Newborn Hearing Screening and Early Intervention in the United States

1
Newborn 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

2
National 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

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Why 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

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Hearing loss is sufficiently frequent in the
screened population Incidence per 1000 of
various screenable congenital conditions
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Speech 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

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Moeller(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.
7
Yoshinaga-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)
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Newborn 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

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Two 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

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Discovery 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

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OAEs

• 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

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OAE screening
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OAEs 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

15
Sample 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
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Automated Auditory Brainstem Response (A-ABR)
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A-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

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2006 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

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National 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

20
Estimates 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
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The 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

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• What about babies who pass the newborn screen but
  are at risk for late-onset progressive hearing
  loss?

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Important 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

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JCIH (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

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JCIH (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?)

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JCIH (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

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JCIH (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

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Signs of unidentified childhood hearing loss

• Lack of awareness to sound
• Speech and language delay
• Poor speech intelligibility
• Distractibility
• Behavior problems

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Case 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

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CDE 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

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Note how misleading right DPOAEs are
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Recordings of speech at day of fitting binaural
BTE hearing aids and one month post-fitting
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Loss 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

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Recommended Reading

• Year 2007 position statement of the Joint
  Committee on Infant Hearing
• http//www.cdc.gov/ncbddd/ehdi/documents/JCIH_2007
  .pdf