Neonatal Hearing

Screening
Ron Christian Neil T. Rodriguez, MD
Second Year Resident
Mary Johnston Hospital
Objectives
• To discuss briefly hearing loss in general
• To discuss the indicators for hearing assessment in newborns
• To discuss neonatal hearing screening, the screening methods, and
the law which govern its use
Hearing Loss
• Normal hearing range: 0 to 20
dB
• Can occur at any age
• Based on severity:
• Mild (20-30 dB hearing level)
• Moderate (30-50 dB hearing level)
• Moderately severe (50-70 dB
hearing level)
• Severe (75-85 dB hearing level)
• Profound (> 85 dB hearing level)
Hearing Loss
• Can be of two types: Peripheral or Central
• Peripheral  can be Conductive or Sensorineural or Mixed
• Conductive  most common type in children; caused by dysfuction in sound
transmission to the external or middle ear
• Sensorineural  caused by damage or maldevelopment of structures in the
inner ear
• Central/Retrocochlear  auditory deficit originating along the central
auditory nervous system pathways from the 8th cranial nerve to the
cerebral cortex (rare in children)
Causes of Hearing Loss
• Most conductive hearing loss (CHL) is acquired, with accumulation of
fluid in the middle ear as the main cause
• Congenital CHL is often due to anomalies in the pinna or external ear,
tympanic membrane, and ossicles. Choleastoma or masses in the ear can also
present as congenital CHL
• Sensorineural hearing loss (SNHL) is both acquired or congenital.
Acquired can be genetic, infectious, anatomic, traumatic, ototoxic, or
idiopathic in nature.
• Among infectious causes, the most common cause of congenital SNHL is
cytomegalovirus, whereas rubella is now uncommon due to improvement in
vaccination efforts
Hearing Loss
• Impact of hearing loss is greatest on infants who are yet to develop
language
• Infants with a prenatal or perinatal history that puts them at risk or
those who have failed a formal hearing screening should be evaluated
by an experienced clinical audiologist until a reliable assessment of
auditory sensitivity has been obtained
Hearing Loss
• According to the Joint Committee on Infant Hearing, the following are
risk indicators for neonates (from birth to 28 days):
• Illness or condition which would require admission of 48 hours or greater at
the NICU
• Stigmata or other findings associated with a syndrome known to include SNHL
or CHL
• Strong family history
• Ear and craniofacial anatomical deformities
• In utero infections
Hearing Loss
• According to the Joint Committee on Infant Hearing (JCIH), the following are risk
indicators for infants (from 28 days to 2 years):
• Developmental delay
• Strong family history
• Stigmata or other findings in syndromes known to include SNHL or CHL
• Postnatal infections such as bacterial meningitis
• In utero infections
• Syndromes associated with progressive hearing loss (ex. Osteoporosis, neurofibromatosis)
• Neurodegenerative disorders (ex. Hunter syndrome) or sensorimotor neuropathies (ex.
Charcot-Marie-Tooth syndrome)
• Head trauma
• Recurrent or persistent otitis media with effusion for at least 3 months
Hearing Loss
Hearing Screening
• According to the Philippine Pediatric Society (PPS), detection of
hearing impairment at 3 months and intervention/rehabilitation at 6
months of life has been shown to prevent or reduce many
consequences of hearing deficits
• If undetected, hearing loss may:
• Impair language development
• Decrease academic performance
• Cause personal and social maladjustments
• Emotional difficulties
Hearing Screening
• According to the American Academy of Pediatrics (AAP), there are five
essential elements to an effective universal hearing screening:
• Screening
• Tracking and follow up
• Identification
• Intervention
• Evaluation
Hearing Screening
• Recommended for children who are suspected to have hearing loss
• Recommended techniques include otoacoustic emissions (OAEs) and
auditory brainstem evoked responses (ABRs)
• OAEs are used in most newborn hearing screening programs  quick, easy to
administer and inexpensive
• ABRs are also used in newborn hearing screening but are more expensive
than OAEs. It is reserved often for neonates who fail the OAEs as it can further
determine the severity and cause of the hearing loss
• A failed OAE is defined as having no response in sounds with 30-40 dB,
regardless of cause of hearing loss
Hearing Screening
• According to the AAP and the JCIH, hearing screening is most
recommended to be done no later than 1 month of age
• The basis for this recommendation is to maximize social, emotional, and
linguistic outcomes for children who are deaf or hard of hearing
• JCIH recognizes OAE and ABR as the screening tools of choice in determining
potential hearing loss in infants
• The Early Hearing Detection and Intervention (EHDI) is established by the
CDC with a “1-3-6 goal”:
• Screen no later than 1 month of age
• Diagnose no later than 3 months
• Enroll in early intervention programs no later than 6 months
Hearing Screening in the Philippines
• Hearing loss is the 3rd leading cause of disability, affecting 13% of
population
Hearing Screening in the Philippines
• R.A. 9709 (An Act Establishing a Universal Newborn Hearing
Screening Program for the Prevention, Early Diagnosis, and
Intervention of Hearing Loss)
• All newborns of the Philippines, regardless if high risk or non-high
risk, with consent of the parent/s or guardian/s, must undergo
hearing screening. A consent form is not necessary; however, if the
family wishes not to do the screening, a refusal form must be
obtained
R.A. 9709
• Timing of screening
• On or after 24 hours after birth before the infant is discharged from the
hospital
• If not hospital born, must be screened not more than 3 months of age
• For purposes of PhilHealth reimbursement, babies must be screened within 2
months of age
• In the Philippines, both OAEs and AABRs are accepted means in
hearing screening.
• In the event that both are unavailable, screening via the Reflexive Behavioral
Test or “Baah” test is done
Hearing Screening
• Otoacoustic Emission (OAE)
• A miniature earphone and microphone
are placed in the ear. Sounds are played
and a response is measured. If the ear
reacts, a response can be measured in
the ear canal by the microphone. When
a baby has a hearing loss, no response
can be measured on the OAE test.
• Because the OAE response is generated
by the outer hair cells in the cochlea
before it reaches the eighth nerve, it is
often referred to as a “pre-neural”
response.
Hearing Screening
• Auditory Brainstem Response
(ABR)
• Sounds are played to the baby's
ears after electrodes are placed on
the baby's head to detect
responses. This screening
measures how the hearing nerve
responds to sounds and can
identify babies who have a hearing
loss.
Alternative Hearing Screening Tests
• Reflexive Behavioral Test/ “Baah”
test
• Used in health centers with trained
individuals but no available health
professionals nor OAE or ARB
• Sound is played 1-2 feet from the
child’s ear and a response is noted to
have been evoked or not. If with
response, the test passes. If no
response, the test fails and patient
must be referred to a secondary center
• A Level 1 questionnaire is provided to
the testers
Alternative Hearing Screening Tests
• For centers with no OAE or ARB
but has health professionals:
• “Baah” test can also be used
• Infant Milestone Related to
Hearing Checklist
• Level 2 questionnaire is answered
Stop Criteria for Non-High Risk Neonates
who Underwent OAE
• Assuming that screening conditions are adequate (quiet baby, quiet room,
acceptable probe fit):
• OAE screening in the well-baby, roomed-in with mother
• Two (2) screening sessions of no more than three (3) screens per ear are
recommended, for a total of a maximum six (6) screens per ear. The screening sessions
should be conducted several hours apart.
• If result of the first test of the first session is “PASS” then the patient is declared “PASS”
for that ear. There is no need for a second session.
• If the result of the first test of the first session is “REFER” then 2 more tests can be
done for that session. If the results of the three tests are “REFER” then a second
session is conducted at least 2 hours later.
• A baby who had a “REFER” result on OAE should not be rescreened with AABR but
rather should proceed to a diagnostic ABR and/or -ASSR
Stop Criteria for High Risk Neonates who
Underwent OAE
• Assuming that the screen conditions are adequate (quiet baby with
minimal movement, quiet room, acceptable electrode impedance and
headphone placement):
• Baby should be screened close to the time of discharge.
• If the baby is less than five (< 5) days old, follow the well-baby protocol.
• If the baby is at least five (≥5) days of age, recommended stop criteria are one
(1) screen per ear.
• A baby who had a “REFER” result on OAE should not be rescreened with
AABR but rather should proceed to a diagnostic ABR and/or auditory steady
state response (ASSR)
Stop Criteria
Follow Up Screening for “Refer” Patients
• Follow-up testing must be done within one to three (1–3) months.
Rescreening of infants should include re-evaluation of both ears
even if only one (1) ear failed at initial screen.
• For OAE outpatient rescreening, 3 screening per ear is recommended
• For AABR outpatient rescreening, 1 screen per ear is recommended.
• Formal auditory brainstem response, ASSR, and/or behavioral
audiometric tests (if available) are recommended for infants who do
not pass 2nd screening within three (3) months.
Follow Up Screening for “Refer” Patients
• All infants with identified hearing loss should be referred by the
primary health care professional to a board-certified ENT specialist
within six (6) months after detection of hearing impairment for
further management by a multidisciplinary team. The same primary
health care professional should refer to other specialists and other
professionals for continuing care.
• Intervention in the form of hearing aid fitting, hearing and behavioral
rehabilitation must be recommended within 6 -12 months after
consult with an ENT specialist.
Summary
• Hearing loss occurs in any age group. Hearing loss can be central and peripheral, with
peripheral being either conductive or sensorineural
• Causes of hearing loss can be congenital or acquired, with majority being acquired
• Impact of hearing loss is more seen in infants who are developing language. Red flag signs
depending on age group will warrant testing
• In the Philippines, we have the R.A. 9079, which established the Universal Newborn Hearing
Screening Program
• OAE and ARBs are the most common types of testing used, with alternatives practiced in the
event either are unavailable
• Patients can pass or fail a test. If a patient fails, he/she is referred to another institution for
further testing after a set amount of months. If the patient still fails, he/she must be seen by
a multi-disciplinary team, and evaluated for use of hearing aids and behavioral evaluation.
Summary
• Screening and follow up (if patient fails the initial screening test) is
important
• As per the EHDI, we have the 1-3-6 goal (Screen no later than 1 month of age.
Diagnose no later than 3 months. Enroll in early intervention programs no
later than 6 months)
• As per the PPS, we should screen no later than 3 months of age, and do
intervention no later than 6 months of age
Integration in Our Community
• Many of our patients were born either at lying in clinics or even at
home, most especially due to the pandemic
• We can increase awareness of this by promoting the importance of
hearing screening (and the detriments of not doing so) via social
media and by promoting these to expectant mothers who go for
prenatal check up at the OPD or community clinics
• We can also inform them the availability of the hearing screening
program at our hospital or at other nearby facilities capable of doing
the screening if they either live farther than our hospital or cannot
afford our hearing screening
References
• Nelsons Textbook of Pediatrics (21st Edition)
• Universal Newborn Hearing Screening and Intervention Act of 2009
Manual of Operations
• PPS Policy Statement on Neonatal Hearing Screening, Series 2004,
Vol. 1, Number 5
• Resource Guide for Early Hearing Detection and Intervention (2021),
Chapter 2
THANK YOU!