Early Hearing Detection and Intervention in

Infants and Young children

 Hearing Loss
Characterized broadly by degree, configuration and
type,
Degree: amount of hearing loss in relationship
to normal auditory function
Configuration: overall shape or pattern of the
hearing loss as displayed on the conventional
audiogram in dB HL as a function of frequency
Type: site (location) of the auditory disorder
 Degree: Categories of HL
Normal -10 to 15 dB HL
Borderline (Minimal) 16 to 25 dB HL
Mild 26 to 40 dB HL
Moderate 41 to 55 dB HL
Moderate-severe 56 to 70 dB HL
Severe 71 to 90 dB HL
Profound > 90 dB HL

New York State Department of Health

 Hearing Loss:
Characterized by Ear(s) Affected
Bilateral
Unilateral (in one ear only)
 Clinical signs of Hearing Loss
Delayed early language milestones
Unintelligible speech
Uncharacteristic voice patterns
Child turns TV volume very loud
Child only responds to loud sounds/words or
in a very quiet environment
 Myths about hearing and early
speech language delay
We dont have to worry because:
His older brothers and sisters talk for him
Boys develop speech much later than girls
Twins always have language delays
Grandma says that her Dad did not speak until
he was 3 years old.
She has great motor milestones
I know he hears because he gets upset every
time I turn on the vacuum.
 genetic and environmental
causes of congenital hearing loss
 Risk Factors for Infants- birth to 28 days

Any illness requiring admission to the NICU for

> 48 hours.
Stigmata associated with a syndrome known to
include SNHL or conductive HL
Craniofacial anomalies including the pinna and
ear canal
In utero infections including CMV, herpes,
toxoplasmosis and rubella
Family history of permanent HL
 Most Common Specific Environmental Risk Causes

CMV > 1 risk factor

Meningitis Perinatal Asphyxia
Prematurity < 1500 g Ototoxic medications
Some Infants pass their hearing screen and have
late onset hearing loss

Some of these infants have a risk

factor and some have no risk factor
known to the family or physician.
 Risk indicators from 29 d to 2 years of age

Stigmata or Syndrome associated with HL

In-utero infections
Postnatal infections
Neonatal indicators such as ECMO
Neurodegenerative disorders
Head trauma
Recurrent or persistent OM
Causes of Permanent Hearing Loss in
100 Infants

50% Environmental 50
50% Genetic
30% syndromic 30
20% >non syndromic 20
 Genetic Causes

Single gene Connexin 26

Gene + environment Mitochondrial + ototoxic
Gene + gene Gene + other gene
 The Connexin 26 Gene
It is estimated that 50-80 % of all autosomal
recessive congenital deafness may to due to
mutations in th3e Cx-26 gene on chromosome
13q11-q12
The Cx gene produces a gap junction protein
expressed between the outer hair cells and
supporting cells and is involved with auditory
transduction.
The estimated carrier frequency in the general
population is 1 in 31. (Estivill et al, 1998)
New technologies used in hearing
screening
 Otoacoustic Emissions (OAE)
Types of OAE
Spontaneous OAEs
Evoked OAEs
Transient OAE (TEOAE, TOAE, or click-OAE:
COAE)
Distortion Product (DPOAE)
 Screening Technologies - Neonates
Evoked Otoacoustic Emissions
EOAE Advantages: EOAE Disadvantages:
Quick Sensitive to ear canal and
Inexpensive middle ear conditions
Sensitive to noise (internal
Frequency-specific
& external)
Identifies cochlear and Cannot identify neural
conductive losses: mild- disorders including auditory
mod? neuropathy
Pass-refer screening High fail rates in some
devices available programs.
 Device Options for OAE Screening
Types
Handheld
Portable screening devices
PC-based hybrids
PC-based clinical systems
 Auditory Brainstem Response (ABR)
Recording (through surface
electrodes) of the micro-volt
electrical activity generated
by the cochlea and
transmitted by the auditory
nerve and brainstem
pathways in response to
brief clicks.
Clicks produce a
synchronized response from
neural fibers; a tracing of the
response is a series of waves
 Screening Technologies Neonates
Screening (Automated) Auditory Brainstem
Response
ABR Advantages: ABR Disadvantages:
Identifies cochlear, Test time
conductive and neural
losses: ? mild-mod. Disposable costs
Pass-refer screening Infant state/myogenic
devices. Some test both artifact
ears simultaneously. Requires electrode
Relatively insensitive to prep, placement &
transient ear canal, middle removal
ear & external noise. Click can miss unusual
? Lower fail rate than OAE configurations of HL
Examples of screening
ABR technology
A failed hearing screen may be a false positive or an
actual fail. These 2 findings need totally different
management.

Therefore, an accurate diagnosis of normal hearing,

SNHL, auditory neuropathy or conductive loss is
important as soon as possible to minimize
parental stress and to decrease the time interval
between screen fail and starting treatment.

Parents of late identified children have feelings of

guilt and frustration.
Early Identification of Hearing Loss
is Important

Delayed identification, even of mild HL

results in
language delays
developmental skill delays, and
behavior problems.
Subsequent delays in literacy, and
academic performance
 There are dramatic benefits associated with early
identification and intervention for hearing loss
before 6 months of age.

Children identified and receiving services < 6 m

have larger vocabularies, better comprehension
and better expressive language than children
identified > 6 m.
 The Medical Workup

Complete prenatal & perinatal hx

Family Hx of onset of HL < age 30
Physical for stigmata, ear tabs, cleft
palate, cardiac, skeletal, microcephaly
Refer to ENT / CT of temporal bones
Refer to Genetics and Opthalmology
 Antenatal history- maternal illness during the
pregnancy or delivery

Neonatal complications, prematurity,

jaundice, asphyxia, assisted ventilation, ECMO
 Examination for Causes
Evaluate for dysmorphic features, minor and
major stigmata and syndromes
Other anomalies visual, facial, endocrine,
cardiac, kidney, hair, and skin
Particular attention to the head and neck. HL
may be associated with abn. pinna, atresia or
stenosis of the ear canal, ear tags, and bony
growths in the ear canal.
Cleft lip and palate may have middle ear fluid
What to ask about family history ?
Is there a family hx of onset of permanent HL
< 30 years of age ( over 3 generations)

Are there other family members with

syndromes or anomalies ?
 Amplification Choices for Families
including Hearing Aids, FM Systems
and Cochlear Implantation
 What is the Goal of
Hearing Aid Fitting
Ensure children receive full-time use and consistent
audibility of the speech signal at safe and comfortable
listening levels as soon as hearing loss is confirmed.

For newborns identified ,

fit hearing aids within one month of
confirmation of hearing loss, preferably before
4 months and no later than 6 months of age.
 Which infants are candidates for amplification?

Significant, permanent Some children with

bilateral peripheral unilateral hearing loss,
hearing loss. and minimal HL
Mild hearing loss in Need based on
some cases audiogram plus
additional information:
Family choice
other disabilities/on-
going medical issues
performance
 Pre-Selection -
Childrens Hearing Aids Should Include:

Binaural fitting unless clear

contraindication
Behind-the-ear style of choice
 Pre-Selection -
Childrens Hearing Aids Should Include:

Flexible response characteristics

Compression to limit overall output
sound pressure level of the hearing
aid (safety and comfort)
 Directional Microphone Technology

Improves directional hearing abilities

Hear parent speaking from front; noise from
shopping mall at back of child reduced.
 Multiple Memory Hearing Instruments

Allows storage of
more than one
electroacoustic
response setting
Allows switching
between memories
for various listening
situations
 Digital Signal Processing (DSP) Technology

Newer hearing aids that use digital

processing of incoming
demonstrate better performance of digital
instruments over conventional hearing
aids in either adults or children
Considerably more expensive
 What Other Features Should
Childrens Hearing Aids Include?

Safety-related features: tamper

resistant
battery compartment
volume control
Physical fit (size) and color
Earmolds made of soft material
 What are the Candidacy Criteria for
a Cochlear Implant?
Limited benefit from conventional amplification
following a minimum of 3-6 months use
May be sooner following deafness from meningitis
Profound hearing loss:
12-18 months
Severe-to-Profound hearing loss
>18 months
Motivated, involved family with child enrolled in an
intervention program emphasizing spoken (oral-
aural) language development
 Cochlear Implants
Acoustic signal picked up by microphone located in headset
worn at ear level
Cord carries sound from microphone to a speech processor
Speech processor digitizes sound into coded signals
Coded signals sent up to the transmitting coil
Coil sends coded signals as FM radio signals to CI under the
skin
CI delivers electrical energy to the electrode array inserted
into the cochlea
Electrodes along the array stimulate remaining nerve fibers
in cochlea
 Benefits
Similar to early amplification provision,
children implanted at early ages with more
experience tend to do better than older
children who receive implants after greater
period of deafness
Hearing Aids
 What is a Hearing aid?
A hearing aid is a device used to regulate and
amplify sound for the user
Hearing aids are used by the partially deaf but
not the completely deaf
They can greatly improve the hearing of someone
partially deaf but it cannot cure or help complete
deafness.
Placed either in and or around the ear
They have 4 major parts: the
microphone, battery, amplifier,
and receiver
 Type of hearing aids

BTE (Behind-the-Ear)
Fits behind pinna(ear)
Consists of case, earmold, &
tube
Sound routed from case to
earmold
Mini BTE
 ITE (In-the-Ear)
Mild to severe hearing
loss
Completely inside outer
ear
Not recommended for
children
Capable of added features
telecoil
 ITC(In-the-Canal)
Mild to moderate hearing
loss
Customized to recipient
Almost completely
hidden
Difficult to adjust and
change battery
 CIC(Completely-in-Canal)
Mild to moderate hearing
loss
Smaller than ITC
Difficult to adjust and add
features
More expensive
 The hearing aid today cont.
The newest hearing aids are small
and self-adjust to allow clearer
hearing. They also adjust when the
user enters loud areas allowing the
users inner-ear not to be harmed
The newest hearing aids can
wirelessly receive sounds from
electronic devices such as computers
and T.V.s
The digital hearing aid has become
the main type of hearing aid used, as
it is more versatile and produces
clearer sounds
 How it Works
The hearing aid works by the
microphone receiving the
sound and converting the
sound into electric impulses
using the battery
The hearing aid than sends the
impulses to the amplifier
which amplifies the impulses
to the desired level and send
them to the receiver.
The receiver than converts the
Impulses into amplified sound
Waves which vibrate and
stimulate the hair follicles in
the inner ear to allow hearing
 Bone Anchored Hearing Aid
Hearing aid that uses bone conduction to transmit
sound vibrations through the skull and to the cochlea.
Design includes a titanium implant, an external
abutment that connects to the implant, and a sound
processing device that connects to the abutment.
The sound processing device picks up external sounds
and transmits them through the skull via the implant.
This bypasses the external ear canal, which allows
users with conductive hearing loss to hear again.
Bone Anchored Hearing Aid
 BAHA
Mixed and Conductive Hearing Loss
 BAHA(Bone Anchored
Hearing Aid)
Bypass normal hearing
system
Helps severe
sensorineural,
condutive, or mixed
Attach to bones in
middle ear
Bypasses auditory
canal and middle ear
 BAHA
A sound processor picks up sound vibrations.
An abutment is attached to the sound processor and the implant. The
abutment transfers the sound vibrations from the processor to the implant.

Titanium implant is placed in the mastoid process, where it fuses with the
living bone (osseointegration). The implant transfers the sound vibrations
to the functioning cochlea.

This creates direct (percutaneous) bone conduction. In contrast, traditional

BC hearing aids connect indirectly to the bone through unbroken skin
(transcutaneous) and work by exerting pressure against the skull.
 BAHA
Types of hearing loss
the BAHA system can help
Mixed and Conductive hearing loss
unilateral or bilateral fitting
examples such as:
Chronic otitis media
Congenital atresia
Cholesteatoma
Middle ear dysfunction/disease
External otitis
 Thank You