

As of the end of 1999, 14 of 71 birthing hospitals were screening newborns for hearing loss prior to hospital discharge. Six hospitals were screening all newborns for hearing loss, while eight were screening high risk infants and/or infants in the neonatal intensive care unit. In total, only 7.3% of the 79,640 births in Washington State last year were screened for hearing loss prior to hospital discharge.

However, four additional hospitals have since implemented universal newborn hearing screening programs in 2000. (Refer to Appendix E for map indicating hospitals currently screening) With these additions, and with the expectation that others will soon follow, it’s expected that at least 30% of newborns will be screened for hearing loss prior to hospital discharge by the end of calendar year 2000. Hospitals currently screening newborns for hearing loss have attempted to assure that appropriate diagnostic and intervention services are received. Some use commercially-available tracking software or databases developed in-house, and others use the manual "paper trail" method. However, there is currently no statewide tracking system in Washington.

The availability of audiologic and intervention services varies across Washington State, due to the large and diverse geographic area and the Cascade Mountain Range that divides the largely rural eastern part of the state from the more populated western part. Two programs, both located in the western half of the state where the three most populated counties are located, provide comprehensive audiological and intervention services for infants and toddlers with hearing loss and their families. In other areas of the state, families must rely on the limited services available locally or travel to Western Washington.

Approximately 53% of the school districts in Washington offer special education programs to children under the age of three. Two of the districts provide birth-to-three services specifically for children with hearing loss. Children with special needs may also be served through developmental disabilities centers, neuromuscular programs, regional genetic clinics, and other programs. Eligible children may also receive early intervention services through the Infant Toddler Early Intervention Program (ITEIP, the IDEA, Part C Program) or services through the Children with Special Health Care Needs Program, both organized at the county level.

Washington State hopes to achieve statewide universal newborn hearing screening, coordinated with appropriate diagnosis and intervention, for all infants by 2002. Efforts in both the public and private sector to develop a coordinated EHDDI infrastructure will allow this goal to be achieved.

The Washington State Department of Health (DOH)

DOH recently purchased five Transient Evoked Otoacoustic Emissions (TEOAE) screening units that will be given to hospitals through a Request for Partners (RFP) process. Hospitals outlining a sustainable program with plans for ensuring that all three components of an EHDDI program are addressed-- detection, diagnosis, and intervention—will be given the screening equipment. (Refer to Appendix F for a draft of the Request for Partners application)

DOH also plans to convene three workgroups to define "best practices" in the areas of detection, diagnosis, and intervention. The first workgroup will be composed of hospital staff and physicians involved in current and planned universal newborn screening programs, and this group will be charged with developing "best practices" for screening protocols and setting standards for referral/false positive rates. The second workgroup will include pediatric audiologists and will be charged with developing standards for diagnostic evaluation. The third workgroup will be composed of individuals who are responsible for the provision of intervention services for infants with hearing loss and consumers. This group will be responsible for developing "best practices" in the area of intervention, including the determination of what types of referrals should be made (e.g. to ITEIP, genetics, support groups, etc.) Convening such workgroups is expected to enhance the EHDDI infrastructure in Washington State. Furthermore, all three sets of best practices will help define data elements to be collected through the tracking system.

Lion’s Sight & Hearing Foundation of Washington & Northern Idaho(LSHF)/ Children’s Hospital & Regional Medical Center (CHRMC)

In the private sector, the Lion’s Sight and Hearing Foundation of Washington and Northern Idaho has funded Susan Norton, PhD, CCC-A of Children’s Hospital & Regional Medical Center (CHRMC) in Seattle to provide direct technical assistance to hospitals in implementing EHDDI programs. Dr. Norton is also planning statewide workshops on various topics, including: (1) early intervention services for infants 0-12 months with hearing loss and (2) hearing aid fitting for infants and toddlers. Additionally, CHRMC has recently conducted a survey of audiologists in an effort to compile a list of pediatric audiologists qualified to perform diagnostic evaluation for infants referred from screening. LSHF and CHRMC are significant partners with DOH in building a coordinated EHDDI infrastructure in Washington.

The implementation of EHDDI within Washington State is admittedly in its early phases. If awarded, this proposal will allow DOH to develop a coordinated and standardized system for tracking the status and progress of infants through the three components of an EHDDI system. As CHRMC continues to assist hospitals in establishing screening programs, the tracking system developed through DOH will enable for monitoring trends and geographic clusters of hearing loss. It will be possible to determine false positive rates, loss to follow up rates, and differences between hospitals.

Challenges to Developing An EHDDI Tracking & Surveillance System

Implementing an EHDDI Tracking & Surveillance System is not without challenges. Some of the anticipated challenges include:

• Participation: hearing screening is not mandated in Washington. Therefore, all hospital reporting to the EHDDI Tracking & Surveillance System will be voluntary.
• Informed consent: since hearing screening is not mandated in Washington, DOH can only collect data from and disclose information to health care providers and facilities involved in the care of the infant (Washington State Health Care Information Access and Disclosure Act, Chapter 70.02.050 Revised Code of Washington). DOH cannot provide information to individuals not involved in the care of an infant (i.e. potential audiologists or interventionists) without informed consent. In addition, DOH cannot contact parents directly without informed consent.
• Reporting protocols: some states have documented that hospitals report screening information only on those infants screened so that the rate of screening appears higher than it actually is. Reporting on every infant, regardless of whether he/she was screened, is necessary to understand the number of children not being screened. Reporting protocols must be clearly understood, so that reporting is complete.
• Loss to follow up: loss to follow up is one of the largest problems for any tracking system, and is especially problematic for this type of tracking system since data on three components must be collected: detection, diagnosis, and intervention. Efforts to continuously improve the EHDDI infrastructure to assure that infants receive coordinated services in the appropriate time frames will be necessary for combating loss to follow up.
• Missing data elements: when reports are received, it is likely that some data will be missing. Missing demographic data elements may prohibit multiple reports on a particular infant to be linked. DOH must either attempt to obtain the missing data, which will be difficult and time consuming, and/or take into account the missing data during data analyses.
• Unclear definitions: reported data must be accurate. Data elements must be established, agreed upon, and clearly defined for all reporters.
• Ascertainment of late onset or progressive hearing loss: methods of collecting data on late onset or progressive hearing loss need to be developed; such methods have not been addressed.

While all of these factors present significant challenges to a successful EHDDI Tracking & Surveillance System, DOH’s prior experience in maintaining tracking systems will be of great benefit in overcoming these challenges.

DOH’s Office of Newborn Screening (NBS) has maintained a successful tracking system since 1977. NBS monitors the entire population to assure that all newborns are screened for four disorders--Congenital Hypthryoidism (CH), Congenital Adrenal Hyperplasia (CAH), Phenylketunuria (PKU), and hemoglobinopathies (such as Sickle Cell)-- and immediately follows up on abnormal screening results so that affected infants receive prompt diagnosis and appropriate treatment. For the past 23 years, over 99% of infants born in Washington have been screened by the blood spot screening program, and all affected infants have been diagnosed and treated in a timely fashion. The NBS program also evaluates the short and long term outcomes and provides communication and education regarding the program.

The success of this program has resulted from years of hard work in implementing several quality assurance measures, and establishing relationships with hospitals, physicians, and other people involved in the birthing process (i.e. midwifes). Hospitals not only send blood spots for every infant born in the state to DOH for testing on a daily basis, but they send birth rosters of every baby born in that particular hospital on a weekly basis. This allows NBS to match birth rosters to samples received and ensure that all infants are screened. If a child is listed on a birth roster but no sample exists, the NBS Program contacts the hospital to request that the infant’s providers collect a sample. NBS reinforces the program’s success by providing quarterly and annual reports to all birthing hospitals indicating their performance.

NBS has also successfully educated midwives of the importance of newborn screening, so even infants of home births (1% of all births) are screened. Additionally, NBS has been successful in getting physicians to send a specimen to DOH for a recommended, but not required, routine second newborn screen for infants between 7 and 14 days of age. Currently about 90% of infants receive the recommended second screen.

EHDDI is sufficiently different from routine newborn screening so that the existing NBS tracking system will not work perfectly for EHDDI Tracking & Surveillance. However, the EHDDI Tracking and Surveillance System will be integrated with NBS Tracking, since both rely on hospitals to provide initial screening data. In addition to integration with NBS tracking, the EHDDI Tracking and Surveillance System will include an easy-to-use web-based component for audiologists to provide data to DOH on the latter two EHDDI components—diagnosis and intervention. Once the web-based site is established, it can be easily adapted to allow for the collection of data concerning children identified with late onset or progressive hearing loss.

The integration of EHDDI with NBS tracking will take place during Phase I of system development. The creation of the web-based reporting component will occur during Phase II of system development. The "lessons learned" from existing NBS tracking will be extremely valuable in development of the EHDDI Tracking & Surveillance System.

B. Goals & Objectives

The following goals and objectives describe the nature, scope and structure of the web-based tracking system integrated with the existing newborn blood spot screening program for EHDDI proposed for Washington State. There are four broad goals, each containing measurable process and outcome objectives that characterize the performance and impact of the project.

1. Develop an EHDDI Tracking & Surveillance System in Washington State that is integrated with the existing NBS tracking system and includes a web-based reporting component for diagnostic evaluation and intervention referral information.
   1. By October 15, 2000, implement a contract for the development of the EHDDI Tracking & Surveillance System.
   2. By June 1, 2001, complete development and testing of Phase I of the EHDDI Tracking & Surveillance System with at least five birthing hospitals (identified through RFP process).
   3. By August 1, 2001, implement Phase I of the EHDDI Tracking & Surveillance System.
   4. By November 1, 2001 (Year 02), complete development and testing of Phase II of the EHDDI Tracking & Surveillance System with at least one diagnostic center.
   5. By January 1, 2002 (Year 02) implement Phase II of the EHDDI Tracking & Surveillance System.
   6. By April 1, 2004 (Year 04) prepare an article for publication which describes the EHDDI Tracking & Surveillance System development process, and includes initial findings and recommendations.

2. Initiate and improve statewide reporting for EHDDI that includes each of the three newborn screening components – detection, diagnosis and intervention.
   1. By November 1, 2000, establish a directory of birthing hospitals and physicians currently performing newborn hearing screening. (to be performed quarterly)
   2. By December 1, 2000, finalize recommendations from the "best practice" workgroups and disseminate to stakeholders broadly.
   3. By January 1, 2001, develop draft addendum to newborn screening blood spot collection card for the purposes of obtaining information about hearing screening and obtain input from nurses and technicians currently in charge of universal newborn hearing screening programs about proposed addendum.
   4. By February 1, 2001 develop a list of diagnostic centers across the state, based upon the CHRMC audiologist survey and "best practice" findings.
   5. By March 1, 2001, finalize an addendum to the state’s NBS blood spot card for collecting data on newborn hearing screening.
   6. By April 1, 2001, develop reporting procedures for hospitals and physicians screening.
   7. By June 1, 2001 disseminate addendum, reporting procedures, "best practice" recommendations, and list of diagnostic centers to all hospitals in the state to and physicians screening (well-child/second screens).
   8. By July 1, 2001, utilize the newborn screening physician database to send all pediatricians throughout the state reporting procedures, "best practice" recommendations, and a list of diagnostic centers.
   9. By September 1, 2001 (Year 02), develop reporting procedures for diagnostic centers.
   10. By December 1, 2001 (Year 02), provide training and technical assistance to all eligible pediatric audiologists at diagnostic centers about reporting procedures through the state web-based EHDDI tracking system and their role in ensuring a high quality EHDDI tracking system.
   11. By October 1, 2002 (Year 03) convene a workgroup to make recommendations regarding data collection for infants/children with late onset or progressive hearing loss and how these data can be most effectively analyzed and used.

3. Assure the timeliness and quality of detection, diagnosis, and referral to intervention services initially for those infants born in hospitals that provide screening and ultimately statewide as screening becomes universal.
   1. By June 1, 2001, develop a preliminary DOH follow-up protocol for infants (1) referred from screening for a second screen; (2) referred from screening for diagnostic evaluation; (3) not screened. (will be revised on an on-going basis as necessary)
   2. By January 1, 2002 (Year 02), determine by hospital: false positive rates; % of newborns screened/missed, % newborns referred for diagnostic confirmation; % seen for diagnostic evaluation by three months of age (if possible at this time); % infants with confirmed hearing loss and % referred to intervention services (if possible at this time). (to be performed and reported to hospitals quarterly).
   3. By May 1, 2002 (Year 02), determine by diagnostic center: % of infants seen for diagnostic confirmation by three months of age, % of infants with confirmed hearing loss, and % of infants referred to intervention by six months of age. (to be performed and reported to diagnostic centers quarterly)
   4. By August 1, 2002 (Year 02) compare the EHDDI tracking system data related to newborns receiving screening with the EHDDI tracking data related to older children identified with hearing-loss to determine if additional or targeted public approaches may be indicated (e.g., if most two year olds being diagnosed come from an area with one large birthing center that does not perform universal newborn hearing screening, then enhanced efforts to persuade this institution to initiate universal newborn hearing screening would ensue). (to be performed quarterly)
   5. By March 1, 2003 (Year 03), develop the first EHDDI Tracking & Surveillance System report with an evaluation of progress and initial data findings, and disseminate to hospitals, physicians, pediatric audiologists at diagnostic centers, and other stakeholders. (to be performed annually)

4. Evaluate reporter and consumer satisfaction with the EHDDI Tracking & Surveillance System.
   1. By January 1, 2002 (Year 02) determine the overall percentage of hospitals and diagnostic centers reporting (to be performed annually)
   2. By June 1, 2002 (Year 02) conduct interviews/focus groups with hospital staff, physicians, pediatric audiologists regarding the web-based EHDDI tracking system (i.e., user friendliness, ease of reporting, etc.)
   3. By June 1, 2003 (Year 03), conduct a parent satisfaction survey of at least 20% of parents who had a child undergo universal newborn hearing screening and of whom at least half were referred for diagnostic confirmation services to determine parent satisfaction with the tracking system and services received.

C. Description of Program & Methodology

1. Develop an EHDDI Tracking & Surveillance System in Washington State that is integrated with the existing NBS tracking system and includes a web-based reporting component for the diagnostic confirmation and intervention referral information.

   This proposal utilizes current processes of the NBS program to obtain information about screening prior to hospital discharge. Additionally, a web-based reporting component will be developed that diagnostic centers will use to report data about the infants referred for diagnostic evaluation and referral to appropriate intervention. This approach is expected to maximize the success and sustainability of the system. Details about proposed system development can be found in Appendix G, the EHDDI Tracking & Surveillance System Concept Plan .

   The proposed web-based component will build upon the DOH Perimeter Security Project (PSP). The PSP, scheduled to be complete by October 2000, is being designed to support secure Internet and web-based applications. Components of the project include: (1) the development of a secured network communications infrastructure connecting DOH to other State of Washington agencies, hospitals, clinics, and others; (2) agency firewalls and demilitarized zone; and (3) an intrusion detection system. Therefore, information submitted to DOH via the web will be protected by this new extremely confidential and secure infrastructure.

   DOH anticipates sharing information about the development process, along with initial findings and recommendations, by preparing an article for publication in the fourth year of the grant.

2. Initiate and improve statewide reporting for EHDDI that includes each of the three newborn screening components – detection, diagnosis and intervention.

   An addendum to the current NBS blood spot collection card will be utilized for reporting about hearing screening. Elements on the addendum will likely include: screening results--whether the infant passed, needs a repeat screen, was referred for audiologic evauation, or refused screening (by ear); as well as type of test administered--Auditory Brainstem Response (ABR), Transient Evoked Otoacoustic Emissions (TEOAE), Distortion Product Otoacoustic Emissions (DPOAE). The current NBS blood spot collection card is included in Appendix H.

   In the first quarter and every quarter thereafter, a list of all birthing hospitals/physicians conducting hearing screening will be established. Hospitals will receive the addendum to the NBS blood spot collection card and will be encouraged to begin reporting. Facilities where a two stage screening protocol is used will be identified, and given additional "addendums" for the purpose of reporting hearing screening. In some cases, the second screen is being performed at a hospital, but in a few cases, it is being performed at the first well-child visit. In the latter case, physicians will need to be mailed "addendums." The NBS Program already has a database of physicians that can easily be utilized for these purposes.

   A list of pediatric audiologists who are qualified to perform diagnostic evaluations is currently being compiled by the LSHF/CHRMC EHDDI project. This list may be updated as the "best practices" for diagnosis are completed by an audiologist workgroup in the first quarter. It is anticipated that there will be approximately ten to twelve qualified diagnostic centers across the state. During the second year, training and technical assistance will be provided to all diagnostic centers in the state about reporting through the web-based component. Specific data elements to be collected will likely include: diagnostic tests performed; type and degree of hearing loss; whether amplification was fit; and type of early intervention services the child was referred to. The "best practice" workgroups in diagnosis and intervention are expected to further define data elements to be collected. Furthermore, input from the CDC will be incorporated into the system.

   In the third year, a workgroup of audiologists will be convened to discuss mechanisms for ascertaining infants/children with late onset or progressive hearing loss within the EHDDI Tracking & Surveillance System. It’s anticipated that simple adaptations to the EHDDI Tracking & Surveillance System to enable the collection of these data can be initiated.

3. Assure the timeliness and quality of detection, diagnosis, and referral to intervention services initially for those infants born in hospitals that provide screening and ultimately statewide as screening becomes universal.

   In the first quarter, the Office of Newborn Screening will identify a follow-up coordinator whose work will include EHDDI follow-up. For a complete description of the anticipated operation of the EHDDI Tracking & Surveillance System to assure this goal is attained, refer to the "Operational Scenarios" section of the EHDDI Tracking & Surveillance System Concept Plan in Appendix G.

   During the second year, hospitals and diagnostic centers will be given feedback regarding their performance (i.e screening rates, false positive rates, # of diagnostic evaluations performed). Feedback has proven very successful in boosting hospital performance for blood spot screening and is expected to do the same for newborn hearing screening.

   Data analysis will also be performed for an annual report that will be disseminated broadly across the state. The data will identify areas for systematic improvement, and DOH will work with its collaborators to make the necessary improvements to the EHDDI infrastructure.

   Refer to the Evaluation Plan for a more detailed description of data collection and analysis for quarterly performance reports and the annual report.

4. 4. Evaluate user and consumer satisfaction with the EHDDI Tracking & Surveillance System.

   At the end of the first year and annually thereafter, reporter participation will be assessed. Focus groups and/or interviews with users of the tracking system (hospital staff, physicians, pediatric audiologists) will be performed to assess satisfaction with the system and processes. Consumers (parents) will also be surveyed to identify areas for systematic improvement. Refer to the Evaluation Plan for a more complete description of these activities.

   A detailed workplan and timeline outlining the activities that will be undertaken to achieve each of the above four goals and corresponding objectives is included in Appendix I.

D. Evaluation Plan

Monitoring of the EHDDI Tracking System Project Activities

During Year 01 of the EHDDI Tracking System project, staff will be monitoring activities as outlined in the work plan. Monitoring will be done to determine if the activities are being done on time, for their degree of completeness, and to ensure high quality work has been performed. EHDDI project staff will also anticipate sharing the results of monitoring efforts and/or conducting further evaluation efforts during the anticipated annual CDC site visit.

Evaluating the Success of the EHDDI Tracking System

Inherent in any useful data or tracking system is the ability to accomplish the stated goals. With EHDDI, the ultimate goal is to be able to ensure that all newborns are screened for hearing loss and that those identified with probable hearing-loss receive diagnostic confirmation and are linked to the appropriate intervention services. However, to reach these goals and objectives, there are several additional areas that need to be assessed.

Quantitative measures will include: participation of hospitals and diagnostic centers; completeness of reporting; referral and false positive rates; and the percent of infants receiving detection, diagnosis, and intervention services within recommended time frames. Performance in these areas will be compared to standards developed by the "best practices" workgroups. For example, the "best practices" workgroups for detection and/or diagnosis will develop acceptable referral/false positive rates.

Qualitative measures will include input from users of the system (hospitals, physicians, nurse midwives, and diagnostic centers) and those affected by the system (parents). A specific timeline for evaluation activities is included below:

• By January 1, 2002 (Year 02), and annually thereafter, determine the overall percentage of hospitals and diagnostic centers reporting.
• By January 1, 2002 (Year 02), determine by hospital: false positive rates; % of newborns screened/missed, % newborns referred for diagnostic confirmation; % seen for diagnostic evaluation by three months of age (if possible at this time); % infants with confirmed hearing loss and % referred in intervention services (if possible at this time). (to be performed and reported to hospitals quarterly).
• By May 1, 2002 (Year 02), determine by diagnostic center: % of infants seen for diagnostic confirmation by three months of age; % of infants with confirmed hearing loss; and % of infants referred to intervention by six months of age. (to be performed and reported to diagnostic centers quarterly)
• By June 1, 2002 (Year 02), conduct interviews/focus group with hospital staff, physicians, pediatric audiologists regarding the web-based EHDDI tracking system (i.e., user friendliness, ease of reporting, etc.)
• By August 1, 2002 (Year 02), compare the EHDDI tracking system data related to newborns receiving screening with the EHDDI tracking data related to older children identified with hearing-loss to determine if additional or targeted public approaches may be indicated (e.g., if most two year olds being diagnosed come from an area with one large birthing center that does not perform universal newborn hearing screening, then enhanced efforts to persuade this institution to initiate universal newborn hearing screening would ensue).
• By January 1, 2003 (Year 03), determine the completeness of the reporting by participating hospitals (i.e., number of babies born, % who received hearing screening, % not reported, commonly missed data elements, etc.) and incorporate these findings in the NBS annual report on hospital performance.
• By January 1, 2003 (Year 03), determine the % of newborns statewide who were screened prior to one month of age, the % of newborns referred for diagnostic confirmation by an audiologist by three months of age, and the % of infants enrolled in intervention services by six months of age (to be performed and reported annually).
• By June 1, 2002 (Year 03), conduct a parent satisfaction survey of at least 20% of parents who had a child undergo universal newborn hearing screening and of whom at least half were referred for diagnostic confirmation services to determine parent satisfaction with the tracking system and services received.

E. Collaborative Efforts

Collaboration with stakeholders will take place primarily through two channels:

"Best Practice" Workgroups

Three workgroups will be formed to identify "best practices" in the area of screening, diagnosis, and intervention. The "best practices" will assist in defining the data elements to be collected.

• The first workgroup will be comprised of hospital staff involved in current or planned newborn hearing screening programs as well as physicians. This group will be responsible for making recommendations about appropriate screening protocols and setting standards for referral/false positive rates. As much as possible, the DOH responsibilities will replicate existing NBS protocols.
• The second workgroup will be comprised of audiologists who will be charged with developing "best practices" in the area of diagnosis. These audiologists will represent several facilities and geographic locations, and will play a key role in enhancing communication about these issues in their own communities throughout the state.

• A third workgroup will be comprised of individuals with expertise and experience in the area of intervention, who will be responsible for developing "best practices" in the area of intervention. These individuals will also represent several organizations and agencies, including consumer support groups.

EHDDI WA Listserve

Communication about the EHDDI Tracking and Surveillance System will also occur through the EHDDI Washington listserve that was developed in 1999. This email discussion group has over 125 members, including hospital administrators and program staff, professional organization representatives, audiologists, state agency contacts, and interventionists in the public and private sector. The listserve is maintained by a staff member in the Genetic Services Section and members can be added on an on-going basis. It is expected that information about this project will be conveyed through the listserve, and that feedback will also be obtained in this manner.

In addition, DOH communicates informally with many key collaborators, providing updates for newsletters or community meetings.

Refer to Appendix J for key EHDDI collaborators that have indicated support for the development of an EHDDI Tracking & Surveillance System. All of these hospitals, diagnostic centers, organizations, and agencies will be involved in the channels of collaboration described above.

F. Staffing and Management System

For the successful implementation and completion of the project, staff from two separate offices within DOH will be utilized: (1) the Genetic Services Section, part of the Division of Community and Family Health; (2) the Office of Newborn Screening, part of the Division of Epidemiology, Health Statistics and Public Health Laboratories. Furthermore, staff from the DOH Division of Information Resource Management (DIRM) will provide consultation to project staff in the contracting, development, testing, and maintenance stages of the EHDDI Surveillance and Tracking System. Leadership for this project will reside with the Genetic Services Section.

Within the Genetic Services Section:

• Debra Lochner Doyle, MS, CGC is the Manager of the Genetic Services Section and will be the Principal Investigator. Ms. Doyle has been involved with several successful HRSA and CDC grants, and brings to the project extensive experience in project management. She is currently the Principal Investigator on a CDC funded project entitled State Capacity Projects for Assessing and Preventing Secondary Conditions Associated with Disability and Promoting the Health of Persons with Disabilities.

• Sarah Forquer, MPH, a Health Services Consultant in the Genetic Services Section, will be the EHDDI Liaison. Ms. Forquer has been involved in early hearing loss detection, diagnosis, and intervention activities at the DOH for the past year. Ms. Forquer established and maintains the EHDDI WA listserve and has represented DOH on this issue at state and community meetings. She has demonstrated experience in systems development, data management, and contract management through her work in the areas of birth defects surveillance, and quality assurance of Washington State’s Regional Genetic Clinics.

• A Project Coordinator will be hired to work within the Genetic Services Section. The project coordinator will carry out the daily tasks associated with the development and maintenance of the EHDDI Tracking and Surveillance System. He/she will be required to have good organization, communication, and data management skills. Prior experience in grants management and a background in audiology or communication sciences will be preferred.

Within the Office of Newborn Screening:

• Mike Glass, Director of the Office of Newborn Screening, will be a Project Consultant. Mr. Glass has over 10 years experience in managing a successful screening and tracking program. He will provide consultation regarding communication with data reporters, follow-up protocols, and maintaining a tracking and surveillance database.

• A Health Program Specialist 2 within the Office of Newborn Screening will be identified to provide follow-up for infants identified through the EHDDI Tracking and Surveillance System. This person will be familiar with follow-up protocols for blood spot screening, and will bring experience in communicating with hospitals and physicians to obtain necessary data and to ensure timely and appropriate intervention services.

(Refer to Appendix K for CVs and/or job descriptions for positions that do not yet have a person hired or assigned.)

G. Organizational Location of the EHDDI Project within DOH

This project will be a collaborative effort between the Genetic Services Section and the Office of Newborn Screening. Both offices’ staff have a long track record of working collaboratively.

Prior to 1994, the newborn screening follow-up program staff were part of the Genetic Services Section. A re-organization occurred and NBS follow-up staff were joined with the NBS laboratory staff to unify the program. However, the NBS follow-up staff and the Genetic Services Section staff have continued to partner on many projects and in many forums. For example, in the 1998 legislative session, DOH initiated efforts to revise the state’s newborn screening laws. The Genetics Services Section was asked to be the lead but relied heavily on the expertise and input from NBS staff. In addition, GSS staff attend the Clinical Laboratory Advisory Committee meetings and NBS staff attend the Genetic Advisory Committee meetings. Finally, staff from both offices frequently exchange emails, phone calls and other forms of correspondence in an effort to ensure maximum communication and collaboration.

DOH recognizes that not all congenital hearing loss is genetic or hereditary. In addition, the department is very sensitive to the fact that some people may erroneously fear that the location of the EHDDI project within the GSS may suggest a desire to "prevent" hearing loss (i.e., fear eugenic motivations). The truth, however, is that the GSS Mission is to "ensure access to high quality comprehensive genetic health care for all Washington residents," and genetic health care is broadly defined to include all aspects of health care required for an individual or family with, or at risk of, a genetic disorder or birth defect. It is the philosophy of the Genetic Services Section staff that genetic services can be useful for secondary and tertiary prevention efforts and include much more than prenatal diagnosis.

It is also worth noting that approximately 60-68% of non-syndromic congenital hearing loss can be attributed to a genetic etiology. But even within the group that can be attributed to a syndrome, multiple differential diagnoses are seen. GSS staff are accustomed to working with many different conditions and illnesses that impact people and families and working with people of all ages and racial/ethnic backgrounds since genetic disorders know no boundaries. As a result, GSS staff are familiar with the myriad of existing intervention services statewide.

Both the GSS and the NBS offices are located in the greater Seattle area while the majority of DOH staff are located in Olympia. Past needs assessment efforts to identify existing speech and hearing related services in the state reveal that the majority of services (clinical and advocacy) are located in the Seattle area. In addition, nearly 60% of the state’s population reside along the I-5 corridor between Snohomish and Pierce County (Everett, Seattle, and Tacoma). Therefore, DOH anticipates that the majority of the constituents and advisors to the project will live in or around (within 45 miles) the Seattle area.

The GSS office will relocate to a larger office in closer proximity to the SeaTac Airport in September of 2000. The new GSS office will not only afford the program with additional staff space but will also include additional meeting space. A shuttle service is expected to operate from the new office to the airport (approximately 8 minutes away) which will facilitate ease in meetings with constituents and stakeholders from around the state (i.e., they can be flown into SeaTac, picked up at the airport, delivered to the meeting location/office space, and returned for their flight home following the meeting). With this in mind, housing the project with the GSS located in the Seattle area should serve to be more cost effective to the program over its five year term. For these reasons collectively, DOH feels that the project would best be executed through the GSS.

(Refer to Appendix L for organizational charts for DOH and the project.)

Accessibilty of Project Facilities and Services to Persons with Disabilities

DOH policies and procedures are in compliance with the Americans with Disabilities Act of 1990, Section 503 and 504 of te Rehabilitation Act of 1973 and the Washington State Laws Against Discrimination, regarding employment related reasonable accomodation, equal access and non-discrimination for people with disabilities.

Diversity of Workforce

DOH is an equal opportunity employer and will not discriminate against anyone because of race, creed, color, sex, age, religion, national origin, marital status, sexual orientation, disability, or disabled Vietnam era veteran’s status.

H. Human Subjects Review

The State of Washington will not be conducting any activities that will put human subjects at risk during Year 01 of this cooperative agreement. Thus, no applications are required to the State, Department of Health/Department of Social and Health Services jointly sponsored Institutional Review Board. Past surveys of parents regarding their knowledge or satisfaction with state newborn screening practices have been deemed "program evaluation" by the DOH/DSHS IRB and as such have been excluded from the usual review application and approval process. If during Year 01, or in subsequent years of the cooperative agreement, human subjects review is required, applications will be submitted to the appropriate DOH/DSHS review board. The project PI will keep CDC informed of any activities that may require human subjects review.

Endnotes

¹ Northern, J., Downs, M. 1994. Universal screening for infant hearing impairment: Necessary, beneficial, and justifiable. Audiology Today, 1994; 6:10-13.

² Mayer, C., Witte, J., Hildmann, A., Hennecke, K., Schunck, K., Mauk, K., Franke, U., Fahnstich, H.m Rabe, H., Rosssi, R., Hartmann, S., Gortner, L. Pediatrics, 1999; 40: 900-904.

³ Mason, J., Herrmann, K. Universal infant hearing screening by automated auditory brainstem response measurement. Pediatrics, 1998; 101: 221-228.

⁴ Mehl, A., Thomson, V. Newborn hearing screening: the great omission. Pediatrics, 1998; 101 (1) URL: http://www.pediatrics.org/cgi/content/full/101/1/e4

⁵ Elssman, S., Matkin, N., Sabo, M. Early identification of congenital sensory neural hearing impairment. The Hearing Journal, 1987; 40: 13-18.

⁶ Harrison, M., Roush, J. Age of suspicion, identification, and intervention for infants and young children with hearing loss: a national study. Ear and Hearing, 1996; 17: 56-62

⁷ Mace, A., Wallace, K., Whan, M., Stelmachowic, P. 1991. Relevant factors in the identification of hearing loss. Ear and Hearing, 1991; 12: 287-93.