Project Title Nebraska’s Early Hearing Detection and Intervention, Integrated Tracking and Surveillance Project.
Project Number CFDA #93.251
Project Director Julie Thorell, RN, BSN, NBHS Program Coordinator
Organization Name Nebraska Department of Health and Human Services
Address:
301 Centennial Mall South, P.O. Box 95044
Lincoln, Nebraska 68509-5044
Contact Person:
Julie Thorell, Program Coordinator
Phone (402) 471-6770
Fax (402) 471-0749
E-mail: julie.thorell@hhss.state.ne.us
Project Period: 4 years
From: 03/31/01 to 03/30/05
ABSTRACT
Organizational Setting: The Nebraska Department of Health and Human Services
Purpose: The Nebraska Department of Health and Human Services (HHS) is applying for funding to establish a surveillance and tracking program for Nebraska’s early Hearing Detection and Intervention (EHDI) Program, that will assist to ensure that an infant diagnosed with a hearing loss, will receive early diagnosis and intervention.
Few Nebraska hospitals are screening newborns for hearing loss. However, the State passed legislation on April 10, 2000 that will greatly enhance our ability to make screening available statewide to all newborns. This legislation recognizes the importance of screening, referral for diagnostic testing, and referral to early intervention programs, providing education to parents and professionals, as well as the need for tracking and surveillance components.
Likewise, while Nebraska has the infrastructure to obtain the screenings and has an excellent array of service providers, we lack a centralized tracking and surveillance system to be sure all infants receive quality screening, appropriate referral, follow-up testing and evaluation, and linkage with appropriate intervention services. Nebraska’s metabolic screening program’s centralized tracking and follow-up system provides a model for universal newborn hearing screening in Nebraska. This grant will provide funds to develop or acquire a computerized central tracking and referral system that will be the main tool in facilitating follow-up. A Universal Newborn Hearing Screening Advisory Committee has made tremendous progress in developing screening methods, referral protocols, tracking and surveillance components, and education for parents and professionals. This Committee has been charged to assist the HHS in a) assuring the computerized data system minimizes duplication of data entry at the hospital (thereby reducing costs and probability for error), b) determining ways to integrate or link this data with other relevant public health data systems such as the metabolic newborn screening database, birth defects registry, electronic birth certificate registry, and MatCHIM and c) is capable of producing the reports necessary to facilitate follow-up and function for quality assurance purposes.
A resource directory and referral guidelines will be developed that will include a direct connection with Nebraska’s Early Intervention Service Coordination system, to facilitate the families of infants who fail the screening test, connection with providers for further testing, evaluation, diagnosis and intervention or treatment. The Newborn Hearing Screening Program central tracking system is located in HHS. Services coordination for Early Intervention is also located in the same agency. Early Intervention Services are provided by the Nebraska Department of Education (co-lead with HHS for Nebraska’s Early Intervention System). Successful statewide programs for universal newborn hearing screening, audiologic diagnosis, and early intervention depend on data-reporting strategies that facilitate transition of infants and families through a system of care. Development of complete systems of care must become a priority for universal newborn hearing screening to provide its ultimate benefit. Nebraska’s Universal Newborn Hearing Screening Programs are not fully developed. Key issues that are impeding development of these systems may be lack of tracking and reporting systems, lack of standardized guidelines for screening, diagnostic audiologic assessment, hearing aid fitting for very young infants, and lack of understanding about the compelling need for intervention in the earliest months of life.
PROJECT NARRATIVE
1. Purpose of Project:
The purpose of this project is for the development of complete systems of care in order for universal newborn hearing screening to provide its ultimate benefit. Nebraska passed the Infant Hearing Act (LB 950) that will prompt Nebraska toward a successful statewide program of universal newborn hearing screening (UNHS). The State of Nebraska seeks support for the purposes of planning, developing and implementing a sustainable statewide universal newborn hearing-screening program. Elements of the intended program include: 1) physiological screening prior to hospital discharge, 2) coordination with the child’s medical home, family support and existing state and community based programs, 3) audiological evaluation by three months of age, and 4) enrollment in appropriate early intervention by six months of age. Audiologic diagnosis and early intervention depend on data-reporting strategies that facilitate transition of infants and families through a system of care, from the initial screening, to audiologic diagnostic services, and then into early intervention programs. Key issues impeding development of these systems is: the lack of tracking and reporting systems, lack of standardized guidelines for screening, diagnostic audiologic assessment and hearing aid fitting for the very young infants, lack of understanding about the compelling need for intervention, and lack of coordinating services from birth to diagnosis, early identification and early intervention. Challenges in addressing these key issues are compounded by the following factors: over 50% of Nebraska’s hospitals have less than 100 births per year, and reduced availability of treatment and diagnosis centers in the western two-thirds of the state. Of Nebraska’s 93 counties, only seven percent of audiologists and ten percent of speech pathologists, are located in the Western part of Nebraska that is less heavily populated. This
creates an additional burden of travel when families seek specialized evaluation and treatment. Nebraska is a geographically large state, measuring 387 miles across. The state’s area is 77,227 square miles, almost 20% larger than all of New England. Despite Nebraska’s vastness, 79 of the 93 counties (85%) are classified as rural or small urban (less than 9,999 people). Approximately 67% of Nebraska’s total population reside in rural or small urban areas. Of the 69 birthing facilities in Nebraska, 97% are located in counties with less than 9,999 people. About 80% of all births occur in the two metropolitan areas of Omaha and Lincoln. Currently only five of the ten hospitals in these metropolitan areas are performing infant hearing screenings. These metropolitan hospitals are anticipated to implement universal newborn hearing screening with in the next two years. Most (86%) of the birthing hospitals in rural and small urban areas of Nebraska are not conducting screening at this time, and are anticipated to conduct initial screening with less expensive equipment in the future. This approach will generate referral rates between six and ten percent, based on published studies.
Of the approximately 24,000 births in Nebraska in 1999, 26% underwent hearing screening prior to nursery discharge. Limited information is available on the number of infants who were actually born with a significant hearing loss, however, using national estimates based on an incidence level of 1-3:1000, Nebraska will have an expected 25-75 infants born each year with an invisible significant hearing loss.
2. Organizational Experience and Capacity:
The coordinating team for planning, developing and implementing the newborn hearing screening project will be based at the Health and Human Services System HHSS/ Newborn Hearing Screening (NBHS) Program. The NBHS Program within the HHSS has hired a Program Coordinator to manage the UNHS program, including the tracking and surveillance systems. This coordinator will report to the State Genetics Coordinator. This Genetics Coordinator currently directs the Nebraska Newborn (metabolic) Screening Program (NNSP). The NNSP has facilitated the state’s transition away from multiple laboratories, conducting
unstandardized tests without adequate means of tracking and referral. Now the state is able to document one of the most successful follow-up and tracking systems in the country. The state adopted standardized laboratory protocols through an extensive collaborative process facilitated by the NNSP. In addition, it implemented computerized data gathering and data transmission technology with technical assistance from the (federal) Health and Human Services Select Panel on Newborn Screening. Internally the NNSP developed an intensive process to track all infants to assure prompt, appropriate follow-up of abnormal, suspect or missing tests. Greater than 99%of newborns have received an appropriate metabolic screen in each of the past three years. The average age at intervention, (age when diagnosis was made and treatment was started) for infants with phenylketonuria (PKU) in Nebraska has been under 6 days of age each of the last three years, (100% by 7 days of age). Nationally less than 20% of infants identified with PKU are treated by 7 days or less. The program has addressed multiple “special situations” including facilitating screening of infants born at home (in 1999: 78 of 87 known home births infants were screened or >89%); assuring that specimens collected too early or that are otherwise unsatisfactory are repeated at appropriate times (in 1999: 216 of 227 or >95%). Uncommon in other states, but present in Nebraska since January 1999, is an electronic linkage for a match between the birth certificate registry and the NNSP electronic database to assist in the tracking for every registered infant.
In addition, the NNSP has used data for quality assurance purposes and educational strategies to improve its metabolic screening system. For several years, ongoing monitoring for unsatisfactory specimen collection has resulted in immediate training of targeted hospital personnel, resulting in a statewide specimen rejection rate of 0.1% or less each of the last three years. Communication facilitated by the NNSP via quarterly metabolic screening laboratory meetings, and monitoring of false positive rates over time resulted in timely modification of laboratory instrumentation and laboratory cut-off values to reduce the false positive rate yet provide sufficient assurance newborns positive for congenital hypothyroidism would not be missed.
A similar model for the UNHS program will facilitate successful screening for Nebraska’s infants. This experience will assist in developing UNHS protocols for follow-up and referral as well as in using tracking and surveillance data for monitoring. The surveillance and tracking system will include methods to monitor for special situations associated with attempting to assure an appropriate hearing screening for all infants prior to hospital discharge. There is a great deal of work to be done to ensure a comprehensive UNHS system in Nebraska, and the centralized computer based tracking and surveillance system will be one of the most critical tools to make it successful.
The state’s capacity to conduct newborn metabolic screening illustrates its ability to conduct universal newborn hearing screening. Currently, each hospital in Nebraska is submitting newborn blood specimens to their designated laboratory for analysis. Positive results are reported to the child’s physician, and follow-up activities are initiated for each infant suspected of having a metabolic disorder. Follow-up activities continue until the results of a retest are normal or there is a definitive diagnosis. These activities are supported by an information system that maintains information about both normal and abnormal results, rescreening results and diagnostic information on every baby screened in Nebraska; and generates form letters and management reports for the follow-up process.
3. Administration Structure:
Curriculum vitae/biographical sketches, found in Appendices C, are for the lead team Collaborating on this project. The UNHS Program Coordinator will be the central figure coordinating all activities and will operate under the supervision of the State Genetic Coordinator, and with the advice of the Administrator of Family Health in HHSS, and HHSS Medical Advisor. Other state programs assisting the Advisory Committee are: Medically Handicapped Children’s Program, Early Intervention Program, Nebraska Department of Education, Data Management Services, and Vital Statistics.
HHS/NBHS’s Advisory Committee has been formed with representation from many groups. These individuals and the group they represent, are listed in the table below:
| Individuals of Advisory Committee | Group/Facility Representing |
| Lois Harlan | Omaha Tribe of Nebraska |
| Steve Boney, Ph.D. | Audiologist, Barkley Center for Communications, University of Nebraska at Lincoln |
| Floyda Clayton, RN | OB Director rural community hospital |
| Margaret A. Coleman | Nebraska Commission for the Deaf & Hard of Hearing |
| Mary Jo Miller Grandfield, RN | Santee Sioux Tribe of Nebraska |
| Katie Foster, Ph.D. | Audiologist with public school system, early intervention |
| Joel Garjardo | Nebraska Minority Public Health Association, Hispanic Community Center |
| Michael Gorga, Ph.D. | Audiologist, Boys Town National Research Hospital |
| Judy Halstead, MS, LMHP | Services Coordinator and Quality Improvement Supervisor, Representing Senator Dennis Byars |
| Jeff Hoffman, CCC-A | Audiologist with Nebr. Heat Start and Early Head Start,
Nebraska Training Technical Assistance Liaison |
| John Jirka, MD | Neonatologist @ Children’s Hospital, Nebraska Perinatal Association |
| Lora Langley, RN, BSN | Ponca Tribe of Nebraska |
| Lynn Lowry, RN | Director of PHN, Winnebago Tribe of Nebraska |
| Leslie Marsh, RN | Coordinator of hearing screening, central Nebraska Hospital serving high percentage of minority population |
| Renea McWilliams | Parent |
| Mary Pat Moeller, MS | Speech Pathologist, Boys Town National Research Hospital |
| Jill Ramsey | Speech Pathologist, Director of Deaf and Hard of Hearing Outreach |
| Stacie Ray | Parent |
| G. Bradley Schaefer, MD | Geneticist, Chief Medical Genetics and Rehabilitation Medicine, University of Nebraska Medical Center |
| Monica Seeland | Nebraska Association of Hospitals and Health Systems |
| Britt Thedinger, MD | Otolaryngologist, Ear Specialists of Omaha |
| Donald M. Uzendoski, MD | Nebraska Medical Association |
| Denise Wright | Service Coordination and Early Intervention Special Education Coordinator, western Nebraska |
This Advisory Committee is providing expert advice and support to the state’s UNHS program based on their own knowledge and experiences as well as information provided to them by the Nebraska UNHS program. The NBHS Advisory Committee has divided into four work groups with content specific expertise, to make recommendations and develop protocols in areas of screen methods; referral protocols; tracking systems; and education. These work groups also address diagnostic evaluation/assessment protocols, and integration into early intervention services coordination and service provision. Other areas of concern that the Advisory Committee is addressing are: financing, data systems, patient information and informed consent/dissent, and public and professional education. The Advisory Committee has already made tremendous progress. During the first two months, it has developed draft definitions for screening methods, a draft recommended audiological diagnostic protocol, proposed algorithm for referral protocols, and a defined data set of required data for tracking and surveillance. JCIH Standards have been integrated into our methods of screening, referral protocols, and data format for tracking and surveillance.
4. Available Resources:
The Nebraska Department of Health and Human Services has hired a Program Coordinator, who is stationed at the Nebraska State Office Building, Lincoln, Nebraska. Costs to be assumed by HHS are: salaries for Program Coordinator, and .20 FTE Administrative Assistant support, benefits, travel, supplies of copying, postage, office supplies and advisory committee meeting supplies, equipment of computer, printer and software for program coordinator and administrative assistant. The HHS will reimburse the Advisory Committee, to include meals, meeting room rentals, mileage, and as necessary overnight room charges.
Nebraska was one of the first states to mandate services through the schools for infants’ 0-3 years of age having disabilities. An extensive educational service system exists across the state that can help facilitate services for children with hearing loss. Furthermore, the state has available to it the expertise of the Boys Town National research Hospital in Omaha, Nebraska, a nationally and internationally recognized center dedicated to serving infants and children with hearing loss.
Nebraska’s Children’s Special Health Care Needs (CSHCN) population receives sub-specialty care through the Title V funded Medically Handicapped Children’s Program (MHCP) that recruits health care professionals to form sub-specialty clinics to travel in teams to rural areas of Nebraska. These teams include nutritionists, physical therapists, medical social workers, sub-specialty physicians and nurses. In addition, Nebraska’s educational system has divided Nebraska into Educational Service Units (ESU). Each ESU has a number of trained speech pathologists, school nurses, audiologists, physical therapists, and psychologists.
The current status of the system available for service coordination, and educational services in Nebraska is via the “Early Intervention System”. Early Intervention services are available from Services Coordinators employed and supervised by specialists across Nebraska’s 29 planning region teams within Educational Service Units (ESUs), hospitals or other agencies under the broad umbrella of the Nebraska Department of Health and Human Services. The individuals who provide the coordination are specially trained in the knowledge of resources and services for children with disabilities and their families and coordination of those services with other individuals and agencies.
Early Intervention Service Coordinators are also uniquely positioned to facilitate referrals for resources, specialized consultation and direct service delivery with the Nebraska Department of Education’s Director of Outreach Services for Sensory Impaired and/or one or more of the four Regional programs for the Deaf and Hard of Hearing. Certified Educators of the Deaf and/or Speech-Language Pathologists are able to deliver itinerant services in the child’s home or other natural environment. Referral for additional services and/or for further evaluations may also be appropriate. Educational Audiologists are employed by some school districts, and there are centers available that specialize in speech and language assessment for children who are deaf or hard of hearing. Counseling services for families are available in some areas of the state.
In addition, resources such as lending libraries of books, videotapes and equipment are available through Nebraska Department of Education, the Regional Programs, Nebraska Commission for the Deaf and Hard of Hearing, ESUs and other agencies within the various planning regions to assist children who are deaf or hard of hearing and their families. Access to the regional services of the National Center for Hearing Assessment and Management’s resource and technical assistance of Les Schmeltz of Iowa is currently a great asset in forming of the screening methods, referral protocol and tracking system.
Collaborative efforts are in place to assist Mary Pat Moeller, MS with Boys Town National Research Hospital, who has received funding from the NID CD/NIH grant. The overall goal of her project is to provide information that will close existing gaps between identification of hearing loss and first interventions for children and their families. The three specific aims for the overall goal are: (1) to increase the knowledge and change the attitudes of health care providers regarding newborn hearing screening and referral, (2) to increase the skills of clinicians in implementing developmentally appropriate, relationship-focused interventions with infants and their families, and (3) to increase parents’ understanding and self-efficacy related to the complex issues surrounding the diagnosis of hearing loss, genetics of hearing loss, and amplification options for infants and young children. In an effort to improve the newborn hearing screening process, educational efforts will reach the goals as outlined by Moeller.
The resources identified above will provide the state with a system that (1) includes reasonable assurances that infants with hearing loss are identified early in life and (2) bring these children to the attention of health-care and educational providers who are prepared to serve children with hearing loss. There is significant need for a functional centralized data system to facilitate referral for further testing, diagnostic evaluation and entry into a service system that in many ways is already in place.
5. Identification of the Target Population and Service Availability:
The targeted population for this initiative includes all occurrent births in Nebraska. Although this population based activity is intended to provide newborn hearing screening to 95% of children born in Nebraska by December 1, 2003, there is at present, voluntary newborn hearing screening in only 9 of the 69 birthing facilities/hospitals in Nebraska. The grant will target all infants receiving a hearing screen in the hospital and subsequently focus on those infants and their families who do not pass the hearing screen, who need a diagnostic evaluation, and who need early intervention services. Currently there are gaps in services in many regions of the state and significant barriers to services due to lack of funding, professional expertise in deafness, transportation issues and language barriers. This project will specifically target planning, development, and implementation of UNHS components to ensure diagnosis by three months, coordination of services and enrollment in early intervention by six months of age.
One major demographic trend is the substantial growth of racial/ethnic minority populations. During the decade of the 1980s, the racial/ethnic minority population rose by 23% while there was minor 0.5% decrease in the white population. This implies a substantial growth of minorities in our state. Since 1990, the racial/ethnic minority population expanded by 395% while the white population increased by four percent. In general, the minority population tends to be younger, have lower incomes, higher poverty, and less insurance coverage. They are also more likely to be employed in high risk occupations such as meat packing plants and farm labor. As a result, these population groups often experience difficulty gaining timely access to health and medical services. Even when services are available, language and cultural barriers prevent effective utilization of these services. As more and more immigrants from Mexico relocate to the United States, there is an increased concern for rubella outbreaks. Mexico does not routinely vaccinate for rubella, and past experience has been shown of the possibility of congenital rubella syndrome.
Poverty in Nebraska decreased slightly from 11.1% in 1989 to 9.8% in 1995. The national rate in 1995 was 13.8%. Nebraska has approximately 12.6% of children living in poverty, compared to the national rate of 20.8%. Poverty rates vary by geographic area. In general, the north central and northwestern counties experienced the greatest poverty rates, with some pockets along the southern tier of counties. The counties with the highest poverty rates were Thurston (24%), Sheridan (18%), and Dawes (17%). These three counties are much higher than even the national average.
Nebraska’s racial and ethnic minority groups are greatly over-represented in the lower income categories. According to the 1990 U.S. Census, the proportion of minority residents living in households with incomes below 100% of the federally-designated poverty level ranged from a low 20,1% for Asian Americans to nearly half 46.2% for Native Americans living in the state. In comparison, only 9.9% of white Nebraskans lived in poverty. In addition, the proportion of the population with incomes below the poverty level has increased for every racial/ethnic minority group since the 1980 census. Disparity in economic status across the races still remains a challenge for Nebraska.
Differences in poverty rates between whites and racial/ethnic minority residents of Nebraska was particularly striking for households with children. For children under five years of age, the proportion living in households with incomes below 100% of poverty level ranged from about 25% for Asian Americans to about 65% for Native Americans, compared to 13.8% of white children in this age group. Among older children aged 5 to 17 years of age, about 10% of white children live in poverty. Rates for minority children in this age group ranged from 14.5% for Asian Americans to more than 50% for Native Americans.
Newborn hearing screening services are available at the hospitals listed below. Resources for rescreening and diagnostic audiology vary by community. Diagnostic audiologists reside mainly in the two metropolitan cities, (60% of the 109 audiologists available or licensed in Nebraska.) Surveys have been sent to audiologists to inquire of their availability and desire to serve the infant population. To further illustrate that newborn hearing screening is not yet widely practiced in Nebraska, the following 1999 data is presented as reported from the 7 birthing hospitals known to be conducting hearing screening of newborns prior to discharge during that year.
| City/Hospital (Universal or targeted screen) | # 1999 births | # and % of births screened prior to hospital discharge | # failed screen (% of screened infants) | Of # failed, # for whom diagnosis is known | # known diagnosed with hearing loss. |
| Hastings, Mary Lanning
(universal) |
643 | 643 | 5
7% |
2 | 1
|
| Lincoln, St. Elizabeth (targeted) | 2,140 | 163
7.6% |
17
10.4% |
Unknown (probably 15 normal, 2 referred) | Unknown |
| Norfolk, Faith Regional (targeted) | 797 | 284
35% |
17 rescreened
5.9% |
4/? | 2 known
|
| Omaha, Bergen (universal) | 2,282 | 2,240
98.1% |
34
1.5% |
Unknown | Unknown
|
| Omaha, Methodist/
Children’s (universal) |
2,971 | 2,572
86.5% |
361
14% |
Unknown | Unknown |
| Omaha, St. Joseph (targeted) | 1,020 | 157
15.3% |
6
3.8% |
Unknown | Unknown
|
| Omaha, University (targeted) | 1,583 | 265
16.7% |
17
6.4% |
Unknown | Unknown
|
Note that essentially no information is available regarding follow-up compliance or the number of babies with confirmed hearing loss, even for these larger birthing facilities whereeither targeted or universal newborn hearing screenings are conducted. This data shows that 6,334 infants were screened out of 24,018 Nebraska births, or 26% of births. In the absence of an adequate tracking system, we have almost no data on the extent or types of hearing losses confirmed, the distribution of suspected or confirmed etiologies, the proportion of infants failing screening who receive confirmatory testing, or the number of infants with confirmed loss at follow-up testing. A tracking system is needed to initially focus on ensuring infants are adequately followed up with timely and accurate diagnosis, and entered into treatment as appropriate. The follow-up tracking system is also needed to incorporate long-term tracking to monitor the effect of various interventions.
Since obtaining data for the hospitals doing newborn hearing screening in 1999, there have been reports by six additional hospitals that are performing universal or targeted newborn hearing screening. Those hospitals performing newborn hearing screening, with number of births noted are; Columbus/Columbus Community Hospital, universal(523); Fremont/Fremont Area Medical Center, universal (429); Grand Island/St. Francis Hospital, targeted (940); North Platte/Great Plains Medical Center, universal (550); Omaha/Alegent Health Care-Immanual Medical Center, universal (749); and Wayne/Providence Medical Center, post discharge universal (112).
6.Needs Assessment:
Many challenges face Nebraska as it plans, develops and implements a universal newborn Hearing screening program. Among these, are access issues defined by :
The availability of medical specialty services vary and tend to exist only in the larger or more urban areas of Nebraska. Pediatricians and laryngologists are scarcely available in the western 2/3 of the state. In many communities, newborns requiring referrals, or evaluations from pediatricians, or oto-laryngologists, as well as audiological diagnostic evaluation, face issues of access to appropriate services and this barrier reduces the likelihood of timely clinical follow-up, referral, diagnosis and intervention.
Successful statewide programs for UNHS, audiological diagnosis and early intervention depend on data reporting strategies that facilitate transition of infants and families through a system of care. In any case, the data that are collected must be sufficient to identify, locate and connect babies in need of follow-up.
Recent studies completed by Mary Pat Moeller, MS (Boys Town National Research Hospital), examined the relationship between age of enrollment in early intervention services and specific language development outcomes measured at 5 years of age in a group of children with sensorineural hearing loss. Findings reported from this study reveal that a statistically significant negative correlation of r = -. 46 (P<. 01) was found between the variable of age of enrollment and vocabulary skills measured at 5 years of age. Therefore, earlier enrolment in intervention services was associated with significantly stronger language outcomes at 5 years of age. Children enrolled before 11 months of age had stronger vocabulary and verbal reasoning skills at 5 years of age than did later-enrolled children. In contrast, average vocabulary scores for later-enrolled children (e.g., >24 months old) were 1.0 to 1.5 SD below their hearing peers. Such delays can be expected to interfere with academic development and understanding in the classroom. (5)
Development of complete systems of care must become a priority for UNHS to provide its ultimate benefit. The success of a screening program depends on effective follow-up and tracking for infants that have been identified as needing further assessment. Follow-up systems are necessary to assure that families receive appropriate and timely services. Data management systems for tracking infants through the referral process are being developed at the hospital and state levels. A significant gap appears to exist after newborns are screened/re-screened by participating facilities. In surveying those 9 facilities providing universal newborn hearing screening, there is just cause for concern expressed by birthing facilities/hospital staff, that they may not be able to provide follow-up services. This may be due to the above listed challenges, or it may be due to the need to educate the professional and public community. Families with children who need follow-up for hearing screening failure, require information, support and encouragement to continue on to diagnostic evaluation, and if necessary, intervention. These children need to be connected to their medical home, which in Nebraska may often be a community based provider. The potential exists for delayed or missing connections from the birthing facilities to the medical home, diagnostics, deaf education, and community based resources.
Two very clear needs emerge from what data there is available regarding the status of newborn hearing screening in Nebraska. First, a comprehensive UNHS program should be planned, devised, and implemented under the guidance of the HHS/NBHS Advisory Committee, which includes representatives of the statewide stakeholders and experts, families, and persons who experience hearing impairments. Defined work groups require input from additional stakeholders and experts to draft protocols for areas such as diagnostics, early intervention or data systems for tracking and follow-up. Secondly, since the level of voluntary hearing screening in Nebraska should reach 95% of births by December 1, 2003, the challenge is to quickly develop an interim strategy to ensure all children who need rescreening, diagnostic audiological evaluation and/or early intervention services receive them by 6 months of age. Each child who fails screening at the birth facility must be tracked and followed to resolution via networking with all appropriate resources on a case-by-case basis. Mechanisms and protocols will be developed to inform and educate families about the significance of positive hearing screens, and the need for follow-up, and to obtain consent for sharing screening results with the State NBHS program. The Program Coordinator would then track progress and ensure appropriate referrals to services and intervention in collaboration with the family, medical home, audiologists, early intervention and other resources.
Every child in America should have a medical home. In other words, every child should have a primary health care provider who knows the child individually, cares about him/her, is aware of his/her needs, and is capable of providing culturally sensitive and appropriate medical services. In order to assure appropriate follow-up of each child, hospitals will be asked to identify the child’s medical home before hospital discharge.
Children born at home are currently not receiving information about the importance of hearing screening at birth, and the early identification of hearing loss, nor are they receiving a hearing screen. It is imperative that a system of checks and balances be developed to provide hearing screening, and follow-up to all infants born in Nebraska. Collaborative efforts with the Newborn Screening Program (metabolic) and Vital Records will be carried out to ensure that parents of all infants born at home are sent information as well as a letter to inform them of the available resources for the infant and family’s use.
Nebraska faces three main challenges in implementing UNHS. First, screening and reporting practices must be improved among a group of 13 hospitals. Secondly, screening must be implemented by 60 additional hospitals to achieve universal screening. And thirdly, follow-up is incomplete on nearly all of the infants suspected with hearing loss. By education of professionals and hospitals of the importance of newborn hearing screening and reporting for purposes of intervention, no child should experience a delay in language or cognitive development.
Demographics and geography hinder Nebraska’s direct health care services. Most of the population is located in the eastern portion of the state, where most of the services are also located. Most of Nebraska has been federally designated as health professional shortage areas (HPSAs), Medically Underserved Areas (MUAs), and Medically Underserved Populations (MUPs). In 1999, over half (50/93) of Nebraska’s counties have been designated, either in full or in part, as primary care HPSAs. These shortage areas potentially affect more than 25% of Nebraska’s population. Based on 1998 Census estimates, more than 12% of the state’s population lives directly within a HPSA. In addition, 73% of Nebraska’s 93 counties have been designated, in full or in part, as containing MUAs or MUPs. Over 22% of the state’s population live within the designated areas and are potentially affected by a shortage of health services. In addition to federally designated HPSA, Nebraska has state designated HPSAs. Within state designated HPSAs, a high degree of shortage exists in each of the defined health specialization.Two-thirds of Nebraska’s counties currently have a shortage of family practice physicians (62/93), 95% have a shortage of pediatricians (89/93), and 91% have a shortage of OB/GYNs (85/93). Of the 36 pediatric specialists (representing 11 different specialty areas) in Nebraska, all but five are located in Douglas County. All but one pediatric specialist practiced in the Eastern part of the state. In addition to the 36 pediatric specialists, Nebraska has 202 pediatricians (total 238) located in 18 counties.
7. Collaboration and Coordination:
Coordination of people and services is required to implement the highest quality early intervention program, and ensure seamless transition of the infant and their family into appropriate early intervention. HHS/NBHS collaborates with many partners both public and private in the normal course of its activities. The UNHS initiative will require building upon existing relationships, and developing of new ones. Extensive collaboration between the Nebraska Health and Human Services System and the Nebraska Department of Education, and the Nebraska Association of Hospitals and Health Systems will occur throughout the implementation of the UNHS. The creation of a statewide Advisory Committee includes those listed previously, and should formalize relationships to carry out the planning, implementation and evaluation of the newborn hearing screening program. The Advisory Committee will be of assistance with the: a) development of guidelines for universal newborn hearing screening methods, referral protocols, and diagnostic guidelines, b) development of the resource directory, c) advise on the tracking and surveillance system, and d) through sharing of progress reports and data with the advisory committee for ongoing quality improvement.
Although voluntary newborn hearing screening is expected to occur in about 40% of Nebraska births during year 1, (2001), links to diagnostics and intervention are not established and will require the collaboration of internal and external, public and private partners. Linkages to other entities of the HHSS/NBHS will alleviate duplication of data entry and promote utilization of early intervention services. Research of the possibility of electronic data sharing with Electronic Birth Certificates, Nebraska Newborn Screening Program, Immunizations, and Birth Defects Registry is being carried out. Establishing screening protocol and methods of referral will be the primary focus of the UNHS implementation in Nebraska.
8. Goals and Objectives:
8.1 Goal 1
Develop and implement a computer-based surveillance and tracking system that can be used by all hospitals conducting newborn hearing screening and the centralized newborn hearing-screening program to facilitate follow-up testing. This system will facilitate tracking for purposes of appropriate referral, diagnosis, and treatment of all infants with known or suspected hearing loss, and to provide quality assurance data for ongoing monitoring and improvement of the system. (This process to be completed with the assistance of the Advisory Committee)
Objectives
8.1.1.1 Advertise/post request for proposal for vendor’s for computerized electronic database tracking and surveillance system.(2months)
8.1.1.2 Gather and provide information to the Advisory Committee on quality comprehensive universal newborn hearing screening systems including: technical assistance available, education of public and professional audience, patient education, resource directories, screening methodologies, referral systems, data tracking systems.(ongoing)
8.1.1.3 Explore options for transmission of data from hospitals to the central tracking program, using a cost effectiveness model for evaluation criteria. (2months,ongoing)
8.1.1.4 Evaluate existing database systems available on the market via live demonstration, using the following criteria: Functionality, Compatibility, Security, Support, and Cost. (2months).
8.1.1.5 Based on recommendations of the Advisory Committee, arrangements for Health and Human Services to purchase an existing software system for use in hospitals, and the central tracking program, based at the NHHSS.(2-3months)
8.1.1.6 With Advisory Committee input, develop standardized protocols for data transmission, and follow-up (tracking and referral) and a resource directory and communicate these to each hospital conducting newborn hearing screening.(ongoing)
8.1.1.7 Purchase software, install and train personnel at one pilot site, and at the centralized tracking program on data entry and transmission, as well as functional aspects of software. (3-6months)
8.1.1.8 Develop electronic linkages between newborn hearing centralized tracking database, metabolic newborn screening database, birth certificate registry and birth defects registry for purposes of monthly match.
8.1.1.9 Software is installed and personnel are trained at all sites conducting newborn hearing screening. As new sites initiate newborn hearing screening, the software will be installed and training carried out. (6-9months, ongoing).
Activities to accomplish goal 8.1 Goal 1 will include the following: develop a state database to monitor the progress of newborn hearing screening in the state, provide statewide quality control indicators, compare state database of newborn hearing screening to Electronic Birth Certificate (EBC), NNSP (metabolic), Immunizations, and Birth Defects Registry, and assess the feasibility of state legislation to mandate universal newborn hearing screening.
8.2 Goal 2
Increase professional and public awareness of the importance of newborn hearing screening.
Objectives
8.2.1 Conduct focus groups to identify appropriate information and public awareness activities (6months and ongoing).
8.2.2 Research availability of culturally appropriate UNHS Public Service Announcements, and develop videotapes, newspaper articles, electronic information and fact sheets for local media in collaboration with statewide deaf and hard of hearing consumer organizations and minority groups (6months, ongoing).
8.2.3 Increase demand for hearing screening through public information materials made available to birthing classes (ongoing).
8.2.4 Conduct needs assessment to clarify specific areas of education and training needs, of hospital staff, audiologists, pediatricians, and general practitioners (3months, ongoing).
8.2.5 Explore distance learning options, including Internet, video conferencing, remote interpreting facilities, and teleconferencing (1 year, ongoing).
8.2.6 Research avenues of funding to support education of hospitals, audiologists, Early Intervention Team, and physicians (1 year, ongoing).
Activities to accomplish 8.2 Goal 2, to include: distribution of newborn hearing screening informational brochures to all hospitals to use for promotion UNHS to expectant parents, provide information to Early Intervention Coordinating Services, develop parent education materials on website, and education of physicians and nurses about the program. Educational materials will include how results will be forwarded for follow-up and tracking
9. Required Resources:
The Nebraska Health and Human Services has hired a UNHS Program Coordinator to oversee the planning, development and implementation of the statewide program, statewide planning, and facilitation of the Advisory Committee. Clerical support of a .25 FTE Administrative Assistant will be required to compliment the work of the Coordinator, the Advisory Committee and the evolving statewide program and plan. Funding for travel is required to convene the statewide advisory committee in addition to allowing the coordinator to travel to communities to interact, educate and work with hospitals performing or considering performing voluntary hearing screening. Funds are also budgeted for an annual universal newborn hearing screening meeting. Possibility of use for teleconferencing may be an effective way to reduce meeting costs and improve productivity. As such, monies are required in each project year to support teleconferencing by the Advisory Committee and its workgroups. Contractual services will be required to develop and produce educational materials.
Finally, a resource for diagnostic testing and travel is being requested to cover costs for children without third party payers who are not receiving services from the early intervention system. This will allow families to avail themselves of timely diagnostic services which can be accessed more directly through the Program Coordinator and/or the child’s medical home. All alternate resources, including use of Part C funds, will be exhausted before using these diagnostic funds.
10. Project Methodology
The UNHS Program Coordinator will be responsible for coordinating the program and the activities of the UNHS Advisory Committee. To assist in the knowledge and skill development of the UNHS Program, the Program Coordinator will attend two 2-day meetings sponsored by the Centers for Disease Control on Early Hearing Detection and Intervention. The Advisory Committee provides expert advice and support to the state’s UNHS program based on their own knowledge and experiences as well as information provided to them by the Nebraska UNHS program. The UNHS Program Coordinator and State Genetic Coordinator will facilitate communications about the plans for and progress on Nebraska’s centralized computer based tracking and surveillance system to the appropriate providers as well as inform them of how they can submit input and questions to the committee.
The Advisory committee is to accomplish two main goals. One being, to adopt appropriate approved screening methods and follow-up testing protocols for use in Nebraska, as well as guidelines for early intervention referral. The Committee is providing input on follow-up procedures and referral of infants with suspected hearing loss, or for whom results are inconclusive. It is recognized that follow-up and referral will be more complex for UNHS than in the case for metabolic screening, due to the availability of multiple providers for repeat hearing tests, diagnostic evaluation, and treatment as well as the multiple options of treatment methods. Therefore, developing information flow-charts that incorporate feed-back loops to the centralized tracking system will be essential to prevent infants from “falling through the cracks.” Another role is to evaluate existing computerized tracking/surveillance software packages that might be adopted or adapted for use by Nebraska hospitals conducting newborn hearing screening. As part of this evaluation, the committee will examine which options is most cost effective for hospitals and which options reduce probability for human error, for example by minimizing duplication of manual data entry or by transmitting data electronically from the hospitals to the state. The staff will seek out packages that can integrate with existing data systems, as these may promote both cost effectiveness and data accuracy for hospitals and the state. In addition, evaluating options that include integration or linkages with the electronic birth certificate registry, metabolic newborn screening database, and birth defects registry will provide a check and balance to help ensure screening, as well as facilitate identification for each of these registries. The Advisory Committee will first consider cost effective ways to integrate data systems, and where this is not possible, make recommendations for linkages between data systems.
The Program Coordinator may call upon members of the Advisory Committee to advise hospitals seeking to establish UNHS. Anticipated questions for hospital administrators and staff include what equipment to use, number of tests in the screening protocol, algorithms, timing of screening, appropriate training and supervision of personnel, and how and by whom results are to be communicated to parents. To encourage consistency in the system, the Department of Health and Human Services Newborn Hearing Screening Program will communicate the Advisory Committee’s guidance to hospitals on issues relevant to referral (e.g. communication with parents, physicians, tracking, etc.).
Recommendations from the Advisory Committee, in regards to Screening methods and Referral Protocols, will be provided to the birthing facilities, allowing each birthing facility to establish their own protocol as long as the birthing facility would: utilize a physiological screening test, (OAE or ABR) to screen for both unilateral and bilateral hearing loss; ensure that the screening is carried out prior to discharge; ensure that infants with a positive screening result are re-screened before discharge; incorporate newborn hearing screening into the hospital’s routine consent procedures; make screening available for infants who are discharged without an initial or follow-up screening; and inform the physicians, who in turn inform the parents of the screening results.
Integral to the success of a comprehensive system of newborn hearing screening will be development of procedures for referral for re-testing and the connecting to service providers. The centralized computer tracking system is the main tool utilized for this purpose. Nebraska is uniquely positioned administratively with the partnership between the Nebraska Health and Human Services System (HHSS) and Nebraska Department of Education (NDE) for implementation of its Early Intervention System (Part C of the Individuals with Disabilities Education Act). Until electronic tracking is available, hospitals will be requested to manually submit information to the HHS/NBHS on each infant born in their facility (paper or diskette). This allows for the Program Coordinator to submit a form letter to the infant’s physician informing him or her of the “refer” test result, and a list of credentialed audiologists included with the letter. If the diagnostic report is not received within 30 days, the physician will be contacted by telephone. If the diagnostic report is not received within 30 days of the telephone call, parents will be notified. During this telephone conference with the parents, education of available services prior to verbal consent for permission to refer to the Early Development Network will be obtained. Parents will have the opportunity to descent, even though UNHS is a standard of care, as this information is being shared for purposes of tracking and follow-up. The Early Development Network will then be contacted, and in return contact the family and arranges for diagnostic evaluation. Referral for Early Intervention is to be initially made by the physician or audiologist who performs the diagnostic evaluation.
The Medically Handicapped Children’s program (Nebraska’s Title V Children with Special Health Care Needs Program) will fill service gaps for infants needing additional diagnostic, medical treatment planning and medical treatment services beyond newborn hearing screening phase, subject to Nebraska Education System responsibilities. These services will be provided through established program criteria and policies which may include further audiology evaluations at specialized pediatric hearing centers, medical services coordination and further diagnostic examinations with pediatric and otolaryngology specialists. Follow-up treatment including surgery and hearing aides may be provided for those eligible for the program subject to private insurance, Medicaid, and “Kids Connection” (Nebraska’s Medicaid expansion for children’s health insurance) eligibility. Early Intervention Service Coordinators will be trained on the availability of the CSHCN program.
Parent education and parent feedback will be an integral part of implementing newborn hearing screening in Nebraska. Education will be provided through written materials for parents. These materials will be distributed at the hospital to parents of newborns. Brochures will be published in the near future, and offered to hospitals that are engaged in UNHS. A resource directory listing relevant Nebraska service providers (including service coordinators, hospitals, physicians, and other hearing impairment treatment providers and parent support groups) will be developed, giving parents access to this information. This directory will include information on financial aid and include a survey to provide a mechanism for parents to provide feedback to the system about their experiences and questions about early hearing loss detection and intervention. The resource directory will be made available by the central tracking system to all hospitals for infants referred for further testing. The UNHS Program Coordinator will work to incorporate the directory on the HHSS web site and seek to inform families about this web site resource. In addition to information about service availability through the public school system utilized by the 29 Early Intervention regions, parent education will also include information about additional resources available to them and their infant. For example, programs recognized nationally for excellence in speech, language, and hearing diagnosis and treatment such as the Boys Town National Research Hospital in Omaha, Nebraska, and the Barkley Center for Communication Disorders at the University of Nebraska at Lincoln, Nebraska, will be listed among service providers in the resource directory. Recent events place Nebraska in a uniquely favorable position for the purposes of information dissemination. The Boys Town National Research Hospital has been awarded a 3-year grant from the National Institutes of Health (NIH-NIDCD), which will support efforts for information dissemination related to childhood hearing loss. Products produced as a result of this award will help to further our efforts at information dissemination, both to parents/families and health-care providers. The Principal Investigator for this HIH-NIDCD grant (Mary Pat Moeller) serves on the Nebraska Newborn Hearing Screening Advisory Committee as well, and is a collaborator on the education work group ( a sub committee of the Advisory Committee) developing educational materials.
In addition to the guidelines for referral of infants failing the screen (abnormal or inconclusive results), specialized referral guidelines are being developed by the Advisory Committee for further comprehensive genetic diagnosis and treatment services for infants suspected to have possible genetic conditions with implications for the family or infant. The resource directory will include Nebraska genetic service providers including the Munroe-Meyer Institute for Genetics and Rehabilitation (MMIGR) at the University of Nebraska Medical Center in Omaha, Nebraska.
It is important that all children with a congenital hearing loss have a formal evaluation to define the etiology of the hearing loss. Defining the etiology provides crucial information for the family besides answering the question “Why did it happen?” Knowing the cause of the hearing loss can also answer questions about prognosis (“What do we expect for the future?”) and recurrence risk (“Will it happen again?”). One out of every 2000 newborns has a heritable hearing loss. Of these, 75-80% is inherited in an autosomal recessive manner, 15% are autosomal dominant, 3% are X-linked and 2% are mitochondrial. Thus, every child identified with a congenital hearing loss should receive a formal neuro-sensory genetic work-up in an attempt to define the genetic basis of its hearing loss.
Seventy percent of heritable hearing loss is non-syndromic (i.e. no other associated medical problems) while 30% have an identifiable syndrome. It is equally important to identify the presence of a genetic syndrome in order to look for associated medical problems that necessitate screening and/or intervention. For example, below are listed the most common causes of syndromic hearing loss and the associated major medical concerns:
| Hearing Loss Syndrome | Associated Medical Condition |
| Alport | Nephritis, renal failure |
| Branchio-oto-renal | Renal anomalies, branchial cysts |
| Jervell and Lange-Nielsen | Sudden cardiac death |
| Pendred | Hypothyroidism |
| Neurofibromatosis type 2 | Tumors |
| Usher | Visual impairment, balance problems |
| Waardenburg | Limb anomalies, Hirschsprung disease |
| Mitochondrial syndromes | Seizures, strokes, diabetes, visual problems. |
Given this information, it is not enough to simply screen for hearing loss in the newborn. A system must be in place to triage and evaluate infants identified with hearing loss. In Nebraska, there are organized genetic services along a “hub and spoke” model. The entire state is canvassed by out-reach clinics in which children can be referred for initial evaluations and counseling. In addition, there has been developed a quaternary referral clinic, the “Neuro-
sensory Genetics Clinic” for evaluation of complex cases. This clinic is a joint effort of experts at the University of Nebraska Medical Center and the Boys Town National Research Hospital. Specialties that staff this clinic include: clinical genetics, genetic counseling, molecular genetics, audiology, vestibular specialists, otolaryngology, speech pathology, and ophthalmology. As part of the follow-up system, all families with infants suspected to have hearing loss would be provided information about the availability of this comprehensive diagnostic service.
In summary, through procedures developed on the basis of input from the Advisory Committee, linkages between hospitals, primary care physicians, centralized tracking system in HHS, and referrals to the Early Intervention Service Regions and Regional Programs for Deaf and Hard of Hearing will be established so infants who fail the screen can be connected for comprehensive diagnostic evaluations leading to appropriate diagnosis and treatment.
As Nebraska embarks on implementation of the Infant Hearing Act, and as the tracking and referral system is established, the program will utilize meetings held by the six Planning Regions and the four Regional Programs for Deaf and Hard of Hearing to educate those involved with service coordination and referral about the system established for infant hearing screening, tracking and referral.
11. Evaluation Plan:
To evaluate this project, HHS will use a process evaluation to monitor progress on each of the activities and objectives. A quarterly progress report assessing timeliness, completeness and the success of the project will be produced by the UNHS Program Coordinator and made available to the Advisory Committee, the HHSS Policy Cabinet, and the Legislature. An important component of this evaluation will be a compilation and summary of parent comments returned from the survey included in their resource packets. In addition as hospitals begin screening and join the electronic tracking and monitoring system, the outcomes will be evaluated on the following data points (relative to each other):
The evaluation will look at the quality of data received, the components of the follow-up system that need strengthening, and which processes are more successful in helping HHS achieve the goal of at least 95% of infants born in Nebraska being screened by December 1, 2003. A final report produced by the UNHS Program Coordinator will be distributed to all birthing hospitals, and confirmatory test providers known to the program. Process evaluation and review will include 1) progress on development of a Universal Newborn Hearing Screening Plan for Nebraska, with recommendations being provided by the Advisory Committee on screening methods and referral protocols, and 2) status of a system in the state which ensures the timely screening, diagnosis, referral and intervention services for Nebraska’s infants. This report will describe the tracking system developed, protocols for data transmission, and follow-up, guidelines for referral for further testing, diagnosis, neuro-sensory evaluation and treatment/intervention providers. Ongoing consultation will be maintained with the Advisory Committee, hospitals, audiologists, confirmatory testing facilities, and early intervention programs.
Program impact will be monitored by outcomes to the identified goals and objectives listed in section 8. Evaluation of successful completion of the project goals using a variety of tools including survey data, phone consultations and verification of hospital reports, birth certificate data, Part C Follow-up, and statistical analysis. The number of births screened, completion of diagnostic audiology by three months of age for screening failures, and age at enrollment in early intervention will be tracked and monitored. The enrollment of infants diagnosed with hearing loss should increase significantly across the statewide Early Intervention programs. Additional financial and staff resources might be required in the future for all services involved with UNHS. Focus groups will be used to evaluate success of public awareness campaigns and parent satisfaction surveys will be used to assess program effectiveness. The demand for genetics evaluations will also increase substantially as genetics clinics are routinely used for newborns identified with congenital hearing loss. Improved identification and enrollment of Part C children prior to 12 months of age will also occur as a result of a comprehensive universal hearing screening program. Early identification and early intervention for all Nebraska children and for disabilities beyond congenital hearing loss is likely to be enhanced by the implementation of a comprehensive, collaborative universal newborn hearing screening program in Nebraska.