|Henry Wentworth (at 17 months), whose hearing loss was identified and who receives services through the Massachusetts EHDI program, enjoys the flowers in Chicago's Millennium Park. "Flower" was one of Henry’s first signed words.
"In June of 2007 my son Henry had his hearing screened shortly after he was born. I remember the sinking feeling I felt when the screener told my wife and me that neither of Henry's ears passed. We were told that the screen is not a perfect test, but that Henry should be seen by an audiologist.
"Weeks later at Henry's audiology appointment, we learned that Henry had a hearing loss. It was difficult, both practically and emotionally, to put this into perspective. During this confusing time, we received a call from a parent outreach specialist at the Massachusetts Department of Public Health, Universal Newborn Hearing Screening Program, to discuss Henry's diagnosis and the intervention services that we could get for him. The outreach specialist helped us to identify and organize the next steps in our plan for Henry's care. In addition, talking with another parent of a child with hearing loss helped us realize we weren't the only ones going through this, and bolstered our confidence that we could be strong enough to give Henry a good start and a good life."
Connecting families to other families is a guiding principle of the Massachusetts Universal Newborn Hearing Screening Program. The program is committed to being a coordinated, comprehensive, family-centered, culturally competent system of care that addresses the needs of families whose infants receive newborn hearing screening services in Massachusetts. Audiologists praise the program's determination to ensure that every baby—regardless of a family's language or changing location—receives screening and, equally as important, appropriate diagnostic and intervention services.
Comprehensive State Law
Massachusetts recently celebrated the 10-year anniversary of passage of Chapter 243 of the Acts of 1998, An Act Providing for Hearing Screening of Newborns, one of the strongest newborn hearing screening laws in the nation. The law calls for an early hearing detection and intervention (EHDI) program that requires:
- A hearing screening test to be performed on all newborn infants in the birthing facility prior to discharge
- All health insurers to cover newborn hearing screening with the Commonwealth as payer of last resort
- Hospital newborn hearing screening protocols approved by the Department of Public Health
- Training and supervision of hospital newborn hearing screening personnel by a licensed audiologist
- Referral for audiological follow-up at centers approved by the Department of Public Health, with the Commonwealth as payer of last resort for testing
- An Advisory Committee for statewide newborn hearing screening
Six major components have contributed to the success of the Massachusetts program: engaging stakeholders, screening, diagnosis, intervention, data management, and outreach and follow-up.
Engaging All Stakeholders
The Massachusetts Department of Public Health (DPH) recognizes the importance of engaging key stakeholders in the development of new programs. The Massachusetts Universal Newborn Hearing Screening Program was developed in collaboration with a dedicated community of parents, consumers who are deaf and hard of hearing, legislators, audiologists, physicians, hospital staff, early intervention and specialty-service providers, public health officials, geneticists, and others. The strength of the program is due to their hard work and commitment to the principle of early identification of hearing loss in the newborn population. The Advisory Committee has overseen the development of regulations, guidelines, policies, data collection, and quality assurance efforts, which include ongoing data analysis to understand disparities in receiving follow-up care and access to intervention services, surveys to understand families' level of anxiety and satisfaction, and participation in multi-state evaluation of loss to follow-up in state EHDI Programs.
Representatives from each of the 29 DPH-approved audiological diagnostic centers meet three times per year. "Both the Advisory Board meetings and the diagnostic test centers meetings have had an extremely beneficial effect on interaction among service providers in Massachusetts," said Marilyn Neault, director of habilitative audiology at Children's Hospital Boston. "The stakeholders who care about children with hearing loss have come to respect and inspire one another across the state, to the benefit of the children and families served."
Identifying key contacts in each facility (e.g., newborn hearing screening program directors, audiologists, and data staff) and maintaining contact with them are crucial. It is important to establish guidelines/regulations to ensure screening protocols are carried out consistently throughout the state. Clear communication policies also are key, with families receiving screening results orally and in writing in the family's preferred language.
It is essential that infants who fail the screen receive follow-up. When infants fail the screen, the medical home should be notified and an appointment made for audiological testing prior to discharge. In Massachusetts, follow-up testing is performed exclusively at DPH-approved audiological diagnostic centers.
There are approximately 79,000 births in Massachusetts each year and the newborn hearing screening rate from 2003 to 2006 has been approximately 99% (Centers for Disease Control and Prevention [CDC], 2003, 2004, 2005, 2006). In less than five years (data collected July 2001–December 2006), more than 1,000 infants were diagnosed with permanent hearing loss. The median age of diagnosis in 2006 was 1.25 months of age for newborns who failed their hearing screening(s); 92.8% of newborns who failed their hearing screening(s) received follow-up audiological testing in 2006 (7.2% were lost to follow-up). The CDC estimates that nationally, more than 46% of infants who failed hearing screening in 2006 were lost to follow-up or their results could not be documented (CDC, 2008).
States should identify appropriate audiological follow-up services. Massachusetts audiological diagnostic center guidelines require facilities to submit protocols for DPH approval. The written guidelines include protocols for staffing and support services, including pediatric ratios; information on follow-up and documentation; capabilities for testing procedures; calibration of equipment; pediatric sedation; adherence with the Americans with Disabilities Act; policies for interpreters; management plan for confirmed hearing loss; fees and services; agreement to provide data; and quality assurance/quality improvement indicators. The centers must demonstrate the capacity to make appropriate referrals to specialty care and EI, in collaboration with the child's medical home. If amplification is recommended, plans should be in place to fit the device within one month of diagnosis. The guidelines also require the directors of the centers to meet three times per year for trainings developed by the EHDI program.
According to the Joint Committee on Infant Hearing (JCIH), the goal of EHDI is to maximize linguistic competence and literacy development for children who are deaf or hard of hearing. Infants with hearing loss should receive appropriate intervention at no later than 6 months of age. Professionals with expertise in hearing loss should provide the intervention services.
In Massachusetts, all children with hearing loss are eligible for early intervention. Of the 226 infants identified with hearing loss in Massachusetts in 2006, 152 (67.3%) were enrolled in EI (20 were deceased, declined services, or moved out of jurisdiction). Fifty-four infants (23.9%) were not documented as receiving early intervention services in Massachusetts and CDC statistics indicated 33.9% nationally were not documented as receiving services. According to analysis of data (Liu et al., 2008), families with infants with milder degrees of hearing loss or unilateral hearing loss were less likely to utilize EI services compared to families of children who have more severe loss of hearing.
The JCIH recommends uniform state registries and national information databases that incorporate standardized methodology, reporting, and system evaluation. The success of state EHDI programs is measured by minimizing the number of infants who become lost to follow-up/lost to documentation (LTF/LTD).
Massachusetts has a statewide surveillance and tracking system created to assist staff in implementing and evaluating newborn hearing screening program activities. The system has three components: electronic birth certificate (EBC), audiological evaluation report, and family intake record. The EBC provides screening results, and demographic and medical information for all infants screened for hearing loss in Massachusetts, and is linked to early intervention data to ensure families receive services. Routine reports are distributed to hospitals and audiological centers to complete missing data and monitor quality.
Outreach and Follow-up
Assuring a low false-positive screening rate is a critical component of performing effective outreach. The Massachusetts EHDI staff use data collected to ensure screening is performed and to call families to confirm that an appointment is made when newborns fail a hearing screen. Staff stay in touch with the family until a diagnosis is achieved and assist audiologists in tracking missed appointments. Massachusetts EHDI staff developed a parent information kit that is provided to each family by the audiologist at diagnosis. The parent outreach specialist calls each family soon after diagnosis to connect the family to other state resources and provides support and technical assistance. The parent outreach specialist stays in contact with the family until the family is enrolled in early intervention and continues to be available for support as needed. Families are connected to ongoing support through the Massachusetts Commission for the Deaf and Hard of Hearing, early intervention programs, and Family TIES, a parent-to-parent match program.
Delivering culturally competent services will improve care and assist in meeting the EHDI goals. Programs and providers should value diversity, increase cultural awareness of staff, and employ a diverse, culturally and linguistically competent workforce. The Massachusetts EHDI Program uses EBC information to identify ethnicity and the preferred languages of parents, including American Sign Language (ASL), and tailors outreach efforts accordingly. Staff seek to be responsive to each family's needs.
A strong EHDI program will ensure that infants with hearing loss have a medical home. Communication with the medical home should occur at all stages of the EHDI process. According to the JCIH, the infant's pediatrician or other primary health care professional is responsible for monitoring the general health, development, and well-being of the infant. That professional must ensure that the audiological assessment is conducted on infants who do not pass a hearing screen and must initiate referrals for medical specialty evaluations. Infants with confirmed hearing loss should be evaluated by an otolaryngologist who has knowledge in pediatric hearing loss and have at least one examination to assess visual acuity by an ophthalmologist experienced in evaluating infants.
In addition, families should be offered a genetics consultation. Each state has a designated American Academy of Pediatrics champion who works with the state to ensure resources about hearing loss are available to providers.
Quality Assurance and Quality Improvement
Parental satisfaction is evaluated at all levels of the EHDI system in Massachusetts. In a recent study conducted in the state, parents of newborns who had been screened for hearing were asked if they were to have another baby, would they want to have the baby's hearing screened. Ninety-nine percent of families responded "yes" to this question. The study also indicated that parents were grateful for identification of hearing loss and early intervention services, and supported newborn hearing screening nationwide (MacNeil, Farrell, Liu, & Stone, 2007).
Additionally, Massachusetts uses data to understand disparities in care, including factors that correllate with a greater risk of some infants becoming lost to follow-up. Infants born to mothers who were nonwhite, covered by public insurance, smokers during pregnancy, or residing in western, northeastern, or southeastern Massachusetts were at higher risk of becoming lost to follow-up on audiologic evaluation.
Of children with hearing loss, those with a unilateral, mild, or moderate degree of hearing loss, normal birth weight, or living in the southeastern or Boston region were more likely to go without early intervention services (Liu et al., 2008).
Massachusetts data clearly indicate that some infants who pass their newborn hearing screen are later identified with hearing loss. EHDI programs must remain vigilant to ensure that children with later-onset hearing loss are identified as early as possible and are connected to services. The JCIH risk criteria should be used and developmental milestones, hearing skills, and parental/caregiver concern should be monitored by the child's medical home.
"Having a strong centralized database and follow-up procedure is key to any good screening program, but the Massachusetts Department of Public Health's EHDI Program sets the gold standard," according to Neault.
"Given a legislative mandate with minimal funding, a small staff excels in grant procurement and implementation for data management and follow-up. They keep high professional standards while doggedly chasing every baby whose screening result has not been resolved, no matter what language the parent speaks or how many times the last name or address have changed.
"The audiologists in Massachusetts are well aware how fortunate we are to have such a strong DPH driving the success of our EHDI program," Neault said. "We see the results every day in our work, serving toddlers with early-identified hearing loss whose language development is on par with their hearing peers."
EHDI Success/Henry at 20 Months
The system of care in Massachusetts has contributed to the early identification of Henry's hearing loss. His father, Richard Wentworth, said, "Henry is currently seen twice a week by his early interventionists and also attends an EI playgroup twice a week. He wears bilateral hearing aids and is comfortable using the growing number of signs that he has learned (upwards of 45 words at last count) to communicate needs and feelings, and to describe what he sees.
"Recently, Henry has begun using more verbal cues. His receptive language, both verbal and signed, is excellent. He loves reading time and enjoys meeting new people. In short, Henry is exactly where he needs to be, and we couldn't be prouder of him!"
The story of Henry and his family—and the stories of hundreds of other young children in the state—are the driving force behind the Massachusetts EHDI initiative.