Jcih 2007
Jcih 2007
Jcih 2007
The online version of this article, along with updated information and services,
is located on the World Wide Web at:
http://www.pediatrics.org/cgi/content/full/120/4/898
Abbreviations
JCIH—Joint Committee on Infant
Hearing EHDI— early hearing
2007 JCIH POSITION STATEMENT UPDATES detection and intervention
ABR—auditory brainstem response
The following are highlights of updates made since the 2000 JCIH statement3: CMV— cytomegalovirus
1. Definition of targeted hearing loss ECMO— extracorporeal
membrane oxygenation
0● The definition has been expanded from congenital permanent bilateral, AAP—American Academy of Pediatrics
uni-lateral sensory, or permanent conductive hearing loss to include MCHB—Maternal and Child Health Bureau
HRSA—Health Resources and Services
neural hearing loss (eg, “auditory neuropathy/dyssynchrony”) in infantsAdministration
admitted to the NICU. NIDCD—National Institute on Deafness
and Other Communication Disorders
CDC—Centers for Disease Control and
Prevention
2. Hearing-screening and -rescreening protocols
UNHS— universal newborn
0● Separate protocols are recommended for NICU and well-infanthearing screening
OAE— otoacoustic emission IFSP—
nurseries. NICU infants admitted for more than 5 days are toindividualized family service plan OME
have auditory brainstem response (ABR) included as part of— otitis media with effusion FM—
frequency modulation DSHPSHWA—
their screening so that neural hearing loss will not be missed. Directors of Speech and Hearing
Programs in State Health and Welfare
0● For infants who do not pass automated ABR testing in the NICU, referralAgencies
should be made directly to an audiologist for rescreening and, when indi- GPRA—Government
cated, comprehensive evaluation including ABR. Performance and Results Act
OMB—Office of Management
0● For rescreening, a complete screening on both ears is recommended, and Budgets
even if only 1 ear failed the initial screening. PEDIATRICS (ISSN Numbers: Print, 0031-
4005; Online, 1098-4275). Copyright © 2007 by
0● For readmissions in the first month of life for all infants (NICU or well the American Academy of Pediatrics
infant), when there are conditions associated with potential hearing loss
(eg, hyper-
8. Information infrastructure
0● Infants who do not pass the speech-
language por-tion of a medical home 0● States should implement data-
global screening or for whom there is a management and -tracking systems as
concern regarding hearing or lan-guage part of an integrated child health
should be referred for speech-language information system to monitor the quality
eval-uation and audiology assessment. of EHDI services and provide
recommendations for improving systems
7. Communication of care.
0● The birth hospital, in collaboration with
the state EHDI coordinator, should ensure PEDIATRICS Volume 120, Number 4, October 2007 899
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0● An effective link between health and education900 AMERICAN ACADEMY OF PEDIATRICS
professionals is needed to ensure successful transi-and follow-up services, the Centers for Disease Control and
tion and to determine outcomes of children withPrevention (CDC) to develop data and tracking sys-tems, and
hearing loss for planning and establishing publicthe NIDCD to support research in EHDI. By 2005, every state
health policy. had implemented a newborn hearing-screening program, and
approximately 95% of newborn infants in the United States
were screened for hearing loss before hospital discharge.
BACKGROUND Congress recommended cooperation and collaboration among
It has long been recognized that unidentified hearing loss atseveral federal agencies and advocacy organizations to facilitate
birth can adversely affect speech and language development as and support the development of state EHDI systems.
well as academic achievement and so-cial-emotional
development. Historically, moderate-to-severe hearing loss in EHDI programs throughout the United States have
young children was not detected until well beyond the newborn demonstrated not only the feasibility of universal new-born
period, and it was not unusual for diagnosis of milder hearing hearing screening (UNHS) but also the benefits of early
loss and unilat-eral hearing loss to be delayed until childrenidentification and intervention. There is a growing body of
reached school age. literature indicating that when identification and intervention
occur at no later than 6 months of age for newborn infants who
In the late 1980s, Dr C. Everett Koop, then US Sur-geonare deaf or hard of hearing, the infants perform as much as 20 to
General, on learning of new technology, encour-aged detection40 percentile points higher on school-related measures
(vocabulary, articu-lation, intelligibility, social adjustment, and
of hearing loss to be included in the Healthy People 20004 goals
10–13
for the nation. In 1988, the Maternal and Child Health Bureaubehav-ior). Still, many important challenges remain. De-
(MCHB), a division of the US Health Resources and Services spite the fact that approximately 95% of newborn infants have
Administration (HRSA), funded pilot projects in Rhode Island,their hearing screened in the United States, almost half of
Utah, and Hawaii to test the feasibility of a universal statewide newborn infants who do not pass the initial screening do not
screening program to screen newborn infants for hear-ing losshave appropriate follow-up to either confirm the presence of a
before hospital discharge. The National Institutes of Health,hearing loss and/or initiate appropriate early intervention
through the National Institute on Deafness and Otherservices (see www.infanthearing.org,
Communication Disorders (NIDCD), issued in 1993 awww.cdc.gov/ncbddd/ehdi, and www.nidcd.nih.gov/health).
consensus statement on early identification of hearing
impairment in infants and young children.5 In the statement the State EHDI coordinators report system-wide problems
authors concluded that all infants admitted to the NICU should including failure to communicate information to families in a
be screened for hearing loss before hospital discharge and thatculturally sensitive and understandable format at all stages of
universal screening should be implemented for all infantsthe EHDI process, lack of integrated state data-management
within the first 3 months of life.4 In its 1994 position statement,and -tracking systems, and a shortage of facilities and personnel
the JCIH endorsed the goal of universal detection of infants with the experience and exper-tise needed to provide follow-up
with hearing loss and encouraged continuing research andfor infants who are referred from newborn screening
14
development to improve methods for identification of andprograms. Available data indicate that a significant number of
intervention for hearing loss. The AAP released a statementchildren who need further assessment do not receive
6,7
that recommended newborn hearing screen-ing and interventionappropriate fol-low-up evaluations. However, the outlook is
in 1999.8 In 2000, citing advances in screening technology, theimproving as EHDI programs focus on the importance of
JCIH endorsed the univer-sal screening of all infants through anstrength-ening follow-up and intervention.
integrated, inter-disciplinary system of EHDI.3 The Healthy
People 2010 goals included an objective to “increase the
proportion of newborns who are screened for hearing loss byPRINCIPLES
one month, have audiological evaluation by 3 months, and areAll children with hearing loss should have access to resources
enrolled in appropriate intervention services by 6 months.”9 necessary to reach their maximum potential. The following
principles provide the foundation for ef-fective EHDI systems
and have been updated and ex-panded since the 2000 JCIH
position statement.
The ensuing years have seen remarkable expansion in
newborn hearing screening. At the time of the National 1. All infants should have access to hearing screening
Institutes of Health consensus statement, only 11 hospi-tals in using a physiologic measure at no later than 1 month
the United States were screening more than 90% of their of age.
newborn infants. In 2000, through the support of Representative
Jim Walsh (R-NY), Congress autho-rized the HRSA to develop
newborn hearing screening 2. All infants who do not pass the initial hearing screen-
ing and the subsequent rescreening should have ap-
propriate audiological and medical evaluations to
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confirm the presence of hearing loss at no later than 3 erate or greater degrees of hearing loss can have signif-icant
months of age. effects on language, speech, academic, and social-emotional
20
3. All infants with confirmed permanent hearing lossdevelopment. High-risk target populations also include infants
should receive early intervention services as soon asin the NICU, because research data have indicated that this
21–23
possible after diagnosis but at no later than 6 monthspopulation is at highest risk of having neural hearing loss.
of age. A simplified, single point of entry into an The JCIH, however, is committed to the goal of iden-tifying
intervention system that is appropriate for childrenall degrees and types of hearing loss in childhood and
with hearing loss is optimal. recognizes the developmental consequences of even mild
degrees of permanent hearing loss. Recent evi-dence, however,
4. The EHDI system should be family centered withhas suggested that current hearing-screening technologies fail to
infant and family rights and privacy guaranteed
identify some infants with mild forms of hearing loss.24,25 In
through informed choice, shared decision-making, and
addition, depending on the screening technology selected,
parental consent in accordance with state and federal
infants with hearing loss related to neural conduction disorders
guidelines. Families should have access to in-
or “auditory neuropathy/auditory dyssynchrony” may not be de-
formation about all intervention and treatment op-tected through a UNHS program. Although the JCIH recognizes
tions and counseling regarding hearing loss.
that these disorders may result in delayed communication,26–28
5. The child and family should have immediate access tocurrently recommended screening algorithms (ie, use of
high-quality technology including hearing aids, co-otoacoustic emission [OAE] testing alone) preclude universal
chlear implants, and other assistive devices when ap-screening for these disorders. Because these disorders typically
propriate. occur in children who require NICU care,21 the JCIH
recommends screening this group with the technology capable
6. All infants and children should be monitored for of detecting au-ditory neuropathy/dyssynchrony: automated
hearing loss in the medical home.15 Continued assess-mentABR mea-surement.
of communication development should be pro-vided by
appropriate professionals to all children with or without risk All infants, regardless of newborn hearing-screening
indicators for hearing loss. outcome, should receive ongoing monitoring for devel-opment
of age-appropriate auditory behaviors and com-munication
7. Appropriate interdisciplinary intervention programs
skills. Any infant who demonstrates delayed auditory and/or
for infants with hearing loss and their families should
communication skills development, even if he or she passed
be provided by professionals who are knowledgeable
newborn hearing screening, should receive an audiological
about childhood hearing loss. Intervention programs
evaluation to rule out hearing loss.
should recognize and build on strengths, informed
choices, traditions, and cultural beliefs of the families.
8. Information systems should be designed and imple-Roles and Responsibilities
mented to interface with electronic health charts andThe success of EHDI programs depends on families working in
should be used to measure outcomes and report thepartnership with professionals as a well-coordinated team. The
effectiveness of EHDI services at the patient, practice,roles and responsibilities of each team member should be well
community, state, and federal levels. defined and clearly under-stood. Essential team members are
the birth hospital, families, pediatricians or primary health care
GUIDELINES FOR EHDI PROGRAMS profession-als (ie, the medical home), audiologists,
The 2007 guidelines were developed to update the 2000 JCIHotolaryngolo-gists, speech-language pathologists, educators of
position statement principles and to support the goals ofchil-dren who are deaf or hard of hearing, and other early
universal access to hearing screening, evalua-tion, andintervention professionals involved in delivering EHDI
intervention for newborn and young infants embodied inservices.29,30 Additional services including genetics, oph-
Healthy People 2010.9 The guidelines provide currentthalmology, developmental pediatrics, service coordina-tion,
information on the development and implemen-tation ofsupportive family education, and counseling should be
successful EHDI systems. available.31
Hearing screening should identify infants with specif-ically
defined hearing loss on the basis of investigations of long-term, The birth hospital is a key member of the team. The birth
developmental consequences of hearing loss in infants,hospital, in collaboration with the state EHDI co-ordinator,
currently available physiologic screening techniques, andshould ensure that parents and primary health care professionals
availability of effective intervention in concert with establishedreceive and understand the hearing-screening results, that
principles of health screen-ing.15–18 Studies have demonstratedparents are provided with appropriate follow-up and resource
that current screen-ing technologies are effective in identifyinginformation, and
hearing loss of moderate and greater degree.19 In addition,
studies of children with permanent hearing loss indicate that
PEDIATRICS Volume 120, Number 4, October 2007 901
mod-
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that each infant is linked to a medical home. 2 The hos-pital902 AMERICAN ACADEMY OF PEDIATRICS
ensures that hearing-screening information is transmitted audiological treatment and management. For the fol-low-up
promptly to the medical home and appro-priate data are component, audiologists provide comprehensive audiological
submitted to the state EHDI coordinator. diagnostic assessment to confirm the exis-tence of the hearing
The most important role for the family of an infant who isloss, ensure that parents understand the significance of the
deaf or hard of hearing is to love, nurture, and communicatehearing loss, evaluate the infant for candidacy for amplification
with the infant. From this foundation, families usually developand other sensory devices and assistive technology, and ensure
prompt referral to early intervention programs. For the
an urgent desire to understand and meet the special needs of
treatment and management component, audiologists provide
their infant. Families gain knowledge, insight, and experience
32
by accessing re-sources and through participation in scheduledtimely fitting and monitoring of amplification devices. Other
early intervention appointments including audiological, med-audiologists may provide diagnostic and auditory treat-ment
ical, habilitative, and educational sessions. This experi-ence canand management services in the educational set-ting and
be enhanced when families choose to become involved withprovide a bridge between the child/family and the audiologist in
parental support groups, people who are deaf or hard of hearing,the clinic setting as well as other service providers.
and/or their children’s deaf or hard-of-hearing peers. InformedAudiologists also provide services as teachers, consultants,
family choices and de-sired outcomes guide all decisions forresearchers, and administrators.
these children. A vital function of the family’s role is ensuring
direct access to communication in the home and the daily Otolaryngologists are physicians whose specialty in-cludes
provision of language-learning opportunities. Over time, thedetermining the etiology of hearing loss; identi-fying related
child benefits from the family’s modeling of partnerships with risk indicators for hearing loss, including syndromes that
professionals and advocating for their rights in all set-tings. Theinvolve the head and neck; and evalu-ating and treating ear
transfer of responsibilities from families to the child developsdiseases. An otolaryngologist with knowledge of childhood
gradually and increases as the child ma-tures, growing inhearing loss can determine if medical and/or surgical
independence and self-advocacy. intervention may be appropri-ate. When medical and/or surgical
intervention is pro-vided, the otolaryngologist is involved in the
long-term monitoring and follow-up with the infant’s medical
Pediatricians, family physicians, and other allied health care
home. The otolaryngologist provides information and
professionals, working in partnership with parents and other
participates in the assessment of candidacy for amplifi-cation,
professionals such as audiologists, therapists, and educators,
assistive devices, and surgical intervention, in-cluding
constitute the infant’s medical home.2 A medical home is
reconstruction, bone-anchored hearing aids, and cochlear
defined as an approach to providing health care services with
implantation.
which care is acces-sible, family centered, continuous,
comprehensive, co-ordinated, compassionate, and culturally
Early intervention professionals are trained in a vari-ety of
competent. The primary health care professional acts in
academic disciplines such as speech-language pa-thology,
partnership with parents in a medical home to identify and
audiology, education of children who are deaf or hard of
access appropriate audiology, intervention, and consultative
hearing, service coordination, or early child-hood special
services that are needed to develop a global plan of appropriate
and necessary health and habilitative care for infants identifiededucation. All individuals who provide ser-vices to infants with
with hearing loss and infants with risk factors for hearing loss.hearing loss should have specialized training and expertise in
All children undergo sur-veillance for auditory skills andthe development of audition, speech, and language. Speech-
language milestones. The infant’s pediatrician, familylanguage pathologists provide both evaluation and intervention
physician, or other pri-mary health care professional is in aservices for language, speech, and cognitive-communication
devel-opment. Educators of children who are deaf or hard of
position to advocate for the child and family.2,16
hearing integrate the development of communicative
competence within a variety of social, linguistic, and
An audiologist is a person who, by virtue of academic
cognitive/academic contexts. Audiologists may provide
degree, clinical training, and license to practice, is qual-ified to
diagnostic and habilitative services within the individu-alized
provide services related to the prevention of hearing loss and
family service plan (IFSP) or school-based individ-ualized
the audiological diagnosis, identifica-tion, assessment, and
education plan. To provide the highest quality of intervention,
nonmedical and nonsurgical treat-ment of persons with
more than 1 provider may be required.
impairment of auditory and ves-tibular function, and to the
prevention of impairments associated with them. Audiologists The care coordinator is an integral member of the EHDI
serve in a number of roles. They provide newborn hearing-team and facilitates the family’s transition from screening to
33
screening program development, management, qualityevaluation to early intervention. This per-son must be a
assessment, service coordination and referral for audiological professional (eg, social worker, teacher, nurse) who is
diagnosis, and knowledgeable about hearing loss. The care coordinator
incorporates the family’s preferences for outcomes into an IFSP
as required by federal legisla-
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tion. The care coordinator supports the family members in theirphysicians and parents, documentation of results in medical
choice of the infant’s communicative develop-ment. Throughcharts, and methods for reporting to state reg-istries and
the IFSP review, the infant’s progress in language, motor,national data sets.
cognitive, and social-emotional devel-opment is monitored. The Physiologic measures must be used to screen new-borns and
care coordinator assists the family in advocating for the infant’sinfants for hearing loss. Such measures in-clude OAE and
unique develop-mental needs. automated ABR testing. Both OAE and automated ABR
technologies provide noninvasive re-cordings of physiologic
The deaf and hard-of-hearing community includes membersactivity underlying normal audi-tory function, both are easily
with direct experience with signed language, spoken language,performed in neonates and infants, and both have been
hearing-aid and cochlear implant use, and other communicationsuccessfully used for UNHS.19,34–37 However, there are
strategies and technologies. Optimally, adults who are deaf orimportant differences between the 2 measures. OAE
hard-of-hearing should play an integral part in the EHDImeasurements are ob-tained from the ear canal by using a
program. Both adults and children in the deaf and hard-of-sensitive micro-phone within a probe assembly that records
hearing community can enrich the family’s experience by serv-cochlear responses to acoustic stimuli. Thus, OAEs reflect the
ing as mentors and role models. Such mentors have experiencestatus of the peripheral auditory system extending to the
in negotiating their way in a hearing world, raising infants orcochlear outer hair cells. In contrast, ABR measurements are
children who are deaf or hard of hear-ing, and providingobtained from surface electrodes that record neural activity
families with a full range of informa-tion about communicationgenerated in the cochlea, auditory nerve, and brainstem in
options, assistive technology, and resources that are available inresponse to acoustic stimuli delivered via an earphone.
the community. Automated ABR measurements reflect the status of the
peripheral auditory system, the eighth nerve, and the brainstem
A successful EHDI program requires collaboration be-tweenauditory pathway.
a variety of public and private institutions and agencies that
assume responsibility for specific compo-nents (eg, screening,
Both OAE and ABR screening technologies can be used to
evaluation, intervention). Roles and responsibilities may differ
detect sensory (cochlear) hearing loss19; how-ever, both
from state to state. Each state has defined a lead coordinating
technologies may be affected by outer or middle-ear
agency with over-sight responsibility. The lead coordinating
dysfunction. Consequently, transient condi-tions of the outer
agency in each state should be responsible for identifying the
and middle ear may result in a “failed” screening-test result in
pub-lic and private funding sources available to develop,
implement, and coordinate EHDI systems. the presence of normal cochlear and/or neural function.38
Moreover, because OAEs are generated within the cochlea,
OAE technology cannot be used to detect neural (eighth nerve
Hearing Screening or auditory brain-stem pathway) dysfunction. Thus, neural
conduction disorders or auditory neuropathy/dyssynchrony
Multidisciplinary teams of professionals, including audi-
without concomitant sensory dysfunction will not be detected
ologists, physicians, and nursing personnel, are needed to
by OAE testing.
establish the UNHS component of EHDI programs. All team
members work together to ensure that screening programs are
of high quality and are successful. An audiologist should be Some infants who pass newborn hearing screening will later
25
involved in each component of the hearing-screening program,demonstrate permanent hearing loss. Al-though this loss may
particularly at the level of statewide implementation and,reflect delayed-onset hearing loss, both ABR and OAE
whenever possible, at the individual hospital level. Hospitalsscreening technologies will miss some hearing loss (eg, mild or
and agencies should also designate a physician to oversee the isolated frequency region losses).
medical aspects of the EHDI program.
Interpretive criteria for pass/fail outcomes should re-flect
39,40
Each team of professionals responsible for the hospi-tal-clear scientific rationale and should be evidence based.
based UNHS program should review the hospital in-frastructureScreening technologies that incorporate auto-mated-response
in relationship to the screening program. Hospital-baseddetection are necessary to eliminate the need for individual test
programs should consider screening technology (ie, OAE orinterpretation, to reduce the effects of screener bias or operator
automated ABR testing); validity of the specific screeningerror on test outcome, and to ensure test consistency across
device; screening protocols, in-cluding the timing of screeninginfants, test condi-tions, and screening personnel.41–45 When
relative to nursery dis-charge; availability of qualified screeningstatistical probability is used to make pass/fail decisions, as is
personnel; suitability of the acoustical and electricalthe case for OAE and automated ABR screening devices, the
environments; follow-up referral criteria; referral pathways forlikelihood of obtaining a pass outcome by chance alone is
follow-up; information management; and quality control andincreased when screening is performed repeatedly.46–48
improvement. Reporting and communication protocols
must be well defined and include the content of reports to PEDIATRICS Volume 120, Number 4, October 2007 903
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This principle must be incorporated into the policies of
rescreening.
904 AMERICAN ACADEMY OF PEDIATRICS
There are no national standards for the calibration of OAE or infants who have spent time in the NICU represent 10% to 15%
ABR instrumentation. Compounding this prob-lem, there is a
of the newborn population.54
lack of uniform performance standards. Manufacturers of
The 2007 JCIH position statement includes neonates at risk
hearing-screening devices do not al-ways provide sufficient
of having neural hearing loss (auditory neuropa-thy/auditory
supporting evidence to validate the specific pass/fail criteria dyssynchrony) in the target population to be identified in the
and/or automated algo-rithms used in their instruments.49 In theNICU,55–57 because there is evidence that neural hearing loss
absence of national standards, audiologists must obtain
results in adverse communica-tion outcomes.22,50 Consequently,
normative data for the instruments and protocols they use.
the JCIH recommends ABR technology as the only appropriate
screening tech-nique for use in the NICU. For infants who do
The JCIH recognizes that there are important issuesnot pass automated ABR testing in the NICU, referral should be
differentiating screening performed in the well-infant nurserymade directly to an audiologist for rescreening and, when
from that performed in the NICU. Although the goals in eachindicated, comprehensive evaluation, including diagnostic ABR
nursery are the same, numerous method-ologic andtesting, rather than for general outpa-tient rescreening.
technological issues must be considered in program design and
pass/fail criteria.
Screening Protocols in the NICU 0● the results of the screening test (pass, did not pass, or
missed) as documented in the hospital medical chart;
An NICU is defined as a facility in which a neonatologist
and
provides primary care for the infant. Newborn units are divided
into 3 categories:
0● communication directly from a representative of the
0● Level I: basic care, well-infant nurseries hospital screening program regarding each infant in its
0● Level II: specialty care by a neonatologist for infants care who did not pass or was missed and recommen-
at moderate risk of serious complications dations for follow-up.
To ensure accountability, individual, community, and stateOpportunities for Interaction With Individuals Who Are Deaf
health and educational programs should assume theor Hard of Hearing
responsibility for coordinated, ongoing measure-ment andIntervention programs should include opportunities for
improvement of EHDI process outcomes. Early interventioninvolvement of individuals who are deaf or hard of hearing in
programs must assess the language, cogni-tive skills, auditoryall aspects of EHDI programs. Because inter-vention programs
skills, speech, vocabulary, and social-emotional development ofserve children with mild-to-profound, unilateral or bilateral,
all children with hearing loss at 6-month intervals during thepermanent conductive, and sen-sory or neural hearing disorders,
first 3 years of life by using assessment tools that have been role models who are deaf or hard of hearing can be significant
standardized on children with normal hearing and norm-assets to an intervention program. These individuals can serve
referenced as-sessment tools that are appropriate to measureon state EHDI advisory boards and be trained as mentors for
progress in verbal and visual language. families and children with hearing loss who choose to seek their
support. Almost all families choose at some time during their
The primary purpose of regular developmental mon-itoringearly childhood programs to seek out both adults and child
is to provide valuable information to parents about the rate ofpeers with hearing loss. Programs should ensure that these
their child’s development as well as programmatic feedbackopportunities are available and can be delivered to families
concerning curriculum deci-sions. Families also becomethrough a variety of com-munications means, such as Web
knowledgeable about expec-tations and milestones of typicalsites, e-mail, newslet-ters, videos, retreats, picnics and other
development of hearing children. Studies have shown that validsocial events, and educational forums for parents.
and reliable documentation of developmental progress is
possible through parent questionnaires, analysis of videotaped
conversational interactions, and clinically administeredProvision of Communication Options
assessments.* Documentation of developmental progressResearch studies thus far of early-identified infants with hearing
should be provided on a regular basis to parents and, withloss have not found significant differences in the developmental
parental release of information, to the medical home andoutcomes by method of communication when measured at 3
audiologist. Although criterion-referenced checklists mayyears of age.† Therefore, a range of options should be offered
provide valuable information for estab-lishing interventionto families in a nonbiased manner. In addition, there have been
strategies and goals, these assess-ment tools alone are notreports of children with successful outcomes for each of the
sufficient for parents and in-tervention professionals todifferent meth-ods of communication. The choice is a dynamic
determine if a child’s developmental progress is comparableprocess on a continuum, differs according to the individual
with his or her hearing peers. needs of each family, and can be adjusted as necessary on the
basis of a child’s rate of progress in developing communication
skills. Programs need to provide families with access to skilled
and experienced early intervention professionals to facilitate
communication and language development in the
Opportunities for Interaction With Other Parents of Children
With Hearing Loss communication option chosen by the family.
Intervention professionals should seek to involve par-ents at
every level of the EHDI process and develop true and
meaningful partnerships with parents. To reflect the value of theSkills of the Early Intervention Professional
contributions that selected parents make to development andAll studies with successful outcomes reported for early-
program components, these parents should be paid asidentified children who are deaf or hard of hearing have
contributing staff members. Parent representatives should beintervention provided by specialists who are trained in parent-
12,90,97
included in all advisory board activities. In many states, parentsinfant intervention services. Early interven-tion programs
have been integral and often have taken leadership roles in theshould develop mechanisms to ensure that early intervention
development of policy, resource material, communicationprofessionals have special skills necessary for providing
mechanisms, mentoring and advocacy opportunities,families with the highest quality of service specific to children
dissemination of information, and interaction with the deafwith hearing loss. Profes-sionals with a background in deaf
community and other individuals who are deaf or hard of education, audiology, and speech-language pathology will
hearing. Parents, often in partnership with people who are deaf typically have the skills needed for providing intervention
and hard of hearing, have also participated in the train-ing ofservices. Profes-sionals should be highly qualified in their
professionals. They should be participants in the regularrespective fields and should be skilled communicators who are
assessment of program services to ensure ongo-ingknowledgeable and sensitive to the importance of en-
improvement and quality assurance.
†Refs 10 –13, 85, 87, 88, 90, 93, and 96.
When early intervention professionals have knowl-edge of thecheck and augment physiologic findings (see www.au-
principles of adult learning, it increases their success with diology.org).
parents and other professionals. The goal of amplification-device fitting is to provide the
infant with maximum access to all of the acoustic features of
Quality of Intervention Services speech within an intensity range that is safe and comfortable.
Children with confirmed hearing loss and their families haveThat is, amplified speech should be comfortably above the
the right to prompt access to quality intervention services. Forinfant’s sensory threshold but below the level of discomfort
newborn infants with confirmed hearing loss, enrollment intoacross the speech fre-quency range for both ears. To accomplish
intervention services should begin as soon after hearing-lossthis in in-fants, amplification-device selection, fitting, and
confirmation as possible and no later than 6 months of age.verifi-cation should be based on a prescriptive procedure that
Successful early interven-tion programs (1) are family centered,incorporates individual real-ear measures that account for each
32
(2) provide fam-ilies with unbiased information on all options infant’s ear-canal acoustics and hearing loss. Validation of the
regarding approaches to communication, (3) monitor develop-benefits of amplification, particularly for speech perception,
ment at 6-month intervals with norm-referenced instru-ments,should be examined in the clinical setting as well as in the
(4) include individuals who are deaf or hard of hearing, (5)child’s typical listening environ-ments. Complementary or
provide services in a natural environment in the home or in thealternative technology, such as frequency modulation (FM)
center, (6) offer high-quality service regardless of where thesystems or cochlear im-plants, may be recommended as the
family lives, (7) obtain informed consent, (8) are sensitive toprimary and/or secondary listening device depending on the
cultural and language dif-ferences and provide accommodationsdegree of the infant’s hearing loss, the goals of auditory
as needed, and habilita-tion, the infant’s acoustic environments, and the fami-
ly’s informed choices.3 Monitoring of amplification, as well as
(9) conduct annual surveys of parent satisfaction. the long-term validation of the appropriateness of the individual
habilitation program, requires ongoing audiological assessment
Intervention for Special Populations of Infants and Young along with electroacoustic, real-ear, and functional checks of
Children the hearing instruments. As the hearing loss becomes more
Developmental monitoring should also occur at regular 6-specifically defined through audiological assessments and as
month intervals for special populations of children with hearingthe child’s ear-canal acoustics change with growth, refinement
loss, including those with minimal and mild bilateral hearingof the individual prescriptive hearing-aid gain and output tar-
loss,98 unilateral hearing loss,99,100 and neural hearing loss,22gets is necessary. Monitoring also includes periodic val-idation
because these children are at risk of having speech and of communication, social-emotional, and cogni-tive
language delay. Research find-ings indicate that approximately development and, later, academic performance to ensure that
one third of children with permanent unilateral loss experience progress is commensurate with the child’s abilities. It is
significant language and academic delays. 99–101 possible that infants and young children with measurable
residual “hearing” (auditory responses) and well-fit
Audiological Habilitation amplification devices may fail to develop auditory skills
Most infants and children with bilateral hearing loss and manynecessary for successful spoken commu-nication. Ongoing
with unilateral hearing loss benefit from some form of personalvalidation of the amplification device is accomplished through
amplification device.32 If the family chooses personalinterdisciplinary evaluation and collaboration with the early
amplification for its infant, hearing-aid selection and fittingintervention team and fam-ily.
should occur within 1 month of initial confirmation of hearing
loss even when additional audiological assessment is ongoing.
Audiological habili-tation services should be provided by an
audiologist who is experienced with these procedures. Delay Cochlear implantation should be given careful con-
between confirmation of the hearing loss and fitting of ansideration for any child who seems to receive limited benefit
ampli-fication device should be minimized.51,102 from a trial with appropriately fitted hearing aids. According to
Hearing-aid fitting proceeds optimally when the re-sults ofUS Food and Drug Administration guidelines, infants with
physiologic audiological assessment including diagnostic ABR,profound bilateral hearing loss are candidates for cochlear
OAE, and tympanometry and medical examination are inimplantation at 12 months of age and children with bilateral
accord. For infants who are below a developmental age of 6severe hearing loss are eligible at 24 months of age. The
months, hearing-aid selection will be based on physiologicpresence of develop-mental conditions (eg, developmental
measures alone. Behavioral threshold assessment with visualdelay, autism) in addition to hearing loss should not, as a rule,
reinforcement audiom-etry should be obtained as soon aspreclude the consideration of cochlear implantation for an
possible to cross- infant or child who is deaf. Benefits from hearing aids and
cochlear implants in children with neural hearing loss
ment of children who have appropriate amplification come and system barriers to be conquered to achieve optimal
devices in early life. EHDI systems in all states in the next 5 years. Follow-up rates
remain poor in many states, and fund-ing for amplification in
0● Expand programs within health, social service, andchildren is inadequate. Funding to support outcome studies is
education agencies associated with early interventionnecessary to guide inter-vention and to determine factors other
and Head Start programs to accommodate the needs of
than hearing loss that affect child development. The ultimate
the increasing numbers of early-identified children.
goal, to op-timize communication, social, academic, and
0● Adapt education systems to capitalize on the abilities vocational outcomes for each child with permanent hearing
of children with hearing loss who have benefited fromloss, must remain paramount.
early identification and intervention.
0● Develop genetic and medical procedures that will de-
termine more rapidly the etiology of hearing loss. JOINT COMMITTEE ON INFANT HEARING
Jackie Busa, BA Judy
0● Ensure transition from Part C (early intervention) toHarrison, MA
Part B (education) services in ways that encourage
Alexander Graham Bell Association for the Deaf and Hard of
family participation and ensure minimal disruption of
Hearing
child and family services.
Jodie Chappell
0● Study the effects of parents’ participation in allChristine Yoshinaga-Itano, PhD
aspects of early intervention. Alison Grimes, AuD
0● Test the utility of a limited national data set and de- American Academy of Audiology
velop nationally accepted indicators of EHDI systemPatrick E. Brookhouser, MD Stephen
performance. Epstein, MD
American Academy of Otolaryngology-Head and Neck
0● Encourage the identification and development of cen- Surgery
ters of expertise in which specialized care is providedAlbert Mehl, MD
in collaboration with local service providers.
Betty Vohr, MD, Chairperson, March 2005–present
0● Obtain the perspectives of individuals who are deaf or American Academy of Pediatrics
hard of hearing in developing policies regarding med- Judith Gravel, PhD, Chairperson, March 2003–March 2005
ical and genetic testing and counseling for families
who carry genes associated with hearing loss.139 Jack Roush, PhD
Judith Widen, PhD
CONCLUSIONS American Speech-Language-Hearing Association Beth
Since the 2000 JCIH statement, tremendous and rapid progressS. Benedict, PhD
has been made in the development of EHDI systems as a majorBobbie Scoggins, EdD
public health initiative. The percent-age of infants screened Council of Education of the Deaf (the member orga-nizations
annually in the United States has increased from 38% to 95%. of the Council on Education of the Deaf include the
The collaboration at all levels of professional organizations, Alexander Graham Bell Association for the Deaf and Hard of
federal and state government, hospitals, medical homes, and Hearing, American Society for Deaf Children, Conference of
families has contributed to this remarkable success. New Educational Administra-tors of Schools and Programs for the
research initiatives to develop more sophisticated screening and Deaf, Convention of American Instructors of the Deaf,
diagnostic technology, improved digital hearing-aid and FM National Associ-ation of the Deaf, and Association of
technologies, speech-processing strategies in co-chlear College Educators of the Deaf and Hard of Hearing)
implants, and early intervention strategies con-tinue. MajorMichelle King, MS, AuD
technological breakthroughs have been made in facilitating theLinda Pippins, MCD David
definitive diagnosis of both ge-netic and nongenetic etiologiesH. Savage, MSC
of hearing loss. In addi-tion, outcomes studies to assess the Directors of Speech and Hearing Programs in State Health
long-term outcomes of special populations, including infants and Welfare Agencies
and children with mild and unilateral hearing loss, neural
hearing loss, and severe or profound hearing loss managed withEX OFFICIOS
cochlear implants, have been providing information on theJill Ackermann, MS
individual and societal impact and the factors that contribute toAmy Gibson, MS, RN
an optimized outcome. It is apparent, how-ever, that there areThomas Tonniges, MD
still serious challenges to be over- American Academy of Pediatrics
Pamela Mason, MEd
American Speech-Language-Hearing Association
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