childhood deafness with all

7/28/2019 Childhood Deafness With All

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Bilateral Childhood Deafness

Aetiology

Dr Kamol Krishna Pramanik

Diagnosis

Dr. H.S. Mobarak Hossain

Management

Dr. Ashfaq Ahmad

Impact on family and PrognosisDr. M.M. Anwar

Role of SAHIC in management of Deaf-mute

Dr Mahmudur Rahman


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Aetiology

Dr.Kamol Krishna PramanikFCPS (ENT)

Registrar

Dept of ENT & Head-Neck surgery

Chittagong medical college hospital


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AetiologyA. Prenatal(Before birth)

B. Perinatal(During birthC. Postnatal(After birth)


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Prenatal causes

Genetic defect

-Michel aplasia

-Mondini aplasia-Schiebe aplasia

-Alexander applasia


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Prenatal contd..

Maternal Infections (TORCHES)

-Toxoplasmosis

-Rubella-CMV

-Herpes

-Syphilis


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Prenatal contd

Drugs during pregnancy-Streptomycin

-Gentamicin

-Tobramycin

-Amikacin

-Quinine-Chloroquin


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Prenatal contd

Others

-Radiation to mother in 1st 3 months

-Nutritional deficiency-Diabetes

-Toxaemia

-Thyroid deficiency


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Perinatal causes

Anoxia

Prematurity

Birth injuryNeonatal jaundice

Neonatal meningitis

Ototoxic drugs


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Postnatal causes

Genetic-Familial progressive sensorineural

deafness

-Certain syndromes like

Alports,Klippel-Feil, Hurler etc.


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Postnatal contd

Non-genetic

Viral infections-Measles,Mumps,Varicella,influenza,

meningitis,encephalitis.

Secretory otitis media

Ototoxic drugs

Trauma

Noise-induced hearing loss.


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Diagnosis

Dr. Mubarok hossain.FCPS(ENT)

Registrar

Dept. of ENT & Head-Neck surgery

Chittagong Medical College and Hospital


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Suspicion of hearing loss

The child sleeps through loud noises.

Fails to develop speech at 1-2 years.

Child with defective speech

Poor performance at school.


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Assessment of hearing

Neonatal screening procedures

1.Aurosal test

2.Auditory response cradle


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Arousal test:

A high frequency narrow band noise is

presented for 2 seconds to infant inlight sleep

Normal hearing infant aroused twice of

three.


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Auditory response cradle

Baby is placed in a cradle

His behaviour (trunk and limbmovement, head jerk, respiration) in

response to auditory stimulus are

monitored by transducers.


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Behavioral observation

audiometry

Moros reflex

Cochleo-palpebral reflex

Cessation reflex


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Moros reflex

Sudden movement of limbs and

extension of head in response to soundof 80-90 dB.

Birth to 2 months


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Auropalpebral reflex:

The child responds by blink to a loud

noiseCessation reflex:

Infant stops activity and start crying in

response to sound of 90 dB.


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Distraction technique

(6-18 months of age)

The test is based on the principle thatthe normal response observed when

sound is presented to a baby is a head

turn to locate the sound source.


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Distraction technique


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Conditioning technique

(6-36 months)

Visual reinforcement audiometry

Play audiometry


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Visual reinforcement audiometry

The child is conditioned to turn his

head to the direction of sound which is

also reinforced by light

The head turn are then noted in

response to the sound stimuli.


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Play audiometry

The child is conditioned to perform an

act ( placing a marble in a box, a

plastic block in a bucket.) when hehears a sound.

It can be done in a free field or by

using head phones.


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Electrophysiological test

( 0-6 months)

Otoacoustic emission

Auditory brain stem response.

Electrocochleography


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Otoacoustic emission

Low intensity sound produced by outer haircell

Produced either spontaneously or in

response to the acoustic stimuli They can be picked by a miniature

microphone

Absence of otoacoustic emission indicatestructurally damaged or non functioningouter hair cell.


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Auditory brain stem response

Records electrical response in cochlearnuclei and its central connection in brain

stem to sound.


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Electrocochleography

Invasive procedure

Records electrical activity generated incochlea by directly placing electrode

needle over promontory.


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Pure tone audiometry

3 years onwards


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Management of Bilateral

Childhood deafness

Dr. Ashfaq Ahmad.

FCPS (ENT)

Assistant Professor.Dept of ENT & Head-Neck Surgery.

Chittagong Medical College


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Detailed history :

-Pre-natal.

-Peri-natal.-Post-natal.

Family history.


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Physical examination :

Ear:

-Congenital bilateral meatal atresia.-OME,

-Wax.

-CSOM

-Status and type of deafness.


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Nose :

-Nasal discharge.

-Posterior rhinoscopy in elderlychildren- Adenoids.

Throat :

-Huge tonsils extended upwards toocclude E.tube producing OME.


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Investigations

-History and meticulous examination

will guide relevant investigations.


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Management

Essential to know-

-The degree and type of deafness.

-Mental status.-Prelingual hearing loss.( before development of speech).

-Post lingual hearing loss(after development of speech).

-Socioeconomic status.


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Aim

Improvement of hearing.

Development of speech.

Development of language.

Adjustment in society.

Useful employment.


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TreatmentDepends upon cause of hearing loss.

Bilateral congenital meatal atresia

-Meatoplasty.

OME -Adenoidectomy.

-Myringotomy.

CSOM -Conservative treatment.

-Myringoplasty. Ototoxic drugs -Withdrawal of the drugs.

Noise induce hearing loss -Withdrawal fromthe noise.


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Aetiology of hearing loss remain

obscure in many cases.


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The children who do not have

serviceable hearing status require-

1. Parental guidance :

-Deaf child- A great emotional shock for the

parents.

-They should be dealt with great sympathy

so as to accept the situation.


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Habilitation of the deaf childdemands a lot from parents

Care and periodic replacement of hearingaid.

Change of ear moulds as child grows.

Follow up visits for revaluation.

Education at home.

Selection of vocation


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2. Hearing aids :

Most of the deaf child have useful portion

of residual hearing which can be made

serviceable by amplification. Hearing aids as early as possible.

Binaural aids.

Hearing aids help to develop lip readingalso.


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3. Development of speech and language :

Communication depends upon--Receptive skill.

-Expressive skill.

Receptive skill through-

-Visual.

-Auditory.

-Tactile faculties.

Expressive skill through--Oral speech.

-Written speech.

-Manual sigh language.


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In hearing impaired children whose

hearing status is poor or totally absent.

For proper communication :

Amplification of sound by hearing aid.

Cochlear implants.

Develop visual means of communication.

Develop tactile means of communication.


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A. Hearing aids :

Best way of communication.

Moderate to severe hearing loss.

Post lingually deaf.

Improve auditory receptor.

Improve speech reading i.e read movement of--Lips,

-Face,

-Natural gastures of hand and body. Expressive skill is encouraged through oral

speech.


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Hearing aids

Bone anchored hearing aid


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Bone anchored hearing aid(BAHA)

Used in conductive or mixed hearing loss

which can not be treated surgically and airconduction aids can not be used due to

discharging ear, aural atresia and canal

stenosis


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Cochlear implant

Bilateral severe to profound hearing loss. Minimal or no benefit from the hearing aids.

Support of family for post implant training program.


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B. Manual communication :

Makes use of sign language.

Finger spelling method.

Abstract ideas are difficult to express.

General public does not understand


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C. Total communication :

Uses all modalities of sensory input-Auditory.

-Visual.

-Tactile.

Develop

-Oral speech .

-Lip reading.

-Sign language.Children with prelingual severe to profounddeafness should undergo training in this

form of communication.


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Education of the deaf

Residential and day school for the deaf

child.

Some deaf children with moderate hearing

loss can be integrated into schools for

normal children with preferential seating in

the class.


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Radiohearing aids :

Have revolutionised education of the deaf.

Microphone and transmitter worn by

teacher, receiver and amplifier by the child.

The child hear the teacher better with out

disturbed by environment.


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Vocational guidance :

The deaf are sincere and good workers.

They can be usefully employed in several

vocations


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Cochlear implant

C hl i l t


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Cochlear implant


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How cochlear implant works


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Impact & prognosis ofbilateral childhood deafness.

Dr. Mostafa Mahfuzul Anwar

FCPS (ENT)

Assistant professor (ENT)

Chittagong Medical College


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Impact on family

Psychological upset

Stage of mourning and shock

DenialGuilt

Acceptance

Constructive action

False beliefs


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Impact on family- cont

Hearing impairment

Speech problems

Problems in schooling

Problems in social interaction

Problems in family life Problems in career


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Social impact

Isolation

Avoidance

Difficulty in interaction Participation in gatherings

Socially handicap

Think as a burden


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Impact on nation

Number is quite high (1 in per 1000)

Dependency Economic loss


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Things to do-

Counseling

Rehabilitation

Career planningNational guideline

Specialized centre- one stop service

Multisectoral approach Social movement


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Prognosis

Conductive losses are recoverable.

Sensorineural loss does not recover.

Some may progress till adulthood. Psychological upset is high.

Child abuse is more common.

With advent of newer technologies, theprognosis is promising.


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childhood deafness with all

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