Remember when high-definition (HD) big-screen televisions first hit the market? The price tags on those sets were pretty high, and some market analysts doubted that consumers would consider HD technology a must-have item. Today, however, HD flat screens can be found in multiple rooms of many households.
Speaking of screens, newborn hearing screening has experienced a similar evolution over the past 15 to 20 years. In the mid-90s, pediatric hearing healthcare practitioners knew we were missing the mark, with the average age at identification of congenital hearing loss hovering around 3 years old (National Institutes of Health Consensus Development Conference Statement, 1993 http://consensus.nih.gov/1993/1993hearinginfantschildren092html.htm), but researchers, experts, and legislators expressed skepticism about universal newborn hearing screening (UNHS) as a viable solution ( Pediatrics 1994;93:330-334 http://pediatrics.aappublications.org/content/93/2/330.abstract).
In spite of these reservations, advocates continued to push for the implementation of universal newborn hearing screening, with numerous researchers demonstrating the feasibility and effectiveness of such programs ( Ear Hear 2000;21:85-91 http://journals.lww.com/ear-hearing/Fulltext/2000/04000/The_New_York_State_Universal_Newborn_Hearing.3.aspx; J Pediatr 1998;133:353-357 http://www.jpeds.com/article/S0022-3476(98)70268-9/abstract; Semin Hear 1993;14:1-118 https://www.thieme-connect.com/products/ejournals/issue/10.1055/s-002-12994).
In the real world of pediatric hearing healthcare, early hearing detection and intervention (EHDI) has been a remarkable success. States are typically screening about 95 percent of infants born in hospital settings, and, in states with effective implementation of UNHS, children's average age at identification of hearing loss is 2 to 3 months ( Ear Hear 2003;24:89-95 http://journals.lww.com/ear-hearing/Fulltext/2003/02000/Trends_in_Age_of_Identification_and_Intervention.10.aspx; Hawaii Med J 1997;56:352-355 http://www.ncbi.nlm.nih.gov/pubmed/9447797; Pediatrics 2010;126[suppl 1]:S52-S58 http://pediatrics.aappublications.org/content/126/Supplement_1/S52.full; J Pediatr 1998;133:353-357 http://www.jpeds.com/article/S0022-3476(98)70268-9/abstract).
We dedicate this column on the contemporary state of universal newborn hearing screening to experts and advocates who have advanced the cause, including, but certainly not limited to: Marion Downs, Karl White, Betty Vohr, the late C. Everett Koop, Beth Prieve, Jay Hall, Lynn Spivak, Martin Hyde, John Bamford, Christine Yoshinaga-Itano, Susan Norton, and Karen Munoz.
1. CHECKING THE SCOREBOARD
The Joint Committee on Infant Hearing (JCIH) has established a one-three-six objective, which stands for screening by 1 month of age; diagnostic assessment, if needed, by 3 months; and intervention, when required, by 6 months.
About 97 percent of infants receive newborn hearing screening, according to a 2009 survey reported by the Centers for Disease Control and Prevention (CDC; bit.ly/CDC-EHDI http://bit.ly/CDC-EHDI). About 94 percent of infants complete the screening process during their first month of life.
Unfortunately, we have not enjoyed quite the same success in the latter two pillars of our one-three-six goal. The CDC reports that diagnostic assessment is completed by three months of age for only 68 percent of infants who do not pass the hearing screening. Furthermore, approximately 40 percent of children are lost to follow-up (bit.ly/CDC-follow-up http://bit.ly/CDC-follow-up).
In addition, of infants who are diagnosed with hearing loss, almost 90 percent are referred for intervention, but only about 67 percent receive the intervention services they need, with the overwhelming majority again lost to follow-up (bit.ly/CDC-follow-up http://bit.ly/CDC-follow-up).
When we implement solid UNHS protocols, we find that referral rates fall well below the four-percent JCIH benchmark ( Pediatrics 2007;120:898-921 http://pediatrics.aappublications.org/content/120/4/898.full). In fact, recent reports indicate that many UNHS programs boast a referral rate of less than one percent or two percent ( Hawaii Med J 1997;56:352-355 http://www.ncbi.nlm.nih.gov/pubmed/9447797; Ear Hear 2000;21:85-91 http://journals.lww.com/ear-hearing/Fulltext/2000/04000/The_New_York_State_Universal_Newborn_Hearing.3.aspx; J Pediatr 1998;133:353-357 http://www.jpeds.com/article/S0022-3476(98)70268-9/abstract).
Current CDC estimates suggest that around 13 percent of children who refer in a universal newborn hearing screening end up having a significant hearing loss (bit.ly/CDC-follow-up http://bit.ly/CDC-follow-up).
In contrast, in the Wyoming EHDI program—which uses a two-stage screening, with the first screen occurring in the hospital newborn nursery and the second screening on an outpatient basis—the rate of referral for diagnostic assessment is 0.3 percent, and 80 percent of infants referred for assessment have permanent congenital hearing loss (The Hearing Review Jan. 1, 2005; bit.ly/WY-EHDI http://bit.ly/WY-EHDI).
2. OAE VS AABR
Otoacoustic emission (OAE) testing and the automated auditory brainstem response (AABR) are the only measures that may be used for universal newborn hearing screening, the JCIH determined. Each measure has advantages and limitations.
AABR is the only screening measure that can identify neural hearing losses, but it may be more likely to miss mild hearing loss or configured hearing loss.
In contrast, OAEs are more likely to catch mild and configured hearing losses. However, the use of otoacoustic emissions on the infant's day of birth means a higher likelihood of a failed screening because the OAE response is more susceptible to attenuation by vernix caseosa in the ear canal ( Int J Audiol 2007;46:680-685 http://informahealthcare.com/doi/abs/10.1080/14992020701459868; Int J Audiol 1994;33:152-164 http://informahealthcare.com/doi/abs/10.3109/00206099409071876).
In terms of which test is more cost-effective, AABR supplies are more expensive than OAE supplies, but many studies suggest a higher failure rate for OAE screening, which results in increased costs for the EHDI system.
3. A PRO'S PROTOCOL
If a child spends more than five days in the neonatal intensive care unit (NICU), then an AABR screening must be conducted rather than OAE, the JCIH recommends in its 2007 Position Statement ( Pediatrics 2007;120:898-921 http://pediatrics.aappublications.org/content/120/4/898.full), addressing the higher incidence of neural hearing loss in the NICU. Babies who fail the AABR screening in the NICU should be referred directly for diagnostic audiology assessment.
In the well-baby nursery, AABR should be the screening tool of choice if screenings are conducted within the first few hours after birth ( Int J Audiol 2007;46:680-685 http://informahealthcare.com/doi/abs/10.1080/14992020701459868; Int J Audiol 1994;33:152-164 http://informahealthcare.com/doi/abs/10.3109/00206099409071876). After about 24 hours, both OAE and AABR have acceptable referral rates.
When rescreening a child who passed the test in one ear and failed in the other, both ears should be screened.
Most hospitals conduct a two-stage screening approach, with the initial screening administered around the first full day after birth or the first 24 hours after birth, and the subsequent screening occurring either immediately before discharge or a week later on an outpatient basis.
Automated auditory brainstem response may be used in both screening stages. Otoacoustic emission screening should never be conducted after a baby fails an initial AABR screening, the JCIH recommended ( Pediatrics 2007;120:898-921 http://pediatrics.aappublications.org/content/120/4/898.full). In that case, the AABR should be repeated.
It is also common for hospitals to use OAE for the initial screen and AABR for the follow-up screen. This approach has been shown to be a good compromise, reducing screening costs and referral rates. However, in one study, children who failed OAE screening and passed the follow-up AABR screening were later determined to have mild sensory hearing loss ( Pediatrics 2005;116:663-672 http://pediatrics.aappublications.org/content/116/3/663.abstract).
To reduce screening costs, some hospitals have moved to a two-stage OAE/OAE screening. When implemented effectively, this approach has been shown to possess a low referral rate ( Int J Audiol 2003;42:443-447 http://informahealthcare.com/doi/abs/10.3109/14992020309081514; J Am Acad Audiol 2004;15:414-425 http://www.ncbi.nlm.nih.gov/pubmed/15341223; Pediatrics 2005;116:663-672 http://pediatrics.aappublications.org/content/116/3/663.abstract).
In Oklahoma, the Newborn Hearing Screening Program is linked with the state's Newborn Metabolic Disorder Screening Program. The first AABR screening takes place 12 hours after birth, optimally, and occurs no earlier than six hours after birth. Then, if needed, a second AABR screening takes place close to 24 hours after birth so that the results of the hearing and metabolic screenings come to the state laboratory at the same time. This approach has significantly improved tracking for follow-up purposes.
4. CURRENT CHALLENGES
An excellent study conducted by Shanna Shulman and other stakeholders found that one of the most significant barriers hampering adequate follow-up in newborn hearing screening was a lack of provider knowledge of the EHDI process ( Pediatrics 2010;126[suppl 1]:S19-S27 http://pediatrics.aappublications.org/content/126/Supplement_1/S19.full). Only one-third of UNHS screeners had received training on how to present information to families of infants who failed hearing screening.
In addition, about one-half of respondents indicated that their UNHS program did not have sufficient access to pediatric audiologists capable of providing accurate diagnostic assessment. Identifying qualified pediatric audiologists and early interventionists was particularly an issue in less populated states.
Many programs reported that pediatricians did not regularly review screening outcomes or that states failed to provide pediatricians with ready access to the results. Also, pediatricians often had a “wait-and-see” attitude about the need to pursue further testing following a failed hearing screening.
Furthermore, families faced challenges to obtaining services. Some did not have transportation to the follow-up appointment or were unable to get insurance coverage to pay for the assessment. When the family does not speak English proficiently, communication can be an issue, and families who moved frequently were sometimes difficult to serve.
Half of the survey respondents reported that hospitals did not provide sufficient information on infants who failed hearing screening, with screening results frequently not including contact information for the family. Also, an adequate network did not exist between providers to share information about infants’ status within the UNHS system.
While the JCIH has identified the medical home as the primary resource responsible for ensuring that every infant receives appropriate EHDI services ( Pediatrics 2007;120:898-921 http://pediatrics.aappublications.org/content/120/4/898.full), approximately 20 percent of infants did not have a documented medical home, Dr. Shulman and colleagues found.
5. CUTTING OUR LOSSES
To address EHDI programs’ less than stellar loss-to-follow-up record, we can start by improving our communication, beginning with the information provided by screeners to the families of infants who fail screening.
The National Center for Hearing Assessment and Management (NCHAM) provides excellent scripts that depict exactly how a screener should counsel caregivers of infants who have passed or failed a hearing screening, or who could not have screening completed.
There are separate scripts for counseling families of infants who are at high risk versus low risk for hearing loss. Information should be provided to families in verbal and written format, ideally in their native language.
Semantics plays a role in the discussion of the results. The term “refer” has often been used to indicate a child who did not pass the hearing screening and needs to be referred for additional testing. We believe that the phrase “did not pass” invokes a stronger call to action to follow up with diagnostic assessment.
It is highly desirable to schedule an appointment for follow-up assessment before the family leaves the screening facility. The screener should reconfirm the names of the infant and caregivers, verify the caregiver's contact information, and obtain names and telephone numbers for at least two additional contacts.
Ideally, screening results should be provided in multiple ways, including documentation in the infant's medical chart and to the state's EHDI program, and written and verbal reports to the medical home and attending physician at the hospital.
In terms of addressing a family's financial constraints, existing state programs, such as Medicaid, Part C Early Intervention, and Title V, should be accessed. Hospitals may identify attending pediatricians who can request pre-authorizations necessary for insurance companies to pay for follow-up assessment. Also, state EHDI programs typically are equipped with resources families can use to receive requisite hearing healthcare services.
State EHDI programs must be staffed with personnel assigned to ensure that children are not lost to follow-up. The Oklahoma Newborn Hearing Screening Program has a follow-up/audiology coordinator who serves as the liaison between the state EHDI system and the organizations, audiologists, early interventionists, etc., who are completing screening and assessment, as well as with parents. Recent reviews indicate the loss to follow-up/loss to documentation rate has steadily decreased in Oklahoma since the addition of this position, dropping as low as 16 percent.
We encourage any audiologist who is interested in providing EHDI services to seek the guidance and mentorship of the many experts in our field. It is also helpful to create peer networks in which difficult cases and clinical protocols may be discussed.
6. KNOWLEDGE IS POWER
If you're overwhelmed by the task of developing systems, materials, and scripts for communicating with families, providers, and your state's UNHS program, do not fret. Many states have their own systems and materials for facilitating the EHDI process. If you need more information, we encourage you to use the follow resources:
- The National Center for Hearing Assessment and Management (NCHAM) is a one-stop shop for EHDI information. It offers an excellent interactive training module, as well as a comprehensive e-book, slide presentations, and peer-reviewed research articles: bit.ly/NCHAM-Training http://bit.ly/NCHAM-Training.
- The Centers for Disease Control and Prevention website is a particularly good place to find UNHS/EHDI statistics and review the progress we are making: bit.ly/CDC-statistics http://bit.ly/CDC-statistics.
- The British Newborn Hearing Screening Program has great information for professionals and families alike: hearing.screening.nhs.uk http://hearing.screening.nhs.uk/.
Families may also be directed to these sites for helpful information:
- Boys Town National Research Hospital: babyhearing.org http://babyhearing.org/.
- Alexander Graham Bell Association for the Deaf and Hard of Hearing: listeningandspokenlanguage.org http://listeningandspokenlanguage.org/.
- John Tracy Clinic: jtc.org http://www.jtc.org/.
- NCHAM: communicatewithyourchild.org http://communicatewithyourchild.org/.
7. NEW TOOLS IN OUR TOOL BAG
Recent research has identified several audiologic measures that may harbor excellent potential to improve the efficacy of UNHS.
For example, a number of studies have examined the chirp stimulus to elicit the auditory brainstem response (ABR). The ABR elicited by the chirp stimulus is typically more robust than that evoked by a click, and it may be easier to record ABR responses closer to behavioral threshold ( J Acoust Soc Am 2010;128:215-223 http://scitation.aip.org/content/asa/journal/jasa/128/1/10.1121/1.3397640). More research is needed to examine the utility of the chirp for UNHS.
Recent research has shown wideband reflectance (WBR) to be a potential tool to identify conductive hearing loss and identify the presence of otitis media with effusion ( Ear Hear 2010;31:221-233 http://journals.lww.com/ear-hearing/Fulltext/2010/04000/Wideband_Reflectance_in_Normal_Caucasian_and.8.aspx; Int J Audiol 2008;47[suppl 1]:S57-S61 http://informahealthcare.com/doi/abs/10.1080/14992020802294057; Ear Hear 2010;31:599-610 http://journals.lww.com/ear-hearing/Fulltext/2010/10000/Wideband_Reflectance_in_Newborns__Normative.2.aspx). In addition, researchers have shown that the WBR can measure acoustic reflex.
Combining OAE and WBR screening measures is feasible, as each measure requires very similar instrumentation. However, further research is needed.
8. GENETICS: PRESENT AND FUTURE
Genetic screening may someday be incorporated into the newborn screening process. A blood sample is already collected to screen for metabolic disorders. This sample could easily be screened for the most common causes of congenital hearing loss, such as Connexin 26 mutations and Pendred syndrome.
For parents of children newly diagnosed with hearing loss, surveys have suggested that the most pressing concern is determining the cause of the hearing loss. Our current knowledge of genetics allows us to identify the cause of most congenital hearing losses.
Furthermore, knowing the etiology of the hearing loss can assist in the provision of treatment and determination of prognosis. Certain genetic causes of hearing loss are associated with other disorders that may require separate intervention.
As our knowledge of the genetics of hearing loss evolves, we may someday be able to identify infants at risk for progressive hearing loss. These infants could receive frequent audiological assessments throughout the first few years of life.
9. MUCH ADO ABOUT NOTHING?
There has been some concern that UNHS may create significant anxiety for parents, particularly when a child fails the hearing screening, and that a failing result may hamper the early parent–child bonding process. A fairly sizable body of published studies has suggested that these concerns may be unwarranted ( Acta Paediatr Suppl 1999;88[suppl 432]:73-75 http://onlinelibrary.wiley.com/doi/10.1111/j.1651-2227.1999.tb01164.x/abstract; Am J Audiol 2000;9:135-141 http://aja.pubs.asha.org/Article.aspx?articleid=1773851; J Matern Fetal Neonatal Med 2013;26:932-935 http://informahealthcare.com/doi/abs/10.3109/14767058.2013.766687).
Although some parents indicate higher stress levels when learning that their baby failed a hearing screening, they generally report that their increased anxiety was temporary and that they felt the hearing screening was important and justified.
The key to reducing anxiety seems to reside in two areas. First, it is very important that parents be informed of the hearing screening prior to its administration so that they can learn about the process and ask questions.
Second, it is important that the screener or attending physician/nurse provides a detailed agenda of what needs to take place following a failed screening. Having a sound and expeditious plan of action is reassuring for the family.
10. THE BOTTOM LINE
Twenty years after the initial skepticism about universal newborn hearing screening, it now is difficult to argue against the merits of the early hearing detection and intervention system. In states that have implemented quality UNHS programs, the age at identification of hearing loss has decreased from approximately 3 years to 3 months.
We also fully understand the benefits of early intervention for outcomes in children with hearing loss ( Ear Hear 2013;34:535-552 http://journals.lww.com/ear-hearing/Fulltext/2013/09000/Outcomes_of_Early__and_Late_Identified_Children_at.2.aspx; Pediatrics 1998;102:1161-1171 http://pediatrics.aappublications.org/content/102/5/1161.full) and the cost savings associated with early identification and intervention.
Two years ago, the federal government authorized funding to continue support of newborn hearing screening for a three-year period. As such, we are fast approaching the time of the next decision on whether to authorize funding for UNHS.
In addition, recent discussions have suggested that sequestration will result in a five percent to 30 percent cut in federal funding appropriated for UNHS. Such a cut would seriously cripple our efforts to operate effective universal newborn hearing screening programs.
Martyn Hyde estimated the increased cost of conducting UNHS compared with the cost of selective screening based on the presence of risk factors associated with hearing loss. (Hyde M. Principles and methods of population hearing screening in EHDI. In: Seewald R, Tharpe AM, eds. Comprehensive Handbook of Pediatric Audiology. San Diego, CA: Plural Publishing; 2010.)
In a birth cohort of 100,000 infants, the increased cost of UNHS is roughly around $18,000 per baby identified, Dr. Hyde estimated. This figure seems quite small compared with the excessive costs necessary to educate and support children who do not receive proper hearing healthcare. In particular, when hearing loss is not identified at birth and children go without early intervention, an additional $420,000 is spent on educational needs ( Semin Hear 1993;14:105-118 https://www.thieme-connect.com/products/ejournals/abstract/10.1055/s-0028-1085108).
When viewed from this perspective, the costs of universal newborn hearing screening are a worthy investment. We must, as a field, continue to advocate for funding to support our UNHS programs.