INTRODUCTION TO HEARING IMPAIRMENT AND COCHLEAR

IMPLANT

Dr. Ghulam SaqulainHead of Dept. of ENT,

Capital Hospital.

Helen Keller

“When you lose your vision, you lose contact with things;

when you lose your hearing, you lose contact with people.”

Patient profile and History

3 years old male childWas admitted on 2nd march 2016Resident of Azad Kashmir Normal birth with immediate cry.According to mother, hearing loss since birth, not

responding to any sound nor able to talk.There was no other congenital anomaly noted.Other milestones were normal

Past History No medical and surgical history

Hearing Assessment

BERA test

Profound SNHL. Hearing loss was stable. BERA was repeated at the interval of 6 months till surgery.

Right ear90 db

Left ear90 db

MRI brain and Temporal bones

Bilateral CP angles and 7th-8th nerve complexes were normal.

The cochlear turns and vestibule were normal bilaterally

There was no other pathology noted in brain

CT scan Brain and temporal bones.

Mastoids were well pneumatized. Middle ears on both sides were normal with intact ossicular chain. Bony cochlea and semicircular canals were also intact.

Plan Since this was a case of profound non serviceable SNHL, Cochlear Implantation was planned

Surgery Patient was operated for Cochlear implantation at capital Hospital on 3rd march 2016 by experienced:Implant Surgeon AudiologistOur team

What is a Hearing Impairment?

Hearing impairment is the decreased ability to hear and discriminate among sounds.

A complete inability to hear also occurs and is quite common such that a person is unable to understand speech even in the presence of amplification.

World Health Organization (WHO) recognizes moderate, severe and profound HL as disabling hearing losses

WHO Definition of a Disability (2000)

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STATISTICS AND DEMOGRAPHICS:

In United States: About 12,000 babies (3 in 1,000) are born with significant hearing impairment.

In United Kingdom:10 million people (1 in 6 of population)By 2031 set to rise to 14.5 million people It is estimated that about 50,000 - 70,000 people use sign language

In Pakistan:

According to census report 1998, 9 % of total population is disabled

According to WHO survey, 10% of total population is suffering from one or other disability. Hearing impairment accounts for 9% of disabilities.

Age of Presentation

Etiology:

InfectionGenetics, Aging, Exposure to noise, Chemicals and Physical trauma. 

Breakdown of causes/risk factors

responsible for increasing burden

of hearing disability and

handicap.

Ear comprises of three main parts playing their own vital role :

Types of Hearing Loss:

Type Area Affected CausesConductive Middle Ear Blockage

DamageInfection

Sensorineural Inner Ear (Hair cells) or auditory nerve

Noise Drug side effectsGeneticsInfectionsAgeing

Conductive Hearing Loss

Sound does not move into inner ear efficiently.

Due to a problem in either outer or middle ear.

May be medically or surgically treated or cured.

Inner ear works fine.

Amplification may help to get sound through the auditory system.

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Conductive Hearing Loss

Sensorineural Hearing Loss

Sound is not interpreted efficiently by inner ear. (Louder doesn’t help!)

May be due to hair cell/nerve damage.

May be due to problem with auditory nerve.

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Sensorineural Hearing Loss

HI is tip of an iceberg:

Impact of Hearing loss

Pre Lingual Hearing Loss

Post Lingual Hearing Loss

Post Lingual Hearing Loss:

Typically:Gradual lossDetected by family

On the one hand, they are fortunate in that they have learned to speak.

On the other hand, they suffer from social isolation.

When the loss is due to heredity causes, total deafness is often the end result.

Pre lingual Hearing Loss:

Depends On: Ear/s involvedDegree /LevelType of hearing loss Age of onset

Mild Hearing Loss: May affects

Speech perception in noise esp. in classroom environment a mild hearing loss is disabling

Behavioral Emotional or social problems

Severe Losses:

Children do not speaking at a normal age Delayed language acquisition; Lack of reaction to loud noises Disordered speech Ignore adults when spoken to

A child who sustains hearing loss early in life may lack the language stimulation and speech development experienced by children who can hear.

Hearing loss stunts imitation of essential speech patterns and sounds, and prevents the individual from monitoring his or her errors.

The academic progress of a child with hearing loss often lags behind that of children who can hear.

It is common for hearing-impaired adolescents to be four or more grades behind their peers.

Infant Screening:

Screening of neonates, preferably before they leave the hospital is recommended and is essential component of UNHS programs

UNHS

This is because language and communication develop rapidly during the first two to three years of life, and undetected hearing impairment can lead to delays in developing these skills.

MANAGEMENT

Mild hearing loss Lip readingHearing aid

Moderate hearing loss Lip reading Sign languageHearing Aid

Severe hearing loss Difficulty even with a hearing aid. Most will lip read and some will use sign language

Profound hearing loss Hearing aids are of little benefit. Most will lip read Cochlear Implantation

Cochlear Implant

A cochlear implant is a small electronic device that provides sound to children and adults who have a severe to profound hearing loss, non serviceable ears (70 to 90 dB) who do not benefit from hearing aids.

Cochlear implants enable sound to reach the brain by effectively bypassing the part of the ear which is damaged, to directly stimulate the hearing nerve electronically.

Who is a Candidate for a Cochlear Implant?

Adults (18 years+)•Severe-to-profound, bilateral sensorineural hearing loss•Less than 50% speech recognition with hearing aids on open-set sentence recognition

Children (12 months - 2 years)•Profound, bilateral sensorineural deafness (> 90 dB HL)•Little or no benefit from hearing aids

Children (2 years - 17 years)•Severe-to-profound, bilateral sensorineural deafness •Little or no benefit from hearing aids

Cochlear Implant Three Parts

Receiver- surgically implanted beneath the skin above the ear. A small wire containing an array of electrodes is threaded through the cochlea.

Microphone-part of the “headpiece” that rest on the outer side of the skin that covers the internal receiver. The microphone picks up sound and transmits it to the speech processor.

Speech Processor-a microcomputer that can be worn behind the ear that transmits the signal to the receiver and electrodes, which then stimulates the auditory nerve fibers, which in transmits the signal to the brain where sound is interpreted.

How is a Cochlear Implant Different From a Hearing Aid

Hearing Aid Cochlear ImplantAcoustically amplify sound. Convert sound into electrical

signals.

Rely on the responsiveness of healthy inner ear sensory cells.

Bypass the inner ear sensory cells and stimulate the hearing nerve directly.

Cochlear Implant Surgery

Operation lasts about 1-2 hours It’s a team approach Performed by otologist Audiologist check the activity of implant peroperatively Speech language pathologist Psychiatrist and psychologist

Pediatric Cochlear Implant Candidacy

Audiologic Evaluation

Medical Evaluation

Speech Language Evaluation

Psychological Evaluation

Educational Evaluation

Bilateral Cochlear Implants

Bilateral Implants Simultaneous or sequential Benefits:

Improved directionality Improved listening in noise Clarity of speech

Possible Positive Effects of Cochlear Implant

Many CI users are able to detect medium to loud sounds including speech sounds and can learn to recognize familiar sounds.

Many CI users find it easier to communicate. More than 50% are able to understand speech without visual cues.

In clinical studies of adults who experienced hearing loss after they had developed speech, 90% reported improved communication without speech reading.

75% reported communicating more effectively when at a dinner party, when driving a car, with family members, with family and when ordering at a restaurant.

Some can talk on a telephone and enjoy music.

Factors Thought to Influence Cochlear implant Success

Length of time the person was deaf before implantation Length of time the implant was utilized Language and Listening skills before implantation Cognitive and attention abilities Intensity of therapy after implantation Type of language programming Resources and involvement of patient’s family

Deaf Culture and Cochlear implant

The Deaf culture is a group of individuals, generally born-deaf, and who communicate with American Sign Language (ASL).

Deaf culture celebrate their deafness, and many, if given the opportunity to hear, would choose to remain deaf because they do not see deafness as a disease or a disability, only as a difference.

If all deaf-born infants are implanted and choose the hearing world, then it will be the demise of a culture

They maintain that hearing parents’ decision to implant deaf children without consulting the Deaf community renders the decision “ill-informed, ill-prepared, ill-advised, ill-founded, and ill-fated.

Team Approach

Parent Commitment

Summary

Cochlear implants are an effective treatment for severe-to-profound sensorineural hearing loss.

A multi-disciplinary approach is necessary when determining cochlear implant candidacy in children.

Post-operative management of a child with a cochlear implant consists of programming of the sound processor and important rehabilitation.

Parent commitment and school support are necessary components to ensure a child’s success with a cochlear implant.

Thank you for your AttentionAny Questions?