11/4/2019

Deafness &

Hearing loss
DR. D. K. KISHORE
AIMST UNIVERSITY, 2017

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Report-2016
World Health Organization

Globally, 360 million people (about 5% of the

world’s population) live with disabling hearing
loss, of whom 32 million are children

The prevalence of hearing loss increases from 1.7% among

children to 7% in adults. More rapidly in adults.
More than 30% of the population is above 65 years

Report-2016
World Health Organization
Nearly 90% of those with hearing loss live in low-
and middle-income countries

More than 1000 million young persons are at increased risk

of developing hearing loss because of the unsafe use of
personal audio devices and exposure to damaging levels of
sound in noisy entertainment venues

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Bad i

mpacts of
hearing loss

Untreated hearing loss has a profound impact

on
both the individuals affected and their families
 The most obvious effect of
childhood hearing loss is on  Hearing loss thus,
communication  undermines or
impedes literacy,
 Deaf children fail to develop self-esteem and
language unless timely
interventions are put in social skills
place

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Adults with unaddressed hearing loss

have higher unemployment rates

 social isolation and  Overall,

loss of autonomy unaddressed
 associated with hearing loss
poses
 anxiety, depression, a considerable cognitive
decline and economic burden dementia.

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THE IMPORTANCE OF PREVENTION

AND INTERVENTION
Untreated ear infections are a common cause of hearing loss
among children and adults.

The use of ototoxic medicines such as aminoglycosides and

certain antimalarial medicines is another preventable cause of 5
hearing loss. Ototoxicity can be prevented through awareness
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HOW WE HEAR?
 1. Sound entering the
ear canal causes the
ear-drum to vibrate.
ions are
 2. The vibrat hrough
transmitted t
the ossicles (small
bones) which intensify
the pressure of the
sound waves and
transmit vibrations to
...

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 3. the oval
window, a
membrane over
the entrance to
the cochlea.
The simultaneous
pulsating
movements of
the round
window
stabilize
pressure within
the inner ear.

 4.The fluid (endolymph) Fluid

which fills the cochlea
transmits the waves along
the scala vestibuli and
round into the scala
tympani, making the
basilar membrane
separating them vibrate.
This membrane contains
hair-like receptor cells
organ of Corti

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Connection with CSF

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Primary auditory cortex (AI) is situated in the

posterior third of the superior temporal gyrus
(also known as Brodmann area 41), next to
Wernicke's area (W). AI is the central region of
the auditory cortex and receives direct
projections from the ascending auditory
pathway, particularly the ventral region of the
medial geniculate body (MGB) in the
thalamus.

Secondary auditory cortex (AII) is located

A - Lateral view showing the distribution of AI and AII and more rostrally in the temporal lobe and
Wernicke's area (W). Auditory cortex projects to the regions
of the frontal lobe involved in motor function for speech (a),
contains Brodmann area 42.
the lips (b), jaw (c), tongue (d), larynx (e) and Broca's area
(B).
B - Frontal view showing AI inside the Sylvian fissure and
Heschl's gyrus. 1 – Inter-hemispherical fissure. 2 - Sylvian
fissure

http://www.cochlea.eu/en/auditory-brain/thalamo-cortex/auditory-cortex-anatomy

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Cochlea
-There are about 17000 hair cells in each ear

How we hear?

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Which frequency sound we can hear ?

 Humans can hear frequencies from 20 Hz up to 20,000
Hz. As we get older, or exposed to loud sounds which
damage our ears (such as loud concerts), the upper
limit decreases
 i.e. higher frequency is affected in older people.

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What is hearing loss or impairment?

 When we can hear but below the normal physiological level.
 What is that level?

 Sound intensity, or loudness, is measured

in decibels (dB). A person with hearing within the normal
range can hear sounds ranging from 0 to 140 dB.
 A whisper is around 25 to 30 dB. Sounds that are louder
than 90 dB can be uncomfortable to hear.

 https://www.cdc.gov/ncbddd/hearingloss/sound.html Jul 24, 2015

Higher the dB/intensity of sound,

the louder we can hear.

 Suppose one cannot hear a whisper sound but can
hear normal conversation.
 Is it normal?  No
 Hearing loss
 How much?

20-25 dB loss. That means, can not hear this intensity sound. But can
hear higher intensity sound e.g. 40 dB

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Type of hearing loss

• Conductive Hearing Loss
Hearing loss caused by something that stops sounds from
getting through the outer or middle ear. This type of
hearing loss can often be treated with medicine or
surgery.
• Sensorineural Hearing Loss
Hearing loss that occurs when there is a problem in the
way the inner ear or hearing nerve works.
• Mixed Hearing Loss
Hearing loss that includes both a conductive and a
sensorineural hearing loss.

Bone conduction (BC): Transmission of sound to cochlea Conductive>

via vibration of skull bone, bypassing the external and BC
middle ear. BC>AC

Air conduction (AC): Transmission of sound to

the cochlea via external air and middle ear
AC
For hearing, sound must reach Sound can travel AC ≥
the cochlea in any way. to cochlea via air 2xBC (N)
or directly via
bone.
Sensorineural
Types (partial)
aring Loss
of He AC>BC

mixe
d

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How can you differentiate conductiv e

and sensory-neural loss?

 Clinically by  Pure Tone Audiometry
 Tuning fork test

Deafness: High Degree of hearing loss

 Deafness is medically defined by the extent of loss of functional hearing and by
dependence upon visual communication.
 The Canadian Association of the Deaf-Association des Sourds du Canada recognizes
a person to be medically/audiologically deaf when that person has little or no
functional hearing and depends upon visual rather than auditory communication.

 “Anyone who cannot understand speech (with or

without hearing aids or other devices) using sound
alone (i.e. no visual cues such as lip- reading) is
deaf.”
If can hear but less than normal?

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Degree of hearing loss

• Mild Hearing Loss
A person with a mild hearing loss may hear some speech sounds but soft
sounds are hard to hear.

• Moderate Hearing Loss

A person with a moderate hearing loss may hear almost no speech when
another person is talking at a normal level.

• Severe Hearing Loss

A person with severe hearing loss will hear no speech when a person is talking
at a normal level and only some loud sounds.

• Profound Hearing Loss

A person with a profound hearing loss will not hear any speech and only very
loud sounds are heard.

Normal hearing • -10 to 15 dB

Slight hearing
loss •16 dB to 25 dB
Mild hearing loss • 25 to 40
Moderate hearing dBHL
loss • 41 to 70
Severe hearing dBHL
loss • 71 to 90 dBHL
Profound hearing
loss • >91+ dBHL

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 Causes, Risk Factors, and Characteristics

• Genes are responsible for hearing loss among 50% to 60% of children with hearing loss. [Read
article]
• About 20% of babies with genetic hearing loss have a “syndrome” (for example, Down syndrome
or Usher syndrome).
• Infections during pregnancy in the mother, other environmental causes, and complications after
birth are responsible for hearing loss among almost 30% of babies with hearing loss. [Read
article]
• Congenital cytomegalovirus (CMV) infection during pregnancy is a preventable risk factor for
hearing loss among children. [Read summary]
• 14% of those exposed to CMV during pregnancy develop sensorineural hearing loss (SNHL) of
some type.
• About 3% to 5% of those exposed to CMV during pregnancy develop bilateral moderate-to-
profound SNHL.
• A 2005 HealthStyles survey by CDC found that only 14% of female respondents had heard of
CMV. [Read summary]
• About one in every four children with hearing loss also is born weighing less than 2,500 grams
(about 5 1/2 pounds). [Read summary]
• According to ongoing tracking in metro Atlanta, the most common developmental disability to
co-occur with hearing loss is intellectual disability (23%), followed by cerebral palsy (10%),
autism spectrum disorder (7%), and/or vision impairment (5%). [Read article]
https://www.cdc.gov/ncbddd/hearingloss/data.html

Causes of hearing loss: outer ear

 Auricle & Ext. auditory
meatus

 Congenital
anomaly e.g.
meatal atresia
 Ear wax, impacted
 Any foreign body
 Tumour
Conductive type loss

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Deformity external ear

impacted wax

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Causes of hearing loss: middle ear

 Eardrum &
 three small bones called ossicles
that send the movement of the
eardrum to the inner ear

 Fluid in the middle ear

from colds
 Ear infection (otitis
media)
 Poor eustachian tube
function

Conductive type loss  Perforated

eardrum

Fluid in the middle ear from colds

Ear infection (otitis media)
Perforated eardrum

Fluid bubbles

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Otosclerosis  A condition of no definite

So, sound cannot travel well aetiology.

into the cochlear fluid.  Genetic predisposition is
 What type of deafness? found.

Due to some abnormal bone

remodelling process-
 Stapes (ossicle) cannot
move well, or becomes
fixed.

Poor /obstructed eustachian tube

function

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Causes of hearing loss: inner ear

 damage to the inner ear (cochlea), or to the nerve pathways from the inner ear
to the brain
 Most of the time, SNHL cannot be medically or surgically corrected
 SNHL reduces the ability to hear faint sounds. Even when speech is loud
enough to hear, it may still be unclear or sound muffled.
 Some possible causes of SNHL:

 Drugs that are toxic to hearing

 Hearing loss that runs in the family (genetic or hereditary)
 Aging
 Benign tumor
 Head trauma
 Malformation of the inner ear
 Exposure to loud noise
Cochlea, nerve, brain

Ototoxic drugs  certain aminoglycoside

antibiotics, such as
> 200 drugs known
gentamicin (family history
Certain medications may increase susceptibility),
can damage the ear,  cancer chemotherapy
resulting in drugs, such as cisplatin
 hearing loss, and carboplatin.
 ringing in the ear,  salicylate (aspirin)
 or balance disorders.  quinine (to treat malaria),
 These drugs are and
considered ototoxic.  loop diuretics (to treat
certain heart and kidney
conditions).

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Aging Good hearing

Genetic/ familial
Bad hearing

vestibular  A vestibular schwannoma

(also known as acoustic
schwannoma neuroma, acoustic
neurinoma, or acoustic
neurilemoma) is a benign,
usually slow-growing
tumor that develops from
the balance and hearing
nerves supplying the inner
ear. The tumor comes from
an overproduction of
Schwann cells

 Most common inner ear

tumour

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Head trauma Michel’s aplasia

complete labyrinthine aplasia (CLA), is a congenital

abnormality of the inner ear

Exposure to loud noise

 sounds can be harmful  Sudden or
when they are too loud,
even for a brief time, or
 Slowly progressive
when they are both loud  Unilateral/ bilateral
and long-lasting. These 

Temporary or sounds can damage

permanent sensitive structures in the

inner ear and cause noise-
induced hearing loss
(NIHL).

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What happens to ear?

 In most cases noise
damages our hair
cells.
 Human hair cells are
unable to regrow.
 So, damage is
permanent. Stereocilia perch atop sensory hair
cells in the inner ear

https://www.nidcd.nih.gov/health/noise-induced-hearing-loss

• The humming of a
Examples? refrigerator
45 decibels
• Normal conversation
60 decibels
 Explosion, • Noise from heavy city traffic
 continuous exposure to 85 decibels
loud sounds over an • Motorcyles
95 decibels
extended period of time,
• An MP3 player at maximum
such as noise generating volume
machine. 105 decibels
 Disco • Sirens
120 decibels
 Earphone • Firecrackers and firearms
150 decibels

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RISK FACTOR
BABIE
FOR
Referral form for neonatal hearing screening

S
Family history
Head trauma
Parental concern
Ototoxic
medication
Severe neonatal
jaundice
(Exchange
transfusion)
APGAR scores of 0-4 at 1 minute or 0-6 at 5 minutes
Associated syndrome
Craniofacial anomalies (eg. Cleft)
In-Utero infection(TORCH)
Mechanical ventilation >5 days
Bacterial meningitis
Neurodegenerative disorders Very
low birth weight (<1.5kg) Ear
abnormalities

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Signs of Hearing Loss In Children

doesn't respond to sound (eg; telephone, TV, car)


 pays attention to vibrations rather than noises.
 doesn't respond to name upon calling
 doesn't repeat any sounds you make.
 doesn't use his or her voice to attract attention.
 doesn't respond or listen to music, stories or
rhymes.
 Delay in speech

 listens to the television or radio at an abnormally high

volume.
 has articulation or speech problems.
 has problems learning.
 complains of earaches.
 seems to speak differently than other children of the same
age.

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Signs of Hearing Loss in Adult

 Difficulty hearing conversations, especially in noisy
background
 Frequently ask people to repeat what they've said
 Misunderstanding what people say
 Finding it difficult to hear on the telephone
 Turning up TV or radio louder than others in the room
prefer
 Removing yourself from conversations because it's too
difficult to hear
 Reading lips so you can try to follow what people are saying
 Experiencing tinnitus (a persistent ringing, buzzing, etc)

HEARING ASSESSMENT
First line Second line
 History
 Quick Examination
 By way of talking to
the patient
 Voice test
 Tuning fork test

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53

BEHAVIORA HEARING IMMITANCE TEST

L ASSESSMEN
TESTING
T
Tympanometry

ELECTROPHYSIOLOGI
CAL TEST
Acoustic Reflex (AR)

Otoacoustic Emission
(OAE) Reflex Decay Test

Auditory brainstem
response (ABR) / BSER

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Distraction
Test

Pediatric VRA

Behavioral
test Play
audiomet
ry
Adult
PTA

Visual Reinforcement Audiometry for infants and toddler

6 months to around 2 to 3 years old Based on behavioural training

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Distraction Test 55

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Visual Reinforcement Audiometry

(VRA)
 9 months to 2 ½ years old
 Child is reinforced (lighted puppet) after
responding to auditory stimulus (head turning)
delivered via loudspeaker, headphone or insert
phone.
 Phases :
 1st phase : conditioning
 2nd phase : testing

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VRA UP 57

SET
TESTER PUPPET

AUDIOMETER

DISTRACTOR
SPEAKER

TABLE

PUPPET

PATIENT

Play Audiometry 58

 2½ to 5 years old
 Most widely used to detect and assess hearing in children
 Adaptation of pure tone audiometry(PTA)
 Similar to testing conducted with adult except the response
required from the child is cast into a game (play format).
 Phases :
 1st phase : conditioning
 2nd phase : testing

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Pure Tone Audiometry (PTA) 59

 Gold Standard Test

 Aim:
 to establish hearing threshold sensitivity across the
range of audible frequencies important for human
communication.
 To determine the lowest intensity that the
listener can “just barely hear” = threshold.

AUDIOMETER 60

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IMMITANCE TEST

TYMPANOMETRY TEST

An objective measure of middle ear function.

 It’s a dynamic measure of acoustic immittance in the
external ear canal as a function of changes in air
pressure in the ear canal. (ANSI,1987)

Tympanometry 62

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Tympanograms (Jerger’s Fluid in middle

ear
63
classification)
Normal -VE pressure
in middle ear

Electrophysiology test 64

 Otoacoustic Emission (OAE)

 A quick screening tool
 From inner ear (cochlea)

 Auditory brainstem response

(ABR/ BSER)
Retro-cochlear (auditory nerve, brain)

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Auditory brainstem response (ABR) 65

 The ABR is generated by the auditory nerve and by structures in the

auditory brainstem.
 The response can be recorded from surface electrodes.
 Can be used as :
 Threshold seeking
 Site of lesion

Response

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Oto-acoustic Emission (OAE) 67

 Low intensity sounds generated by the cochlear that

can quickly be measured with sensitive microphone
placed in the ear canal.
 Widely used as a screening tool for assessing
cochlear function.
 Middle ear pathologies often eliminates the OAEs
response.

 Absence of OAE indicates cochlear or middle ear

pathology

MANAGEMENT - Treatment
 Medicine (e.g. for Ear Infection)
 Non-surgical treatment (wax removal)
 Surgical (Perforated TM, otosclerosis,
cholesteatoma, acoustic neuroma)
 Hearing Devices
 Speech Therapy

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Hearing Aids

What are hearing aids?

Hearing aids are sound-amplifying devices designed

to aid people who have a hearing impairment.

a microphone that picks up
sound;

The plastic tubing

that fits onto the
end of the hear
delivers the

hook transmits the

amplified

electronic sound
sound into the

from the microphone

ear canal;
into the ear mould
a
mplifier makes
the sound
l
ouder

batteries that power

t
he electronic parts 35
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Hearing Aid Range

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Bone Conduction Hearing Aids

Bone-Anchored Hearing Aids

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How hearing aids work?

 By amplifying the sound  But, if cochlea is not and
sending it to functioning well?
cochlear hair cells.  Then,
answer is -
 cochlear implant
 There should be some
normal functioning hair  A cochlear
implant is an
cells. implanted electronic device
that can produce useful hearing sensation by electrically stimulating nerves
 If hair cells are severely inside the inner ear.

damaged?  Cochlear implants are

indicated only for individuals
with severe-profound hearing
 Then we need …….. loss.

cochlear implant
 It doesn’t
amplify
sound but
 It directly
stimulates
the
cochlear
nerve
bypassing
the
damaged
ear

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References 77

 Katz, J., Burkard, R. and Medwetsky, L. (2009). Handbook of clinical

audiology. Philadelphia: Lippincott Williams & Wilkins.
 Stach, B. (1998). Clinical audiology. San Diego, Calif.: Singular Publ.
 Gelfand, S. (1997). Essentials of audiology. New York: Thieme.

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