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Premature Infants Advances in Care and Outcome

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Joint Committee on Infant Hearing
Update 2007
• Objectives:
• Give a brief background of the 38
year history of the JCIH leading to the
8th JCIH Position Statement
• Present key changes in the 2007
Position Statement
JCIH established in 1969
• Composed of representatives of professional
organizations with interest in children with hearing loss
• Original charge: Make recommendations concerning
early identification of children with HL, and newborn
screening. The professional leading that charge
• was Dr Marion Downs.
First Position Statement
• The first one page Position Statement was published in
Pediatrics in 1971.
• It concluded that data at the time were inconsistent and
misleading and therefore universal screening of
newborn infants could not be recommended.
• Risk Factors were not mentioned.
What is Happening in 2007 ?
• 33 babies are born each day in the US with HL
• 12,000 to 16,000 babies are born each year with HL
• > 95% of newborns in the United States
and territories have their hearing
Highlights of JCIH 2007
Position Statement
Definition of Targeted Hearing Loss
Expanded from congenital bilateral and unilateral
sensory or permanent conductive HL
to include
neural hearing loss (auditory
neuropathy/dyssynchrony) in infants admitted to
the NICU > 5 days.
Hearing Screen Protocols
• Separate protocols are therefore recommended
for NICU and well baby nurseries.
• NICU babies >5 days are to have ABR
included as part of their screen so that neural
HL will not be missed
Hearing Screen Protocols
• Screening results should be conveyed
immediately to families so they understand the
outcome and the importance of follow-up when
• For rescreening, a complete evaluation of both
ears is recommended, even if only 1 ear failed
the initial screen.
• For readmissions of infants in the first month of
if there are conditions present which are
associated with potential hearing loss (e.g.
hyperbilirubinemia req. exchange transfusion or
culture + sepsis), a repeat hearing screen is
recommended prior to discharge.
Diagnostic Audiology Evaluation
• Audiologists with skills and expertise in
evaluating infants with hearing loss should
provide audiology diagnostic and habilitation
• At least one ABR is recommended as part of a
complete diagnostic audiology evaluation for
children under 3 years of age for confirmation of
permanent HL, in conjunction with other
measures for validation of HL.
Diagnostic Audiology Evaluation
• Infants with a risk factor for HL should have at
least one diagnostic audiology assessment by 30 m
of age. Infants with risk factors associated with late
onset or progressive loss (eg CMV or ECMO) are
followed more frequently.
• For families who elect amplification, infants
diagnosed with permanent hearing loss should be
fitted with amplification within one month of
Medical Evaluation
• All families should be offered a Genetics
• Every infant with a confirmed HL should have
at least one exam by an ophthalmologist
experienced in evaluating infants. Other
specialty consultations may be indicated.
• The list of risk factors has been reorganized to
a single list to focus on both early and late
onset and/or progressive HL.
Risk Factors for Hearing Loss: 2006
Caregiver concern* regarding hearing, speech,
Family history* of permanent childhood HL.
NICU care of >5 days, or any of following
regardless of length of stay : assisted
ventilation, ototoxic medications, exchange
transfusion, and ECMO*,
Intra-uterine TORCH infections, particularly
Craniofacial anomalies, especially those
involving the pinna, ear canal, ear tags, ear
pits, and temporal bone anomalies
Risk Factors for Hearing Loss , cont
Physical findings associated with a syndrome
known to include permanent HL
Syndromes associated with progressive HL
such as NF, osteopetrosis, Usher’s syndrome
Neurodegenerative disorders*, such as Hunter
Postnatal infections associated with SNHL
especially bacterial meningitis*
Head trauma requiring hospitalization
Surveillance and screening in the Medical Home
• All infants should have regular surveillance
consistent with the pediatric periodicity schedule of:
• auditory skills
• milestones
• parent concerns
• middle ear effusion
Surveillance and screening in the Medical Home
• All infants should have an objective standardized
screen of global development with a validated tool
at 9, 18, and 30 months of age.
• Children who do not pass a medical home global
screen or if there is concern regarding hearing or
language should be referred for speech-language
evaluation and audiology assessment.
Early Intervention
• Families of infants with all degrees of HL
should be offered Early Intervention.
• The recognized point of entry for EI for infants
with a confirmed HL should be linked to EHDI,
and be provided by professionals with expertise
in HL, including educators of the deaf and
speech language professionals.
• Both home-based and center-based options
should be offered as appropriate interventions.
Modes of Communication for
children with permanent HL
• Families should be made aware of all
communication options and available hearing
• Family choice guides the decision making
• Information at all stages of the EHDI process is to
be communicated to the family in a culturally
sensitive and understandable format.
• Hearing screen information, audiology diagnostic
and habilitation information should be transmitted
to the medical home and the state EHDI
Information Infrastructure
• States must develop adequate data management
and tracking systems as part of an integrated
child health information system in order to
• monitor the quality of EHDI services and
• provide recommendations for improving systems
of care.
Information Infrastructure
• A linkage between health and education is
recommended to determine outcomes of children
with hearing loss at school age.
• This is needed for planning and establishing
public health and education policy.
• It is expected that the JCIH 2007 Position
Statement recommendations will facilitate:
The development of more effective and
seamless EHDI systems
Decrease the loss to follow-up
Improve the outcomes for all infants with HL.
• An abbreviated, “user-friendly”
executive summary of the 2007
Statement is available
• Publication date: October 2007
However, Challenges Remain in 2007
• Follow-up rates of screen fails remain ~ 50%
• There is failure to communicate information to
families in a culturally sensitive and understandable
• Lack of integrated state data tracking systems
Challenges, However, Remain in 2007
• A shortage of facilities and personnel with the
experience and expertise needed to provide followup
• A significant number of children who need further
assessment do not receive appropriate evaluations.
The EDHI Challenge
Continues in 2008 with continued efforts to
achieve success needed on multiple levels:
• hospital,
• community,
• state and
• federal
• We all need to take the “EHDI Challenge”
2007 JCIH Membership and Support
AG Bell
Betty Vohr (Chair), Albert Mehl
Stephen Epstein (Vice Chair), Patrick Brookhouser
Judy Widen, Brandt Culpepper
Christie Yoshinaga-Itano, Alison Grimes
Beth Benedict, Bobbie Scoggins
Michelle King /Linda Pippins, David Savage
Jackie Busa, Judy Harrison
Jill Ackermann –
Pam Mason –
Jodi Chappel,Yvonne Sininger
Irene Forsman John EichwaldTom Tonniges –
Jackson Roush, Judy Gravel,
Amy Donahue -
AAP staff
ASHA staff
AAA staff, and AAA ex officio
ASHA ex officio
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