Bilateral Childhood Deafness

Aetiology
Dr Kamol Krishna Pramanik

Diagnosis
Dr. H.S. Mobarak Hossain

Management
Dr. Ashfaq Ahmad

Impact on family and Prognosis

Dr. M.M. Anwar

Role of SAHIC in management of Deaf-mute

Dr Mahmudur Rahman

Aetiology
Dr.Kamol Krishna Pramanik FCPS (ENT)

Registrar
Dept of ENT & Head-Neck surgery Chittagong medical college hospital

Aetiology
A. Prenatal(Before birth) B. Perinatal(During birth C. Postnatal(After birth)

Prenatal causes
Genetic defect -Michel aplasia -Mondini aplasia -Schiebe aplasia -Alexander applasia

Prenatal contd..
Maternal Infections (TORCHES) -Toxoplasmosis -Rubella -CMV -Herpes -Syphilis

Prenatal contd
Drugs during pregnancy
-Streptomycin -Gentamicin -Tobramycin -Amikacin -Quinine -Chloroquin

Prenatal contd
Others
-Radiation to mother in 1st 3 months -Nutritional deficiency -Diabetes -Toxaemia -Thyroid deficiency

Perinatal causes
Anoxia Prematurity Birth injury Neonatal jaundice Neonatal meningitis Ototoxic drugs

Postnatal causes
Genetic
-Familial progressive sensorineural deafness -Certain syndromes like Alports,Klippel-Feil, Hurler etc.

Postnatal contd

Non-genetic Viral infectionsMeasles,Mumps,Varicella,influenza, meningitis,encephalitis. Secretory otitis media Ototoxic drugs Trauma Noise-induced hearing loss.

Diagnosis
Dr. Mubarok hossain.
FCPS(ENT)

Registrar Dept. of ENT & Head-Neck surgery Chittagong Medical College and Hospital

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.

Assessment of hearing
Neonatal

screening procedures

1.Aurosal test 2.Auditory response cradle

Arousal test: A high frequency narrow band noise is presented for 2 seconds to infant in light sleep Normal hearing infant aroused twice of three.

Auditory response cradle

Baby

is placed in a cradle His behaviour (trunk and limb movement, head jerk, respiration) in response to auditory stimulus are monitored by transducers.

Behavioral observation audiometry

Moros

reflex Cochleo-palpebral reflex Cessation reflex

Moros reflex
Sudden

movement of limbs and extension of head in response to sound of 80-90 dB. Birth to 2 months

Auropalpebral reflex: The child responds by blink to a loud noise Cessation reflex: Infant stops activity and start crying in response to sound of 90 dB.

Distraction technique (6-18 months of age)

The

test is based on the principle that the normal response observed when sound is presented to a baby is a head turn to locate the sound source.

Distraction technique

Conditioning technique (6-36 months)

Visual

reinforcement audiometry Play audiometry

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.

Play audiometry
The

child is conditioned to perform an act ( placing a marble in a box, a plastic block in a bucket.) when he hears a sound. It can be done in a free field or by using head phones.

Electrophysiological test ( 0-6 months)

Otoacoustic

emission Auditory brain stem response. Electrocochleography

Otoacoustic emission
Low intensity sound produced by outer hair cell Produced either spontaneously or in response to the acoustic stimuli They can be picked by a miniature microphone Absence of otoacoustic emission indicate structurally damaged or non functioning outer hair cell.

Auditory brain stem response

Records electrical response in cochlear nuclei and its central connection in brain stem to sound.

Electrocochleography
Invasive

procedure Records electrical activity generated in cochlea by directly placing electrode needle over promontory.

Pure tone audiometry

3 years onwards

Management of Bilateral Childhood deafness

Dr. Ashfaq Ahmad.
FCPS (ENT)
Assistant Professor. Dept of ENT & Head-Neck Surgery.

Chittagong Medical College

 Detailed

history :

-Pre-natal. -Peri-natal. -Post-natal.

Family

history.

Physical examination :
Ear :

-Congenital bilateral meatal atresia. -OME, -Wax. -CSOM -Status and type of deafness.

 Nose :

-Nasal discharge. -Posterior rhinoscopy in elderly children- Adenoids.

Throat

-Huge tonsils extended upwards to occlude E.tube producing OME.

Investigations
-History and meticulous examination
will guide relevant investigations .

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.

Aim
Improvement of hearing. Development of speech. Development of language. Adjustment in society. Useful employment.

Treatment
Depends upon cause of hearing loss.

Bilateral congenital meatal atresia -Meatoplasty.

OME CSOM -Adenoidectomy. -Myringotomy. -Conservative treatment. -Myringoplasty.

Ototoxic drugs -Withdrawal of the drugs. Noise induce hearing loss -Withdrawal from

the noise.

Aetiology of hearing loss remain obscure in many cases.

The children who do not have serviceable hearing status require1. Parental guidance : -Deaf child- A great emotional shock for the parents. -They should be dealt with great sympathy so as to accept the situation.

Habilitation of the deaf child demands a lot from parents

Care and periodic replacement of hearing aid. Change of ear moulds as child grows. Follow up visits for revaluation. Education at home. Selection of vocation

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 reading also.

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.

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.

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.

Hearing aids

Bone anchored hearing aid

Used in conductive or mixed hearing loss which can not be treated surgically and air conduction aids can not be used due to discharging ear, aural atresia and canal stenosis

(BAHA)

Cochlear implant

Bilateral severe to profound hearing loss. Minimal or no benefit from the hearing aids. Support of family for post implant training program.

B. Manual communication :
Makes use of sign language. Finger spelling method. Abstract ideas are difficult to express. General public does not understand

C. Total communication :

Uses all modalities of sensory input -Auditory. -Visual. -Tactile. Develop -Oral speech . -Lip reading. -Sign language.

Children with prelingual severe to profound deafness should undergo training in this form of communication.

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.

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.

Vocational guidance :
The deaf are sincere and good workers. They can be usefully employed in several vocations

Cochlear implant

Cochlear implant

How cochlear implant works

Impact & prognosis of bilateral childhood deafness.

Dr. Mostafa Mahfuzul Anwar
FCPS (ENT)

Assistant professor (ENT) Chittagong Medical College

Impact on family
Psychological upset Stage of mourning and shock Denial Guilt Acceptance Constructive action False beliefs

Impact on family- cont

Hearing impairment Speech problems Problems in schooling Problems in social interaction Problems in family life Problems in career

Social impact
Isolation Avoidance

Difficulty

in interaction Participation in gatherings Socially handicap Think as a burden

Impact on nation
Number

is quite high (1 in per 1000) Dependency Economic loss

Things to do Counseling Rehabilitation

Career

planning National guideline Specialized centre- one stop service Multisectoral approach Social movement

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, the prognosis is promising.