The Hearing Impaired Child

To be blind is to be isolated from the world; to be deaf is to be isolated from other people

Dr Mukundan Subramanian
 Introduction
Screening for hearing loss
Causes of childhood deafness
Clinical evaluation of a child with suspected
deafness
Investigations
Audiology
Radiology
Genetic testing
Special tests
Maximizing the potential of the deaf child
Educational support
Conventional air-conduction hearing aids
Bone-conducting hearing devices and active middle
ear implants
Cochlear implants
Key learning points
INTRODUCTION
Hearing impairment -
immense impact on
psychosocial well-being

Implications for deaf children

particularly profound

2 out of every 100 children < 6

years have permanent bilateral
deafness > 60 dB

Unfortunately deafness
remains mostly undiagnosed
in our country

When it gets diagnosed-

usually too late
INTRODUCTION
A childs first experience of the world is the perception of
environmental noise whilst still a foetus

By 20 weeks gestation the human ear is sufficiently developed to

support hearing, and it perceives environmental sounds by 27 weeks

Once born, children are continually exposed to sound and, more

importantly, speech, which immediately begins to shape the
development of the hearing centres within the brain stem and
auditory cortex

Every time a sound stimulates the auditory centres in a very young

child- new connections develop in the brain- Plasticity

By their first birthday, children with normal hearing are already

accomplished linguists able to recognize common words and discern
subtle differences in voice tone

By their fourth birthday, hearing children will have developed a

sophisticated vocabulary of several thousand words and be able use
this to interact effectively with their peers and adults.
INTRODUCTION
Without external stimulation
the development of central
auditory pathways and speech
centres is severely curtailed, and
language, social and academic
milestones will fail to be met

Critical language learning age

birth to 3
A deaf child cannot learn
language- Language can only be
learnt through hearing

As child does not learn

language- child cannot speak-
child becomes MUTE

A child may be born deaf, but is

practically never born mute
 For many children around the
world, a hearing impairment
leads to a life of exclusion, of
dependence and of wasted
potential

Heavy burden on family & society

Almost always treating clinicians

callousness that delays diagnosis
of deafness and makes a deaf
child develop mutism

Irresponsible advices-
WAIT and SEE
Hearing will improve with age

Misleading statements-
deplorable / unpardonable
offence
SCREENING FOR HEARING LOSS
Remedy- Early Identification + Earliest possible
institution of remedial measures

NEONATAL HEARING SCREENING- Perform

hearing test as soon as child is born

Universal Neonatal Hearing Screening

All live births screened within 48 hrs of birth

High Risk Neonatal Hearing Screening

Babies born out of high risk pregnancies
Hearing loss not restricted only to high risk
pregnancies
50% of congenitally deaf babies born out of normal
pregnancies
High Risk Neonatal Hearing Screening

H/o in utero infections: STORCH Any illness requiring hospitalization >

48 hrs or more in 1st 4 weeks of birth
H/o ototoxic drug use by mother
during pregnancy Baby weight <1500 g

H/o Excessive alcohol intake by APGAR below 4 at 1 min, below 6 at 5

mother mins

H/o Prolonged/ hazardous labour Baby born out of consanguineous

marriage
Any illness which necessitates
admission of child in NICU F/H/o permanent hearing loss

Craniofacial AbN/ downs syndr/

Treacher collins synd
International Joint Committee on Infant Hearing Screening ( 4weeks-
2 yrs)

Parental concern regarding development of SLH

Delayed developmental milestones
Identification of any Syndrome- Usher
Bacterial meningitis/ encephalitis
Hyperbilirubinemia/ Pulm HT
Neurodegenerative disorders
H/o head trauma
Recurrent OME > 3 months
Ambiguous neonatal hearing screening test
result
Causes of childhood deafness
Genetics & Childhood deafness

Hearing loss is
termed syndromic
when it is
associated with
other anomalies
Several hundred
syndromes a/w
HL
Connexion 26
Neonatal Hearing Screening
Perform EOAE evoked otoacoustic emission test
within 48 hrs
If child passes the test Normal Hearing at birth (
practically)
BOA- suggested at 8,12,24, 36 months in c/o high
risk neonate
If child fails EOAE test- Retest after cleaning EAC
after 7 days
If child passes- Normal hearing
If child fails- possibility that child has hearing loss
Perform Brain Stem Evoked Response Audiometry
(BERA)
Clinical evaluation of a child with
suspected deafness
Comprehensive medical history
Details of pregnancy, birth, perinatal period and infancy with
reference to common aetiological factors

Thorough ENT clinical exam and detailed systemic exam

Investigations :AUDIOLOGY /RADIOLOGY /GENETIC TESTING

To investigate the cause of hearing loss

To assess for associated morbidity (e.g. syndromic
associations)
To assess the childs suitability for hearing rehabilitation,
including cochlear implantation if appropriate
OAE- neonates
The cochlea not only receives sound
but also produces sounds called OAEs
OAE is a sound generated from within
the inner ear
OAE test measures cochlear function
(outer hair cells)
OAEs generally not found in persons
with hearing worse than 30 dB
Hence OAEs are not usually used in
adulthood, as hearing loss tends to
occur in this age
OAEs not used to fully determine an
individuals auditory thresholds
OAEs test outer hair cell function,
so rare abnormalities of inner hair
cell or auditory nerve will be missed
Behavioral observation audiometry (BOA)
neonates/infants
Assesses hearing acuity by
presenting a sound and
observing the patients
response
The audiologist detects bodily
reactions to sounds such as
body movement, eye widening,
eye opening
The infant is observed for
changes in behavior after
presenting the acoustic
stimulus
This test can rule out
significant hearing loss.
This test cannot be used to
determine auditory thresholds
Visual Reinforcement Audiometry-
5mths to 2 years
This test makes a child respond to
sound he or she hears by connecting
sounds with a visual signal, e.g.,
stuffed animal
Every time a sound is presented to the
child, a stuffed animal follows a few
seconds later
After several times, the child realizes a
pattern and is not interested in the
sound, but only the stuffed animal
The audible sound makes the child
look for the stuffed animal
Therefore, every reaction following a
sound suggests that the previous
sound was audible
The response tends to decrease for
children between 2 and 3 years of age,
because they tend to become adjusted
to the visual signal and therefore do
not pay attention
This test can rule out significant
hearing loss.
This test can be used to determine
some auditory thresholds.
Play Audiometry 2-5 years
The child is trained to
perform a repetitive task
(putting a ring on a cone) in
response to an auditory
stimulus
BERA-Gold Standard. Newborn and at any age if
retrocochlear pathology is suspected
Checks the brains response to
sound
Correlated with hearing
sensitivity in the frequency
range from 1,000 to 4,000 Hz
Series of clicks are delivered to
the patient through earphones.
Clicks stimulate the auditory
pathway of the ear and cause a
series of electrical activity that is
detected by electrodes on the
patients scalp
Results are displayed as a
waveform on a computer
There are five different (I-V)
waveforms generated and each
wave corresponds to specific
sites within the neural auditory
pathway
ECOLI
 Infants with normal hearing
typically show a wave V
Do not assess whether a wave
V latency is normal or
prolonged
Interpret results as Wave V
present, Wave V absent or
Could not be interpreted

Automated detection
algorithms are used in
screening to give a pass/fail
equivalent to about 3035 dB
nHL (normal hearing level).
RADIOLOGY
MRIscan of the inner ear and
internal acoustic meati
firstline radiological
investigation in children with
profound congenital hearing
loss
No exposure to radiation
Scan takes 30 minutes, which
makes it more suitable for
neonates who can be scanned
whilst they sleep
High-resolution computed
tomography (CT) scan of the
temporal bones :
Superior when assessing the
bony structures of the outer,
middle and inner ear
Quicker to perform than an
MRI scan
CT provides less information
about the nerves
Involves a significant radiation
exposure for young children
Its primary role is when
contemplating cochlear
implantation in a child who
may also have anomalies of the
inner ear (e.g. Mondini
dysplasia)
Special Tests
Electrocardiogram (ECG):
Exclude conduction anomalies
Prolonged Q-T interval in children with Jervell
Lange-Nielsen

Renal ultrasound scan:

Branchio-oto-renal or Alports are suspected or if
there are multisystem anomalies

Ophthalmology: Review by a paediatric

ophthalmologist. Problems include
squint and refractive errors
syndromic associations
Ushers,Waardenburg and CHARGE
syndromes, congenital rubella and
cytomegalovirus (CMV).
 Maximizing the potential CONVENTIONAL AIR CONDUCTION
of the deaf child HEARING AIDS
EDUCATIONAL
Hearing aid amplifies
SUPPORT
sound
Create an Microphone/ Amplifier,/
environment in which Power source / Speaker
the childs ability to Evidence exists that
hear can be
maximized hearing aids should be
Communication will provided prior to 6
be easier if months of age to
background noise can maximize language
be minimized and acquisition
people made aware Binaural hearing has
that the childs numerous benefits, some
hearing is poor of which include better
Nonverbal cues take sound localization,
on added
importance- Essential improved speech
that the child can recognition in quiet and
clearly see the noise and general ease of
speaker listening
Cochlear Implants
These are sophisticated
transducers;
Convert sound energy to electric
impulses, which directly
stimulate the auditory nerve via
an electrode array inserted into
the cochlea
Originally, only a single ear was
implanted, but it is now
accepted that bilateral
simultaneous implantation has
many benefits, including
improved speech recognition in
noisy environments and better
sound localization.
Early implantation is to be
encouraged with children with
congenital hearing loss
CI surgery has been extensively
audited and is safe, although
there are risks
Thank you
Early detection of hearing loss is important
Permanent childhood hearing losses require
comprehensive assessment
Early amplification and stimulation are
important
Maintain vigilance for progressive hearing losses
Early cochlear implantation should be
considered for children with severe to profound
hearing loss