Title: Newborn Hearing Screening and Early Intervention in the United States
1Newborn Hearing Screening and Early Intervention
in the United States
- Lenore Holte, Ph.D.
- Department of Speech Pathology and Audiology
- Department of Pediatrics
- University of Iowa
2National Goals for Hearing Screening (1-3-6)
adopted by NIH, CDC, AAP
- All newborns will be screened for hearing loss
before one month of age, preferably before
hospital discharge - All infants who screen positive will have a
diagnostic audiological evaluation before 3
months of age - All infants identified with a hearing loss will
receive appropriate intervention before 6 months
of age
3Why should we screen the hearing of all newborns?
- Multiple studies (1960s and 1970s) demonstrating
that average reading level of deaf adults is 4th
to 5th grade - Hard-of-hearing students also score significantly
below hearing peers on tests of verbal skills - Critical period for language learning
- Former high-risk screening programs identified
only half of all congenital hearing loss
4Hearing loss is sufficiently frequent in the
screened population Incidence per 1000 of
various screenable congenital conditions
5Speech and language delay is treatable or
preventable Effect of age of identification of
congenital hearing loss on later speech and
language abilities
- Yoshinaga-Itano (1998) and Moeller (2000) found
that the most important factor determining
language abilities at ages 3 and 5, respectively,
was age at which hearing loss was identified and
intervention begun
6Moeller(2000) Receptive vocabulary scores at age
5 years as a function of age of enrollment in
intervention (in months). Mean for
normal-hearing age-matched peers is 100.
7Yoshinaga-Itano et al. (1998) Mean total
language quotient at 36 months by age of
identification of hearing loss (before or after 6
months) and cognition (lt or gt CQ80)
8Newborn hearing screening programs
- Screen every newborn during birth admission
- Most programs recommend outpatient re-screen of
those who do not pass the birth admission screen
at 2-6 weeks of age. - Any infant who does not pass the re-screen needs
a diagnostic Auditory Brainstem Response (ABR)
test - Any infant diagnosed with hearing loss needs
evaluation by pediatric otolaryngologist
9Two primary technologies use in newborn hearing
screening
- 1. Otoacoustic emissions (OAEs)
- 2. Automated Auditory Brainstem response (A-ABR)
- Choice of most cost-effective method depends on
number of babies in the nursery and presence or
absence of high-risk newborns - Both can miss mild hearing loss
10Discovery of otoacoustic emissions (OAEs) a
revolution in auditory physiology
- Kemps (1978) advances in microphone technology
led to evidence of previously postulated active
mechanisms in the cochlea - OAE generators are the outer hair cells in the
cochlea
11(No Transcript)
12OAEs
- Sounds generated in the cochlea and recorded in
the external ear canal with a sensitive
microphone. - Not recorded in ears with outer hair cell loss in
the cochlea. This represents the majority of
sensorineural hearing loss. - Not recorded if there is too much noise in the
environment or if the infant is too active
13OAE screening
14OAEs in newborn hearing screening
- Involves placement of soft probe in ear canal
- Cost of equipment and disposables is lower than
A-ABR - Affected by transient middle ear effusion or the
presence of vernix in the ear canal - For this reason, characterized by higher false
positive rate than A-ABR
15Sample DPOAE pass/fail criteria (these are
default settings on current AudX and most widely
used in Iowa, based on Gorga et al., 1997). Must
pass 3 of 4 F2 frequencies to pass screen
16Automated Auditory Brainstem Response (A-ABR)
17A-ABR in newborn hearing screening
- Cost of equipment and disposables higher than OAE
- Due to placement of electrodes, more
time-consuming than OAE - More specific than OAE
182006 United States national NHS follow-up
- National Data 43 States, 3 territories, 1
district - 3,305,772 births
- 91.2 received screen
- 2.2 did not pass final hearing screen 60,436
infants
19National data 2006 Intervention Status
- 3,343 children identified with hearing loss
- 86.7 with hearing loss referred to Part C EI
- 57.7 receiving Part C EI
- 7.7 receiving Non-Part C EI
20Estimates of Causes of Deafness at Birth and at
Four Years in the United States
Morton C and Nance W. N Engl J Med
20063542151-2164
21The Joint Committee on Infant Hearing (JCIH)
- Established in 1969
- Original charge was to make recommendations for
early identification of children with hearing
loss - Representatives from organizations with interest
and expertise with children with hearing loss - The 2007 statement was their 7th published
statement - Endorsed universal screening in 1994.
- Still publish risk factors lists, especially to
monitor kids at risk for late-onset loss
22- What about babies who pass the newborn screen but
are at risk for late-onset progressive hearing
loss?
23Important points in JCIH (2000) position statement
- Targeted congenital unilateral or bilateral
sensorineural or permanent conductive hearing
loss - Diagnose by 3 months
- Intervention by 6 months
- Monitor children with high-risk factors, even if
they pass the newborn screen (called for an
evaluation every 6 months until kid is 3 years
this would be impossible!) - Information systems for quality control
- Medical home
- Family choice in intervention
24JCIH (2000) Risk indicators for newborns
(collapsed 6 previous indicators from 1994 into
4)
- 1. family history of childhood hearing loss
- 2. in utero infection
- 3. craniofacial anomalies
- 4. NICU admission of 48 hours or more
- 5. syndrome associated with hearing loss
- Problem monitoring all of these babies every 6
months until age 3 will exceed the capacity of
audiologists to provide diagnostic evaluations
25JCIH (2007) position statement Some changes from
2000
- Target population will include auditory
neuropathy/dyssynchrony. This means using AABR in
NICUs - Rescreen both ears of unilateral refers
- Monitor those at risk for late-onset hearing loss
on an individualized schedule, with at least one
audiological evaluation by 24-30 months of age
those with congenital CMV or ECMO more frequent
(every 6 months?)
26JCIH (2007) Risk factors. indicates greater
concern for delayed onset hearing loss
- Caregiver concern re hearing, speech, language
or developmental delay - Family history of permanent childhood hearing
loss - NICU stay of gt5 days, which may include ECMO,
assisted ventilation, exposure to ototoxic
medications and hyperbilirubinemia requiring
exchange transfusion - In-utero infections, such as CMV, herpes,
rubella, syphilis and toxoplasmosis - Craniofacial anomalies
27JCIH (2007) Risk factors. indicates greater
concern for delayed onset hearing loss
- Syndromes associated with hearing loss or
progressive or late onset hearing loss such as
neurofibromatosis, osteopetrosis and Ushers
syndrome. Other frequent syndromes include
Waardenburg, Alport, Pendred and Jervell
Lange-Nielson - Neurodegenerative disorders such as Hunter or
sensory motor neuropathies - Postnatal infections associated with SNHL
including bacterial and viral (esp. herpes and
varicella) meningitis - Head trauma esp. basal skull/temporal bone
fracture - Chemotherapy
28Signs of unidentified childhood hearing loss
- Lack of awareness to sound
- Speech and language delay
- Poor speech intelligibility
- Distractibility
- Behavior problems
29Case study no known newborn hearing screen
- 4 year, 11-month-old girl referred to CDD for
evaluation of possible ADHD and concerns about
speech intelligibility - no known newborn screen
- mother and maternal grandmother have hearing loss
- 1 set of tubes, after which family noticed
improvement in responsiveness to sound
30CDE at CDD
- Parents report she is fidgety and distractible
- Parents estimate her speech is lt20 intelligible
- average nonverbal cognitive abilities
- Speech evaluation poor articulation skills and
good intonation patterns
31Note how misleading right DPOAEs are
32Recordings of speech at day of fitting binaural
BTE hearing aids and one month post-fitting
33Loss to follow-up
- Despite legislative mandate of universal newborn
hearing screening in 38 states, current data from
the CDC indicate that about half of all newborns
who are deaf or hard-of-hearing are lost to
follow-up - Intervention begins with hearing aids, enrollment
in an early intervention program for deaf and
hard-of-hearing infants, and decisions by the
family about communication methods
34Recommended Reading
- Year 2007 position statement of the Joint
Committee on Infant Hearing - http//www.cdc.gov/ncbddd/ehdi/documents/JCIH_2007
.pdf