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Nelson HD, Bougatsos C, Nygren P. Universal Newborn Hearing Screening: Systematic Review to Update the 2001 U.S. Preventive Services Task Force Recommendation [Internet]. Rockville (MD): Agency for Healthcare Research and Quality (US); 2008 Jul. (Evidence Syntheses, No. 62.)

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Universal Newborn Hearing Screening: Systematic Review to Update the 2001 U.S. Preventive Services Task Force Recommendation [Internet].

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1Introduction

Purpose of Review and Prior USPSTF Recommendation

This systematic evidence review updates a prior review for the U.S. Preventive Services Task Force (USPSTF) on universal newborn hearing screening (UNHS) to detect moderate to severe permanent, bilateral congenital hearing loss (PCHL).1, 2 In 2001, based on results of a prior systematic evidence review,3, 4 the USPSTF concluded that the evidence was insufficient to recommend for or against routine screening of newborns for PCHL during the postpartum hospitalization (I Recommendation).

The USPSTF provided additional specific conclusions about the evidence. They determined that methods of screening using otoacoustic emissions (OAEs) and auditory brainstem response (ABR) are highly accurate for identifying PCHL in newborns, and UNHS leads to earlier identification and treatment of infants with PCHL. They found that evidence was inconclusive regarding whether earlier treatment resulting from newborn screening leads to clinically important improvement in speech and language skills at age 3 years and beyond because existing studies had design limitations. The USPSTF considered that earlier identification and intervention may improve the quality of life during the first year of life, and prevent regret by the family over delayed diagnosis of PCHL, but limited data addressing these benefits existed. They were not able to determine whether potential benefits outweighed the potential harms of false-positive tests.

This update focuses on critical evidence gaps that were unresolved at the time of the 2001 recommendation, and utilizes the format and methods of the prior systematic evidence review.3, 4

Condition Definition

The Joint Committee on Infant Hearing (JNIH), comprised of representatives from audiology, otolaryngology, pediatrics, education, and state speech and hearing programs, provides position statements and establishes practice standards for early identification, intervention, and follow-up care for infants and young children with hearing loss. According to the JCIH, hearing screening should identify infants at risk for specifically defined hearing loss that interferes with development.5, 6 The targeted hearing loss for UNHS programs is permanent sensory or conductive hearing loss averaging 30 to 40 decibels (dB) or more in the frequency region important for speech recognition (approximately 500 through 4000 Hertz [Hz]). The focus of UNHS is on congenital as opposed to acquired or progressive hearing loss that may not be detected in the newborn period. The term “hearing impairment” is commonly used outside the U.S. instead of “hearing loss.” These terms refer to the same condition and are both used in this review. (A list of all abbreviations is located in Appendix A.)

Prevalence and Burden of Disease

The rate of PCHL among newborns ranges from 1 to 3 per 1,000 live births.710 Hearing loss occurs more frequently than other newborn conditions for which newborns are routinely screened.11 Compared to children with normal hearing, those with hearing loss have more difficulty learning vocabulary, grammar, word order, idiomatic expressions, and other aspects of verbal communication.12 Hearing loss in children is also associated with delayed language, learning, and speech development, and with low educational attainment.5 Hearing disorders have also been associated with increased behavior problems, decreased psychosocial well-being, and poor adaptive skills.1315

Risk Factors and High Risk Groups

Risk factors associated with a higher incidence of PCHL include neonatal intensive care unit (NICU) admission for 2 or more days; Usher's syndrome, Waardenburg's syndrome, or other syndromes associated with hearing loss; family history of hereditary childhood sensorineural hearing loss; craniofacial abnormalities; and congenital infections such as cytomegalovirus, toxoplasmosis, bacterial meningitis, syphilis, herpes, or rubella.5 However, approximately 50% of infants with PCHL do not have any known risk factors.10, 1620

Current Clinical Practice

Practice standards were set in the U.S. by the JCIH in their 2000 and 2007 position statements.5, 6 In their statements, the JCIH endorsed integrated, interdisciplinary state and national systems of UNHS, evaluation, and family-centered intervention. They recommended that all infants should have access to UNHS and be screened before age 1 month. Infants not passing the screening test should undergo audiologic and medical evaluations before age 3 months, and infants with confirmed hearing loss should receive appropriate intervention before age 6 months (Figure 1). In addition, all infants with risk indicators should undergo periodic monitoring for 3 years. The 2007 statement expands screening protocols for NICU infants, and provides additional guidance for the diagnostic audiology evaluation, the medical evaluation, early intervention, surveillance, communication, and tracking.6

Figure 1. Process of Screening and Follow-up.

Figure

Figure 1. Process of Screening and Follow-up.

According to the American Speech-Language-Hearing Association, 39 U.S. states have enacted legislation related to UNHS.21 The laws are similar in their intent to promote the early identification of hearing loss, but differ in their requirements. These differences include whether screening is mandated, and in what hospitals, or encouraged by the state, how and if the results are required to be reported, and funding mechanisms.22 In addition to state legislation regarding screening, the federal Individuals with Disabilities Education Act (IDEA) requires states to develop and implement statewide systems of early intervention services for infants and toddlers. This Act requires that infants and toddlers with disabilities be identified and evaluated using risk criteria and appropriate audiologic screening techniques.21

Thirty states and territories receive funding from the Early Hearing Detection and Intervention (EHDI) program at the CDC. Some of the funded states have state UNHS legislation while others do not. All states, Puerto Rico, Guam, and the Commonwealth of the Northern Marianas and Palau have received funds to develop and implement UNHS and intervention programs through HRSA's Maternal and Child Health Bureau beginning in 2000.

Screening Tests and Diagnosis

Hearing screening of newborns involves use of objective physiologic measures. Currently, otoacoustic emissions (OAEs) and/or auditory brainstem response (ABR) are most often used to detect sensory or conductive hearing loss.21 Both technologies are noninvasive recordings of physiologic activities that are easily recorded in newborns and are highly correlated with the degree of peripheral hearing sensitivity. In UNHS programs, a 2-step process using OAE followed by ABR in those who failed the first test is often used to improve test performance. In a large trial using this approach, screening test sensitivity and specificity were 0.92 and 0.98, and the positive and negative likelihood ratios were 61 and 0.08, respectively.17

Otoacoustic emissions are low intensity sounds from the cochlea resulting from stimulation by audible sounds. The outer hair cells of the cochlea vibrate, and the vibration produces a low intensity sound that echoes back into the middle ear. This sound can be measured with a small probe inserted into the ear canal. The presence of transient middle ear fluid can affect the performance of this test resulting in a non pass result. The ABR is an auditory evoked potential that originates from the cochlea and is not affected by middle ear fluid. It consists of a series of peaks corresponding to the neural response to an auditory stimulus along the auditory pathway between the auditory nerve and the rostral brainstem. Electrodes are placed on the head, and brain wave activity in response to sound is recorded.21

Newborn hearing screening is generally well accepted and tolerated by parents. Rates of refusals in a U.S. community-based health system were reported as 7 of 8,707 during the first 10 months of 2007 (Personal communication Providence Health & Services [written] December 6, 2007). Under ideal conditions, instruments designed specifically for newborns can test and record findings on sleeping infants in under 5 minutes.

Infants not passing the newborn screening tests are referred for confirmatory testing for diagnosis of PCHL. Referral rates are lower in programs using dedicated technicians rather than volunteers and students.23 The American Academy of Pediatrics has set a referral standard of <4% of all screened newborns, and some hospitals use this measure to monitor quality of the screening program. Confirmation requires a more extensive evaluation by an audiologist using behavioral as well as technologic (AOE/ABR) methods. Although the American Academy of Pediatrics has set a standard of 95% for compliance with follow up testing, this rate varies depending on tracking systems and local services.

The procedure at one state with a tracking system includes the following (Personal communication [written] October 6, 2005).24 At the diagnostic follow-up, the child receive at least a diagnostic OAE evaluation bilaterally and high frequency (1000 Hz) tympanometry. If tests are within normal limits, the child is no longer followed by the state tracking system unless there are risk factors and/or remarkable history (such as family history, syndromes, eventful NICU stay, etc). If the child does not test within normal limits at the diagnostic follow-up, it is recommended that they received a threshold ABR evaluation. This includes click thresholds and frequency specific information at least at 500 and 4000 Hz, and bone conduction thresholds if necessary (normal range on diagnostic ABR is considered 30 dB hearing level and below). This test should be scheduled and completed by 3 months of age.

Treatment

The JCIH recommends that early intervention services should be designed to meet the individualized needs of the infant and family including acquisition of communication competence, social skills, emotional well-being, and positive self-esteem.5 Early intervention includes evaluation for amplification or sensory devices, surgical and medical evaluation, and communication assessment and therapy. Use of cochlear implants in infants has become more available for appropriate candidates in recent years, and is usually considered in those with severe to profound hearing loss after inadequate response to hearing aids.2528 Research in neurological and auditory cortical development suggests that early verses late implantation may be linked to more normal cortical auditory pathway development.2931

Recommendations of Other Groups

Recommendations of other groups are summarized in Table 1. Most recommendations support the JCIH 2000 statement5 specifying UNHS for all newborns, diagnostic testing by age 3 months for those not passing the screening test, and intervention by age 6 months for those with confirmed hearing loss.

Table 1. Recommendations of Other Groups.

Table 1

Recommendations of Other Groups.

Analytic Framework and Key Questions

Evidence reviews for the USPSTF follow a specific methodology32 beginning with the development of an analytic framework and key questions in collaboration with members of the USPSTF. The analytic framework represents an outline of the evidence review and includes the patient population, interventions, outcomes, and adverse effects of the screening process (Figure 2). Corresponding key questions examine a chain of evidence about the effectiveness and potential adverse effects of UNHS and subsequent early intervention. This systematic review updates the evidence from the prior 2001 USPSTF review for the following key questions:

1.

Among infants identified by UNHS who would not be identified by targeted screening, does initiating treatment prior to age 6 months improve language and communication outcomes?

2.

Compared with targeted screening, does UNHS increase the chance that treatment will be initiated by age 6 months for average risk infants? For high risk infants?

3.

What are the adverse effects of UNHS and early treatment?

Figure 2. Universal Newborn Hearing Screening Analytic Framework and Key Questions.

Figure

Figure 2. Universal Newborn Hearing Screening Analytic Framework and Key Questions.

For this review, targeted screening indicates selective screening of newborns based on the presence of risk factors or associated conditions. High-risk newborns are those with risk factors known to be associated with PCHL and/or newborns admitted to the NICU.

Selected key questions addressed in the prior report3, 4 were not updated in this report because they were adequately addressed by existing evidence:

1a.

Can UNHS accurately diagnose moderate to severe sensorineural hearing impairment?

What are the sensitivity and false negative rate of screening tests?

What are the specificity, false positive rate, and predictive value of screening tests?

1b.

Compared with selective screening, how many more cases are identified?

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