This document was developed as
part of the National Institute on Deafness and Other Communication Disorders
(NIDCD) Workshop on Universal Newborn Hearing Screening held in Chevy Chase,
Maryland on September 4-5, 1997. Members of the Working Group on Early
Identification of Hearing Impairment in attendance at this workshop and who
participated in the writing of this document included: G. Pamela Burch-Sims,
Ph.D., Tennessee State University; Richard A. Chole, M.D., Ph.D., University of
California; Allan Diefendorf, Ph.D., Indiana University School of Medicine;
Karen Doyle, M.D., Ph.D., University of California at Irvine; Stephen Epstein,
M.D., Wheaton, Maryland; Judith Gravel, Ph.D., Albert Einstein College of
Medicine; Deborah Hayes, Ph.D., Children's Hospital, Denver; Linda Hood, Ph.D.,
Louisiana State University Medical Center; Susan Jerger, Ph.D., The University
of Texas at Dallas; Mary Pat Moeller, M.S., Boys Town National Research
Hospital; Susan Norton, Ph.D., Children's Hospital and Medical Center; Beth
Prieve, Ph.D., Syracuse University; Patricia Stelmachowicz, Ph.D., Boys Town
National Research Hospital; Judith Widen, Ph.D., University of Kansas Medical
Center; and Christine Yoshinaga-Itano, Ph.D., University of Colorado.
Introduction A National Institutes of Health Consensus Conference held in March of 1993
recommended hearing screening of all newborns, termed universal newborn hearing
screening. Access to the largest possible number of newborns is necessary to
promote early identification of hearing impairment for all infants and
subsequent referral for diagnosis and intervention. The best opportunity for
achieving this goal appears to be provided by the development of hearing
screening programs for newborns in hospital nurseries or in birthing centers,
prior to discharge. The successful implementation of this pro-active approach
should lead to a greater likelihood that a child with hearing impairment will
enjoy academic, social, and vocational success. Recent data indicate that the
direct cost of universal newborn hearing screening programs is comparable to the
direct cost of universal screening programs for other congenital conditions such
as hypothyroidism, phenylketonuria (PKU), and hemoglobinopathies.
A variety of current technologies are available to identify hearing
impairment in the first days of life. Two of the current methodologies generally
established as effective for universal newborn hearing screening are auditory
brainstem responses (ABR) and evoked otoacoustic emissions (EOAE). The focus of
this document is to recommend acceptable procedures that can be used by States
for universal newborn hearing screening. It is important to note, however, that
a newborn hearing screening program is only one component of a comprehensive
approach to the management of childhood hearing impairment. The process also
requires follow-up diagnostic services, counseling, intervention programs, and
parental educational programs. This comprehensive process must be administered
by a multidisciplinary team consisting of individuals such as audiologists,
physicians, educators, speech/language pathologists, nurses, and parents.
Current Status of Statewide
Systems for Universal Newborn Hearing Screening In concert with recommendations of NIH/NIDCD and the Joint Committee on
Infant Hearing (JCIH)*, current statewide programs generally employ ABR and/or
EOAE for hearing screening. These techniques are physiological measures of the
status of the peripheral auditory system that are highly correlated with hearing
status. The techniques permit the identification of infants with communicatively
significant hearing impairment without referring large numbers of normally
hearing infants for unnecessary follow-up testing.
Acceptable
Protocols The initial hearing screening should be carried out before 3 months of age to
ensure that intervention can begin between birth and 6 months of age. The
initial screening should represent only one component of an overall
identification and intervention program for children with hearing impairment.
The overall screening program should include provisions for: 1) tracking of
infants who are referred from the initial screening; 2) follow-up diagnostic
testing; and, 3) intervention for those infants with confirmed hearing
impairment. These programmatic goals can best be achieved through a
multi-disciplinary approach including, but not limited to, primary health care
providers, neonatologists, audiologists, otolaryngologists, educators,
speech-language pathologists, psychologists, and parents. In addition, an
important determiner of the success of universal hearing screening programs is
the effective, timely and sensitive communication of the results and of any
necessary follow-up recommendations to parents or other legal guardians and to
primary care providers.
Finally, because some hearing impairments develop during early childhood, the
Working Group recommends that children continue to be monitored for hearing
impairment and that all children undergo hearing screening upon entering school
and periodically thereafter.
Summary *Footnote 1. JCIH is a multidisciplinary committee composed of
representatives from the American Academy of Audiology, American Academy of
Otolaryngology-HNS, American Academy of Pediatrics, American
Speech-Language-Hearing Association, Council for Education of the Deaf, and
Directors of Speech and Hearing Programs in State Health and Welfare
Departments.
The
early identification of children with hearing impairment is an important public
health objective in the United States. Each year in this country, approximately
1.5 to 3 per 1,000 children are born with significant hearing impairment. With
an annual birth rate of approximately 4 million infants, this prevalence rate
translates into as many as 33 children per day born with hearing impairment.
Currently, many of these children are not identified until the second year of
life or later despite advances in the technology available for the early
detection of hearing impairment. This delay in identification contrasts with
some available statistics from other developed countries where the age of
identification has been reduced to less than one year of age. The consequences
of a late diagnosis of hearing impairment are significant delays in spoken
language and literacy. Without appropriate and timely identification and
intervention, early childhood hearing impairment interferes with the development
of oral/aural communication, impedes academic performance, and results in
long-term vocational consequences.
The development of
statewide systems for universal newborn hearing screening has been addressed by
three approaches, namely legislative mandate, voluntary initiative, and federal
support. Legislative mandates have been used to initiate some form of newborn
hearing screening in approximately 20 states. In four of the states (Rhode
Island, Hawaii, Colorado, and Mississippi), the legislation specifically
addresses the screening of all newborns. In the 16 remaining states, however,
the legislation addresses the identification of risk status in infants, which
indirectly identifies a pool of infants who are at-risk for hearing impairment
and who should be screened. A limitation of this latter approach is that
screening programs restricted to infants with risk factors for hearing
impairment identify only 50% of infants with significant hearing impairment.
Thus, health care professionals in several states are attempting to initiate
hearing screening services for all newborns, including neonates without risk
factors. With regard to voluntary initiatives, voluntary programs exist in
several of the states without legislative mandates. For example, Wyoming has
achieved effective universal newborn hearing screening without legislation.
Numerous local voluntary programs within individual communities or hospitals
also exist. Finally, with regard to federal support, funds from the Bureau of
Maternal and Child Health have allowed 17 states to commit to achieving
universal hearing screening by the year 2000. When fully operational, these 17
states will screen more than 1,000,000 newborns a year.
Acceptable hearing screening protocols should have
specific response attributes and measurement characteristics. Some of these
desired response and methodological features are the following:
Based on a review of published data, a
physiological response implemented with objective response criteria best meets
the above requirements. Acceptable approaches include: 1) auditory brainstem
response (ABR); 2) either transient evoked otoacoustic emissions (TEOAE) or
distortion product evoked otoacoustic emissions (DPOAE); or, 3) a combination of
OAE and ABR. Future research may yield additional objective, physiological
measures that could advance universal newborn hearing screening. A variety of
non-auditory factors may influence the outcome of any of the current hearing
screening approaches. These include test environment, infant state, infant
medical status, and age. The skill and commitment of the examiner are also
important factors influencing the screening test results. Studies have
documented that the actual screening can be carried out effectively by a wide
variety of personnel with appropriate training, such as nurses or volunteers.
Training and quality assurance measures are vital components for the efficiency
and overall effectiveness of screening programs.
As many as
12,000 infants are born each year in the United States with hearing impairment.
Many of these children are not identified as being hearing impaired until they
are 2 years of age or older. A delay in the diagnosis of hearing impairment
leads to delays in language acquisition and academic achievement. Early
identification of and appropriate intervention for children with hearing
impairment leads to improvements in speech and language development in affected
children, thereby improving the likelihood of positive social, emotional,
cognitive, and academic development. Identification of and intervention for
infants prior to 6 months of age seems to result in the most favorable outcomes.
Therefore, the Working Group recommends that a system of universal hearing
screening within newborn nurseries be instituted. Universal screening can be
achieved with low cost methods that successfully differentiate newborns with
hearing impairment from newborns with normal hearing. The chosen screening
method should be simple and the response should be reproducible; the method
should be capable of being administered by a variety of adequately trained
personnel. At the present time, the Working Group concurs that one or more of
the following screening strategies are suitable for application to infant
screening: 1) auditory brainstem response (ABR); 2) either transient evoked
otoacoustic emissions (TEOAE) or distortion product evoked otoacoustic emissions
(DPOAE); or, 3) a combination of OAE and ABR. Universal screening of newborns
will lead to the referral of more infants for diagnosis and intervention.
Comprehensive intervention and management programs are an essential part of a
universal hearing screening program.