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Transcript of DEAFNESS Mbbhagat
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Dr. MAHENDRA B. BHAGATMB, DNB (PED), PGD-HM, DIM
PEDIATRICIAN
SURAT
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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 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.
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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 speechand 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 educationallysignificant hearing loss (USA)
About 25% of students who are deaf or hard
of hearing have another disabling condition7 November 2010 9
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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/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
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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
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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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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 basedon 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 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
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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 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
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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 asynchronized response from
neural fibers; a tracing of the
response is a series of waves
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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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m e of reete o o
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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
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
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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
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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 HL7 November 2010 43
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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
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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, 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
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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 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
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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 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
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t
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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
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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 7 November 2010 57
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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 isalso 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 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
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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 whoare 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 7 November 2010 64
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W hat is it like to ³hear´ a
hand?
You have to be deaf tounderstand.
± A poem by W illard 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 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
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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
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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 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
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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 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.
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