Early Identification of Hearing Loss: The Best of Times...The Worst of Times
The current status of newborn hearing screening and intervention programs in the United States is analogous to the opening lines of Charles Dickens’ novel, A Tale of Two Cities: “It was the best of times, it was the worst of times, it was the age of wisdom, it was the age of foolishness.”
Every day in the U.S., 33 babies are born with significant permanent hearing loss. That is more than 12,000 babies per year, making hearing loss the most frequent birth defect in America. If a baby’s hearing loss is not identified early, it will interfere with the infant’s ability to develop speech, language and cognitive skills, to make friends and interact with family members and, ultimately, to succeed in school and all other aspects of life. As just one example of how devastating hearing loss can be, consider the following. Twenty years ago, before the advent of newborn hearing screening, the average deaf adult in the U.S. read at the third grade level, despite the fact that our educational systems were spending tens of thousands of dollars each year in special education services for each deaf child to try to overcome the deficits caused by permanent hearing loss.
The importance of identifying permanent hearing loss as early as possible had been recognized for many years but little progress was made until recently. The issue was given increased emphasis in 1989 by Dr. C. Everett Koop in his post as U.S. surgeon general. Recognizing that technological advancements had created the right circumstances to significantly reduce the age at which children with hearing loss could be identified, Dr. Koop issued the following challenge to the country:
“Early intervention with hearing impaired children results in improved language development, increased academic success and increased lifetime earnings … and actually saves money since hearing impaired children who receive early help require less costly special education services later. … I am optimistic. I foresee a time in this country ... when no child reaches his or her first birthday with an undetected hearing impairment.”
At the time Koop issued the challenge, less than three percent of all newborns were being screened for hearing loss and there were very few audiologists who had the skills, experience and equipment to accurately diagnose hearing loss in one-month-old babies. A few years later in 1993, the National Institutes of Health held a Consensus Development Conference on Early Identification of Hearing Impairment in Infants and Young Children, the resulting recommendation of which was “screening of all newborns ... for hearing impairment prior to [hospital] discharge.”
Since that time, there has been steady growth in the number of newborns being screened for hearing loss. The National Center for Hearing Assessment and Management recently reported that approximately 95 percent of all newborns in the U.S. are now being screened for hearing loss (Figure 1).
Such progress, to use the words of Dickens, has made this “the best of times” for infants and toddlers with hearing loss and their families. The expansion of newborn hearing screening, coupled with technological improvements in equipment and techniques for audiological diagnosis, has also dramatically increased the probability that newborns with congenital hearing loss will be identified at much earlier ages than was the case 20 years ago. When newborn hearing screening programs are effectively implemented, the average age at identification drops from two to three years old to two to three months old. By dramatically lowering the age of identification, many more children with hearing loss are able to have access to auditory input and begin developing language at much younger ages than was previously possible. Families are also able to receive support services earlier, which reduces stress and enables them to be more effective participants in their child’s educational activities.
Another positive development is that 41 states have now passed legislation related to newborn hearing screening, which has resulted in increased coordination between state agencies, enhanced data reporting systems for more effective tracking of babies who need services and use of more comprehensive educational and public awareness activities. Stimulated by a relatively small amount of federal money, every state and territory has also established an early hearing detection and intervention (EHDI) program, in most cases as a part of the Department of Health. These agencies are responsible for developing a statewide EHDI program that screens all newborns, completing diagnostic evaluations before three months of age for all infants who do not pass the screening and enrolling all children with permanent hearing loss in appropriate early intervention programs before six months of age.
Even though a great deal of progress has been made, it would be wrong to conclude that we are anywhere close to accomplishing what Dr. Koop challenged us to do in 1989. Many babies who are referred from newborn hearing screening are lost to follow-up (Figure 2). For these children and their families, it’s “the worst of times.” It is clearly unacceptable that approximately one-third of babies who do not pass the newborn hearing screening test are not known to get the follow-up they need.
Another problem is that most of the legislation passed by states related to newborn hearing screening has been unfunded mandates, making it very difficult to develop the systems of care needed to meet the needs of these infants and toddlers. Although federal funding has enabled states to begin to develop statewide EHDI systems, each year for the past four years, the president’s budget has proposed to eliminate federal funding for newborn hearing screening and intervention programs. Such cuts would jeopardize the progress that states have made during the last decade. Congress has always restored the money for state EHDI programs in the approved budget but the uncertainty about whether the money will be there makes it difficult for states to plan ahead. (For an up-to-date report of the status of federal funding for newborn hearing screening programs, visit www.agbell.org.)
Another reason that this is still “the worst of times” for many families is that the nation’s early intervention system is not yet well prepared to provide high quality services to the increasing number of deaf or hard of hearing children who are being identified at ever younger ages. Although each state and territory decides on the specific components of their early intervention system, a significant amount of funding and guidance for these efforts comes from Part C of the Individuals with Disabilities Education Act that was recently reauthorized by Congress. In a recent letter sent to all Part C state program directors by the U.S. Department of Education and the U.S. Department of Health and Human Services, federal officials noted that there is a “growing national crisis in the provision of essential early intervention and healthcare services for infants and toddlers with hearing loss.” The letter continued: “Studies have demonstrated that when hearing loss of any degree, including mild bilateral or unilateral hearing is not adequately diagnosed and addressed, the hearing loss can adversely affect the speech, language, academic, emotional and psychosocial development of young children. Although efforts to identify and evaluate hearing loss in young children have improved, there is still anecdotal evidence to suggest that many young children with hearing loss may not be receiving the early intervention or other services they need in a timely manner that will enable them to enter preschool and school ready to succeed.”
Part of the reason that the current early intervention system frequently does not meet the needs of children with permanent hearing loss is that the Part C system was developed 20 years ago at a time when the majority of children with permanent hearing loss were not being identified until they were at least two years old. Furthermore, at that time the technology for digital hearing aids and cochlear implants was not as well developed as it is now. Not surprisingly, the kinds of services being provided 20 years ago were very different. Consequently, very few Part C early intervention providers in the system today have the specialized training necessary to provide state-of-the-art services to infants and toddlers with hearing loss. Prior to the widespread implementation of universal newborn hearing screening programs, the vast majority of children with hearing loss who were identified before three years of age were those with severe or profound bilateral hearing losses. Today, many infants and toddlers with mild, moderate or even unilateral hearing losses are being identified before three months of age. These children require different services than those needed by children with bilateral severe/profound hearing losses.
To make matters worse, federal funding to states for early intervention programs has actually declined in terms of real dollars available. Though there is currently about $1,500 in federal funding for each child with permanent hearing loss being identified in newborn hearing screening programs, in real dollars at the 1991 level, it amounts to about $1,000.
Just because 95 percent of all babies are now being screened does not mean Koop’s challenge has
been met. Technological developments in screening equipment have provided the tools that enable us to do quick, inexpensive and effective newborn hearing screening, but that screening has not yet been successfully linked to systems of precise diagnosis, early intervention, medical care and family support. With the help of federal funding, states have made great progress in putting those systems into place but the job is far from finished and continued federal funding, along with increased support from states, is essential to continue making progress.
Newborn hearing screening is a simple and inexpensive procedure that results in dramatic benefits for the babies who are identified early and provided with appropriate services, and their families. Having the potential to make such a difference in the lives of children and their families means that “the best of times” is within our grasp. For the children and families for whom that potential is not achieved, it will remain “the worst of times.”
