RHODE ISLAND'S CDC EHDI GRANT (2001)

GRANT ABSTRACT

Title of the Grant Project: Cooperative Agreement for Early Hearing Detection and Intervention (EHDI) Tracking, Research, and Integration
Project Title: Enhancing Rhode Island's Early Hearing Detection and Intervention (EHDI) Efforts
Organization: Rhode Island Department of Health, 3 Capitol Hill, Providence, RI 02908
Project Director: Peter R. Simon, MD, MPH telephone: 401-222-5928

The overall aim of this Level II project is to enhance the tracking, research and integration of the Early Hearing Detection and Intervention (EHDI) system. This will be accomplished primarily by improving the information systems infrastructure to support a sustainable, integrated system of screening, diagnostic and habilitation services. More specifically, project goals focus on: 1) expanding the integration of the EDHI system into public health data systems, and 2)establishing comprehensive and coordinated service and reporting systems to assure EHDI provides access to a seamless system of preventive, diagnostic and therapeutic services for all children.

Rhode Island has been a leader in the development and implementation of both newborn hearing assessment and integrated public health information systems. As the first state in the nation to mandate and implement universal newborn hearing assessment and one of the first states to create and implement an integrated pediatric public health information system, known as KIDSNET, Rhode Island has demonstrated its capacity to undertake innovative and creative projects that meet public health needs. The Rhode Island Hearing Assessment Program (RIHAP), administered by the Women and Infants' Hospital, manages all components of the statewide newborn hearing assessment program. Challenges to date for the program include: a DOS based information system, difficulty obtaining diagnostic information on children with progressive or late onset hearing loss, and inefficient coordination and communication with audiologists. KIDSNET adminsitered by the Division of Family Health, integrates information from 9 different public health programs, including RIHAP, into a single child record accessible to enrolled primary care providers. Challenges to date for KIDSNET include provider enrollment, DOS based software, duplicate data entry by newborn programs, linking records, and data quality.

This project, overseen by the RI Department of Health (HEALTH), will be a colloborative effort of HEALTH, the RIHAP program at Women and Infants' Hospital, and the RI School for the Deaf. Proposed activities will help to eliminate many of the challenges faced by both programs. Further integration, such as implementing state of the art technology, improving methods to capture data into both RITRACK and KIDSNET, providing audiologists access to KIDSNET, and enhancing coordination among newborn/early childhood programs will improve the completeness and timeliness of detecting and intervening with children diagnosed with hearing loss. Researching the pyscho-social effects on families in conjunction with CDC and other Level II recipients will be undertaken, as well as studies to better understand the genetic causes of hearing loss.

GRANT NARRATIVE

Problem and Current Status: Each year, 99% of the approximately 13,000 infants born in Rhode Island are screened for hearing loss with a 1-2% initial fail rate. Despite this successful initial screening effort, there are infants who need repeat screening or follow-up audiology but are lost to follow-up. In addition, although hospital birth records are verified against hearing screening records, there is no electronic means to assure that all infants have been screened for hearing loss. Also, little is known about parents perceptions and adaptive behaviors related to screening and follow-up or the incidence of known genetic causes of hearing loss in Rhode Island and how these relate to outcomes for infants and families.

Since the 1993 legislative session in Rhode Island mandated universal newborn hearing screening, the Rhode Island Department of Health (HEALTH) has been working to integrate the RI Hearing Assessment Program (RIHAP) with other components of newborn screening mandated under existing state or federal law. Programmatic responsibilities for newborn screening in RI (hearing, bloodspot, Part-C/Childfind) have been consolidated in the Division of Family Health, in the Office of Children's Preventive Services under our KIDSNET unit, which is working to integrate all newborn screening data (hearing, blood spot, developmental risk) in a way that facilitates communication and coordination among newborn screening programs, primary care providers (Medical Homes), and specialty care providers.

Redundant data entry at the birth hospitals occurs as a result of separate systems. Accurate matching of data from system to system presents an additional challenge. Since HEALTH's electronic birth certificate information system (VR2000) is currently undergoing significant change, an opportunity has been created to consolidate some of the data entry for hearing screening with other newborn screening activities. Demographic data will only need to be entered once, with each program adding only program specific data (i.e. hearing screening results). This data can then be downloaded to the individual program databases. Tracking and referral would continue to occur and be managed through each program. These activities position Rhode Island to improve the efficiency and effectiveness of our EHDI system through participation in this EHDI Cooperative Agreement Program.

KIDSNET is an integrated information system to support tracking and follow-up for nine mandated pediatric preventive services programs: Newborn Screening for poor developmental and health outcomes under Part C IDEA, Blood Spot Newborn Screening, RIHAP, WIC, EI, Lead Screening, Immunization, Home Visiting, and Automated Vital Statistics. KIDSNET now offers electronic access to pediatric primary care sites in RI where children receive vaccines. Newborn hearing screening data is available to primary care providers who participate in KIDSNET, yet many have indicated the system is not user friendly enough to be used for this purpose.

Currently, RITRACK, RIHAP's information system, tracks all births through a two stage screening process to referral for diagnostic audiology. RIHAP refers to EI all infants with suspected hearing loss. Audiologic follow-up is recommended for those infants passing the initial or second screen with risk factors for late onset hearing loss. Our tracking investment in RIHAP presently does not support an extension through the diagnostic process. Audiologists and otolaryngologists do not have access to RITRACK or KIDSNET. Screening hospitals have no automated systems for reporting results of initial testing to RIHAP. Diagnostic outcome information is not captured in RITRACK or KIDSNET. Ascertainment for late onset infant and toddler hearing loss is presently handled through the RI Department of Education Statewide School Screening Program. Their data is not integrated with our surveillance system and their efforts are not always coordinated with newborn screening follow-up and tracking activities.

Efforts are already under way to convene and formalize a "certified" network of audiologists committed to increasing their capacity to address infant and toddler hearing loss. Early Intervention (EI) is now exploring a new mechanism to pay for services provided by "certified" pediatric audiologists who would have additional skills and commitments for data sharing and coordination.

Under an MCHB funded project addressing pscho-emotional issues associated with the screening and diagnosis of hearing loss in a newborn, discussion with audiologists has already begun. "First Connections" is building a database of qualitative assessments examining opportunities for improving follow-up and EI effort. A pediatric Audiology Task Force has been convened to explore best practices and to increase capacity to address infant hearing loss. Ideas for additional services or changes in existing protocols may be introduced under research protocols developed as a part of this cooperative agreement.

The ability to generate enthusiasm amongst our EHDI systems stakeholders for this newborn screening effort depends on our increasing capacity for sharing information and documenting the performance of our programs and system using population-based information. The capacity of screening hospitals, RIHAP, audiologists and otolaryngologists to coordinate services after a child is referred will be enhanced by additional investments in RIHAP/EI service integration as well as information management and utilization made possible under this cooperative agreement.

Improved information system infrastructure and data management, both for EHDI and KIDSNET are crucial to developing better communication for care coordination, improved surveillance, and assurance that all newborns are screened and receive timely and complete follow-up. A powerful information system with accurate, timely information, that is user friendly is needed to address these areas of concern and build upon the strong newborn screening foundation in Rhode Island.

HEALTH is prepared to utilize the resources of this cooperative agreement to improve and enhance information systems infrastructure for a sustainable, integrated system of screening, diagnostic and habilitation services to improve communicative, cognitive and social outcomes for families and children with hearing loss. We have a commitment to a collaborative continuous quality improvement process focused on positive outcomes for parents and infants.

Program Goals and Objectives

Goal 1: To expand the integration of newborn hearing screening and follow-up data (EHDI) into public health data systems to assure that all RI children receive preventive services in a comprehensive and coordinated manner.

Objective 1A: Establish improved methods for populating RITRACK by consolidating birth hospital data entry and linking all newborn public health data including newborn hearing assessment, newborn bloodspot, newborn developmental risk assessment, and birth certificates to KIDSNET.

Objective 1B: Establish and implement the best method for birth hospitals to submit hearing screening data electronically to RIHAP.

Objective 1C: Develop an analytic plan which will use EHDI outcome data to inform policy makers and care providers on the status of newborns identified with hearing loss and to direct quality improvement efforts.

Goal 2: To establish a comprehensive service and reporting system linking HEALTH to primary and specialty medical providers, and related services (audiology, EI, social support systems) to assure a seamless system of preventive, diagnostic, and therapeutic services to children and their families.

Objective 2A: Increase the number of pediatric primary care providers participating in KIDSNET from 36% to 95%

Objective 2B: Upgrade and Web-enable RITRACK and KIDSNET software to allow both systems to be more user friendly with improved access and easy integration with each other.

Objective 2C: Connect audiologists, and otolaryngologists to KIDSNET to promote the sharing of screening, diagnostic, and care coordination information between themselves, RIHAP, EI and primary care providers to minimize loss to follow-up.

Objective 2D: Develop standardized reporting forms and implement a tracking system for identifying and diagnosing newborns with late onset or progressive hearing loss and link that system to RIHAP, KIDSNET or the birth defects registry.

Goal 3: To assure that 100% of RI children receive all newborn screening and related services including newborn hearing assessment, newborn bloodspot screening and newborn developmental risk assessment as mandated by law.

Objective 3A: Establish policies and procedures to assure that all newborns in RI receive all newborn screening services including hearing assessment.

Objective 3B: Institute a continuous quality improvement plan for all the newborn screening services including newborn hearing assessment which is based on analytic data and addresses concerns identified by parents and professionals

Objective 3C: Undertake an economic analysis of current and forecasted program costs to help reformulate state budgeting policies

Goal 4: To collaborate with a wide variety of partners both within Rhode Island and among other states, as well as with federal and national agencies to build a coordinated EHDI infrastructure, share and standardize data sets and pool data for research purposes.

Objective 4A: Improve the coordination of services among a variety of RI's newborn and early childhood programs, and their diagnostic and treatment partners to assure infants and young children with hearing loss are identified and receive comprehensive and coordinated services.

Objective 4B: Participate in collaborative relationships with other CDC Level I and II recipients as well as national organizations and federal agencies to share information, models of integration, research methods, data and other relevant matters.

Goal 5: To identify genetic causes of hearing loss

Objective 5A: To document the incidence of Connexin26 and mitochondrial gene defects and characterize the hearing loss in children with non-syndromic hearing loss

Goal 6 (Research): To identify psychosocial effects of EHDI in an effort to improve outcomes for infants and families

Objective 6A: To identify factors that influence a parent's ability to adapt his/her behaviors in a positive manner after receiving a hearing loss diagnosis for his/her infant (or toddler).

Objective 6B: To conduct a follow up study on the impact of false positive screening results on families.

Description of Program and Methodology

The Rhode Island Hearing Assessment Program (RIHAP): In 1993 Rhode Island became the first state in the nation to mandate and implement universal newborn hearing screening. RIHAP, based at Women and Infants Hospital in Providence, carries out that public mandate on a daily basis under the direction of HEALTH. The four primary goals of RIHAP include administration of universal newborn hearing screening at each of the 7 birthing hospitals in the state, facilitating early diagnostic identification of permanent hearing loss, providing smooth transitions to EI and education services, and collaborating with parents and professionals.

Following established protocols, newborns are screened by either well- trained hospital technicians or nurses, in the hospital nursery at 6 to 52 hours of age using transient evoked otoacoustic emissions (TEOAE). Infants who do not pass this screen, and have an immediate automated auditory brain stem response (AABR) screen and do not pass, now become part of the RIHAP database tracking system for follow up. Infants who pass and have a medical risk factor (Joint Committee on Infant Hearing Year 2000 Position Statement) are tracked. Infants who pass and do not have a medical risk factor are discharged from the program.

At Women and Infants' Hospital, RIHAP staff process and manage that site's screening results daily. There, patient demographic information is downloaded from the hospital database into the "RITRACK" database daily, and actual computerized screening results for any given infant are available immediately to the audiologist on site. This allows time for a rescreen before discharge only at Women and Infants' Hospital making it easier to meet the Joint Committee on Infant Hearing Year 2000 (JCIH) guidelines which state that infants who failed their initial screen will be re-screened within 1 month of hospital discharge. However, at the other six birthing hospitals, data is submitted via courier once a week on hand written forms (Appendix A) and computer disks to the central RIHAP site at Women and Infants' Hospital for data entry and a sometimes lengthy process of matching and downloading into RITRACK. The RIHAP audiologist reviews and interprets all screening results. Information system integration and electronic transmission capabilities would help to assure that RIHAP consistently meets the JCIH guidelines for timely re-screen and identification of permanent hearing loss.

RIHAP's information management system RITRACK (see appendix A) supports the program by generating reminders to families of their follow up needs, and by tracking patient access to services and outcomes for quality improvement. RIHAP also uses KIDSNET's home visiting program to encourage compliance with follow-up activities when families do not respond to RIHAP letters or phone messages.

Two committees contribute to this system of screening and follow up services: 1) The Rhode Island Infant Hearing Screening Advisory Committee is mandated to provide regulatory oversight to RIHAP. Committee membership includes representatives from audiology, pediatrics, special education, insurance, the deaf community, hospital neonatal nurseries and the Rhode Island Department of Health. A quarterly report is made to the RI Director of Health. 2) The Rhode Island Infant Hearing Screening Follow-Up Committee addresses practical issues facing parents of and professionals working with children who have hearing loss. Committee membership includes representatives from HEALTH, families with children with hearing loss, the deaf community, family physicians, pediatrics, audiologists in private practice and institutional settings, the Rhode Island Department of Education, the University of Rhode Island Communications Disorders Department and advocacy groups. The mission is to identify and address gaps in services and to ease access to services including evaluation, intervention, and habilitation for families and professionals. A subcommittee is an integral part of the RIHAP/EI Quality Review process for children identified through RIHAP with hearing loss. Recent quality improvement initiatives for the Follow-Up Committee include RIHAP's referral process to EI, EI organizational structure changes, EI staff training needs, and web site information for parents and professionals.

The Rhode Island Hearing Center is administered through the RI Department of Education and the RI School for the Deaf. The Center offers diagnostic audiologic services to all children in Rhode Island birth to age 21. It has the mandated responsibility of providing statewide school screening and screens 60-65,000 school-aged children annually. Although not required by the school health regulations, the Hearing Center is committed to providing the program to pre-K children as young as age 3 who are enrolled in nursery schools, preschools, Head Start programs and pre-kindergartens as well. During the current school year, the Hearing Center screened 1,235 children younger than 5 years. These school screenings serve as a safety net for children who are new to the system, who did not undergo newborn screening, or who demonstrate late onset or progressive hearing loss.

In January 2001, a pilot program that included the use of hand held distortion product otoacoustic emissions (6 frequencies) was initiated as the first level of screen. Advantages of using this technology included accepted performance characteristics (sensitivity of 91% to 100% and specificity of 88.5%, L. Christenson 1990), speed of screening, ease of training technicians, and the fact that there is no required behavioral response from the child, eliminating the concerns that a child understand and follow directions. Demonstrated benefits of this revised protocol include increased numbers of children screened daily, utilization of technology used in newborn screening and follow up, and potential completion of the screenings by December, reducing the number of children referred with abnormal tympanograms secondary to colds or upper respiratory infections. Data are still being collected and will be analyzed to assess the false positive and refer rates and to modify protocols.

The Hearing Center is currently investigating ways to improve its data tracking and surveillance through electronic information management systems. Until now, screening results were recorded in the child's school health records by the school nurse and paper copies were brought back to and maintained by the Center. Tracking the children who went on for diagnostic evaluations at facilities other than the Hearing Center or who required educational accommodations based on a diagnosed hearing loss was a near impossibility. Additionally, there was no immediate access to results of newborn hearing screening to know whether children were at risk or lost to follow up.

The Hearing Center is a valuable resource in Rhode Island's EHDI system, however there is no formal linkage to HEALTH, KIDSNET or RIHAP. Incorporating the school screening and follow up services into a broader electronic system will facilitate shared information between HEALTH and Education, improve monitoring/surveillance of children with late onset hearing loss to at least grade 3, and standardize reporting from the school screenings.

RI Pediatric Audiology Task Force: The RI Audiology Task Force, is comprised of hospital audiology departments, private practices and those associated with large Otolaryngologist group practices. This group has identified several key issues including the need for improved access and interagency communication and information: 1) Since audiologists are currently not participating in KIDSNET, they are not able to retrieve readily accessible information about patients such as newborn hearing screening results, participation in EI or home visiting programs, and 2) there is a need for standardized reporting forms that are clear and informative to other providers including primary care physicians and sites.

Family Guidance Program: The Family Guidance program serves children with hearing loss, birth to age 3 years in Rhode Island through a cooperative agreement between the RI Dept of Health and the RI Dept of Education. Staffed by teachers of the deaf, speech language pathologists, educational audiologists and language specialists, it offers an array of center-based and home-based services individually designed for each family and child's unique needs. Services include parent support groups, family training to develop effective communication with their child, speech-language-auditory activities, weekly sign language classes, coordination and participation in the IFSP and IEP plans. The mission is to provide families with guidance and information, which will support them in making decisions and advocating for their child. It is designed to enhance parents' competence and strives to reflect the culture, values, and social characteristics of which the family is a part.

First Connections Resource and Training Project is a RI Department of Education and RI Department of Health collaboration, supported by HRSA. The primary goal is to address parental/familial psycho-emotional needs arising from the diagnosis of hearing loss by refining Rhode Island's EHDI system into one that is well-informed, coordinated, linked and prepared to deliver expert services. Identifying parental needs and perspectives of the current system through qualitative analysis (focus groups and questionnaires), informing mental health professionals and creating integrated programmatic means to provide family support, strengthening pediatric audiologic services, and defining the medical home for children with hearing loss are key goals of the grant. Peter Simon, MD is principal investigator and Ellen Kurtzer-White, MS, CCC-A is project director. The research component of this application will interface and build on efforts initiated in First Connections.

The Rhode Island Department of Health Programs and Resources:

KIDSNET- One of the most critical resources available to this project and upon which much of the further data integration will be built is KIDSNET. KIDSNET is a population based public health data system with information on children's preventive health services with data on all Rhode Island children born on or after January 1, 1997. Currently it links data from nine different programs into one child record using a data warehouse model (Appendix B). Newborn Blood Spot results will soon be incorporated into each child's record. KIDNET allows for on-line, point-of-service access by PCP's and other authorized users.

Universal Newborn Screening for Developmental Risk- Through this program, every infant born in RI is screened for developmental risks as mandated under the Individuals with Disabilities Education Act - Part C/Child Find. Screening criteria include demographic, clinical, and psychosocial risk factors. Data is entered into a laptop at the hospital then downloaded to KIDSNET on a daily basis to create a record in KIDSNET. "At risk" babies are referred to HEALTH's Family Outreach Home Visiting Program (FOP).

Family Outreach Program) - This HEALTH program, contracted to Visiting Nurse agencies, provides home visits to families with "at risk" newborns consisting of a home assessment and preventive health education. Using a family support model, community referrals and linkages are made. At 6 to 9 months a developmental screening is conducted. If warranted, a referral is made for services. This program provides follow-up to children behind in preventive services, as well as hard to reach newborns in need of immediate services such as confirmatory newborn hearing or laboratory testing and helps assure that children are linked to a medical home.

Early Intervention (EI)- HEALTH recently expanded the number of community based agencies contracted to provide services to enhance capacity. Services, which were once provided on a regional basis, are now provided statewide by providers. These agencies work closely with the newborn screening programs and will coordinate with Comprehensive Evaluation Diagnosis Assessment Referral and Reevaluation (CEDARR) centers.

CEDARR - Development of CEDARR Family Centers is a recent initiative developed by the State's Medicaid Agency, the RI Department of Human Services (DHS). The CEDARR initiative will help focus Medicaid resources on high-risk populations through improving the appropriateness of care, supporting a more positive family centered system of care, promoting clinical excellence, improving outcomes, and promoting overall cost effectiveness for Medicaid eligible children with special needs. In addition, the CEDARR initiative will establish the means to support new and expanded services in critical areas that currently do not exist or are limited. Specialized clinical evaluations and coordination of care services will be provided. They will serve as a resource for families of children with special needs including those with hearing loss. The focus on effective case management is related to what KIDSNET does and real interagency collaboration has been part of the development.

Newborn Screening Blood Spot Program - Historically, the Blood Spot Program has not had an automated tracking system. As a participant in the New England Newborn Screening Program (NENSP), a regional newborn screening laboratory, RI's Newborn Screening staff can access NENSP's new tracking system for specimens obtained in RI. Summary screening results will be sent to KIDSNET and made available to PCP's and other authorized users through KIDSNET.

Birth Defects Surveillance - HEALTH was awarded funding by CDC to establish a birth defects surveillance system. This will provide a significant amount of data on genetic conditions and potentially hearing loss and will be developed in collaboration with KIDSNET.

Vital Records- HEALTH is reengineering its automated birth certificate system, making it an ideal time to reevaluate relationships between newborn screening data systems and Vital Records. As part of the implementation, HEALTH will integrate the data collection components of the newborn screening programs and birth certificates.

Methodology (see Appendix C for project timeline)

Goal 1, Objective A: (Consolidating data entry and linking newborn data via KIDSNET)

Initial funding to create newborn developmental risk and hearing screening data entry modules in the VR2000 system has been awarded by HRSA, Maternal Child Health Bureau. To supplement that effort, the following methods are proposed:

1) Create a workgroup with representation from each newborn screening program to develop a more efficient means of populating the RITRACK data system with newborn hearing screening results.

2) Implement integrated data entry VR2000 system within each hospital with easy access for data entry of hearing screening results. This includes coordinating newborn developmental risk assessment, birth certificate, newborn screening, and blood spot collection activities at the program level to assure results are efficiently and accurately collected, entered and linked in the data system.

Goal 1, Objective B (electronic submission of hearing screening data from outlying hospitals)

1) Each of the birthing hospitals will require updated, user-friendly, screening equipment, computers, modems, and data entry resources.

2) Hearing screen results will be electronically transmitted to the tertiary site. A duplicate paper system will be continued until outcomes review demonstrates that the electronic system is timely and accurate according to the evaluative components listed below.

Goal 1, Objective C (Analytic data plan)

1) Create a data subcommittee of the Rhode Island Infant Hearing Advisory Committee to develop a process and set priorities to identify and obtain outcome data which will be used to set standards of care for the program. This sub-committee will help to identify what data should be used, assess the quality of the data, suggest how it should be analyzed and then shared. A data usage plan outlining the above will be created.

2) Link data between the RITRACK system and KIDSNET to identify children with positive newborn hearing screens who are not linked to a medical home, who have not received other preventive services, or who are receiving and/or home visiting services.

3) Assist in the development of a continuous quality improvement plan by recommending which EHDI outcome data should be used to assess needs and measure progress.

Goal 2, Objective A (Increase # of pediatric primary care providers participating in KIDSNET)

A KIDSNET strategic planning process held during the fall of 2000, resulted in the recommendation that KIDSNET focus on getting providers online and assuring a high quality of data before linking specialty care providers or upgrading the system. In order to enroll the remaining 59% of primary care providers, the following strategies will be implemented:

1) Hire additional provider liaison (consultant) to help visit provider offices, educate physician offices about KIDSNET, facilitate the enrollment process, trouble shoot problems, and provide feedback as needed. The consultant provider liaison will work closely with the existing provider liaison and will be responsible for helping to connect specialty care providers including audiologists and otolaryngologists.

2) Work closely with the three major managed care plans in the state. Two of the three plans have approached KIDSNET asking how they can assist in getting full provider participation. Examples of ideas that have been proposed include using KIDSNET as the data source for HEDIS measures and helping practices analyze workflow to incorporate methods to send immunization data easily.

3) Target local communities for complete KIDSNET participation. KIDSNET's provider liaisons will recruit all of the providers within the same local area, trying to engage community support as a whole. This may foster a sense of group participation as well as some competition.

4) Work with Head Start agencies, daycare centers and school nurse teachers who have difficulty accessing immunization and lead screening results for school entry requirements to develop strategies for KIDSNET to support the need for this documentation. These methods are likely to be incentives for providers to join with KIDSNET.

Goal 2, Objective B (Upgrade and Web-enable RITRACK and KIDSNET)

Both RITRACK and KIDSNET are DOS based systems in great need of a software upgrade. In order to evaluate how best to do this for each system, keeping in mind that both systems need to link with each other, the new VR2000 birth certificate system, and any new system implemented at the School for the Deaf, the following steps will be taken:

1) Hire an information systems consultant to explore options for upgrading and web-enabling both systems (convert existing software, redesign software, purchase already existing software), identify pros and cons of each option, determine best methods, identify project costs, and provide recommendations for each system in a technology plan.

2) Implement the technology plans during years two through four. This would likely involve contracting with an IS implementation team to convert the systems to newer technology. The IS consultant would be retained to oversee this process.

Goal 2, Objective C (Connect audiologists, and otolaryngologists to KIDSNET)

1) Hold focus groups and planning meetings with the Audiology Task Force for input and feedback on what information they would like to retrieve through KIDSNET and/or other means and why the information is needed and to develop standardized data reports back to KIDSNET and RIHAP on diagnostic evaluations (see below), and other case management and care coordination issues.

2) Develop a data sharing policy using established KIDSNET protocols to assure that shared data is on need to know basis, conforms with programmatic, state and federal confidentiality laws, and meets KIDSNET participating program 's approval.

3) KIDSNET staff will purchase and install KIDSNET computers, modems and communications software packages in audiologists' departments and practices and train the audiologists on using the system during years three and four.

Goal 2, Objective D (Forms and tracking system for late onset/progressive hearing loss)

In addition to the need to track diagnostic and subsequent screening results (RIHAP receives approximately 80% of diagnostic ABR results), infants are identified with medical risk factors and referred for VRA follow up for possible late onset or progressive hearing loss. RIHAP receives follow-up results on approximately 30% of these children . To develop an improved tracking system:

1) RIHAP staff will work with the Audiology Task Force to develop a standard reporting form to document diagnostic hearing results including those for children with risk factors for hearing loss who passed their newborn screening test. RITRACK's testing and follow up results fields will provide components for inclusion. This will help RIHAP as well as the states birth defects registry identify children with hearing loss even if it is late onset or progressive.

2) Provide computers, KIDSNET access, and training to participating Rhode Island audiologists who see children identified for follow-up through RIHAP. Participating audiologists will be able to access information on the children they are evaluating, as well as data enter directly into KIDSNET or submit results on a form.

Goal 3, Objective A (Assure that all newborns receive all newborn screening services)

Planning and some of the initial groundwork needed to identify ways to assure universal screening occurred as part of a HRSA funded genetics planning grant and continuation funds for this effort have been awarded. To accomplish this objective, KIDSNET must cross check other newborn screening data against hearing screening results. The process is outlined below.

1) A report which identifies newborns in KIDSNET with no evidence of hearing screening is currently being tested and will be generated on an ongoing basis. Because KIDSNET is connected to the birth certificate system, infants not born in the maternity hospitals where screening is done, such as home births, can also be identified.

2) Once the exception report is in place, a KIDSNET/RIHAP workgroup will design and implement a follow-up protocol. The report will be reviewed by KIDSNET staff for accuracy and data matching problems. If a child truly appears as though they never received a hearing screen, they will be referred back to the RIHAP program for outreach and if needed to the Family Outreach Program (HEALTH's home visiting and family support program).

Goal 3, Objective B (Continuous quality improvement plan for all newborn screening)

With much of the newborn screening activities occurring in the birthing hospitals and the consolidated management, quality improvement efforts can be looked at from a broad perspective. Methods will be:

1) Identify sources of data, such as the KIDSNET exception reports on unscreened infants, which exist or could be collected to inform CQI process.

2) Identify system processes which are critical to successful screening.

3) Create and implement a CQI plan with input from RIHAP, the Hearing Screening Follow-up Committee, Rhode Island Chapters of the American Academy of Pediatric and Academy of Family Practitioners, audiologists, otolarangologists, EI, and other relevant partners.

4) Connect hearing screening CQI to the Division of Family's Health's effort to create a division wide CQI team to monitor MCH performance measures.

Goal 3, Objective C (Economic analysis of current and forecasted program costs)

RI State law authorizes HEALTH to charge hospitals a newborn hearing assessment fee. Hospitals then bill insurers for the cost of the test. HEALTH has regulatory authority to adjust the fee to reflect program costs. The collected fees are returned to the State's general treasury from which a budget allocation is given to the program. In order to sustain a comprehensive, well-coordinated and integrated EHDI system, it is important to identify the true program costs. Through this grant, we will:

1) Hire a health economist to undertake a newborn hearing screening cost analysis including screening, follow-up, tracking, administration, education, and quality assurance. Costs associated with diagnosis and treatment will be estimated separately.

2) Use the cost study as justification to adjust the State's newborn hearing assessment screening fee to reflect true costs

Goal 4, Objective A (Improve service coordination among newborn/early childhood programs)

The RI Infant Hearing follow-up committee will establish a Care Coordination sub-committee with representation from RIHAP, KIDSNET, Audiology, Otolaryngology, RI School For The Deaf, the primary care community, parents and other critical partners. The committee will meet at least quarterly and will do the following:

1) Adopt diagnostic and treatment standards

2) Develop performance indicators to monitor adherence to above standards

3) Identify methods for training relevant providers about the range of available services (home visiting, parent groups, etc), and navigating the EHDI system.

4) Establish billing procedures and agreements for audiology services

Goal 4, Objective B (Collaborate with other CDC Level I and II recipients and others)

1) Staff at Women and Infants' Hospital and the RI School For The Deaf will work closely with staff at CDC and other recipients of Level II cooperative agreements on selected research projects.

2) Project staff, especially KIDSNET staff, will continue to attend and present at national meetings focused on the integration of public health information systems, such as CDC's National Immunization Registry Conference, Data Utilization and Enhancement Grantee meetings, All Kids Count Connections Collaborative, Genetic Services Implementation Grantees meetings, Neonatal Newborn Screening Symposiums etc.

3) The Project Director, Peter Simon, MD and Research Investigators Betty Vohr, MD and Ellen Kurtzer-White, MS, will continue to participate in national committees, pediatric and medical associations, and workgroups especially those related to newborn hearing screening, Children with Special Health Care Needs, medical homes, etc.

Goal 5, Objective A (Document the incidence of Connexin26 and mitochondrial gene defects)

Connexin 26 and Mitochondrial Gene Defect Analysis:

(See appendix D for detailed methodology)

1) Families will be informed of the study and informed consent obtained.

2) DNA can be successfully extracted from buccal smears. We will obtain buccal smears from newly identified children with non-syndromic hearing loss in the first 3 years of the study, seeking to analyze the Connexin 26 and mitochondrial gene for mutations. A cheek swab will be obtained and submitted to the Center for Human Genetics.

3) Identification of a specific gene defect in the children studied will identify those children at risk of progressive hearing loss. It will also identify the cause of the hearing loss, allow the provision of accurate genetic counseling, and ascertainment of carriers in affected families.

4) Genetic counseling will be offered to all families at the RI Hospital Department of Genetics.

Research Methodology

Two distinct research questions are proposed under the goal of addressing psychological and family issues related to newborn hearing screening, diagnosis and intervention. See Appendix E for detailed research plans which will be submitted for human subjects review.

Objective 6A (Identify factors influencing adaptive parental behavior after HL diagnosis)

The first objective is to describe the expectations and intervention experiences reported by parents of young children with diagnosed hearing loss that positively impacted their sense of empowerment and enablement to create language learning environments for their child. A greater understanding of parents' perspectives on what was most useful will lead to refinement of the diagnostic process and intervention program, resulting in enhanced child and family outcomes. Because existing programs for deaf children and their families are generally designed on the basis of what experts believe they should contain, rather than on what parents wish to receive (Bernstein and Barta, 1998; Harrison, et al 1998), parental needs, expectations and desires will be investigated to increase the efficacy of EI programs (Haas and Corwley 1982) and to increase parents' sense of empowerment.

Objective 6B: (Follow up study on the impact of false positive screening results on families)

The second research objective addresses the question of the psycho-emotional impact of false positive hearing screening results on families, including the potential for vulnerable child syndrome. Studies of other types of medical newborn screening have reported that false positive results produced increased levels of parental anxiety, and that there may be residual effects 12 to 24 months after the screen, even after the family is informed the child is well. Although RIHAP's initial fail rate is laudable at 1-2%, hundreds of families are given false positive first screen results.

1) These families will be given the opportunity to participate in the study and will be asked to complete surveys, questionnaires and questions during an interview when their child reaches 6, 18 and 30 months of age.

Collaborative Efforts (see appendix F for letters of support)

Collaborative efforts to assure the quality of our EHDI system include the following:

1) Children's Cabinet: Since 1992, the Children's Cabinet has been the forum for improving collaboration and coordination of state and federally funded services to families in RI. From the Children's Cabinet collaboration has come our 1994 Medicaid Waiver, CEDARRS, and joint funding for our Family Outreach Program, the home visiting component of KIDSNET providing outreach for RIHAP. This collaboration was instrumental in the recent reorganization and expansion of EI which will enable some of the service integration between RIHAP follow-up, EI, and KIDSNET.

2) HEALTH : KIDSNET itself represents a collaboration around data sharing and service coordination between RIHAP, Blood Spot Newborn Screening, WIC, Immunization, Lead and EI Programs. These are documented in the KIDSNET Policy Manual (Appendix G). KIDSNET has established data sharing agreements with each of its participating programs and all users must sign confidentiality agreements. The Division of Family Health is preparing to integrate quality improvement activities across its categorically funded Offices in the near future using the Title V Performance indicator requiring all children with special needs to have a "Medical Home." We are partnering with our Vital Statistics Office on a redesign of our existing automated vital record and have been a part of the analytical arm of our vital statistics activities for years (MCH Data Book). Through the Parent Consultant Program, we are collaborating with the RI Parent Information Network, a U.S. Department of Education funded parent information service, to assure that our system is family centered and culturally sensitive.

3) Department of Human Services : KIDSNET is now evaluating options for data sharing agreements with Head Start at the request of the Department of Human Services.

4) Independent service providers: KIDSNET has a data sharing agreement with the HELP Lead Safe Center, a Medicaid Waiver financed "enhanced case management" provider serving families with lead poisoned children.

5) EHDI system: The RIHAP Follow-up Committee, described earlier, meets bimonthly around efforts to improve the quality of RIHAP and to identify systems barriers to quality to be addressed by HEALTH at a higher policy level. "First Connections" is an MCHB funded project that grew out of the RIHAP Follow-up Committee findings of serious gaps in access to mental health services for families of newly identified deaf infants. Under this project, efforts are already under way to expand the routine reporting of diagnostic data from audiologists. The RIHAP/Audiology Network will explore the development of certification standards for pediatric audiology within EI and will create an opportunity to enhance the financing of audiology as a part of the EI Program. Not only will this raise pediatric audiology standards within the EHDI system, it will create a financing stream to make it feasible for practitioners to acquire necessary equipment and to maintain and enhance their skills to appropriately evaluate infants and toddlers with hearing losses. The Rhode Island School for the Deaf has been providing audiologic support to RIHAP in interpreting infant hearing screenings and these programs have a long standing history of mutual collaboration and support.

6) Birthing Hospitals: RIHAP is responsible for equipping and training all screeners in the hospitals where infants are delivered in RI. Close working relationships have been established at each hospital, as they also have for newborn blood spot and developmental risk screening.

7) Medicaid Managed Care Organizations (MCOs): A prototype of collaborative continuous quality improvement is now operating to improve lead screening rates in 18 month olds.

Participants include Medicaid, each of the MCO's with 1115 Waiver populations, the local Chapter of the AAP, our Childhood Lead Poisoning Prevention Program and KIDSNET staff. Already we have seen the early fruits of this effort in the offers of assistance to KIDSNET from Neighborhood Health Plan, United Health Plans of New England and Blue Cross/Blue Shield RI.

8) Multi-site research and demonstration projects: We are willing to commit to pooling data and developing data standards collaboratively as we have in the past under our existing CDC Cooperative Agreement on Prevention of Communication Disability. We are participant's in the National Center for EHDI in Utah. Drs. Simon and Vohr have worked with Karl White continuously since their first efforts to develop Acoustic Evoked Emissions as a screening tool in the 80's. Drs. Vohr and Simon and Ellen Amore sit on the Directors Genetics Screening Advisory Task Force. Dr. Simon continues to represent RI at the New England Regional Genetics Group.

Evaluation Plan

Goal 1, Objective A (Consolidating data entry and linking newborn data via KIDSNET)

1) Interim process measures include keeping workgroup meeting minutes.

2) A written summary including options for populating the RITRACK system with hearing screening results and a recommended best option will be submitted by the workgroup chair.

3) Interim process measures on elimination of redundant data entry, and improved data linkage include a detailed design plan and successful testing of the VR2000 software.

4) Outcome measures include reduced data entry time, fewer file transfers to KIDSNET and shorter error reports (non-matched) needing manual resolution.

Goal 1, Objective B(electronic submission of hearing screening data from hospitals)

1) Time frames for installation of computers and modems will be set and met.

2) A duplicate paper system will continue until >95% of infant's records are generated by the level 1 data staff, >95% of the hearing screen data is electronically transmitted on day of screening, and RITRACK to KIDSNET records match >95% of the time.

3) Results will be entered into RITRACK within three days of the completed hearing screen. Results will be uploaded into KIDSNET within 3 days of RITRACK entry.

4) Quality Review reports will demonstrate that the JCIH guidelines for re-screen are being met.

5) Other outcome measures include reduced data entry time for screening and data staffs, significantly reduced time for rescreens to be scheduled, more "real time" availability of hearing screen information to families, physicians, and other service providers.

Goal 1, Objective C (Data usage plan)

1) The creation and activities of a data sub-committee will be monitored through meeting minutes and a roster of sub-committee members.

2) The written data usage plan will document the priorities, outcomes and standards developed by the sub-committee.

Goal 2, Objective A (Increase pediatric care providers participating in KIDSNET)

1) KIDSNET monitors rates of enrollment and system usage by physicians by reviewing the number of user ID's issued and modem bank logs.

2) The majority of providers are currently more likely to use reports and less likely to use the system for direct patient care. Periodic formal feedback from providers regarding usage especially as it relates to newborn screening data will be sought through focus groups and one-on-one interviews. Written meeting minutes will be kept.

3) Memorandum of Understanding will be developed with the three major Managed Care Organizations outlining joint efforts to rollout KIDSNET.

4) The provider liaison will monitor enrollment at the community level in order to determine if a strategy for engaging an entire community and its providers proved to be beneficial.

Goal 2, Objective B (Upgrade and Web-enable RITRACK and KIDSNET)

1) Interim measures to monitor progress toward this objective will include hiring of a contract employee, and minutes of meetings between the contract employee, KIDSNET staff, IS staff, RIHAP, the RI School for the Deaf, Physicians and their Office staff. Draft technology plans will be reviewed and modified as needed.

2) The final measure will be a complete technology plan that can be used to create an RFP to upgrade and web-enable the KIDSNET and RITRACK systems.

Goal 2, Objective C (Connect audiologists, and otolaryngologists to KIDSNET)

1) Audiology Task Force meeting minutes will document progress on determining information to be available to audiologists through KIDSNET or other means in accordance with program policies and confidentiality laws

2) Completed audiology security access and user profiles

3) Draft and then final data sharing policy will be prepared.

4) Monitor the number of audiologists trained on KIDSNET with computer access who are submitting data.

Goal 2, Objective D (Forms and tracking system for late onset/progressive hearing loss)

1) Interim evaluation measures include minutes of Audiology Task Force meetings and drafts of a standard reporting form.

2) Final reporting form completed and # of audiologists reporting the RIHAP/KIDSNET using the form will be tracked.

3) % of records with missing follow-up screening and final diagnosis will be tracked.

4) Data on hearing loss sent to RIHAP, KIDSNET and the birth defects registry.

5) # of audiologists with computers, KIDSNET access, and training who are participating in KIDSNET and submitting data will be monitored. Time frames for installation of computers, KIDSNET, and training will be set and met.

6) Initial refer and rescreen refer rate will be analyzed

7) Cross check of data entered by school screening with RIHAP and EI to determine those children in need of continued intervention or services. Quality Review process will include record and report reviews to insure that children in need of continued intervention or services are being followed.

Goal 3, Objective A (Assure that all newborns receive all newborn screening services)

1) Reports will be generated to identify 2 week-old babies who never receive a hearing screen. Numbers on the report will be tracked over time.

2) A full case review will occur to determine how and why each baby missed the screening so that systems issues can be identified and addressed.

3) Annual statistics of missed screenings will also be used to evaluate progress.

Goal 3, Objective B (Continuous quality improvement plan for all newborn screening)

1) Meeting minutes and draft CQI plans will serve as interim progress measures.

2) Once completed, a checklist of components will be used to assess progress toward complete implementation.

3) In order to monitor the effectiveness of the plan and assure improved outcomes, data for the different components will be collected and analyzed over time. Examples include reviewing number of newborns initially missed for screening, numbers of children lost to follow-up, and false positive rates.

Goal 3, Objective C (Economic analysis of current and forecasted program costs)

1) Interim measures will include contract in place with health economist for cost analysis and drafts.

2) Final analysis will be submitted in written form.

3) State's newborn hearing assessment screening fee adjusted if needed to reflect costs.

Goal 4, Objective A (Improve coordination among newborn/early childhood programs)

1) Meeting minutes will be used to document activities of the subcommittee

2) Adopted standards will be written up and shared with care providers

3) Certification standards for audiology services will be documented and implemented with polices and procedures for billing of services

4) Signed written agreements will be in place between certified audiology practices and

5) Performance indicators will be incorporated into HEALTH's Newborn screening CQI plan

6) Training opportunities will be initiated and attendance tracked.

Goal 4, Objective B (Collaborate with other CDC Level I and II recipients and others)

1) Participation in and documented exchange of information among other level I and level II recipients will be used to monitor collaborative efforts.

2) Presentations and attendance at national meetings as well as participation in national and local committees related to topics of this grant will be tracked.

Goal 5, Objective A (Document the incidence of Connexin26 and mitochondrial gene defects)

Children who have mutations in the Connexin 26 gene will be at greater risk of having progressive hearing loss.

1) Identify the incidence of Connexin 26 and mitochondrial gene defects associated with permanent hearing loss.

2) Categorical analyses and ANCOVAS to determine if children with the Connexin 26 gene defects or the mitochondrial mutations have differences in their audiograms or progression of hearing loss.

Goal 6, Objective A (Identify factors influencing adaptive parental behavior after HL diagnosis)

1) Qualitative measures including videotaped interviews and open-ended questions will be used to evaluate parental expectations and positive experiences at 3 distinct junctures of identification and intervention: the diagnosis of hearing loss; the initial IFSP; and the transition planning meeting at age 30 months.

2) Quantitative measures will also be used including the Family Empowerment Scales questionnaires by P. Koren and other questionnaires.

Goal 6 Objective B (Study on the impact of false positive screening results on families)

1) Qualitative measures will be based on videotaped parent interview over time to describe themes that emerge.

2) Quantitative data will be taken from a variety of parent surveys addressing coping and stress.

Staffing and Management System (See Appendix H for resumes)

The proposed program staff are highly experienced and have participated in the creation and implementation of KIDSNET over the last eight years. The experience gained through this process has been invaluable and will allow HEALTH to continue as a leader in the area of integrated public health information systems. KIDSNET staff has grown from 3 FTEs to 10 FTEs within the last 3-5 years. Other available resources including Robert Childs, HEALTH's Chief of Information Systems, Samara Viner-Brown, the DFH's Chief of Data and Evaluation (responsible for Birth Defect Surveillance), and several staff from the Office of Vital Records will provide in-kind support. Roles of key project personnel for whom funding is requested are described in the budget justification and in Appendix I. Additional key personnel whose efforts on this project are in kind contributions are described below and in detail in Appendix I:

HEALTH: Peter Simon, MD, Medical Director for KIDSNET, will act as project director for this proposal, bringing medical and policy leadership. William H. Hollinshead, MD, Medical Director of the Division of Family Health, will also provide leadership support. Ellen Amore, MS, Newborn Screening Manager, will work on EHDI/Newborn Screening data integration and quality assurance. Amy Zimmerman, MPH, Office Chief of Children's Preventive Services, and Deputy Chief Kim Salisbury-Keith, MBA, will also provide planning and policy leadership as well as management of KIDSNET operations and data quality components.

Women and Infants' Hospital: Betty R. Vohr, M.D., Medical Director of RIHAP, will be a key participant in developing the collaborative efforts with other states and on research design, methods, analyses and integration. Bonnie England. R.N., RIHAP Administrator, will advise on data base changes, the submission of reports, and the integration of data sets. Cheryl McDermott, CCC-A, is the audiology coordinator for RIHAP, responsible for supervision of all newborn hearing screening, interpretation of results, and reporting quality indicators to HEALTH. She will provide audiology support to the research and genetics activities and participate in the work groups and collaborative efforts with community audiologists.

RI School for the Deaf: Peter Blackwell, Principal, will provide leadership support and participate in EHDI information system integration activities. Ellen Kurtzer-White, M.S., Director of "First Connections" Project, and Mary Jane Johnson M.S., Family Guidance Program teacher, will be co-principal investigators. Mary Jane will act as a liaison to families and as a trainer/consultant for the research assistant and coder. She will participate in the core meetings. Deborah Topol, BA, a diagnostic linguist at the RI School for the Deaf, will monitor assessments and protocols coding for communicative interaction.

Organizational Structure and Facilities (see appendix J for organizational charts)

Organizational Structure at HEALTH: Within HEALTH, the Division of Family Health (DFH) is organized into five sections: Office of Children's Preventive Services (OCPS), Office for Children with Special Health Care Needs (OCSHCN), which includes EI, Office of the Supplemental Food Program for Women, Infants and Children (WIC), Adolescent and Young Adult Health Unit (AYA), and the Office of the Medical Director (OMD), which includes the Parent Consultant Program, Communication and Policy Unit, Data and Evaluation Unit, and Fiscal and Administration Unit.

The OCPS oversees the KIDSNET, RIHAP, Newborn Blood Spot Screening, Newborn Developmental Risk Assessment, Immunization, and Lead Screening Programs. Program management, administration, daily operations, and long term planning for these programs are approached in a coordinated manner and integrated where feasible. KIDNSET receives data from all of the above programs as well as EI, WIC, and the Family Outreach Home visiting program. The Office of Vital Records within the Division of Management Services also updates KIDSNET with birth certificate data.

One aspect of KIDSNET, which does not reside in the Division of Family Health, is technical oversight of the information system itself. HEALTH has a centralized Office for Information Systems (OIS) located in the Division of Management Services. Information System staff are assigned to KIDSNET. This arrangement keeps the program aspects in the Division where that expertise lies while allowing the technical components to be overseen by individuals with information system expertise.

Organizational Structure of RIHAP and Women and Infants Hospital:

Women and Infants Hospital is part of the Care New England system of care providing services to families in Rhode Island, southeastern Massachusetts and eastern Connecticut and is committed to providing comprehensive women's and newborn health care. The Rhode Island Hearing Assessment Program (RIHAP) is part of Women and Infants Hospital's Department of Pediatrics, and is directed by HEALTH to manage the state-wide newborn hearing screening program. See attached RIHAP organizational chart "Administrative Staff". RIHAP staff are directly responsible to the RIHAP Medical Director, the Chief of the Department of Pediatrics, and to hospital administration.

Newborn hearing screening is provided at each of the seven Rhode Island birthing hospitals by each hospital's own trained staff. However, only the RIHAP staff based at Women and Infants manage the screening data and follow up procedures for infants who failed or missed their hearing screens, or for infants with medical risk factors who will be followed after hospital discharge. In a collaborative effort, the RIHAP Medical Director and program administrators provide screening equipment and supplies, and training and support to the hearing screening staffs at all of Rhode Island's birthing hospitals. Additionally, all hospitals receive regular quality review reports and are included in the program's quality improvement initiatives. Ultimately, hearing screen data from all Rhode Island newborns is provided to the Department of Health through the state-wide KIDSNET data system.

Organizational Structure of the Rhode Island School for the Deaf: The Rhode Island School for the Deaf is part of the RI Department of Education and provides education for children with hearing loss from birth though high school. As described earlier, the Family Guidance Center, the Hearing Center and the First Connections program are based at the RI School for the Deaf.

Human Subjects Review

Research involving human subjects will comply with DHHS regulations. Active assurances for this project include Rhode Island Department of Health Multiple Project Assurance #1481, Women and Infants' Hospital Federalwide Assurance #IRB00000746.

These are important studies which will provide new information associated with false positives, a diagnosis of hearing loss, and factors which contribute to optimal outcomes for the family. The risks involved are minimal. Dr. Vohr has had extensive experience conducting longitudinal clinical studies on high risk populations of infants and children and has a strong support team. She has been the coordinator for NICHD Neonatal Research Network Follow-up studies since 1997 and successfully met the requirements for certification in Human Subjects Protection in Research in accordance with the National Institutes of Health mandate (see appendix K). Project activities related to human subjects are as follows:

1) During years 1 and 2 we will enroll 50 infants who have false positive hearing screen results.

During years 1,2,3, and 4 it is estimated that approximately 20 newborns with hearing will be enrolled through the Family Guidance Program. A total of 80 infants with hearing loss will be enrolled during the study. A full time research assistant trained by Dr. Vohr and the program coordinator will obtain informed consents in the hospital or at the Family Guidance Program working in collaboration with Mary Jane Johnson, the Director of the Parent Guidance Program and Ellen Kurtzer-White. Parents of newly diagnosed infants will be approached by the audiologist at RIHAP. If the parent agrees, the research assistant will provide additional information and obtain informed consent. Separate informed consents will be submitted to the IRB at WIH for False/positives, children with hearing loss, and genetic studies. Three copies will be signed: one for the parent, one for the hospital record and one for the research file. An interpreter will be used as needed. A separate informed consent will be used for the Connexin 26 and mitochondrial gene study and will be offered only to families with a child identified with non-syndromic HL. We estimate 50% male gender and 20-25% minority (see Appendix L).

2) Assessments of children will include diagnostic hearing tests, mother infant interaction, and behavioral assessments. If any evidence of behavior problems, abuse or neglect is identified, the appropriate referrals will be made.

3) Families of children with HL will participate in focus groups and complete standard interviews and questionnaires.

4) Children with non-syndromic hearing loss may have an assessment for the Connexin 26 and mitochondrial gene defects if the parents provide permission. Parents may opt not to receive feedback on the genetic testing. The DNA will be obtained from buccal smears. We have experience with this methodology. There is minimal discomfort associated with a buccal smear and we do not anticipate any problems. At the most, there may be a tiny drop of blood.

5) The Family Resource Scale, Family Support Scale, Impact on Family, Parenting Stress Inventory, CDI and Child Behavior Check List will be administered to the mothers. We have experience with all of these questionnaires. The impact on the Family of HL will be administered to families with a child with HL.

6) All records will be kept confidential with the use of unique identifiers. Parents must sign for release of all confidential information. No identifying information will be included on any reports. Parents who agree to have the genetic studies will be referred for genetic counseling. Data is entered in a room which is locked when not in use. All study data collected will be entered on a weekly basis and there will be a weekly staff meeting to monitor the accuracy of procedures, protocols, and data collection. Access to data is granted to authorized users and requires a log-in password. All data used for publications or presentations will not use patient names. Only group data analyses are used.