American Speech-Language-Hearing Association

Expert Panel Recommendations on Newborn Hearing Screening

These Expert Panel Recommendations (EPR) on Newborn Hearing Screening were developed by the American Speech-Language-Hearing Association (ASHA) Newborn Hearing Screening Working Group. Members of the working group included Beth Prieve (chair), Kathryn Beauchaine, Diane Sabo, Anne Marie Tharpe, and Anne Oyler (ex officio). ASHA Vice Presidents for Professional Practices in Audiology Jaynee Handelsman (2010-2012) and Donna Fisher Smiley (2013-present) served as the monitoring officers.

Executive Summary

Screening all newborns for hearing loss and providing them with rehabilitation as soon as indicated are advocated by professional organizations in the United States and worldwide (Joint Committee on Infant Hearing [JCIH], 2007; National Institutes of Health [NIH], 1993; World Health Organization [WHO], 2010. Evidence-based systematic reviews (EBSRs) indicate that universal newborn hearing screening (UNHS) has reduced the age of identification of hearing loss and improved language outcomes for those identified (U.S. Preventive Services Task Force [USPSTF], 2002, 2008; Institute for Quality and Efficiency in Healthcare [IQWIG], 2007). ASHA's Newborn Hearing Screening Working Group, hereafter referred to as the Working Group, along with the National Center for Evidence-Based Practice (N-CEP), conducted an EBSR that addressed the effectiveness of newborn hearing screening tools (Evidence-Based Systematic Review of Newborn Hearing Screening Using Behavioral Audiometric Threshold as a Gold Standard [PDF]). The main conclusion of the EBSR was that both otoacoustic emissions (OAEs) and auditory brainstem response (ABR) had a moderate likelihood of identifying permanent hearing loss as confirmed by behavioral thresholds later in life. Additional questions regarding optimal stimulus and response characteristics, test/re-test reliability, screening personnel, and parental stress associated with failed newborn hearing screening could not be addressed through an EBSR because of lack of quality literature. Therefore, in the absence of guidance from the EBSR, the Working Group presents expert panel recommendations that address various aspects of universal newborn hearing screening procedures, follow-up, and future research directions. These recommendations are based on a consensus regarding best practices drawn from published literature (for details on the EBSR process, see Evidence-Based Systematic Review of Newborn Hearing Screening Using Behavioral Audiometric Threshold as a Gold Standard [PDF]).

Definitions

Diagnostic evaluation
Comprehensive examination of hearing to determine if hearing loss is present and, if so, the type, degree, and configuration.
Early Hearing Detection and Intervention (EHDI) program
Program designed to identify newborns with hearing loss by universal newborn hearing screening and to enroll identified newborns in early intervention programs.
Fail or refer
Screening results that indicate a likelihood of hearing loss that requires a re-screening or diagnostic evaluation.
Negative Likelihood Ratio (LR-)
Probability that a newborn with hearing loss will pass a newborn hearing screening divided by probability of a pass for a newborn who does not have a hearing loss (modified from ASHA Evidence-Based Practice Glossary).
Newborn Hearing Screening (NBHS)
Screening conducted in the newborn period.
Newborns
Infants from birth to 30 days of age; for purposes of this document, those undergoing newborn hearing screening, even if they are older than 30 days.
Pass
Screening result for both ears that meets or exceeds set criteria and requires no follow-up, unless risk indicators for late-onset or progressive hearing loss exist.
Permanent Childhood Hearing Loss (PCHL)
All permanent bilateral or unilateral conductive, sensory, neural, or mixed types of hearing loss.
Positive Likelihood Ratio (LR+)
Probability that a person with hearing loss will obtain a result in the positive (affected) range, divided by the probability that a person without the hearing loss will have a result in the affected range (modified from ASHA Evidence-Based Practice Glossary).
Sensitivity
Probability of a positive test when the disorder is truly present, as measured by a gold standard test (Hyde, 2010).
Screening
Examination of seemingly asymptomatic persons to determine if they are likely or unlikely to exhibit the disorder of interest.
Specificity
Probability of a negative test when the disorder is truly absent, as measured by a gold standard (Hyde, 2010).
Universal Newborn Hearing Screening (UNHS)
Screening every newborn for hearing loss.

About This Document

These guidelines are intended for EHDI professionals who participate in the UNHS process. They should be regarded as recommendations for practice, not standards. Every EHDI program has unique characteristics that influence an approach to the newborn hearing screening process. These guidelines supersede the Guidelines for Screening for Hearing Loss—Neonates and Young Infants, Birth through 6 months section of Guidelines for Audiologic Screening (ASHA, 1997). The specific procedural recommendations provided herein are supported by the best available research evidence at this time and by professional experience. Further study of these guidelines is encouraged to evaluate the efficacy of the procedures recommended.

Although it is recognized that newborn hearing screening is but one part of a comprehensive EHDI program of service, this document focuses exclusively on protocols and procedures for newborn screening and refers the reader to other documents for additional guidance in EHDI activities. Specifically, Guidelines for Audiologists Providing Informational and Adjustment Counseling to Families of Infants and Young Children With Hearing Loss Birth to 5 Years of Age (ASHA, 2008) discusses the critical role audiologists play in the provision of family-focused counseling within the context of pediatric hearing health-care delivery. Guidelines for the Audiologic Assessment of Children From Birth to 5 Years of Age (ASHA, 2004) and Audiologic Guidelines for the Assessment of Hearing in Infants and Young Children (American Academy of Audiology [AAA], 2013) outline specific procedures and protocols for serving young children with hearing loss. Information on loss-to-follow-up can be found in Loss to Follow-Up in Early Hearing Detection and Intervention (ASHA, 2008). Guidance on fitting hearing aids to infants and children can be found in Pediatric Amplification (AAA, 2013). The Supplement to the JCIH 2007 Position Statement: Principles and Guidelines for Early Intervention After Confirmation That a Child Is Deaf or Hard of Hearing (2007) provides additional guidance for EHDI programs.

Introduction

It is our recommendation that all newborns be screened for hearing loss. The expected outcome of newborn hearing screening is the identification of newborns who are likely to have hearing loss and require diagnostic evaluation. There are conditions that can be identified at birth that cause late-onset hearing loss and, thus, a second goal of UNHS is to identify those newborns for ongoing monitoring (JCIH, 2007).

JCIH (2007) recommends a time line that includes a hearing screening to be completed no later than 1 month of age. For those newborns who fail the newborn hearing screening, the diagnostic audiologic evaluation should be completed as soon as possible, but no later than when the child is 3 months of age. Newborns diagnosed with hearing loss who need intervention should have hearing technology fitted within 1 month of the diagnosis and be enrolled in an early intervention program no later than at 6 months of age. This 1-3-6 time line emphasizes the importance of moving as quickly as possible beyond the screening to diagnosis and treatment. Newborns in neonatal intensive care units (NICUs) do not meet this time line as a result of medical conditions that cause them to be hospitalized for long periods of time and prevent timely screening or follow-up (Spivak, Sokol, Auerbach, & Gershkovik, 2009). In addition, as noted in a previously published ASHA guideline on audiologic counseling, these benchmarks must be balanced with the reality that certain issues/factors—such as medically compromised/fragile newborns, individual coping styles, family systems, and cultural differences—may not always permit adherence to these guidelines (ASHA, 2008).

Children who pass the newborn hearing screening, but have risk factors for late-onset hearing loss, require ongoing audiologic monitoring. In addition, because some children develop hearing loss and do not have apparent risk factors, it is recommended that all children receive systematic hearing screening through adolescence (Harlor & Bower, 2009). Screenings could be completed as part of their periodic screenings through the medical home, in early education settings (e.g., Early Head Start), or through school screening programs. Furthermore, at a minimum, it is recommended that, in addition to NBHS, hearing screening be provided to all children upon enrollment in intervention programs and school entry.

Targeted Hearing Level

The goal of NBHS is to identify newborns who have PCHL. The recommended hearing level targeted for identification in the newborn period is mild-moderate degree or greater in one or both ears within the mid-to-high frequency range (JCIH, 2007). The exact hearing level is difficult to define for several reasons. First, screening equipment manufacturers choose stimulus levels and characteristics for their own equipment. Second, the stimulus levels and characteristics of screening equipment typically cannot be adjusted by the operator. Finally, and important, there are no calibration standards for transient stimuli or for determining the actual stimulus levels in ear canals of newborns; stimulus levels are greater for newborns than older children and adults, because of the small size of newborns' ear canals (e.g., Kruger & Ruben, 1987). In addition, transducers, which affect the spectral content of stimuli, vary across screening equipment.

Some newborns with congenital hearing loss pass the newborn hearing screening with current screening equipment and protocols (e.g., J. Johnson et al., 2005). Consequently, passing a screening does not ensure normal hearing across the frequency range. For example, ABR uses a transient, broad-frequency stimulus with primary contributions from the mid- to high-frequency range of hearing. Therefore, newborns with "islands" of normal to near-normal hearing within those broad spectra might pass the hearing screen. Although minimal and frequency-specific hearing losses are not targeted by newborn hearing screening programs, they have the potential to interfere with speech and psychoeducational development of children (e.g., Yoshinaga-Itano, DeConde Johnson, Carpenter, & Stredler Brown, 2008). It is the recommendation of the Working Group that, until such time as newborn hearing screening tools are available to detect minimal and mild levels of hearing loss, all reports of newborn hearing screening results reflect this limitation in screening. In addition, children who passed hearing screening as newborns should undergo ongoing surveillance of speech and language development. Minimally, these screenings should occur for all children referred for early intervention services and upon school entry. For more information, refer to the American Academy of Pediatrics Recommendations for Preventive Pediatric Health Care [PDF] (2008).

Protocols

The primary factor that will influence screening technology selection and the protocol to follow is the population to be screened—newborns cared for in the well-infant nursery (WIN) or those in the NICU. Another factor that influences the selection of screening technology and protocol is the personnel conducting the screening. If screening is conducted by anyone other than an audiologist, the equipment must be automated (i.e., use set parameters and provide pass/fail results that do not require interpretation). The number of newborns screened can influence a protocol as well. Protocols for screening large numbers of newborns should minimize test time and disposable costs. Table 1 summarizes protocols used in NBHS programs and reported in the published literature. It is the opinion of the Working Group that any of these protocols can be used, provided the protocol is chosen carefully—to meet a hospital's needs and with the protocol's limitations in mind. Section VII describes general procedures to follow, regardless of the protocol chosen.

Table 1. Published NBHS Protocols Consistent With JCIH Guidelines
Protocol Population Primary Advantages Limitations Equipment Costs

A: ABR only

Figure 1-WIN [PDF]

Figure 2-NICU [PDF]

NICU or WIN

Lower fail rate than for OAEs only 6; will aid in detecting neural hearing loss

Misses some mild hearing loss 3(unless low stimulus level is used)

ABR

Disposable costs higher than for OAEs only; initial test time greater than OAEs only 6

B: OAEs only

Figure 3 [PDF]

WIN

Low 6 disposable costs

Misses neural hearing loss (low prevalence in WIN) 1

OAEs

Disposable and test time low; follow-up costs higher than for ABR due to high inpatient refer rate 6

C: Two Tier—OAEs with ABR rescreen only if OAE is failed

Figure 4 [PDF]

WIN

Lower fail rate than for OAEs only 6

Misses some mild hearing loss 3

ABR + OAEs

Disposable costs vary depending on OAEs fail rate; protocol balances high inpatient referral rate and cost of ABR 5,6

D: Two Technology—ABR and OAEs

Figure 5 [PDF]

NICU or WIN

Will aid in detecting neural hearing loss 4

Time and cost intensive compared to other protocols 1

ABR + OAEs

Disposable cost and test time higher than for one-technology test or two-tier tests 1,4

1 Berg et al.,2011; 2 Gravel et al., 2005; 3 J. Johnson et al., 2005; 4 Kirkim et al., 2008 ; 5Meier et al., 2004; 6 Vohr et al., 2001

A. Screening With ABR Only

Background

ABR has been used to screen hearing in newborns for more than 30 years, with early reports focused on testing in the NICU using conventional ABR. An ASHA EBSR evaluated the effectiveness of ABR in identifying PCHL using behavioral audiometric thresholds as the gold standard (see Evidence-Based Systematic Review of Newborn Hearing Screening Using Behavioral Audiometric Threshold as a Gold Standard [PDF]). The EBSR found that most studies reporting the effectiveness of ABR to identify childhood hearing loss were performed before 2000 using conventional ABR in newborns cared for in the NICU. In 50% of studies, it was found that ABR was at least moderately effective in identifying hearing loss. Based on the ASHA EBSR, the sensitivity of ABR for detecting permanent hearing loss using behavioral audiometric threshold as a gold standard ranged from 45% to 100% (Shimizu et al., 1990; Smyth, Scott, & Tudehope, 1990; Stevens et al., 1990). Specificity ranged from 71.3% to 99.3% (Durieux-Smith, Picton, Bernard, MacMurray, & Goodman, 1991; Savio, Cecilia Perez-Abalo, Gaya, Hernandez, & Mijares, 2006). Positive likelihood ratios ranged from 3.28 to 87.9.

Currently, most NBHS programs use automated devices. Automated ABR differs from conventional ABR in that waveforms are automatically evaluated using criteria that have been developed and set by manufacturers, thus allowing for non-audiologists to conduct and record the results. Based on manufacturer-specific algorithms, the output of the screening is reported as either "pass" or "refer." Published results from UNHS programs report referral rates of 0.2%-3.1% (Barsky-Firkser & Sun, 1997; Lin, Shu, Lee, Lin, & Lin, 2007; Mason & Hermann, 1998; Stewart et al., 2000). A large-scale, statewide study reported that referral for diagnostic ABR based on automated ABR screening was <2% (Stewart et al., 2000); hence, ABR has gained widespread acceptance as a screening tool for all newborns. ABR screening is recommended by JCIH (2007) for newborns cared for in the NICU for more than 5 days, because they are at greater risk for neural hearing loss.

Procedure

Infants can be tested in the nursery or a quiet room. Apply electrodes according to manufacturer and equipment requirements. Visually inspect the outer part of the ear canal to ensure that the canal is patent and clear of debris and place transducers. The infant should be asleep or resting quietly for the test, with the newborn positioned to reduce muscle artifact. Screen each ear.

Flow charts are presented for infants cared for in the NICU (see Figure 1 [PDF]) and the WIN (see Figure 2 [PDF]) separately. For all newborns who pass the newborn hearing screening, the process is similar in the WIN and NICU. That is, if both ears pass the ABR screening and the newborn has no identified risk factors for late-onset or progressive hearing loss, then the screening is complete and developmental hearing and speech/language skills should be monitored in the medical home. If an infant passes the screen and has risk factors for late-onset or progressive hearing loss, then the infant should have hearing skills and speech and language milestones monitored (for further discussion, see Section XII).

For newborns who fail the hearing screen, a referral to a pediatric audiologist for diagnostic ABR is needed; however, a repeat screen can be obtained prior to referral to an audiologist for diagnostic testing. Newborns cared for in the WIN who fail the initial screen should be rescreened either prior to discharge or after discharge on an outpatient basis within 1 month of age (but not both). Infants who have been in the NICU for more than 5 days can be referred directly to an audiologist for a diagnostic ABR after the first failed screen or can be rescreened prior to discharge. A newborn cared for in the NICU for less than 5 days who has failed one screening can be screened a second time before hospital discharge or as an outpatient within 1 month of discharge, but not both. Repeatedly screening an infant is not recommended (see VII: General Procedures). All infants (in the WIN and NICU) who fail the rescreen should be referred to an audiologist who specializes in pediatric testing for a diagnostic audiologic evaluation.

The timing of the second screen (inpatient or outpatient) is dependent on the individual hospital or community and, in large part, on the resources available for a second screening. In some communities, it may be appropriate to refer to an audiologist for an outpatient re-screen, followed by diagnostic ABR if the newborn fails. If the second screening cannot be completed within the first month of life, it is appropriate to refer for a diagnostic evaluation after one failed screen.

As noted above, it is recommended that this protocol be used with automated ABR, but this protocol can also be used with conventional ABR. If conventional ABR is used, the screening and interpretation should be done by an audiologist who adheres to strict operator and interpretation protocols.

There are no EBSRs to determine the best stimulus levels for identification of infants at risk for hearing loss. However, the recommended parameters noted below have been used with large numbers of newborns (Lin et al., 2007).

Stimuli

  • Most automated equipment presents click stimuli at 35 dB nHL at a rate of 30-37/s. If the equipment allows a choice of stimulus levels, the screening program audiologist can decide to screen using low click levels.

Stopping Criteria For Pass

For automated ABR, manufacturers use their own proprietary stopping rule, based on a template comparison or statistical algorithms. Most instrumentation does not allow for operators to change the stopping rule criteria.

Advantages of ABR Compared With OAEs

  • An ABR screening accesses more central structures of the auditory system than OAE screening, allowing for detection of neurologic involvement.
  • Compared with OAE screening, ABR screening results are thought to be less susceptible to false positives due to ear-canal debris (e.g., Vohr et al., 2001). However, ABR can have false positives for hearing loss, if the infant is neurologically compromised, but has normal hearing.

Disadvantages of ABR Compared With OAEs

  • ABR test time has been reported to be longer than OAE testing in some studies, with the hypothesis being that it takes time for traditional electrode application (e.g., Berg et al., 2011; Meier, Narabyashi, Probst, & Schmuziger, 2004; Norton et al., 2000).
  • ABR screening can be less cost effective than OAE screening, because of the higher cost of disposables (electrodes and disposable earphones, largely) and increased personnel time (Berg et al., 2011). There are reports that this cost imbalance can be stabilized with a lower refer rate, resulting in reportedly lower or similar overall program cost (Meier et al., 2004; Vohr et al., 2001).

B. Screening With OAEs Only

Background

Otoacoustic emissions, either transient-evoked OAEs (TEOAEs) or distortion product OAEs (DPOAEs), can be used to screen hearing for infants cared for in the WIN. OAE-only protocols have been used for over 20 years for UNHS (e.g., White, Vohr, & Behrens, 1993). Large UNHS programs using OAEs have reported referral at time of hospital discharge to be 6.5%-13% (Vohr, Carty, Moore, & Letourneau, 1998; Vohr et al., 2001; Watkin, 1996); however, after outpatient re-screen, referral for diagnostic ABR ranges can be 1%-1.5% (Attias et al., 2006; Chen, Yi, Chen, Dong, Yang, & Fu, 2012; Vohr et al., 1998; Watkin, 1996). Because OAEs are sensitive to residual outer- and middle-ear debris that may be present after birth (e.g., Chang, Vohr, Norton, & Lekas, 1993; Doyle, Rodgers, Fujikawa, & Newman, 2000), most OAE screening protocols involve an outpatient re-screening for newborns who fail screening before hospital discharge. Based on the ASHA EBSR (Evidence-Based Systematic Review of Newborn Hearing Screening Using Behavioral Audiometric Threshold as a Gold Standard [PDF]), the sensitivity of OAEs for detecting hearing loss using behavioral audiometric threshold as a gold standard ranged from 55% to 100% (Ari-Even Roth et al., 2008; Stevens et al., 1990). Specificity ranged from 71% to 91% (Ari-Even Roth et al., 2008; Apostolopoulos, Psarommatis, Tsakanikos, Dellagrammatikas, & Douniadakis, 1999). Positive likelihood ratios ranged from 3.1 to 10.2.

A flowchart for this protocol is illustrated in Figure 3 [PDF].

Procedure

OAEs should be measured in a quiet environment (e.g., in a quiet room with no one speaking and minimal background noise). Visually inspect the outer part of the ear canal to ensure that the canal is patent and clear of debris and place probe. A snug probe fit is essential for valid and reliable recordings. The infant should be resting quietly. Screen each ear.

For OAE screenings, the stimulus level is calibrated in each ear according to manufacturer's specifications. After stimulus level requirements are met, OAEs are collected to meet stopping criteria. If OAEs do not appear to be present, the probe should be taken out and inspected to see if it is blocked with debris. If the probe is occluded with debris, the probe should be cleaned, the tip should be changed, and another screening should be completed. If the probe is clean and clear of debris, the screen is complete.

If the infant passes the newborn hearing screening, the hearing screening is complete. If one or both ears do not pass the screening, both ears should be re-screened before hospital discharge. If both ears do not pass upon a second screen, the infant should return for an outpatient OAE re-screening at no later than 1 month of age. The newborn should be re-screened no more than one time as an outpatient. At the outpatient re-screening, if the infant meets criteria in both ears, the infant passes the newborn hearing screening. If the infant passes the screen and has risk factors for late-onset or progressive hearing loss, then the infant should be followed for late-onset or progressive hearing loss (see "Detection of late-onset hearing loss" in Section XII).

If upon the second screening the baby does not pass for both ears, the newborn is referred to an audiologist who specializes in pediatrics for diagnostic audiologic testing.

There are no EBSRs to determine the best stimulus levels, stopping criteria, and criteria for pass/fail for OAE screening in newborns. However, the published parameters noted below have been used with large numbers of individuals.

Stimuli

  • TEOAEs using a high-level click, approximately 80 dB pSPL, and a subtraction (sometimes referred to as non-linear) paradigm to reduce stimulus artifact (e.g., Norton et al., 2000; Spivak et al., 2000; Vohr et al., 1998; Watkin et al., 1996) is recommended.
  • DPOAEs using mid-level stimuli are recommended (e.g., f1 primary = 65 dB SPL and f2 primary = 55 dB SPL), because they have been shown to have better sensitivity and specificity than high-level stimuli (Norton et al., 2000; Stover, Gorga, Neely, & Montoya, 1996; Sun, Jung, Kim, & Randolph, 1996).

Stopping Criteria for Pass

Passing criteria are likely to be set by the manufacturer. If pass criteria can be changed, the program audiologist should ensure that TEOAEs and DPOAEs are tested at a minimum of three test frequencies—2000, 3000, and 4000 Hz. These values are recommended, because they have excellent sensitivity and specificity (Norton et al., 2000; Harrison & Norton, 1999; Hussain, Gorga, Neely, Keefe, & Peters, 1998; Gorga et al., 1997; Gorga et al., 2005). Because OAEs are sensitive to hearing loss at 4000 Hz, it is recommended that inclusion of this frequency in pass criteria be routine, not optional.

It is recommended that the signal-to-noise ratio (SNR) for these three frequency bands (TEOAEs) or for these three f2 frequencies (DPOAEs) be used for pass criteria. The SNRs for TEOAEs in these bands should be at least 4 dB, but preferably 6 dB (Norton et al., 2000; Wessex Universal Neonatal Hearing Screening Trial Group, 1998). An alternative is to use percentage reproducibility between waveforms of 75% in 2, 3, and 4 kHz bands for pass criteria (Vohr et al., 1998). DPOAE SNR criteria are more varied, depending on whether mean SNR is calculated, or mean plus one, or two standard deviations (SDs) of noise. For mean plus two SDs, 3 dB has been used (e.g., Norton et al., 2000). For comparison to mean noise, 6 dB is more typical; however, manufacturers will often have their own criteria programmed into the screening equipment. In addition, a minimum absolute DPOAE level of -5 dB SPL should be imposed (e.g., Gorga et al., 1997). However, it must be noted that this level was chosen based on a population that did not include newborns.

TEOAEs: A minimum of 50 averages should be collected before testing is terminated.

DPOAEs: DPOAEs are often terminated based on signal-to-noise ratio. Rather than a minimum number of averages, manufacturers choose a minimum length of averaging time.

Advantages as Compared With ABR

  • Disposable supplies for OAE screenings are often less expensive than those for ABR. Also, test time has been reported to be shorter than ABR test time (e.g., Berg et al., 2011).

Disadvantages as Compared With ABR

  • OAEs do not detect neural hearing losses.
  • OAEs have a higher refer rate from inpatient screening compared with the rate for ABRs (e.g., Lin et al., 2007; Vohr et al., 2001). To achieve a low referral rate for those needing diagnostic testing, outpatient re-screening is advised for newborns who fail inpatient screening. The screening process is complete after the outpatient screening, before referral to an audiologist skilled in pediatric diagnostic audiologic testing. A protocol must be in place to capture those who failed inpatient screening to attend the outpatient re-screening.
  • OAEs may be reduced or absent due to outer- and middle-ear debris (e.g., Doyle et al., 2000).

C. Two-Tier Screening: OAEs Followed by ABR When the OAE Test is Not Passed

Background

In an effort to reduce overall program costs, a two-tier screening approach can be used. With this approach, OAE screening is completed first, with an ABR done only for those newborns who do not pass the OAE screen. This protocol was recommended by the National Institute on Deafness and Other Communication Disorders Health Development Conference and published as Early Identification of Hearing Impairment in Infants and Young Children (NIH, 1993). Large-scale studies have reported refer rates for two-tier screening to be less than 1% (e.g., Wessex Universal Neonatal Hearing Screening Trial Group, 1998; Spivak et al., 2000). Another large study (21,279 newborns) used TEOAEs followed by ABR and reported 92% sensitivity and 98% specificity for bilateral, PCHL = 40 dB HL confirmed at 8 years of age (Kennedy, McCann, Campbell, Kimm, & Thornton, 2005). Some studies have found this protocol to be more cost efficient than OAE programs (Lin et al., 2005; Lin, Shu, Lee, Lin, & Lin, 2007), and others have found them to be similar in cost (Meier et al., 2004; Vohr et al., 2001). The initial rationale behind this approach is that OAEs have lower disposables cost and are faster to perform compared with disposables cost and efficiency of ABR. However, inpatient OAE screening has a higher fail rate than ABR. Performing OAE screening first reduces the number of newborns who would go on to have a more time-consuming and expensive ABR screen.

Procedure

The flowchart for this protocol is illustrated in Figure 4 [PDF]. Both ears are tested with OAEs first. Each ear must pass OAE screening. If the infant passes the OAE test in both ears, no further testing is required. If the infant passes the screen and has risk factors for late-onset or progressive hearing loss, then the infant should be followed (see "Detection of late-onset hearing loss," in Section XII).

If the OAE is failed in one or both ears, one OAE re-screen can be conducted or the ear/ears can be re-screened in the same test session with ABR without performing an OAE re-screen. If the ear fails again with the OAE re-screen, an ABR screening test is conducted on the same ear/ears within the same session. If the ABR is a pass, the infant has passed the hearing screening. If one or both ears do not pass the ABR, one of two alternatives can be done. The newborn can be referred for outpatient ABR re-screen, or the newborn can be referred directly for diagnostic audiologic testing.

In Figure 4 [PDF], the scenario is provided if the newborn gets discharged after the initial OAE screen. In this case, the newborn returns for an outpatient OAE screen, followed by a screening ABR in the same session if the newborn does not pass OAE screening. If the newborn fails the ABR screen, a referral is made for diagnostic audiologic testing. If the newborn passes and has no identified risk factors for hearing loss, the screening is complete. If the infant passes the screen and has risk factors for late-onset or progressive hearing loss, then the infant should be followed for late-onset or progressive hearing loss (see "Detection of late-onset hearing loss," in Section XII).

Stimuli

  • Stimuli are the same as those recommended for ABR and OAEs (above).

Response Criteria

  • Response criteria are the same as those recommended for ABR and OAEs (above).

Advantages as Compared With ABR and OAEs Only

  • Refer rate from inpatient screening is lower than for OAE only.
  • Cost of program may be lower than using ABR only.

Disadvantages as Compared With ABR and OAEs Only

  • Two pieces of equipment are needed, although some manufacturers have both tests in one piece of equipment. Personnel must be trained on two procedures.
  • Because the tests used currently differ in detecting different degrees of hearing loss, a baby may not pass the OAE test but pass the ABR; hence, the test may miss some mild hearing loss (J. Johnson et al., 2005).
  • This protocol will not detect neural hearing loss.

D. Two Technologies ABR and OAEs

Background

Some studies have explored using both OAEs and ABR for newborn hearing screening (Berg et al., 2011; Berg, Spitzer, Towers, Bartosiewicz, & Diamond, 2005; Kirkim et al., 2008). In this protocol, newborns must pass both an OAE screening and an ABR screening. The rationale behind it is that OAEs are more sensitive to cochlear hearing loss and ABR will identify inner hair cell and neural lesions through the brainstem. Inpatient refer rates for this protocol, which were based on OAE-pass and ABR-fail, are reported to be 24% in the NICU (Berg et al., 2005) and 0.92% in the WIN (Berg et al., 2011). In a group of 22,786 newborns (nursery type not specified), 9.4% was the inpatient refer rate. Ultimately, 78 newborns had follow-up physiological testing, and it was found that 10 had abnormal/absent ABR, present OAEs, and absent acoustic reflexes (Kirkim et al., 2008). There are no sensitivity and specificity data or comparisons of screening with behavioral audiometric hearing thresholds using this protocol.

Procedure

The flowchart for this protocol is illustrated in Figure 5 [PDF]. Both ears of newborns are tested with ABR and OAEs. Each ear must pass both tests to be considered a "pass." If the infant passes the screens but has risk factors for late-onset or progressive hearing loss, then the infant should be followed for late-onset or progressive hearing loss (see "Detection of late-onset hearing loss," in Section XII).

Newborns who fail screening for one or both technologies can be re-screened with that technology (technologies) once before hospital discharge or in an outpatient setting, but not both. If the newborn fails one or both of the second screenings in one or both ears, the newborn is referred for outpatient diagnostic testing.

Stimuli

  • Stimuli are the same as those recommended for ABR and OAEs (above).

Response Criteria

  • Response criteria are the same as those recommended for ABR and OAEs (above).

Advantages as Compared to ABR Only and OAEs Only

  • The advantage of this screening protocol is that OAEs may be sensitive to mild hearing loss and ABR screens for neural hearing loss.

Disadvantages as Compared With ABR Only and OAEs Only

  • The disadvantage to this protocol is that the hospital needs to purchase screening equipment that can perform both measures (purchase and maintain two pieces of equipment or one device that does both tests). Also, personnel must be trained on both technologies. In addition, it takes more time (Berg et al., 2011), because two procedures are done. Fail rates may be higher, because two screening tests must be passed (Berg et al., 2005; Kirkim et al., 2008).

General Procedures for All Protocols

Standard Universal Precautions Apply

All procedures must ensure the safety of the patient and clinician and adhere to universal health precautions (e.g., prevention of bodily injury and transmission of infectious disease). Decontamination, cleaning, disinfection, and sterilization of multiple-use equipment before reuse must be carried out according to facility-specific infection control policies and procedures and according to manufacturer's instructions (Bolyard et al., 1998; Centers for Disease Control and Prevention [CDC], 2011).

Screening

Newborns in the WIN should be screened as close as possible to hospital discharge and at least prior to 1 month of age. Screen NICU newborns when they are ready for discharge and/or when they are medically stable. For newborns transferred from one hospital to another, state laws or hospital protocols may vary, depending on which hospital is responsible for screening.

The screening can be done in a nursery or in a quiet room. A sound booth is not needed for hearing screening. The infant should rest quietly or sleep for the test. The preferred method for testing is to have the newborn resting quietly in his/her bassinette, although if needed, the newborn can be held. Both ears should be screened.

Care should be taken to screen newborns no more times than recommended in the protocol. The probability of erroneously getting a pass outcome by chance increases with every screen. Therefore, the chance of passing infants who have hearing loss increases the more times they are screened (see JCIH, 2007, for more information).

Pass/fail indications: The newborn must pass the screening in both ears to be considered a "pass." If the newborn fails one ear, both ears must be screened during the re-screening. If the newborn passes the screening or the re-screening and has no identified risk factors for late-onset or progressive hearing loss, then the screening is complete. If the newborn passes the screen and has risk factors for late-onset or progressive hearing loss, then the newborn's hearing should be followed during early childhood (Harlor & Bower, 2009; JCIH, 2007; ASHA, 2005), as discussed in Section XII, "Detection of late-onset hearing loss."

Parental Right to Refuse

If a parent objects to the mandatory hearing screening, it should be noted in the medical chart. Numerous professionals have argued that a state policy of performing screening without obtaining parental permission, or at least informing parents about screening, is at least bad policy, but may also violate constitutional protections of the parental role (American Academy of Pediatrics, 1995; Berge, 1992; Clayton, 1992; Fleischman, Post, & Dubler, 1994). Although many hospitals have global consents for all newborn screening procedures, screeners and all personnel should be aware of hospital and state regulations regarding parental refusal.

Personnel

Audiologist Roles and Responsibilities in Newborn Hearing Screening

Audiologists have a role in the guidance, development, implementation, and oversight of NBHS programs. They are responsible for training screeners and monitoring program outcomes. Training screeners is critical and ongoing, as high staff turnover can impact the quality of the newborn hearing screening process. In addition, audiologists may perform screenings in the hospital and conduct the follow-up diagnostic hearing evaluations. Audiologists provide counseling for families of children with hearing loss. For additional information, see Guidelines for Audiologists Providing Informational and Adjustment Counseling to Families of Infants and Young Children With Hearing Loss Birth to 5 Years of Age (ASHA).

Screener Roles and Characteristics

Different types of personnel can perform newborn hearing screenings. A screener may be an audiologist, a nurse, a paraprofessional, or a volunteer trained in newborn hearing screening. Regardless of background, all screeners must be trained in use of the technology and protocol used in the program. Screeners should be familiar with, and competent to perform, the screening methods and adhere to JCIH guidelines, EHDI process, and hospital/clinic protocols, including standard precautions and patient confidentiality standards. The screeners report their findings according to hospital and state EHDI requirements. If using conventional ABR equipment for screening purposes, audiologists must interpret waveforms.

Documentation and Dissemination

Patient records should follow a documentation standard and be in full compliance with the Health Insurance Portability and Accountability Act (HIPAA, 1996; PL 104-191).

Documentation is necessary for any procedure conducted on an infant, including newborn hearing screening. Documentation of the screening results may be as simple as recording the outcome of the test for each ear, any recommendations, the date and time, and the signature of the personnel conducting the screening. In addition to documentation, the results need to be disseminated to the parents, preferably in written format, and to the newborn's physician according to hospital procedures, and all HIPAA guidelines for confidentiality must be followed. Should an infant not pass the hearing screen, the baby's physician needs to be informed immediately and information given to the parents about agency options for diagnostic audiological testing and how to schedule an appointment in order to ensure timely follow-up (ASHA, 2008).

Some states stipulate the information that needs to be reported to the appropriate governing body (e.g., state EHDI program). State guidelines should be referenced to ensure accurate reporting. In addition, communication with all stakeholders is an important component of EHDI programs and impacts success at all levels.

Program Evaluation

Ongoing monitoring of performance of the UNHS program is important to ensure high quality and cost efficiency. Each program should develop its own set of goals and use indicators representing a range of variables that indicate quality. JCIH suggests minimal standards to allow programs to evaluate their performance relative to screening and diagnosis timelines.

  • The benchmark for the percentage of newborns who complete screening (inpatient and outpatient screen) by 1 month of age is 95%.
  • No more than 4% of newborns should be referred for diagnostic audiologic evaluation.
  • Newborns who have failed the screening should have a comprehensive diagnostic audiologic evaluation by 3 months of age; the benchmark is 90%.
  • For families choosing amplification for their infant, amplification should be obtained within 1 month of hearing loss confirmation; the benchmark is 95%.

For a complete listing of indicators that also include early intervention, see www.JCIH.org.

In addition to program evaluations, states can evaluate their programs as they report the statewide findings to the CDC. Examples of data collected annually by the CDC include:

  • Number of newborns screened
  • Number of missed screens, including home births and transferred newborns
  • Number of newborns not screened due to parental refusal
  • Number of newborns passing the screening prior to discharge
  • Number of newborns discharged not passing screening in one or both ears
  • Number of newborns passing outpatient (re)screening
  • Number of newborns not passing outpatient screening in one or both ears
  • Number of infants returning for diagnosis and severity of hearing loss
  • Part C early intervention enrollment

View additional information about NBHS data collection by the CDC.

Education and Counseling

The importance of the information and counseling provided to families before and after hearing screening cannot be overstated. If families are not aware of the hearing screening results and implications, regardless of screening outcome, the screening program is ineffective. Audiologists are referred to "Guidelines for Audiologists Providing Informational and Adjustment Counseling to Families of Infants and Young Children With Hearing Loss Birth to 5 Years of Age." This document clearly delineates the audiologist's role in counseling families of children in this age group who have hearing loss.

Parents can be directed to resources to learn more about hearing screening and follow-up, including the CDC "Screening and Diagnosis" website and Boys Town National Research Hospital's website "My Baby's Hearing."

Challenges Facing Newborn Hearing Screening Programs

Calibration

There are several issues with stimulus calibration that affect the ability of screening equipment to screen accurately for mild hearing loss. First, there is no calibration standard in the United States for click stimuli, which are used in both transient-evoked OAE and ABR screening. Second, signals are measured in couplers that are based on the adult ear. The ear canal and middle ear of newborns are different from those of adults, so the stimuli delivered to the newborn ear will be considerably different in frequency content and higher in level compared with those calibrated for an adult ear.

For OAEs, there are differences in signal calibration, calculation of the noise floor, and response determination algorithm differences between manufacturers. These differences could affect the ability to accurately predict the level of hearing loss detected.

Out-of-Hospital Births

Although births outside of hospitals are challenging, a large number of newborns are not born in hospitals. In 2009, over 29,000 newborns were born at home. According to the CDC, there was a 29% increase in the number of newborns born at home between 2004 and 2009. Medical communities must provide a protocol for newborns who are not born in a hospital to be screened by 1 month of age.

Timely Follow-up

Based on current data from EHDI programs throughout the United States, nearly half (34.9%) of infants born in 2011 who did not pass the final newborn hearing screen did not complete follow-up and were categorized as loss to follow-up (LTF)/lost due to documentation (LTD; CDC, 2013). This finding is especially concerning, because these "lost" newborns are at higher risk for hearing loss than the general population. That is, they have already failed a hearing screen, putting them at higher risk than those babies who passed the screen. The newborns LTF/LTD are at risk of delayed intervention, and early intervention has been linked to improved communication outcomes for children with hearing loss relative to those children who are identified later in life (Moeller, 2000; Yoshinaga-Itano, Sedey, Coulter, & Mehl, 1998). Therefore, reducing the LTF/LTD rate is paramount to ensuring a successful EHDI program.

Resolving numerous challenges to achieving the National EHDI goals of screening by 1 month, diagnosis of hearing loss by 3 months, and intervention by 6 months depends on efficient and effective follow-up for infants who do not pass NBHS. Known barriers to follow-up include limited access to audiologists with pediatric expertise, long wait times for appointments, multiple re-screenings and evaluations prior to confirmation of loss, medical co-morbidities—especially otitis media, and disbelief by parents who observe their babies responding to environmental sounds (Folsom et al., 2000; Holte et al., 2012; Moeller, White, & Shisler, 2006).

Detection of Late-onset Hearing Loss

Infants can pass the newborn hearing screening and develop hearing loss later in life, whether or not they have risk indicators for hearing loss (e.g., Bess et al., 1998; JCIH, 2007). In addition, not all risk indicators will be identified by the time a newborn is 1 month of age, when the newborn hearing process ends. One study based in the United Kingdom found that, of 33,860 children entering kindergarten with hearing loss, approximately 50% were identified by the newborn hearing screening program (Watkin & Baldwin, 2011). The authors concluded that, although some children with hearing loss had missed their newborn hearing screening, a proportion of them developed hearing loss after the newborn screening period.

Because the onset of hearing loss can occur at any time in a child's life, developmental milestones, hearing skills, and speech and language milestones should be monitored in all children, consistent with the American Academy of Pediatrics schedule [PDF]. Children who have previously passed a NBHS, but who have risk indicators for hearing loss, should be referred for an audiologic evaluation between 24 and 30 months of age. Infants having risk factors associated with a high prevalence of late-onset hearing loss, such as cytomegalovirus (CMV) and extracorporeal membrane oxygenation (ECMO), should have audiologic testing on a more frequent basis. If parents have concerns regarding their child's speech and language development, the child should be referred for audiologic testing and speech and language evaluation by a speech-language pathologist (JCIH, 2007).

Funding Issues

Sufficient funding to support the continuation of NBHS programs is a growing challenge. At the level of the hospital, the costs that need to be considered include those associated with the tests—equipment, disposables, and staff time. Staff time includes program management, which may include data entry, data analysis, follow-up activities, and monitoring outcomes. Newborn screening funding varies from state to state, with some states procuring funding from fees, Medicaid, Title V MCH Block Grant funding, and state general revenue funding (K. Johnson, Lloyd-Puryear, Mann, & Ramos, 2006). The trend is to identify funding sources other than grants to ensure program continuity should grant support become unavailable. At a state level, sufficient resources and personnel are necessary to ensure quality monitoring of the programs in the state.

Future Considerations

Stimulus Levels, Response Processing and Passing Criteria, and Protocols

Research is needed on pass/fail rates and sensitivity/specificity for different levels of stimuli used for newborn hearing screening. Not all equipment provides flexibility at the administrative level to change stimulus level or passing criteria, which may differ among hospitals depending on the targeted hearing level goal of the screening program. One important consideration for ABR is that a click level of 35 dB nHL, which is standard for many programs, will pass some newborns with mild hearing loss (J. Johnson et al., 2005). One research study on screening method used a custom 30 dB nHL click (Norton et al., 2000), but field-based research using lower stimulus levels is needed.

Response processing refers to the manner in which the screening equipment processes the response (e.g., filtering, amplification, averaging, etc.). Research on different types of ABR wave detection (e.g., template vs. statistical) is needed to determine its effect on NBHS outcomes. In addition, research is needed on the effect that different passing criteria for ABRs and OAEs have on NBHS outcomes.

Finally, it is important to determine the sensitivity and specificity of different NBHS protocols, once the targeted type of hearing loss and hearing level have been carefully defined. For example, if a program wants to detect all types of mild hearing loss, perhaps a protocol using TEOAEs to target mild hearing loss and a high-level click to target neural hearing loss should be examined. Another research area is to determine whether TEOAEs and DPOAEs target similar levels of hearing loss in a NBHS program.

Technology

Implications of new screening tools should be considered as they emerge. Three examples are auditory steady state response (ASSR), chirp stimuli to evoke ABRs, and middle-ear/acoustic reflex thresholds. Although ASSR is not a new technology, it has not been used widely for newborn hearing screening. In studies using small numbers of newborns, it has been shown to be as effective as ABR in identifying hearing loss (Savio et al., 2006). Likewise, chirp stimuli, which are short-duration stimuli that change frequency over time, have been found to evoke larger ABR waves, which may make it possible to determine if an ABR is present in a short period of time and/or at lower stimulus levels (Ferm, Lightfoot, & Stevens, 2013; Mühler, Rahne, & Verhey, 2012). Emerging research on wideband middle-ear immittance, which includes both reflectance and absorbance, has shown it is likely that some newborns do not pass newborn hearing screening using OAEs because of sound-conduction problems related to outer/middle ear dysfunction (Sanford et al., 2009; Hunter, Feeney, Lapsley Miller, Jeng, & Bohning, 2010). Adding a middle-ear measure to screening protocols might allow determination of type of hearing loss more effectively, resulting in improved screening outcomes. Finally, new research into newborn acoustic reflex thresholds may elucidate their use in the screening or diagnostic process (Kei, 2012).

Non-traditional Hearing Screening Programs

In addition to hearing screening in the newborn period, it is reasonable to consider screening for those factors (e.g., genetic, viral, etc.) that are known to cause childhood hearing loss. Two examples of such screening programs that are currently being considered for widespread use are noted here.

Congenital Cytomegalovirus

cCMV infection has the potential to cause devastating long-term health consequences, including neurological deficits, hearing loss, and vision problems (CDC, 2011). However, treatment and careful monitoring for progression of disease only occurs in those infants who show symptoms of this infection at birth, a mere 10% of this population (CDC, 2011). The identification of cCMV in the remaining 90% of asymptomatic infants will go undetected until a universal screening protocol specifically for cCMV is established.

SNHL is the most common sequela of this infection, yet current newborn hearing screening protocols detect only up to 50% of cases because of the late-onset and progressive nature of the hearing loss (Fowler, Dahle, Boppana, & Pass, 1999). Furthermore, SNHL occurs more frequently in symptomatic infants compared with asymptomatic or presymptomatic infants. This subset of infants with symptomatic cCMV will likely be identified at birth and closely monitored. It has been estimated that as many as 25% of children with asymptomatic cCMV at birth will develop SNHL (Dahle et al., 2000; Iwasaki, Yamashita, Maeda, Misawa, & Mineta, 2007); however, parents and health care providers are unaware of the need to closely monitor them, which results in delayed diagnosis of hearing loss and a high likelihood the etiology of the loss will not be determined. That is, testing for cCMV is only effective when the blood or urine used for testing is collected within the first 2 to 3 weeks of life (deVries, Vossen, Kroes, & van der Zeijst, 2011; Lanari et al., 2006; Lazzarotto, Guerra, Lanari, Gabrielli, & Landini, 2008; Soetens et al., 2008). Thus, if a child is found to have hearing loss later in life, etiology cannot be determined unless a secretion sample has been stored and can be accessed for testing once hearing loss is identified.

Recently, Boppana et al. (2011) reported that procedures for mass screening of cCMV are possible. Saliva specimens obtained at birth and analyzed through polymerase chain reaction (PCR) assay have high sensitivity and specificity in determining the presence of cCMV infection. Furthermore, saliva PCR assays are easily amenable to large-scale screenings of newborns (Boppana et al., 2011). Although studies are needed to determine the feasibility of widespread CMV screening and treatment effectiveness, this remains a promising area of development for newborn screening programs.

Genetics

The initiation of newborn hearing screening has gone a long way toward improving the developmental outcomes of children with hearing loss. However, for this approach to be successful, hearing loss must be present and detectable at birth as there is no systematic hearing screening program after the newborn period. Although it is recommended practice that children with identified hearing loss receive genetic testing (JCIH, 2007; American College of Medical Genetics [ACMG], 2002), it is not yet routine practice that we conduct genetic testing in the newborn period to identify genetic causes of late-onset or progressive hearing losses.

Over 110 chromosomal loci and more than 65 genes have been identified to cause hearing loss (Van Camp & Smith, 2011), and some of these are associated with late-onset or progressive hearing loss. Most notably, Pendred syndrome and GJB2 deafness are associated with such losses. In addition, specific MTRNR1 mutations predispose to an idiosyncratic adverse event of hearing loss caused by aminoglycosides, which can occur with single doses and treatment regimens that do not exceed the recommended therapeutic window (Guan, 2011). It has been predicted that screening for three causes of late-onset or progressive hearing loss (i.e., Pendred syndrome, GJB2 deafness, MTRNR1), along with cCMV, will result in the detection of about 60% of all infants in whom late-onset hearing loss develops (Morton & Nance, 2006).

There are numerous financial, interpretation, and ethical reasons to enter into genetic screening with caution. However, there is every reason to believe that, in the future, a combined approach of hearing screening and molecular genetic testing will assist in the early detection of late-onset, pre-lingual hearing loss in children.

Telepractice

Telepractice is a broad term that encompasses any medically-related activity involving an element of distance. This approach can support the provision of quality health care through telecommunications technology to underserved populations throughout the United Stated and abroad. Services delivered through the use of telecommunication technology should be equivalent in quality to services delivered face to face. The equipment, connectivity, software, hardware, and network compatibility should be appropriate for the particular service being delivered and able to address the unique needs of the patient. As more professionals begin to utilize this technology, it is incumbent upon audiologists to comply with all laws and rules governing the maintenance of patient records, including confidentiality requirements and licensure. Patients should be informed about how services offered via telepractice differ from face-to-face services. In a number of professional policy documents, ASHA has provided guidance to its members who embark on different implementations of telepractice.

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