Centers for Disease Control & Prevention EHDI Grants (2000):
Hawai'i

GRANT ABSTRACT
• Grant Narrative

The Hawai`i Department of Health (DOH) is currently funded for "Tracking, Integration and Research for Early Screening, Assessment and Intervention" (EASI) for Level I activities, and requests additional funds for Level II research activities. The DOH meets eligibility criteria of having a centralized, electronic, newborn hearing screening database with approximately 88% of all live births in the state, and a Hawaii birth rate of approximately 17,500 per year.

A. EHDI tracking and surveillance program

Hawaii's Newborn Hearing Screening Program (NHSP) began under a 1990 law which mandated the DOH to develop methodology to establish a statewide program and develop guidelines for screening, diagnosis, and monitoring of infants and children to age 3 years with hearing loss. Screening began in two hospitals in 1992, was provided in all birthing facilities by 1999, and is now standard medical practice for newborn care in Hawaii. The newborn hearing screening law was strengthened in 2001 with amendments that mandated screening for all newborn infants, reporting of screening results by birthing facilities to the DOH, and adoption of rules for screening, evaluation, and intervention by the DOH.

Early Hearing Detection and Intervention (EHDI) tracking and surveillance is essential in working toward the Healthy People 2010 Objective 28-11 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 early intervention services by age 6 months.

1. Challenges, barriers, and problems. The status of the EHDI tracking/surveillance system is described in Section IIIA. Major challenges include:

• The current system is labor-intensive, requires substantial manual effort to update and maintain, and has a problem of some inaccuracy in manual data entry.
• There is substantial duplication at hospitals in entry of similar information for newborn hearing screening, newborn metabolic screening, birth certificates, and other data systems.
• Multiple data systems (birth certificate, newborn hearing screening, newborn metabolic screening, early intervention) have information on the same child and are not linked. Problems with linkage include different timing for entry and transmission of information, confidentiality concerns, and proprietary systems.
• Hawaii's present EHDI tracking/surveillance database is limited in usefulness because it does not allow the ready analysis of data for certain purposes, such as formulation of outcome/ performance measures or for epidemiological studies.

2. Need for funding. No additional funds for the EHDI tracking and surveillance program is requested, since Hawaii has funding from the CDC EHDI Level I cooperative agreement.

3. Target population. All 15 birth hospitals provide newborn hearing screening. Data for 2000 showed that of the 17,592 births, 97.7% were screened, 91.1% passed hospital initial screening, and 97.4% completed the screening/identification process. Data also showed that 84.4% who needed rescreening returned, 1.1% were referred for audiological evaluation, and 60 infants were identified with hearing loss. See Appendix B for the newborn hearing screening protocol. Informed consent is not required for screening, but refusals must be documented.

4. Status of the current EHDI tracking and surveillance system. See Section IIIA.

5. Diagnostic facilities and intervention services. Most audiologists (and all equipment) needed for complete infant audiological assessment are available only on Oahu. Infants/children from the Neighbor Islands who need audiology assessments have to travel to Oahu, with the state NHSP providing assistance for airfare and transportation as needed. Audiologists are available on Neighbor Islands for amplification.

The Early Intervention Section (EIS) assures that infants and toddlers age 0-3 years who are developmentally delayed receive appropriate early intervention (EI) services as specified under Part C of the Individuals with Disabilities Education Act (IDEA). Statewide services include: information and referral, care coordination/family support, EI services (including audiology, nutrition, vision services, special education, speech language, occupational therapy, physical therapy, social work, psychological services, assistive technology), and respite services. EI services are available on all islands. Services are provided for all levels and types of disabilities, including hearing loss.

6.Integration of EHDI tracking/surveillance system with other newborn screening program activities. See Section IIIA.

B. Genetic and other causes of hearing loss

About 1 in 500 infants have congenital mild to profound hearing loss (prelingual hearing loss). Genetic causes account for at least 50% of prelingual hearing loss. Of these, approximately 80% are non-syndromic and are inherited in an autosomal recessive manner. Of the non-syndromic recessive hearing loss, approximately 50% are caused by mutations of a single gene, the GJB2 gene encoding the connexin 26 protein. Thus, mutations in the connexin 26 gene account for approximately 20% of all prelingual hearing loss and are a common cause of prelingual hearing loss.

There are other genetic causes. Approximately 70 different disease genes producing hearing loss have been either mapped or cloned. Several genes causing syndromic hearing loss (e.g., Wardenburg syndrome and some forms of Usher syndrome) have been identified.

Non-genetic acquired causes include: in utero infection such as cytomegalovirus, craniofacial anomalies, hyperbilirubinemia, ototoxic medications, and other causes.

For a family of a child with hearing loss, knowing whether the cause is genetic may help them to understand the risks in future pregnancies.

A study by Cohn (1999) suggested that for newborns with confirmed hearing loss, connexin 26 testing may help in defining a group in which approximately 60% have severe-profound hearing loss and require aggressive language intervention.

The prevalence of different mutations of the connexin 26 may depend on the population, e.g., as shown for American Jews (see Cohn 1999) and Koreans (Park 2000).

Studying genetic and other causes of hearing loss in children identified through newborn hearing screening in Hawaii will contribute to the CDC national study of rates of genetic causes of hearing loss in this population. Identifying ethnic differences may help in determining whether a genetic evaluation for hearing loss should be done for all children identified through newborn hearing screening, or whether it should be done on a targeted basis. Hawaii has a unique multi-ethnic population - of the resident births in 2000, the three largest groups were: Native Hawaiian or Other Pacific Islander-42.1%, Asian-34.4%, White-16.1%. The Native Hawaiian birth population was 35.6%.

C. Effectiveness of early identification and intervention for children with hearing loss

Two published studies by Yoshinaga-Itano (1995, 1998) and Mueller (2000) demonstrated that infants who were deaf or hard-of-hearing, who were identified early and provided intervention, demonstrated higher levels of language acquisition than did infants who were identified later. In these studies, all children received services in specialized group settings.

In rural areas and in some isolated states like Hawaii, a specialized setting is not financially feasible due to the small number of children with hearing loss. Services in Hawaii are frequently provided in non-specialized settings by interventionists who work with children who have all types of disabilities. These interventionists have not been trained specifically to work with children with hearing loss and their backgrounds, knowledge-bases, and approaches vary widely.

Data from other sources (Hatfield N, 2001) suggest that early identification of hearing loss in itself does not guarantee positive outcomes. EI services provided to families of early-identified infants must be designed to meet the individualized needs of the child and family, be provided by qualified specialists, and be accessible to the family. The Joint Committee on Infant Hearing Year 2000 Position Statement describes six principles of effective early intervention: developmental timing, program intensity, direct learning, program breadth and flexibility, recognition of individual differences, and environmental support and family involvement.

Evaluation data from one of Washington State's two regional specialized programs for families with deaf/hard of hearing infants and toddlers have yielded consistent differences in outcomes for children depending on the 'intensity' of services received and level of family involvement (Hatfield N, 2001). Specialized services offered to all families included audiological follow-up and hearing aid fitting, individual habilitation sessions offered in families' homes or at the center, parent support groups, communication play groups for toddlers and siblings, family sign language classes, and individual parent counseling. Families enrolled in the program lived in an area immediately surrounding the program, as well as outlying areas up to a 90-minute drive distance. Analysis of service histories for the 80-85% of children who showed "normal" development, with those of children who did not reach age-appropriate levels, indicated that the intensity of services received by the two groups differed. For families who had one or fewer "contacts per week", children's receptive/expressive language skills lagged behind their chronological age or cognitive level. This correlation between service history and children's language performance was maintained for families living in both urban or rural areas, with positive outcomes for children of families who participated in many program activities, and poorer outcomes for children of families who were unable to participate. These data suggest that even when comprehensive services are offered and provided by a highly trained and experienced staff, there are differences in outcomes for children related to service intensity. This is likely to hold true regardless how early a child's hearing loss is identified.

Hawaii's EI services are provided in "non-specialized" settings for children with any level and type of developmental disability, including hearing loss. Hawaii has 12 years of experience in providing EI services under Part C of IDEA; expansion of EI services in home and community settings as part of the Part C focus on providing services in "natural environments" with non-disabled children; and is comparable to other rural areas in the nation which have challenges inherent in providing appropriate services to a widely-dispersed population for a low-incidence condition. The results of this study will contribute to the knowledge of the effectiveness of EI in a non-specialized setting for young children with hearing loss identified through newborn hearing screening. This data may help in state and national efforts to improve outcomes.

SECTION II. GOALS AND OBJECTIVES

See Table 1.

SECTION III. DESCRIPTION OF PROGRAM AND METHODOLOGY

A. Level I activities

Activities #1-6 below are part of the integrated efforts of the DOH/FHSD/CSHNB Early Intervention Section (EIS), Newborn Hearing Screening Program (NHSP), Newborn Metabolic Screening Program (NBMSP), Hawaii Early Childhood Hearing Detection and Intervention (HECHDI) Project, and the EASI-I Project (CDC Level I grant) for infrastructure development to support integrated data and tracking systems. Activities #7 and 8 are specific for EASI-I.

1. Implementation of newborn hearing screening tracking and surveillance system. Status: Hawaii has used a statewide data management system called HI*TRACK since 1995. HI*TRACK is a DOS-based software developed by the National Center for Hearing Assessment and Management at Utah State University. Hospital newborn hearing screening programs record all births, hearing screening results, and audiological testing for children who failed their hearing screening. Twice a month hospitals transfer their data, via floppy disk, to the State where data are merged into a single cumulative database. Every six months, the NHSP staff cross check state and local data for accuracy. The NHSP is able to generate reports on hospital performance that is used to prove feedback to hospitals regarding their performance.

Fourteen of 15 birthing facilities (accounting for 88.6% of births) report their newborn hearing screening results to the NHSP. To increase to 100% reporting, the DOH/CSHNB successfully proposed and advocated for an amendment to the newborn hearing screening law which required birthing facilities to report screening results to the DOH.

Activities in progress: a) NHSP will be testing the Windows-based upgrade to HI*TRACK, install it at all hospitals, provide training on its use, and assess the capacity of the upgraded system to resolve current problems including reporting and querying capabilities. b) Accuracy of newborn hearing screening database is being ascertained through comparison with newborn metabolic screening, birth certificate, and stand-alone HI*TRACK hospital databases. c) Fields relating to hearing assessment process completion and hearing status will be incorporated in the new EI management information system (MIS) currently under development. d) Administrative rules for the amended newborn hearing screening law are being written..

2. Methods of populating the database. Status: To investigate the possibility of linking HI*TRACK and birth certificate databases, a retrospective linkage was done. A deterministic matching procedure used the variables of child's last name, child's birthdate, mother's last name, mother's first name, birthing facility code, birth time. Five separate merged files were made using different variables, and then combined into a final matched file. The 1999 newborn hearing screening database had a 83% successful match, and the 2000 database had a 80% match. Activities in progress: The benefits and difficulties of utilizing newborn metabolic screening data to populate the newborn hearing screening database are being investigated. This integration may increase accuracy of data since the newborn metabolic screening database seems to have more accurate names, birthdates, and items. However, the timing for screening and transmission of the two databases are different. Other possibilities are using the electronic birth certificate application file to populate the newborn hearing screening database or continuing retrospective linkages to establish comprehensiveness of newborn hearing screening coverage.

3. Standardizing data from multiple sources. Status: Hawaii has a standardized newborn hearing screening database, with all birthing hospitals except one using HI*TRACK. Audiological follow up reports are sent to NHSP where data is entered into HI*TRACK. NHSP staff make referrals for EI services and enter referral information into HI*TRACK. HI*TRACK does not permit tracking of EI services. Activities in progress: To track EI services,, NHSP anticipates accessing information on services and developmental outcomes from the new EI MIS.

4. Reporting system. Status: To minimize loss to follow-up, a NHSP study is in progress, with the assistance of the MCH Epidemiologist (see abstract in Appendix G). Examination of 1999 data indicated that approximately 12.9% of infants who did not pass initial hearing screening did not return for rescreening. To determine a demographic risk profile of infants who did not return for follow-up, 1999 HI*TRACK and birth certificate file were linked. Birth weight, education, and ethnic origin of mother were found to be significantly related to completion status (p<0.05). Another activity involves the EIS H-KISS information/referral system to identify children referred for speech/language or other developmental concerns, who have not completed the newborn hearing screening process. Activities in progress: NHSP and H-KISS staff are developing procedures to routinely capture data on children who did not complete needed follow-up.

5. Methods for collecting data on infants/children with late onset or progressive hearing loss. Status: The NHSP has a system to track children with risk factors for late onset hearing loss. The risk criteria are based on the JCIH 1994 and 2000 position statements. If a child has a risk factor(s), the hospital NHSP staff record risk factors in HI*TRACK and send hearing screen results to the state NHSP where a filing system is used to track dates of the next audiological follow up. Follow-up is done either by the child's physician or NHSP to arrange audiological testing.

6. Analytic plan. Status: The newborn hearing screening database is periodically analyzed for percent of infants screened, referred, and evaluated; differences in newborn hearing screening performance between participating birthing hospitals; and lost to follow-up rates. See Appendix G for the 1999 and 2000 summary reports. This analysis has been useful in identifying problems or needed supports for individual hospitals and statewide. Activities in progress: The new EI MIS will make it possible to determine the percent of infants with hearing loss enrolled in EI programs. Future routine analyses of the newborn hearing screening database will include false positive rates, and characteristics of infants with hearing loss, including demographics, residence, hospital, time.

7. Documenting concerns. This is a major EASI I activity. Status: Initial screening often produces false positive results, which may alarm parents. High false positive rates may make physicians less determined to ensure patients get follow-up assessments. Currently these concerns are not systematically documented. Activities in progress: To document parent concerns about newborn hearing screening, EASI I is working with the MCH Epidemiologist to add questions to the Pregnancy Risk Assessment and Monitoring System (PRAMS) survey in 2002. PRAMS is a research project conducted by the DOH/Maternal and Child Health Branch, with support from CDC. The survey, sent to a small number of women who recently had a baby, collects information about their experiences during pregnancy and the first few months after delivery. Draft questions for the PRAMS survey (see Appendix G) will be piloted and results analyzed by Fall 2001.

8. Integrating EHDI data with other data systems. This is a major EASI-I activity. Status: The newborn hearing screening (NHS) and newborn metabolic screening (NMS) data systems are not linked with each other or with other service programs such as early intervention (EI) programs, Public Health Nursing (PHN), or the Children with Special Health Needs Program (CSHNP). Care coordinators may be unaware of hearing or metabolic conditions which require specific follow-up. Research reflecting service usage and outcomes of identified populations is virtually impossible. Activities in progress: An EASI-I goal is to "facilitate referral, care coordination, tracking, surveillance, program monitoring and research by integrating data systems across target programs and linking with Birth Registry data if feasible". EASI-I will integrate information from NHS, NMS, PHN, and CSHNP to ensure that EI-eligible children are monitored for developmental delays and that EI and other providers are aware of their clients' other needs and services. The EASI-I goals, objectives, and activities are in Appendix G.

B. Plan for a study of genetic and other causes of hearing loss

EASI II will investigate the current follow-up protocols used for the evaluation of genetic and other causes of hearing loss. One of the protocols we will be considering is the one currently used by the Utah Department of Health CDC Level II project (see the protocol in Appendix C). We will also be working with the other Level II recipients and the CDC consultants to determine the model protocol to be used for genetic follow-up. In general, the plan, which is subject to change, is:

1. Purpose. The purpose is to determine the etiology of prelingual hearing loss in children identified through newborn hearing screening. Infants with unilateral or bilateral hearing loss of any degree (mild to profound) will be evaluated. In newborns where there is no identified cause for the hearing loss, testing for mutations of the connexin 26 gene and other relevant genes (to be determined when the final protocol is developed) will be offered and performed.

2. Patient Selection Criteria: Infants identified through the newborn hearing screening will be classified as affected with some degree of hearing loss by the DOH NHSP Coordinator. This will be approximately 50 children/year. Once a child has been confirmed as having a hearing loss, the family will be notified by their primary care provider about the option to participate in this study. The age range for eligibility will be newborn to 2 years.

3. Design. The design of the protocol will be determined after consultation with other Level II recipients and the CDC. It is anticipated that our process on how participants enter and proceed through their study will be similar to that of the Utah Department of Health. In the Utah design, after evaluation, a child is determined to have a known acquired cause, recognizable syndrome, or no evidence of cause or syndrome. All families without a well-established etiology, including autosomal dominant and autosomal recessive cases, will be offered connexin 26 mutation testing (genotyping). If connexin 26 shows no mutation, the lab will proceed to test the mitochondrial mutations associated with non-syndromic hearing loss.

4. Study Procedures. The genetic counselor will review medical records, obtain the family pedigree, and provide information about the genetics of hearing loss and the research protocol. The physical examination, additional review of relevant information, and evaluation will be performed by the clinical geneticist. Specimens will be collected and mailed by a designated local laboratory service which is available statewide. DNA analysis will be performed by a laboratory selected on the basis of range of services and feasibility. The University of Utah and University of Iowa DNA laboratories have offered to perform the connexin 26 mutation testing for Hawaii. A follow-up genetic counseling session will be arranged with the family to discuss results, risks for future pregnancies and other family members, and answer any questions or concerns. Laboratory results will be conveyed to the primary care physician only with the family's consent.

5. Data Analysis and Interpretation. Data analysis will use data which has been stripped of participant names and contact information. The subjects will be classified according to cause of prelingual hearing loss, i.e., acquired, genetic, or unidentified. Frequency of the connexin 26 mutation and other mutations, if additional testing is done, in subjects who fall into the established pedigree pattern and the unidentified cause will be determined.

6. Collaboration. We will collaborate with other Level II recipients to develop multi-site research questions and analytic guidelines, and modify the project as necessary in order to allow a pool of standardized anonymized data.

C. Plan for a study on the effectiveness of early identification and intervention in a non-specialized setting for children with hearing loss

1. Conceptual model. The study's conceptual model of direct outcomes, supporting outcomes, and direct and risk factors affecting outcomes is shown in Appendix D. The three direct outcomes of EI services for children with hearing loss are communication acquisition, pre-literacy skills, and social-emotional development. Supporting outcomes include auditory skills, parent/caregiver involvement in the early intervention process, parent knowledge about issues surrounding hearing loss and intervention, and child's overall development. Outcomes are affected by EI services, including type, frequency, provider knowledge and skills, and setting. Family/demographic factors (e.g., parental education, income, ethnicity, language, transportation, etc.) and child factors (e.g., type and degree of hearing loss, mode of communication, other special needs, cognitive ability, etc.) also influence outcomes.

2. Participants. All families of infants and toddlers who are identified through Hawaii newborn hearing screening as having a sensorineural hearing loss and receiving EI services under Part C will be eligible for the study, provided that they are age 1.0, 2.0, or 3.0 years (+ 1 month) at the time of assessment. Children who pass newborn hearing screening and are later diagnosed as having a hearing loss are excluded from the study. An estimated 200 children will be enrolled, based on a prevalence of congenital sensorineural hearing loss of approximately 3/1,000 births, 17,000 births per year, and an estimated 80% enrollment of children with hearing loss in EI services. Length of enrollment in the study will vary according to birthdate and enrollment date.

3. Data collection. Assessment of outcomes and supports will be done during a 3 year period. See Figure 1 for methods of assessment, including criterion-referenced tests, scales, and checklists. Assessments will be done within one month before or after a child's birthday. Speech language pathologists, special education teachers, and occupational therapists (both EI program staff and contracted persons) will do the formal assessments. The child's care coordinator or Research Coordinator will assist in completing other assessments and obtaining other family/child information. Information about a child's EI services will be obtained from a staff log of services, fee-for-service provider records sent to EI programs, and/or data in the new EI MIS. Both prospective data and retrospective data will be gathered.

4. Statistical Analysis. All analyses will be conducted using the Statistical Analysis System version 8.0 (SAS Institute, Cary, NC 1999). Probability ("p") values less than .05 will be considered significant. Descriptive statistics for 1) tests of child skills and development, and for services, stratified by age at testing and 2) for all parent variables, will be reported.

Without an experimental or quasi-experimental design, and with the range of family characteristics and child medical/health conditions expected for this population, determining the effectiveness of intervention must relay on case control methods, i.e., using each child as his or her own control (Campbell et al., 1998). All children will have experienced at least six months of intervention by their first birthday, so measures specific to this study will not be available pre-intervention. However, many programs administer the HELP at admission, so this test will be available both pre- and post- intervention. Measuring the impact of intervention on development is difficult due to maturation effects which are not linear in relation to age. The best (though imperfect) statistic yet developed for this situation (Proportional Change Index or Predicted Performance Score) is a comparison of the ratio of developmental age to chronological age at enrollment, with the ratio of the increase in development over the study period to the change in chronological age ( Wolery, 1983; Hauser-Cram and Kraus, 1991). This statistic is based on the simplifying assumption that pre-enrollment development was linear, at least for periods corresponding to the length of intervention, and that this rate of development would continue over time without intervention. The difference between expected developmental age at a later time based on this ratio, with measured developmental age, will be analyzed over the group of children using a t test for paired measures.

Pairwise correlations between outcomes and supporting variables, outcomes and independent variables, and supporting variables and independent variables, will be reported. To further examine the relative impact of independent and supporting variables on outcomes, regression and/or path analysis will be performed for variables, or categories of variable values, which meet the requirements for this type of analysis and within which sufficient numbers of cases are found to provide power of .8 at the .05 level of significance.

5. Expected benefits of this study. The results of this study will contribute to the knowledge on the effectiveness of early intervention for young children with hearing loss, and will be useful in improving the EHDI service system. Outcomes for young children who receive early intervention services within non-specialty settings will be determined. Key factors that influence outcomes will be identified, and information may be used in refining EI services.

6. Collaboration. We are will to willing to collaborate with other Level II recipients to develop multi-site research questions and analytic guidelines, and modify the project as necessary.

SECTION IV. COLLABORATIVE EFFORTS

Collaboration with other CDC Level II grantees. The EASI-II will collaborate with other CDC EHDI Level II grant recipients and other agencies to develop multi-state research projects that allows for anonymized pooling of standardized data and to develop analytic guidelines. We will appreciate any CDC technical assistance for the research projects.

Advisory Committee. The existing EASI Advisory Committee will advise EASI-II. Members currently include: parents of children with hearing loss, American Academy of Pediatrics Hawaii Chapter, Center for Disabilities Studies, EI programs, Hawaii Center for the Deaf and Blind, Gallaudet University Regional Center, Hawaii Kids Count, DOH/Public Health Nursing Branch, DOH/Genetics Program, and pediatric audiologists. The Hawaii Academy of Audiology and Hawaii Speech, Language and Hearing Association will be added. The Advisory Committee will provide input on the development of the research projects during Year 1, be updated on progress during Years 2 and 3, and provide input on project findings during Year 4.

Genetic research project. Collaboration will include:

- A current Level II grantee is the Utah Department of Health (UDOH) Project with the UDOH Division of Community and Family Health Services and Utah State University. We will use their assistance in developing our genetic protocol. Dr. John Carey is Professor of Pediatrics, Department of Pediatrics, Division of Medical Genetics, University of Utah. He will assist the project during Year 1, by providing continuing education for physicians, audiologists, and other providers, and providing training specific for hearing loss to the project's genetic counselor. He will provide genetic evaluations for children enrolled in study until Hawaii has an alternate geneticist, and will help in the project to transition to the new geneticist.

• The American Academy of Pediatrics Hawaii Chapter and other professional organizations will be provided with information about referring children with hearing loss to the project.
• With CSHNB, the University of Hawaii/School of Medicine/Department of Pediatrics is leading an effort to establish interim genetic services with geneticists from other institutions periodically practicing in Hawaii. The Department of Pediatrics is also working on establishing a full-time clinical geneticist position.
• Within CSHNB, the State Genetics Coordinator Sylvia Au, a board certified genetic counselor, will participate in developing of the genetics research protocol with CDC and other Level II grantees including Utah.
• The NHSP will participate in the recruitment of participants for the genetics project.

Early intervention research project. Collaboration will include:

• Claudia Canady, Ph.D, is a tenured faculty of the Division of Speech Pathology and Audiology, School of Medicine, University of Hawaii, with a specialty in child language development. She will assist in developing the project design and protocols, and will assist with the final interpretation of results. She will develop a training video on the assessment tools (Rosetti, REEL, PLS, McArthur, HELP) for EI program speech language pathologists, special education teachers, and others; review completed assessment forms and provide feedback, and provide training to the Research Coordinator on reviewing assessments.
• Nancy Hatfield, Ph.D., Director of Training and Early Childhood, Washington Sensory Disabilities Services, Washington, has extensive experience in developing programs and service delivery systems for birth-to-three year olds with hearing loss, their families, and service providers. In January 2001, she completed an evaluation of Hawaii EI services for families of infants and toddlers with hearing loss, and provided recommendations on processes, services, training, and capacity-building. For the research project, she will assist in finalizing the protocol, procedures, and data analysis plan. At the end of study, she will assist in interpreting data and developing recommendations for services in a non-specialized setting for children with hearing loss.
• Early Intervention Section (EIS) of CSHNB/DOH. With the assistance of EIS Supervisor, state and state-contracted private early intervention programs will participate in the project planning and implementation. The EI program managers will be convened during the first 6 months of the project to provide input to the research design and implementation details.

Coordinated EHDI infrastructure. The DOH Family Health Services Division (FHSD has received funds for different aspects of the EHDI infrastructure and linkage with other systems. Principal Investigators and Project Directors are aware of each other's activities and collaborate in planning so that efforts are not duplicated. Projects are: (a) EASI (EHDI Level I) to integrate data systems for NHSP, NBMSP, EI programs, and other programs (CDC). (b) "Hawaii Early Childhood Hearing Detection and Intervention" (HECHDI) to further develop and refine the system of screening, assessment, and early intervention services for young children with hearing loss (MCHB/Maternal and Child Health Bureau). (c) "Genetic Awareness, Implementation and Data Project" to increase genetic awareness in Hawaii, implement genetic services for various public health programs, and investigate linking newborn metabolic screening, newborn hearing screening, and birth defects program (MCHB). (d) Data Utilization and Enhancement grant to link vital records data with FHSD program data, develop an integrated Perinatal Data System, and collaborate to develop an integrated perinatal data system (MCHB).

SECTION V. EVALUATION PLAN

Process and Outcome Indicators for the objectives and activities will be tracked (see Table 1).

SECTION VI. STAFFING AND MANAGEMENT SYSTEM

A. Key personnel

SECTION VII. ORGANIZATIONAL STRUCTURE AND FACILITIES

A. Organizational structure.

The Hawaii Department of Health's Family Health Services Division (FHSD), the state agency responsible for the state Title V Maternal and Child Health Block Grant, has 3 branches. The Children with Special Health Needs Branch (CSHNB) includes the Genetics Program, Early Intervention Section, Newborn Hearing Screening Program, and Newborn Metabolic Screening Program. The other two branches are the Maternal and Child Health Branch and Women, Infants, and Children (WIC) Services Branch. Organization charts are shown in Appendix H. The EASI II Research Project will be under the direction of the CSHNB Chief, who will ensure coordination of both the EASI I and EASI II Projects.

B. Facilities, space and equipment. The DOH will provide facilities, space and standard office furniture and equipment for EASI II staff. CSHNB staff are located primarily in two offices in central/east Honolulu. The offices are connected by the DOH intranet. Both offices have conference rooms, telephones, fax and copying equipment.

SECTION VIII. HUMAN SUBJECTS REVIEW

The DOH will comply with the Department of Health and Human Services Regulations (45 CFR Part 46) regarding the protection of human subjects. The approval of the Hawaii DOH Health Institutional Review Board (IRB) will be obtained for both research projects. The Project will ensure that the study is conducted according to the approved protocol, informed consent is obtained, the rights of participants are monitored throughout the project, and all members of the research team are trained in human participation protections.