Update on Newborn

Hearing Screening

R. Alan Grimes, MD, FAAP

Priority Care Pediatrics, LLC

Kansas City, Missouri
Missouri EHDI Chapter Champion
AAP EHDI Task Force Member
Acknowledgements
The speaker acknowledges the major
contributions of staff at the Boys Town
National Research Hospital in the
development of this presentation.

The development of the presentation is

supported by the National Institute on
Deafness and Other Communication
Disorders [(NIDCD/NIH) R25 DC04559; R25
DC006460]
Financial Disclosure Information

I have no relevant financial relationship with

the manufacturers of any commercial
products and/or provider of commercial
services discussed in this CME activity.

I do not intend to discuss an

unapproved/investigative use of a
commercial product/device in my
presentation.
Learning Objectives

Discuss the importance / impact of early

identification of hearing loss
Describe the status of newborn hearing
screening in states and nationally
Review universal newborn hearing screening
(UNHS) techniques
State the Primary Care Physicians role in
Early Hearing Detection and Intervention
(EHDI)
Describe resources to support patient
management and follow up
 National Goals for Hearing
Screening (1-3-6) 1, 2

All infants will access hearing screening using a

physiologic measure
no later than 1 month of age
All infants not passing initial screening and
subsequent rescreening should have confirmatory
audiological and medical evaluations
no later than 3 months of age
All infants with confirmed permanent hearing loss
should receive early intervention as soon as
possible
no later than 6 months of age
 Prerequisites for a Population
Screening Program
YES Condition sufficiently frequent in
screened population
YES Condition serious or fatal without
intervention
YES Condition must be treatable or
preventable
YES Effective follow-up program possible
 Why is early identification of
hearing loss important?
Hearing loss is the most common birth condition
Incidence of Congenital Conditions
(Per 10,000)
35

30
Number per 10,000

25

20

15

10

0
Hearing loss Cleft lip or Down Limb defects Spina bifida Sickle cell PKU
palate syndrome anemia
Congenital Condition Type
 Prevalence of Hearing Loss

Prevalence estimates vary across studies

Estimated that 1 to 3 per 1000 infants will
have permanent sensorineural hearing
loss3, 4
1/1000 from the well baby nursery
10/1000 from the NICU
Rate increases to 6/1000 by school age4
Need for surveillance
 Why is early identification of
hearing loss important?

Previous methods for detecting

hearing loss have been ineffective
High risk screening failed to identify ~
50% of the infants with hearing loss
Large retrospective cohort study5, 6: mean
age of diagnosis 21.6 months
Similar findings reported in US7,8,9
 Vocabulary Development
in Infants 12, 13

400
NH Boys
350 NH Girls
Number of Expressive Words

Toddlers with Hearing Loss

300

250

200

150

100

50

0
12 mos 14 mos 16 mos 18 mos 24 mos
Age

Delays in babble also observed 14, 15

 Reading Comprehension in
Children with Mild-Mod Loss 16

10.0
9.0
8.0
7.0
6.0 Deaf
5.0 Hearing
4.0
3.0
2.0
1.0
8 9 10 11 12 13 14 15 16 17 18

Schildroth, A. N., & Karchmer, M. A. (1986). Deaf children in America, San Diego: College Hill Press.
 Why is early identification of
hearing loss important?
Early identification and intervention
can make a difference
Effects of Age of Identification
on Language Development17
Prospective, longitudinal study of early-
identified infants
30 children with mild-profound hearing loss
(HL) compared to 96 normal hearing (NH)
controls
Children identified < 3 months had stronger
language development at 12-16 months
than those identified > 3 months
Children with HL were delayed compared to
NH infants
 Vocabulary at Age Five by
Age of Intervention19

Significant
Average range
Predictors:

Id Age: 8%

Family
Involvement:
37%
American Academy of Pediatrics (AAP)

Endorsed implementation of
universal newborn hearing
screening in 1999

Defined standards for:

Screening
Tracking & Follow-up
Identification & Intervention
Program Evaluation

Encouraged AAP chapters to

provide leadership in physician
education and newborn screening
in their states
Early Hearing Detection and
Intervention (EHDI)
Endorsed by:
AAP, National Institutes of Health, Maternal
and Child Health, Centers for Disease Control,
Joint Committee on Infant Hearing & in 2008,
the USPSTF
As of 2005, all 50 states implemented
statewide EHDI programs
As of 2006, an average of 95.7% of
newborns were screened nationally
Status of Hearing Screening
in Missouri
 Hearing Screening Techniques

Otoacoustic emissions (OAE)

Auditory brainstem response (ABR)

Two stage screening (OAE + ABR)

Otoacoustic Emissions

Sounds are presented

to the ear canal and a
small microphone
measures the response
in the ear canal
Average test time is
5-15 minutes/baby
Auditory Brainstem Response

Sounds are presented

and surface electrodes
measure brainstem
activity
Average test time 20
min/baby
 OAE + ABR
All babies are screened using OAEs
Those babies who fail the OAE screening
receive an ABR screening prior to leaving the
hospital
Average test time/baby (25-35 min)
Reduces refer rate; useful when follow up is
likely to be difficult or costly
Initial cost of equipment is higher than OAE or
ABR screening alone, but follow-up costs are
less
 2007 JCIH Position on
Screening 2

NICU Well baby nursery

>5 days in NICU Screen with OAE or ABR
ABR should be included Repeat screen when
to screen for neural loss necessary before
Rescreen BOTH ears, discharge
even if only one ear fails When using 2 step
Non pass refer to protocol test order
Audiologist should be OAE then ABR
Readmission rescreen Rescreen BOTH ears,
before discharge even if only one ear fails
 Characteristics of a good
screening program
Refer rate of 1.5-5.0% in well baby nursery
and slightly lower in the NICU (resulting
from 2-stage screening in the hospital)
5.0% = 400 babies per 8000 births
Ongoing training and monitoring program
for personnel
Structured plan for follow up
Ability to track program performance
(important for quality assurance and for
JCAHO requirements)
 What if a baby fails UNHS?

Failure rates range from 1.5-5.0% in

good screening programs

Most babies who fail the initial screening

will actually have normal hearing
For 10 babies that refer, 1 is expected to
have permanent hearing loss
 System challenges:
Loss to Follow Up23

8 New York hospitals,

28% infants who did not pass in-hospital
screening failed to return
Loss to follow up is as high as 50% in some
states
Return rates better for in-hospital fails than
in-hospital misses
Medical Home: Strategies to
Promote Follow Up
At prenatal visit, encourage families to
identify you as follow-up care location
Inform hospital to facilitate communication
of results
Provide checkbox on newborn well child
form/patient chart for hearing screening
results & risk factors
Set up tracking system for infants who do
not pass hearing screening or for those
infants with risk factors
Counseling Parents

Effective communication of results to

families has an influence on follow up
behaviors
Balance between reassurance and
importance of follow up testing
Your child may or may not have a hearing
lossbut lets be sure about it. If further
testing shows hearing loss, the earlier we
get started helping the child, the better.
 Follow Up Testing

Referral for follow-up testing

Repeat OAE and/or ABR screening
If a hearing loss is still suspected
Referral to a pediatric audiologist
Experienced in testing infants & children
Has appropriate equipment to test infants

Frequency specific ABR to estimate

degree and configuration of hearing loss
Early testing can avoid need for sedation
Importance of Intervention in
Outcomes
Early Identification needs to be paired
with early, appropriate and consistent
interventions.
 JCIH 2007 Follow Up
Guidelines 2

EHDI systems should be family-

centered
Families should have:
Access to information on all treatment
options
Counseling regarding hearing loss
Child and family should have:
Immediate access to hearing technologies
Amplification

Hearing aids can be fitted

as young as 1 month of
age
Roles of the Medical Home

Understand testing results at

screening and diagnostic phases &
implications for follow up
Assure follow-up screening; refer for
diagnostic and medical specialty
evaluations (genetics, ophthalmology,
etc.)
Support family in understanding
severity & type of hearing loss
Refer to early intervention
Offer partnership with parents to
identify and develop a plan of health
and habilitative care
 Medical Workup
Complete prenatal & perinatal history
Family Hx of onset of HL < age 30
Physical for stigmata, ear tabs, cleft
palate, cardiac, sketetal, microcephaly
Refer to ENT consider CT of temporal
bones
Refer to Genetics and Ophthalmology
Other: CMV, EKG, Developmental
evaluation
Goals of Early Intervention
Home based services
Optimally, providers have experience & training with the
population and work to:
Establish partnerships with families
Promote family competence & confidence in parenting
child
Support family in providing a language-rich environment in
everyday routines
Support family to become informed decision makers for
the child
Conduct ongoing assessments of outcomes
Adjust interventions as necessary to optimize outcomes
Promote family access to formal and informal supports
Provide culturally competent services
Resources

Early Intervention Contact State EHDI

Coordinator see
www.infanthearing.org
www.nectac.org

Parent-to-Parent www.handsandvoices.org
www.beginningssvsc.com
www.babyhearing.org

Physician support www.aap.org

www.medicalhomeinfo.org
Physician Resources

http://www.medicalhomeinfo.org/screening/hearing.html
ALSO: hearing loss module on
http://www.pedialink.org

http://www.cdc.gov/ncbddd/ehdi/
Chapter Champion Contact

For more information

Chapter Champion (Alan Grimes, MD)
agrimes@pol.net
State EHDI coordinator (Catherine
Harbison)
catherine.harbison@dhss.mo.gov
 Contributors
Mary Pat Moeller, Ph.D., BTNRH Roger Harpster, BTNRH
Pat Stelmachowicz, Ph.D., BTNRH Diane Schmidt, BTNRH
Don Uzendoski, M.D., AAP Chapter Skip Kennedy, BTNRH
Champion, BTNRH Karl White, Ph.D., NCHAM
Leisha Eiten, AUD, BTNRH Michelle Esquivel, MPH, AAP
Staci Gray, PA, BTNRH
Susan Wiley, M.D., AAP EHDI Task Force
Member; Cincinnati Childrens Hospital

Project Supported by the National Institute on Deafness

and Other Communication Disorders (NIDCD/NIH) R25
DC04559; R25 DC006460
For additional information about this presentation or
Universal Newborn
Hearing Screening
contact:

Boys Town National Research Hospital

555 No. 30th St.
Omaha, NE 68131

Dr. Mary Pat Moeller

402/452-5068
E-mail: moeller@boystown.org