DEAFNESS Mbbhagat

8/8/2019 DEAFNESS Mbbhagat

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Dr. MAHENDRA B. BHAGATMB, DNB (PED), PGD-HM, DIM

PEDIATRICIAN

SURAT



Blindness separates

us from things

but

deafness separates us

from people.

7 November 2010 2



It is a continual source of information

about things and happenings within our

immediate physical environment.

It provides warnings signals that areimportant to physical safety.

It gives help to individual in acquiring and

maintaining physical skills

It forms a link with the rest of world andinstinctively becomes an emotional link

that contributes to mental health & social

ease.

7 November 2010 3



> 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

7 November 20104



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.

7 November 20105



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.

7 November 20106



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.

7 November 20107



7 November 2010 8

Implies hearing is defective, but functional

Educational definition ± Those persons

whose hearing makes it difficult, but not

impossible, for them to understand speechand language with or without the use of

hearing aids.



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 educationallysignificant hearing loss (USA)

About 25% of students who are deaf or hard

of hearing have another disabling condition7 November 2010 9



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/loudconversation, 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.7 November 2010

10



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Sound is transmitted as sound waves from theenvironment.

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 electricalimpulses.

These electrical impulses are transmitted to the

auditory nerve and to the brain where they are

interpreted as sound.7 November 2010 12



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.

7 November 201014



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

7 November 201015



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.

7 November 201017



Results in

a reduction in sound level

less ability to hear faint sounds

Multiple audiometric configurations basedon degree and configuration of HL

Any degree of HL

affects speech understanding or ability to

hear clearly

7 November 201018



As a result of exposure to

Abrupt dangerous levels of sound

or

Loud sound over extended periods of

time

7 November 201019



Reaching

hazardous levels of

sound

Extended exposureto 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 thecochlea

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 diseaseNeoplasms ± Vestibular schwannoma

Unknown etiology ± Presbycusis, Meniere

Disease

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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% acquired7 November 2010 26



Prelingually deaf individual

born with insufficient hearing to acquire speech

normally, or

Lost hearing prior to the age at which speech isacquired.

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 spokenlanguage 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 hearingsystem 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.

C APD does not necessarily involve (althoughit may) hearing loss.

C APD 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 parentsor 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 130drugs (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, cancause 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.7 November 2010 34



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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7 November 2010 36

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 asynchronized response from

neural fibers; a tracing of the

response is a series of waves



ADVANTAGES

Identifies cochlear,

conductive and neural

losses: ? mild-mod.

Some test both ears

simultaneously.

Relatively insensitive

to transient ear canal,

middle ear & externalnoise.

? Lower fail rate than

OAE

DISADVANTAGES

Test time

Disposable costs

Infant state/myogenic

artifact

Requires electrode

prep, placement &

removal

Click can miss

unusual configurations

of HL

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7 November 2010 38

m e of reete o o



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

7 November 2010 39



ADVANTAGES

Quick

Inexpensive

Frequency-specific Identifies cochlear

and conductive

losses: mild-mod?

DISADVANTAGES

Sensitive to ear canal

and middle ear

conditions Sensitive to noise

(internal & external)

Cannot identify

neural disorders

including auditory

neuropathy

High fail rates in

some programs..

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Device Options for OAE ScreeningDevice Options for OAE Screening

Types

HandheldHandheld

Portable screening devicesPortable screening devices

PCPC-- based hy brids based hy brids PCPC-- based clinical systems based clinical systems



ADVANTAGES

Low failure rate

Depending on testorder, identifies

cochlear, neural and

conductive losses

Reduced effects of noise

screening devices

available for both

technologies

DISADVANTAGES

Time

Equipment anddisposable costs

7 November 2010 42



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 HL7 November 2010 43



Family history of hereditary or unexplaineddeafness

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 comprehensionand 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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R eading Comprehension Scores of R eading Comprehension Scores of

Hearing and Deaf StudentsHearing and Deaf Students

1.02.0

3.0

4.0

5.0

6.0

7.0

8.0

.0

10.0

8 10 11 12 13 14 15 16 17 18

Deaf

Hearing

Age in Years

G

r a d e

i v

a l

e n t s



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, includingC

T/MRI ±

R/Oanatomic abnormalities and the extent of

inflammatory processes or Neoplasms.

Individualized tests ± TORCH titers in newborn in

NIC

U with IUGR 7 November 2010 48



7 November 2010 49

Audiogram showing bilateral

conductive hearing loss



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 µs¶ or µed¶

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 notunderstand 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

7 November 2010 52



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)

7 November 2010 53



Biologically based MI appears to be at the samerate 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 abuse7 November 2010 54



7 November 2010 55

t



7 November 2010 56

t r ro

Parent GroupsMental Health

Hospital

ScreeningAudiology

P

rima

ryProvider

Child/Family

ENT

GeneticsEducational

intervention

Ther a pists

3rd Party

Payors

Deaf Community

Interpreters Deaf

Services



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 7 November 2010 57



7 November 2010 58

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 isalso damaged.

This is why genetic testing is

often very useful when deciding

what treatment is best for an

individual



GOAL of Hearing Aid fitting ±

Ensure children receive full-time use andconsistent 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. 7 November 2010 59



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.

7 November 2010 60



For children with

bilateral

conductive

hearing loss whoare unable to wear

or benefit from

BTE hearing aids

Children with

unilateral deafness

7 November 2010 61



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.

7 November 2010 62



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

7 November 2010 63



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 7 November 2010 64



W hat is it like to ³hear´ a

hand?

You have to be deaf tounderstand.

± A poem by W illard Madsen

7 November 2010 65



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.

7 November 2010 66



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 auditorynerve

Electrodes are placed directly in the cochlea

Don¶t restore normal hearing

Individual will not hear/understand speech like anormal hearing person

But provides ³useful´ hearing & improved

communication abilities

Also safe, reliable & provides for effective rehab.7 November 2010 67



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4-6 weeks of healing before sound and speechprocessor 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 ³untrained ear´

Requires post-implantation therapy

Process is expensive

Takes time to interpret sound created by

implants7 November 2010 75



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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7 November 2010 77

Training is Everything



7 November 2010 78



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.

7 November 2010 79



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.

7 November 2010 80



Create opportunities for deaf children to mix withtheir peers & others at home, school or in

community

Promote good communication skills

Make sure you have the deaf child¶s attentionbefore 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.7 November 2010 81



All families with a child with congenital

or late onset hearing loss

Families of a child with stigmata or a

syndrome will benefit from theinformation.

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 todevelop to his/her potential.

7 November 2010 83

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