On a recent road trip I passed a big flashing sign on the side of the road that said, “More Road Work Ahead.” A few minutes later, traffic slowed as I passed orange cones, people with fluorescent green vests holding flags and heavy equipment operators, who were all working to build a better road. Not that the existing road was that bad. But there was a need for something even better.
As I drove through the construction zone, it occurred to me that this was an apt metaphor for Early Hearing Detection and Intervention (EHDI) programs: More Road Work Ahead.
Fifteen years ago, in March 1993, the National Institutes of Health held a Consensus Development Conference on Early Identification of Hearing Impairment in Infants and Young Children. Not surprisingly, the panel pointed out how important it was to identify permanent hearing loss as early as possible. But many people were surprised when the panel recommended the “screening of all newborns … for hearing impairment prior to [hospital] discharge.” In practical terms, the panel was recommending the implementation of universal newborn hearing screening programs.
Looking back, the “road trip” we started that day was like turning the crank and climbing into the seat of one of the first Model T Fords for a road trip from New York to San Francisco. Even though the ultimate goal was clear, we didn’t have a map, nor were we sure we had a vehicle that was equal to the task. We didn’t know what we would encounter along the way, what shape the roads were in (or if they even existed), or what kinds of road hazards we would find. During the last 15 years it has often been difficult to find metaphorical gas stations and there have been lots of detours, potholes, storms, flat tires and overheated radiators to boot!
And what a ride it has been! Parents, physicians, audiologists, legislators, educators, state and federal public health staff and family advocates have all been an integral part of the progress. As a result, more than 95 percent of all newborns babies are now being screened for hearing loss before leaving the hospital. The average age at which congenital hearing loss is identified has been reduced to two to three months of age in some statewide EHDI programs and thousands of children with hearing loss are being enrolled in early intervention programs before they are six months old.
During the last 15 years, additional evidence has been gathered showing how important it is for children to develop language and cognitive skills during the first 36 months of life. Research has also shown that when permanent hearing loss is not detected early and treated appropriately, there will often be long-lasting and substantial delays in speech, language, and cognitive and social-emotional development, negatively impacting academic performance and potential. In contrast, infants and young children who are identified early and given prompt and appropriate educational, audiologic and medical services do significantly better than similar children who are identified later.
Viewed from our current perspective, it is clear, however, that we have not yet reached our destination. Nationally, state EHDI programs are unable to document that half of the newborns who fail a newborn hearing screening test receive appropriate follow-up testing. The high cost of appropriate hearing aids and cochlear implants means that they are unavailable to many families whose children need and want them and there is an acute shortage of audiologists who have the skill, experience and equipment to provide comprehensive audiologic services. Many families also have difficulty finding appropriate early intervention programs for their children with permanent hearing loss.
So even though newborn hearing screening has become the medical-legal standard of care in the United States, there is still “More Road Work Ahead” before the destination envisioned by the 1993 NIH Consensus Development Conference is reached.
There is widespread agreement that the most urgent area where more work is needed is making sure that infants and young children who fail their newborn hearing screening tests receive timely and appropriate diagnostic evaluations to determine whether hearing loss is present. Unfortunately, analysis of recent state EHDI data reported to the Centers for Disease Control and Prevention showed that of the 2.2 percent of infants referred for follow-up after newborn screening, only 38.7 percent were documented as having received a diagnostic evaluation. Although some of the infants for whom receipt of a diagnostic evaluation could not be documented might have been evaluated and not reported, these data provide strong evidence that many infants who fail newborn hearing screening are not receiving vital diagnostic and early intervention services. A great deal of effort is being devoted to decreasing loss to follow-up, which occurs for a variety of reasons, including:
d Too many newborns are failing hospital-based hearing screening because of poorly trained screeners, poorly maintained equipment and use of inefficient protocols.
d Parents are not given effective information about initial results, need for follow-up, what to do next, etc.
d Accurate screening results are not shared quickly with the people who need to do follow-up including hospitals, state EHDI programs, healthcare providers, audiologists, early interventionists, etc.
d There is a shortage of pediatric audiologists, particularly in rural/remote areas, because of a lack of training programs and poor reimbursement rates for services to infants and young children.
d Lack of knowledge about current “effective practices” among program managers, healthcare providers and early interventionists.
d Not enough public awareness about the importance of early identification of hearing loss among taxpayers, administrators, extended family, etc.
d Lack of resources committed to EDHI programs for screening, follow-up diagnosis, early intervention, case management, etc.
While improving loss to follow-up among newborns currently being screened in hospital-based hearing screening programs is extremely important, there are other areas where there is still “More Road Work Ahead.” Three examples of such work are described briefly below.
Road Work Project #1
A recent survey of a national sample of 1,968 physicians examined their attitudes, practices and knowledge related to newborn hearing screening and intervention. Even though there is broad agreement about the central role physicians should play in ensuring that congenital hearing loss is identified early and treated appropriately, the results of this survey showed that there are many critical gaps. For example, 53 percent of the respondents did not know that infants who were three months of age or younger could be fit with hearing aids and 18 percent thought it necessary to wait until the child was 12 months of age or older.
There is widespread agreement that all infants and young children who are diagnosed with permanent hearing loss should also be seen by an otolaryngologist, a geneticist and an ophthalmologist. The baby’s primary healthcare provider is in the best position to help the family obtain such services from an appropriate provider. However, in the physician survey, the need for a referral to a geneticist was recognized by only 11 percent of pediatricians, three percent of family physicians and 22 percent of otolaryngologists. Furthermore, the need to refer to an ophthalmologist was recognized by only one percent of pediatricians, zero percent of family physicians and seven percent of otolaryngologists.
In cases where hearing loss is so severe that hearing aids cannot provide sufficient amplification for the acquisition of spoken language, physicians should be prepared to discuss information about cochlear implants with families. However, almost 50 percent of respondents were incorrect about the type and level of hearing loss that would make a child an appropriate candidate for a cochlear implant and almost 70 percent reported a lack of confidence in discussing this surgical procedure with families of such children.
These examples clearly demonstrate the need to better educate physicians about currently recommended practice for infants and young children with hearing loss. Armed with appropriate knowledge and skills, physicians can be a valuable resource to families.
Road Work Project #2
To reduce the number of newborns who need follow-up testing after hearing screening in the hospital, many screening programs have begun using a two-stage protocol in which newborns are screened first with otoacoustic emissions (OAEs) and no additional testing is done for those who pass. Newborns who fail the initial OAE are screened a second time with automated auditory brainstem response (A-ABR). Those who pass the A-ABR are considered to have normal hearing, even though they failed the initial OAE test. A recent multi-center study by Johnson et al. investigated how many infants who failed the OAE and passed the A-ABR had permanent hearing loss at approximately nine months of age. Of 86,634 infants who were screened at seven geographically dispersed birthing centers, using a two-stage OAE/A-ABR hearing screening protocol, 973 infants who failed the OAE but passed the A-ABR completed diagnostic audiologic evaluations when they were an average of 9.3 months of age. The study found that 21 infants (30 ears) who had passed the newborn A-ABR hearing screening had permanent hearing loss when they were eight to 12 months of age. Most of these hearing losses were mild, but still significant enough to interfere noticeably with speech and language development and success in school.
This means that 23 percent of the infants with permanent hearing loss in this group would have been missed if babies who failed the OAE but passed the A-ABR were assumed to have normal hearing. Unfortunately, this is exactly what is happening in most newborn hearing screening programs. These results emphasize the need for administrators of newborn hearing screening programs to think carefully about the screening protocols and equipment being used in their program. Simply screening all of the newborns is not enough. Care must be given to ensuring that the most effective screening protocols and equipment are being used.
Road Work Project #3
In addition to making hospital-based screening programs as efficient as possible, public health officials should be aware of the need to also conduct hearing screenings during the early childhood years. Such screening in daycare and preschool programs or during well-child visits to healthcare providers can be a useful tool for detecting late onset permanent hearing loss, as well as hearing loss that is missed during newborn hearing screening.
The American Speech-Language-Hearing Association estimates that the incidence of permanent hearing loss more than triples, from three per 1,000 to three per 300, by the time children are five years of age. For these older infants, toddlers and preschoolers to be identified in a timely manner, systematic hearing screening at periodic intervals will be necessary because there is good evidence that parents are not good at detecting when their child has a hearing loss.
Unfortunately, after the newborn period, very few children under five are regularly screened for hearing loss using objective screening tools. In a positive step towards the goal of systematic hearing screening during the early childhood years, the American Academy of Pediatrics recently began recommending that all children receive “an objective standardized screening of global development with a validated assessment tool at nine, 18 and 24 to 30 months of age or at any time if the healthcare professional or family has concern … Infants who do not pass the speech-language portion of a medical home global screening or for whom there is a concern regarding hearing or language should be referred for speech-language evaluation and audiology assessment.”
However, implementing hearing screening in physicians’ offices will not be so easy. We know this by how difficult it has been to get healthcare providers to do a quick, subjective hearing screen even when they have a high likelihood of being reimbursed. Specifically, more than 35 percent of all children birth to five years old in the United States are covered by Medicaid. Since its inception, Medicaid has mandated that all eligible children receive early preventive healthcare through the Early and Periodic Screening Diagnosis and Treatment (EPSDT) program but this has never been achieved. A national review of EPSDT revealed that of the 22.9 million children eligible for these services, only 36 percent received a medical screen and only 13 percent received a hearing screen. The report also concluded that the poorest children and those from minority families (which are the children most likely to acquire hearing loss during early childhood), were disproportionately less likely to receive these services.
Another problem is getting healthcare providers and family to follow up once hearing loss has been identified in the healthcare provider’s office. In one recent study, nine pediatric practices were provided with equipment and staff to do hearing screening during well-child visits for three to 19-year-old children. Of the 1,061 children screened, 10 percent failed. Of these, 59 percent had no further evaluation.
Despite such challenges, there are examples of programs that have successfully screened children for hearing loss during the early childhood years. In one such study, young children in Head Start programs were screened for hearing using OAE technology. Of the 3,486 children screened, 95 percent passed and 5 percent (183 children) were referred for a diagnostic evaluation. Of the 119 children who completed a diagnostic evaluation (64 children or 35 percent were lost to follow-up), six had a permanent hearing loss and 74 more had a chronic fluctuating conductive hearing loss that had not previously been detected or treated. Despite the 35 percent lost to follow-up (many of whom were children of migrant workers), some children with hearing loss who would have slipped through the cracks of a system of only newborn hearing screenings were found. These results demonstrate that OAE-based hearing screening of young children can be practical and effective if there is appropriate training, use of the specified protocol and audiologic support.
Otoacoustic emissions testing has proven to be a reliable, easy-to-use screening tool that can be effective in identifying infants, toddlers and preschoolers with permanent hearing loss. Though not used much in healthcare providers’ offices at present, the success of OAE-based hearing screening in Head Start programs suggests that this might also be an effective tool for healthcare providers to use during well-child visits.
Much progress related to early hearing detection and intervention has been made in the last 15 years. But there is still “More Road Work Ahead” if we want to reach the place where all infants and young children with hearing loss are identified early and provided with appropriate audiological, medical and educational intervention that will enable them to reach their full potential. While we continue working to improve existing hospital-based newborn hearing screening programs, we must also pay attention to other opportunities to improve outcomes for infants and young children with hearing loss. The need to educate various stakeholders about recent technological advances, use more effective screening protocols, and expand the settings where hearing screening for infants and young children is done are but three of many examples where more work is needed.
