Dr. MAHENDRA B.

BHAGAT
MB, DNB (PED), PGD-HM, DIM
PEDIATRICIAN
SURAT
 Blindness separates
us from things
but
deafness separates us
from people.

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 It is a continual source of information
about things and happenings within our
immediate physical environment.
 It provides warnings signals that are
important to physical safety.
 It gives help to individual in acquiring and
maintaining physical skills
 It forms a link with the rest of world and
instinctively becomes an emotional link
that contributes to mental health & social
ease.
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 > 280 Million – moderate to profound bilateral
hearing loss (WHO)
 Increased incidence due to increased population
and longer life expectancies.
 80% of deaf & hearing impaired are from low- and
middle-income countries.
 Leading cause of mild to moderate hearing
impairment in children – chronic middle ear
infection.
 50% of deafness/hearing impairment is avoidable
through prevention, early diagnosis and
management

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 Current annual production of hearing aids is
estimated to meet less than 10% of global need.
 Hearing impairment impacts development in
 Speech
 Language
 Education
 Social integration
 Severity of impact related to
 level and type of hearing impairment,
 age of onset, especially if it begins before the age of
speech development.
 Presbycusis is a common problem among the
older individuals.

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 refers to complete or partial loss of the ability
to hear from one or both ears.
 Deaf – the person is not able to hear even
with a hearing aid.
 Hard of hearing – significant hearing loss
that makes special adaptations necessary.

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 Deafness refers to the complete loss of
ability to hear from one or both ears.
 Educational definition
Deafness means a hearing impairment
that is so severe that the child is
impaired in processing linguistic
information through hearing, with or
without amplification/hearing aid, that
adversely affects a child’s educational
performance.

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•Implies hearing is defective, but functional
•Educational definition – Those persons
whose hearing makes it difficult, but not
impossible, for them to understand speech
and language with or without the use of
hearing aids.

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 95 of every 1,000 people have a chronic hearing
loss (1/13-hearing loss, 1/115-deafness in USA)

 The large majority of persons with hearing loss

are adults

 83 out of 1000 children have an educationally

significant hearing loss (USA)

 About 25% of students who are deaf or hard of

hearing have another disabling condition

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 Normal: -10 to 15 dB
 Borderline (Minimal): 15-25 dB HL
 Mild: Would hear soft music from a stereo, but may
not hear whispered conversation (20-40 dB)
 Moderate: Would hear a telephone ringing/loud
conversation, but may be unable to hear a baby
crying (40-55 dB)
 Moderate-severe :(55-70 dB)
 Severe: Would hear drums playing/sound of
vacuum cleaner, but may be unable to hear a dog
barking (70-90 dB)
 Profound: Would hear a plane, but not a telephone
ringing (> 90 dB). Separates deaf from those who
are hard of hearing.
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 Sound is transmitted as sound waves from the
environment.
 These are gathered by the outer ear and sent down
the ear canal to the eardrum.
 Sound waves cause the eardrum to vibrate which sets
the three tiny bones in the middle ear into motion.
 The motion of these bones cause fluid in the inner ear
or cochlea to move. Movement of the fluid in the inner
ear causes the hair cells in the cochlea to move.
 The hair cells transform this movement into electrical
impulses.
 These electrical impulses are transmitted to the
auditory nerve and to the brain where they are
interpreted as sound.
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 Characterized by Ear(s) affected

Unilateral (in one ear only)

Bilateral

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 CENTRAL HL
 CAPD –central auditory processing disorders
 NEURAL
 PERIPHERAL HL
 Conductive - caused by problems with
transmission of sounds
 Sensorineural – caused by auditory nerve
damage
 Mixed – both conductive and Sensorineural
hearing loss.

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 problem - outer or middle ear
 childhood middle ear infection
 TM perforation – trauma, OM
 presence of fluid in the middle ear
 accumulation of ear wax in the external ear
canal/foreign bodies/tumors
 Abnormalities in pinna or bones in ear drum
 Others – OI, osteopetrosis, cholesteatoma
 RESULTS –
 In a reduction in sound level
 Inability to hear faint sounds, but loud sound
may be heard properly.
 Can often be corrected through medicine or
surgery
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Ear canal atresia – conductive HL
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 Problem: inner ear/outer &/or inner hair cells of
cochlea +- 8th nerve
going to the brain
 usually permanent
 requires rehabilitation, with limited success
 Common causes
 aging
 excessive noise
 smoking
 genetic disorders
 infectious disease-measles, mumps, rubella,
TORCHS in mother, meningitis,
 Others – anatomic, traumatic, autoimmune,
ototoxic and idiopathic causes.
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 Results in
 a reduction in sound level
 less ability to hear faint sounds
 Multiple audiometric configurations based
on degree and configuration of HL
 Any degree of HL
 affects speech understanding or ability to
hear clearly

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 As a result of exposure to
Abrupt dangerous levels of sound
or
Loud sound over extended periods of
time

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 Reaching
hazardous levels of
sound
 Extended exposure
to sounds > 85
decibels can cause
hearing loss
 iPods can produce
sounds > 150
decibels!!!

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 Conductive and sensory hearing loss
 Middle and inner ear affected simultaneously.
 Causes
 otosclerosis involving the ossicles and the
cochlea
 transverse and longitudinal temporal bone
fracture
 head trauma
 chronic otitis media +/- effusion
 cholesteatoma and middle ear tumors
 some inner ear malformations

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 Outer ear, middle ear and Cochlea intact
 Deficit in neural transmission (auditory
neuropathy)
 HL – profound and permanent

CENTRAL HL
 Conductive, sensory and neural pathways intact
 Processing deficit at higher levels of CNS
 Hearing loss profound and permanent
 Auditory brainstem implant (ABI) is only possible
help
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 CAUSES
 Developmental and hereditory
 Syndromic – Alport, Usher syndrome
 Non-syndromic – Large vestibular aqueduct
syndrome
 Infectious – otitis media, viral, syphilis
 Drug toxicity – amino-glycosides, loop diuretics

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 Trauma – Head injury, noise-induced,
barotraumas
 Neurologic disorders – Multiple sclerosis
 Vascular & hematologic disorders –
Migraine, sickle cell disease
 Immune Disorders – Polyarteritis nodosa,
HIV
 Bone disorders – Paget’s disease
 Neoplasms – Vestibular schwannoma
 Unknown etiology – Presbycusis, Meniere
Disease
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 Congenital: congenitally deaf; born with
impairment.
 Acquired or adventitious: born normal; suffer
diminished hearing later.
 Presbycusis
 Pre-lingual deafness-HL before development of
speech & language
 Post-lingual deafness – HL after development of
speech & language
 Evidence suggest that normal language
patterns are maintained if deafness occurs after
age 5
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 50% genetic
 75% non-syndromal
 25% syndromal
 75% autosomal recessive (AR) – Usher
syndrome (retinitis pigmentosa, blindness,
SNHL), Pendred syndrome (SNHL, goiter)
 25% autosomal Dominat (AD) – Tracher Collins
(atretic middle and external ears, Mixed HL, cleft
palate, mandibular hypoplasia), Apert, Crouzon
(atretic eustachian tube, cranial bone
abnormalities), neurofibromatosis (acoustic
neuroma)
 50% acquired
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 Prenatal: infections-TORCHS, teratogens-
Radiation, aminoglycosides
 Perinatal: NICU admission-hypoxia,
kernicterus, PFC
 Postnatal: infections, Neoplasms,
ototoxins, trauma (acoustic, blunt,
penetrating), auto-immune (rare in
children)

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 Prelingually deaf individual
 born with insufficient hearing to acquire speech
normally, or
 Lost hearing prior to the age at which speech is
acquired.
 Impact
 Delayed social development
 Delayed language acquisition.
 Inability to pick up auditory social cues.
 Often results in irritability

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 Occurs after the age at which spoken language
normally is acquired (about age 3).
 Effects on speaking, reading, writing, and
speech
 Depends on the individual and the duration of
the hearing loss

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 Is the onset of deafness in older life.
 Hearing loss with aging
 genetic (deafness often runs in families)
 adventitious (from insult to the hearing
system by environmental sound).

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 Sounds often seem less clear and lower in volume.
 Difficulty hearing and understanding speech.
 The speech of others seems mumbled or slurred.
 High-pitched sounds such as "s" and "th" are
difficult to hear and tell apart.
 Conversations are difficult to understand, especially
when there is background noise.
 A man's voice is easier to hear than the higher
pitches of a woman's voice.
 certain sounds seem annoying or overly loud.
 Tinnitus (a ringing, roaring, or hissing sound in one
or both ears) may also occur.

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 Auditory centers of the brain are affected by
 injury, disease, tumor, heredity or unknown
causes.
 CAPD does not necessarily involve (although
it may) hearing loss.
 CAPD involves fine components of sound
 E.g. localization and lateralization, auditory
discrimination, auditory pattern recognition,

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 Deafness can be inherited: if one or both parents
or a relative is born deaf, there is a higher risk that
a child will be born deaf.
 Hearing impairment may also be caused before or
during birth for several reasons. These include:
 Premature birth
 Birth Asphyxia
 Rubella, syphilis or certain other infections in a woman
during pregnancy
 The use of ototoxic drugs - a group of more than 130
drugs (e.g. gentamicin) that can cause damage to the
inner ear incorrectly given, during pregnancy
 Jaundice, which can damage the hearing nerve in a
newborn baby

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 Infectious diseases such as meningitis, measles,
mumps and chronic ear infections –usually
childhood, but also later in life.
 The use of ototoxic drugs at any age, including
some antibiotic and anti-malarial drugs, can cause
damage to the cochlea.
 Head injury or injury to the ear
 Impact – difficulty in hearing in noisy
environment/to combine information from two ears
properly, slow integration of auditory stimuli 
poor attention span, academic or behavioral
problems
 Strategies to cope for older children available,
hence early diagnosis of CAPD must.
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 Neonatal Screening – to detect and treat HL
before 6 months of age, so as to develop
normal speech & language. Compulsory in
USA.
 Two screening test – used singly or in
combination
 Auditory Brainstem response (ABR) – Also
known as
 Screening Auditory Brainstem Response (SABR)
 Automated Auditory Brainstem Response (AABR)
 Brainstem Evoked Response Audiometry (BERA)
 Otoacoustic emissions (OAE)

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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

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 ADVANTAGES  DISADVANTAGES
 Identifies cochlear,  Test time
conductive and neural  Disposable costs
losses: ? mild-mod.  Infant state/myogenic
 Some test both ears artifact
simultaneously.  Requires electrode
 Relatively insensitive prep, placement &
to transient ear canal, removal
middle ear & external  Click can miss
noise. unusual
 ? Lower fail rate than configurations of HL
OAE

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Examples of screening
ABR technology

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 Test measures sound waves produced in the inner
ears. A tiny probe is placed just inside the infant’s
ear canal. It measures the response (echo) when
clicks or tone bursts are played into infant’s ears.
 Recording of an OAE
 Indicates healthy outer/inner ear & cochlea
 Presence highly correlated with normal hearing
sensitivity or no greater than a mild hearing loss
 Absent OAE
 SNHL > 30 dB
 Conductive HL associated with middle or outer ear
abnormality
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 ADVANTAGES  DISADVANTAGES
 Quick  Sensitive to ear canal
 Inexpensive and middle ear
 Frequency-specific conditions
 Sensitive to noise
 Identifies cochlear
(internal & external)
and conductive
 Cannot identify
losses: mild-mod?
neural disorders
including auditory
neuropathy
 High fail rates in some
programs.

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 Device Options for OAE Screening
Types
• Handheld
• Portable screening devices
• PC-based hybrids
• PC-based clinical systems

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 ADVANTAGES  DISADVANTAGE
 Low failure rate S
 Depending on test  Time
order, identifies  Equipment and
cochlear, neural and
disposable costs
conductive losses
 Reduced effects of
noise
 screening devices
available for both
technologies
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 Neonatal intensive care
 Birth wt <2,500?g: All cases
 Birth wt >2,500?g: If medical complications
(asphyxia, seizures, persistent fetal circulation,
intracranial hemorrhage, assisted ventilation > 5
days, hyperbilirubinemia, ototoxic drugs)
 Proven or suspected intrauterine infection
 Bacterial meningitis
 Craniofacial anomalies (microtia, auricular
dysplasia, micrognathia)
 Anomalies of neural crest/ectoderm (widely
spaced eyes; pigmentary defects)/stigmata of
syndromes associated with HL
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 Family history of hereditary or unexplained
deafness
 Recurrent or persistent otitis media with effusion
for > 3 months
 Parental concern about hearing loss, speech or
developmental delay – good predictor
 Delayed speech or language development
 Head trauma associated with loss of
consciousness or skull fractures.
 Other developmental disabilities (mental
retardation, cerebral palsy, autism, blindness)
 H/O prolonged use of ototoxic drugs
 Presence of neuro-degenerative disorders
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 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

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 Delayed identification, even of mild HL results in
 language delays
 developmental skill delays, and
 behavior problems.
 Subsequent delays in literacy, and academic
performance
 Children identified and receiving services < 6 m
have larger vocabularies, better comprehension
and better expressive language than children
identified > 6 m.
 To minimize parental stress and to decrease the
feelings of guilt and frustration, if identified late
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 Reading Comprehension Scores of
Hearing and Deaf Students
10.0 Deaf
9.0 Hearing
Grade Equivalents

8.0
7.0
6.0
5.0
4.0
3.0
2.0
1.0
8 9 10 11 12 13 14 15 16 17 18
Age in Years

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 Audiometry
 Auditory Evoked Brainstem Potential – in
young, neurologically immature or impaired,
behaviorally difficult or severely cognitively
impaired child (MR)
 Tympanometry – assessment of middle ear
function & pathology of its fluid, ossicles and
Eustachian tube.
 Imaging studies, including CT/MRI –R/O
anatomic abnormalities and the extent of
inflammatory processes or Neoplasms.
 Individualized tests – TORCH titers in newborn in
NICU with IUGR
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Audiogram showing bilateral
conductive hearing loss

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 Language development:
 Deaf children may not hear all of the
individual sounds in a word. It is common
for them to leave out word endings like ‘s’ or
‘ed’
 They may have a limited vocabulary because
they cannot hear conversations going on
around them
 They may struggle when one word has
several meanings e.g.. Catch a cold, catch a
ball, the catch on the gate

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 A deaf child may become frustrated if they cannot
communicate effectively or if they do not
understand what is happening around them. This
may affect their opportunity to:
 Learn appropriate social behaviour incidentally
 Communicate effectively with others
 Initiate conversations
 Use appropriate language in differing social
situations
 Be fully included in large groups
 Express their feelings and emotions
 Develop confidence and self esteem

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 Levels of functioning influenced by:
 Degree of hearing loss
 Attitudes of parents and siblings
 Opportunities to acquire a first language
 The presence of other disabilities

 Academic achievement
 Most children with hearing loss have difficulty with all
areas of academic achievement
 Deafness itself imposes no limitations on the cognitive
capabilities of the individual
 Social functioning
 The extent to which a child successfully interacts
depends largely on others’ attitudes and the child’s
ability to communicate in some mutually accepted way
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 Deafness isolates a student from the
world of the majority language as
well as from the world of sound
 To the hearing student, the world is
his classroom
 To a deaf student, education stops
when he steps out of the classroom
(of a special or normal school)

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 Biologically based MI appears to be at the same
rate as hearing people
 Emotional and adjustment disorders several times
higher- in almost 50%
 Language deprivation, cognitive disability
 Neglect at home or in school or community
 Non-acceptance of deafness
 Lack of incidental learning/exposure
 Poor self-control, feeling of shame/guilt
 Lack of social skills, difficulty trusting people, poor inter-
personal interactions
 Abuse – emotional/psychological & physical/sexual
 Failure to become independent from family
 25% higher rate of substance abuse
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 Multidisciplinary approach
Hospital Audiology
Parent Groups
Screening
Mental Health

Primary
3rd Party ENT
Provider
Payors Child/Family

Deaf
Community Educational
Interpreters Deaf intervention
Genetics
Services Therapists
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 Children with hearing loss can develop
verbal speech and language similar to
hearing peers if:
1. The loss is identified early, preferably
before 6 months of age
2. The child has appropriate technology
and uses it consistently
3. The child has appropriate intervention
4. Family is committed to intervention
and language stimulation

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 Wear behind your ear
 Makes sound louder, but not
necessarily clear
 Offers minimal benefits in
noisy environment
 The earlier in a life a child is
fitted, the effectively he will
learn to use it.
 Not effective if inner ear is
also damaged.
 This is why genetic testing is often
very useful when deciding what
treatment is best for an individual

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 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.
 WHEN to fit?
 For newborns identified by UNHS (Universal Neonatal
Hearing Screening), fit hearing aids within one month
of confirmation of hearing loss, preferably before 4
months and no later than 6 months of age.
 Indicated - For infants with significant, permanent,
bilateral peripheral HL, occ. Used in unilateral HL,
abnormal audiogram, family choice, presence of other
disabilities, to improve school performance, etc.

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 The ability to localize from where a sound is coming. This
is very important from a safety perspective.
 A better understanding in noisy environments as
selective listening is achieved more easily.
 Better sound quality as you are hearing in both ears.
 Keeping both ears active. When hearing nerve in one ear
is not used for an extended period of time it may lose its
ability to transmit information
 An increased ability to distinguish between sounds.
 A less tiring and more enjoyable listening experience.
 A sense of balance. When people hear from just one side,
they feel that they have a dead side ”’ which can be a
strange sensation.

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 For children with
bilateral
conductive
hearing loss who
are unable to wear
or benefit from
BTE hearing aids
 Children with
unilateral deafness

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 Processor is placed on the skull bone
 Sound vibrations are passed to the inner ear
structures through the bone.
 Infants & children can wear processor
attached to the softband.

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 Used with hearing aids or cochlear
implant
 Teacher/parent wears wireless
microphone, and speech is heard
consistently even when parent/teacher
moves in room
 Can also be used with other technology
–computer, DVD, IPOD
 Useful when it is difficult to hear even
with amplification over noise &
distance.
 Child able to monitor his/her own voice
or can hear others not using FM
microphone
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 Vibro-tactile devices –
 Communicate sound through physical contact with skin
 Soundbeam/Tact-Aid - makes a vibroacoustic
(vibrotactile) device for experiencing music for the deaf
 Assistive listening devices – a radio link established
between the speaker/teacher & the child can solve
problems caused by distance and noise.
 Speech-to-text translation – using computers
 Text-telephones/fax/emails/instant messages to
communicate between a deaf and a normal or
between 2 deafs
 Use of interpreters – better performance in school
 Use of sign language & lip Reading

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 What is it like to “hear” a
hand?
You have to be deaf to
understand.

– A poem by Willard Madsen

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 For children with severe to profound sensori-
neural hearing loss, 12 months or older.
 When hearing aids do not provide enough power to
support verbal speech and language development.
 When parents desire that their child to
communicate with verbal language.
 Bilateral CI’s are becoming more common.
 Realistic understanding of the risks, benefits and
limitations of implantation.
 A home & educational environment where
oral expression is encouraged/supported.

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 Surgically implanted hearing devices
 For those who have profound hearing loss
 Compensates for the damaged or nonworking
parts of the inner ear
 Bypasses damaged portion of inner ear/hair cells
of cochlea and goes directly towards the auditory
nerve
 Electrodes are placed directly in the cochlea
 Don’t restore normal hearing
 Individual will not hear/understand speech like a
normal hearing person
 But provides “useful” hearing & improved
communication abilities
 Also safe, reliable & provides for effective rehab.
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 4-6 weeks of healing before sound and speech
processor can be used
 Implanting earlier allows for optimal cognitive
development
 Auditory nerve must be intact
 High success rate for language skills when
implanted in children of ages 2-5
 Must be implanted in the good ear
 Better than the “untrained ear”
 Requires post-implantation therapy
 Process is expensive
 Takes time to interpret sound created by
implants
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 Depends on
 Amount of surviving auditory nerve fibers
 Environment where they live
 Motivation
 Multi-disciplinary family-oriented approach
 Regular follow-ups for changes in CI mapping
 If they developed spoken language before going
deaf
 Harder for people who have been deaf since birth
 Have to associate between words and sounds
 Complications – higher incidence of meningitis
in implanted patients (pneumococcal & other
vaccines must)
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Training is Everything

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 Only speak to the patient when he/she can
see your face.
 Always be at eye level.
 Turn off all extraneous sounds e.g. music,
HVAC, etc.
 No backlighting or shadows on your face
 Light face so that facial expressions, lips can
be read.
 Speak to patient before putting on mask
and beginning treatment.

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 Modulate voice - speak slightly louder than
normal, but don't shout.
 Speak at your normal rate - do not exaggerate
sounds.
 Clue the person with the hearing loss about the
topic of the conversation if possible.
 When not understood - rephrase your statement
into shorter simpler sentences
 In restaurants and social gatherings, choose
seats or conversation areas away from crowded
or noisy areas.
 Don’t talk with food or non-food items in mouth.
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 Create opportunities for deaf children to mix with
their peers & others at home, school or in
community
 Promote good communication skills
 Make sure you have the deaf child’s attention
before starting to speak
 Don’t shout! This distorts your lip pattern and
may be painful for hearing aid users
 Don’t talk for ages as it can be tiring for the deaf
child to lip read for long periods
 Use gesture and facial expression to aid
understanding, also use visual clues/aids to
understand better.
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 All families with a child with congenital
or late onset hearing loss
 Families of a child with stigmata or a
syndrome will benefit from the
information.
 Families with a child with non-
syndromic HL want to know the cause ?

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 It is important to respond to concerns
about language immediately !
 Most children with delays of speech and
language respond to appropriate medical,
audiologic, and educational interventions.
 A successful early screening,
identification, and intervention program
will ultimately permit every child with HL to
develop to his/her potential.

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