|
|
Winter 2008
Early Hearing
Detection and Intervention
Best
Practice for Primary Care Providers
Amy Wallin, MD, Iowa Chapter
of the American Academy of Pediatrics
EHDI Advisory Council |
|
Hearing loss is a major
public health concern in the US:
-
Every
year, more than 12,000 babies
are born with a hearing loss, making this the
most common congenital condition.
-
Only 50%
of these children have a risk factor for hearing loss.
-
90% of all
babies born with a hearing loss have two hearing
parents.
|
 |
|
>Early Hearing
and Detection and Intervention 1-3-6 goals
Early Hearing Detection and Intervention (EHDI), a nationwide program of the Centers for
Disease Control and Prevention, promotes “the best possible communication skills
from birth for all children.” The CDC worked with national organizations and
with every state and territory in the US to develop these EHDI 1-3-6 goals:
|
|
Before age
|
All infants will be
|
|
1 month
|
Screened for hearing loss |
|
3 months
|
Given diagnostic audiologic and
medical
evaluation if initial screen raises concerns
|
|
6 months
|
Enrolled in early intervention
services if hearing
loss is found |
|
EHDI and you
Iowa law requires a newborn hearing screen for every child born in Iowa,
whether the child is born at home or in the hospital. Legislation also calls
for mandatory reporting of data, including missed hearing screens, pass or
refer results, primary care provider for child, and parent or guardian
contact information. Hospitals are also encouraged to report known risk
factors for the infant.
Primary care providers
are responsible for ensuring their newborn patients have been screened
before they are one month old. Infants not screened at birth should also be
screened within this time frame. If the initial newborn hearing screen
raises concerns, a re-screen should be scheduled promptly, within one to two
weeks.
Screening techniques
Evoked otoacoustic emissions (OAE) or automated auditory brainstem response
(A-ABR) are the recommended screening techniques in Iowa.
-
OAE screening
measures sound waves or emissions made by the outer hair cells in
the cochlea.
-
A-ABR
screening
uses electrodes placed on the infant’s head to record the brain’s
response to sound
|
|
Two-stage screening is
recommended for infants at higher risk of hearing loss, such as those in
neonatal intensive care units. This approach uses OAE testing first, and
then A-ABR testing second. Two-stage screening identifies mild cases of congenital
hearing loss as well as auditory neuropathy, a disorder characterized by
normal outer hair cell function but abnormal auditory nerve function.
Infants who do not pass
the initial hearing screen or re-screen need a diagnostic pediatric
audiologic evaluation before three months of age. This testing determines
the presence, type, and degree of the baby’s hearing loss. |
 |
If the diagnosis of
hearing loss is confirmed, the child’s health care provider should:
-
Promptly refer the
child and family to early intervention services (Early ACCESS in Iowa,
or Part C), so parents can learn about intervention options and make
timely and well-informed choices for their baby
-
Schedule ENT and
ophthalmology evaluations
-
Offer a referral for
genetic counseling even if the child’s hearing loss has a known
etiology, such as CMV, because a genetic cause may also exist
-
Provide other
medical referrals as indicated, such as developmental pediatrics,
neurology, or an EKG if you suspect long QT syndrome
An infant may need
medical clearance for hearing aids, cochlear implants, or other therapies
chosen by the family. Primary care providers are important sources of
information for families and can offer support and guidance during this
difficult time.
Early intervention
Because hearing plays a
crucial role in the development of cognition, language, and social skills, a
child’s first 6 months are a critical period. For this reason, infants with
hearing loss should be enrolled in early intervention services by 6 months
of age.
n Iowa, Early ACCESS
facilitates early intervention services for infants and children with
hearing loss (contact information is provided in “Resources,” below). Early
ACCESS can provide information about options available to families, and will
work with the family to develop a comprehensive program to support their
decisions about these options. Early ACCESS can also link families with
support groups throughout Iowa.
Late onset hearing
loss
Late onset or progressive hearing loss can develop at
any time in a child’s life. Primary care providers need
to review each child’s risk factors and discuss hearing
monitoring with families. Infants should have periodic
hearing evaluations, especially during the first three
years of life, if they have such risk factors as:
-
Family history of permanent childhood
hearing loss
-
In utero infections
such as cytomegalovirus, herpes, rubella, syphilis,
toxoplasmosis
-
Craniofacial anomalies involving the
pinna, ear canal, ear tags, ear pits; temporal bone anomalies
-
Neonatal intensive care of >5 days,
which may include:
-
Extracorporeal membrane oxygenation (ECMO)
assisted ventilation
-
Hyperbilirubinemia that requires exchange
transfusion
-
Exposure to ototoxic medications (gentamycin,
tobramycin
-
Exposure to loop diuretics (Lasix,
furosemide)
-
Postnatal infection associated with
sensorineural hearing loss, including bacterial or viral meningitis,
herpes, varicella
-
Conditions associated with hearing loss,
such as neurofibromatosis, osteopetrosis, Friedreich’s ataxia
-
Syndromes associated with hearing loss,
including Alport, Charcot-Marie-Tooth, Hunter, Jervell and
Lange-Nielson, Pendred, Usher, and Waardenburg syndromes
-
Head trauma, especially basal skull
or temporal bone fracture requiring hospitalization
-
Chemotherapy
-
Caregiver concern about hearing,
speech, language, or developmental delay
Conclusion
EHDI works to
benefit children and families through the early identification and treatment of
hearing loss. Success relies on the collective efforts of families, primary care
providers, and allied health professionals to provide universal newborn hearing
screening, early intervention, tracking, and surveillance.
For
more information,
please contact
Amy Wallin, MD, Iowa Chapter of the American Academy of Pediatrics, EHDI
Advisory Council, 515-224-4993, wallinal@ihs.org.
Resources
|
|
