DEAFNESS,,,,,HEARING LOSS - PowerPoint Presentation

Slide1

DEAFNESS,,,,,HEARING LOSS

Asst.prof dr.FALIH AL-ANBAKY

Slide2

Hearing impairment,

deafness

, or

hearing loss

refers

to the

inability to

hear or understand

things, either totally or partially

. Symptoms may be

mild, moderate

,

severe or profound

. A patient with mild hearing impairment may have problems understanding speech, especially if there is a lot of noise around, while those with moderate deafness may need a hearing aid. Some people are severely deaf and depend on lip-reading when communicating with others.

People who are profoundly deaf can hear nothing at all

.

In order to communicate spontaneously and rapidly with people, they are totally

reliant on lip-reading and/or sign language

.

Slide3

What is the difference between hearing loss and deafness

?

Hearing loss

refers to a

diminished ability

to hear sounds like other people do,

whil

e

D

eafness

refers to

the inability

to understand speech through hearing even when sound is amplified.

P

rofound deafness

=totally

deaf

means the person cannot hear anything at all; they are unable to detect sound, even at the highest volume possible.

Slide4

A few facts and figures about deafness in UK

10 million people (approx.) in the UK are affected by hearing loss (1 in 6).

6.5 million of these are aged

60

and over.

3.7 million are of

working age

.

Around 2 million people

use hearing aids.

About 800,000 are

severely or profoundly deaf

.

Many people with hearing loss also

have tinnitus

. They may also have

balance difficulties

.

Hearing loss increases sharply with age - about a third of people aged 70+ have a hearing loss

.

But 10 million people make deafness the second largest disability in the UK.

Slide5

How do we hear things?

Slide6

Three Types of Hearing Loss

Conductive hearing loss

-

when hearing loss is due to problems with the ear canal, ear drum, or middle ear and its little bones (the

malleus

,

incus

, and stapes).

Sensorineural

hearing loss (SNHL

)

- when hearing loss is due to problems of the inner ear, also known as nerve-related hearing loss.

Mixed hearing loss

- refers to a combination of conductive and

sensorineural

hearing loss. This means that there may be damage in the outer or middle ear and in the inner ear (cochlea) or auditory nerve.

Slide7

Conductive Hearing Loss

Causes:

Malformation

of outer ear, ear canal, or middle ear structures

Fluid in the

 

middle ear

from colds

(OME).

Ear infection

(

Acute & Chronic

suppurative

otitis media -

Allergies

Poor Eustachian

tube function

.

Perforated

eardrum

.

Benign

or malignant

tumors

in external canal and middle ear.

Impacted earwax

.

Infection in the ear canal

(

Otitis

externa

).

Foreign body in the ear

.

Otosclerosis

.

Tympanosclerosis,,adhesive

otitis

media.

Slide8

Treatments of Conductive Hearing

Loss

According to underlying cause:

-

congenital cause

by surgery or hearing may

be improved with amplification with a bone conduction hearing aid, or a surgically implanted,

osseointegrated

device (for example, the

Baha

or

sound bridge),

or a conventional hearing aid, depending on the status of the hearing nerve

.

Infection

treated medically or surgery

Tumor

by surgery ,radiotherapy

Genetic like

otosclerosis

by drug

flouride

,surgery ,or hearing aids

Slide9

Sensorineural Hearing Loss

Causes:

Head trauma

.

Exposure to loud noise

(noise induced hearing loss,

Acaustic

trauma).

INFECTION

S:-

.

Vir

al causes

:

such

as

Chicken

pox ,Cytomegalovirus ,Mumps, Meningitis,

AIDS - offspring of mothers who had AIDS during pregnancy have a much higher risk of being deaf by the age of 16 years

*

Syphilis *Lyme disease *Tuberculosis (TB

),

experts believe that the medication, streptomycin, used to treat TB may be the key risk factor

Sickle cell disease .

Diabetes

- studies have shown that up to 40% of diabetic patients suffer from some kind of hearing loss **

Hypothyroidism , Arthritis

Ototoxic

drugs

Autoimmune disease

.

Hearing loss that runs in the family

(

hereditory

).

.

Aging (presbycusis

(

.

Malformation of the inner ear

.

Meniere

’

s Diseae

.

Otosclerosis - a hereditary disorder in which a bony growth

invade

the cochlea damage the hair cell.

Tumors

Slide10

Treatment of Sensorineural Hearing Loss

:

SND

from acoustic trauma

(or exposure to excessively loud noise),

by

corticosteroids

to reduce cochlea hair cell swelling and inflammation to improve healing of these injured inner ear structures.

SND

from head trauma or abrupt changes in air pressure such as in airplane

descent, which can cause inner ear fluid compartment rupture or leakage, which can be toxic to the inner ear. There has been variable success with

emergency surgery

when this happens.

SND

be of viral origin

, is an otologic emergency that is medically treated with corticosteroids.

Bilateral progressive hearing loss

over several months, also diagnosed as

autoimmune inner ear disease,

is managed medically

with long-term corticosteroids

and sometimes with drug therapy..

Fluctuating sensorineural hearing loss

may be from unknown cause or associated with

Meniere

’

s

Disease

may be treated medically with a low-sodium diet, diuretics, and corticosteroids. If the vertigo is not medically controlled, then various surgical procedures are used to eliminate the vertigo.

Slide11

Treatment of SND

Sensorineural hearing loss from disease

in the central nervous system may respond to medical management for the specific disease affecting the nervous system. For example, hearing loss secondary to multiple sclerosis may be reversed with treatment for multiple sclerosis.

Sensorineural hearing loss from tumors

of vestibular

nerve

(

Acaustic

neuroma

)

,

by surgery.

Irreversible sensorineural hearing loss

,

the most common form, may be managed

with hearing aids

. When hearing aids are not enough, this type of hearing loss can be surgically treated with

cochlear implants

Slide12

Mixed Hearing Loss

Treatments for Mixed Hearing Loss

R

ecommends taking care of the conductive component first.

(medically

or surgical

(

.

To

ma

k

e the person a better hearing aid candidate, .

Slide13

Degree of

deafnes

,Hearing impairment

There are

four

levels of deafness (possibly

5

in some countries), they are:

Mild deafness or mild hearing impairment

- the patient can only detect sounds from between

25

to

39

decibels (.

Moderate deafness or moderate hearing impairment

- the patient can only detect sounds from between

40

dB and

69

dB

.

Severe deafness

- the person only hears sounds above

70

db to

89

dB

.

.

Profound deafness

- anybody who cannot hear a sound below

90

dB is

profoundly deaf;

Obviously, if the hearing impaired

or

deaf person can read and write, they may also communicate by reading and writing

Slide14

How deafness diagnosed

?

Patients who suspect something is wrong with their hearing will usually go and see their GP (general practitioner, primary care physician) initially.

or ENT

doctor

s.

-

Start with history of

the symptoms

, when they started, whether or not they have gotten worse, whether there is any pain, etc.

A physical examination

- the doctor will look into the patient's ear using an otoscope (aur

o

scope). The

finding should be recorded

Doctors may ask questions regarding the patients hearing

,

which will probably be similar to the ones below:

Do you often find yourself asking people to repeat what they said?

Do you find it hard to understand people on the telephone?

Does the doorbell ring and you did not hear it? If so, does this happen frequently

Slide15

When you chat to people face-to-face, do you have to focus carefully?

Has anybody ever mentioned to you that you might have a problem with your hearing?

When you hear a sound, do you often find it hard to determine where it is coming from

When several people are talking, do you find it hard to understand what one of them is telling you?

Are you often told that the TV, radio or any sound-producing device is too loud?

Do you find the speech of men easier to understand than women's or children's?

Have you often found yourself misunderstanding what other people say to you

Have you hear

ringigng,rushing

,hissing sounds in ears(

Tinitus

).

?

Anybody who answers "yes" to most of the above questions should see their doctor and have their hearing checked.

Slide16

The diagnosis of

deafnes

depend on

folowing

tests:

.

Whispered speech test

.

The

doctor will whisper a combination of numbers and letters from behind

the

patient

and ask him to repeat the combination to check if he can hear anything. Each ear should be tested separately.Tuning fork test. Pure tone audiometry. .Otoacoustic emissions. This test is used to measure your

cochlear function

by recording signals produced by the hair cells.

Auditory brainstem response

.

This test measures the activity of cochlea, auditory nerve and brain when a sound is heard

Slide17

Slide18

Slide19

Slide20

If your hearing loss has a sensorineural cause, a number of other tests can be done to pinpoint where the problem lies.

**

In cases of unilateral

sensorineural

deafness

,

and may be associated with vertigo

tinitus,

need

to do

CTscan and MRI scan. This is to rule out rare causes of hearing loss such as an acoustic neuroma or cholesteatoma or other intracranial (cerebellopontine angle) tumour

Slide21

Otosclerosis

Definition:

a localized disease of the

otic

capsule in which new spongy bone causes

ankylosis

of the footplate of stapes or invades the cochlea.

.

Slide22

Aetiology

:

exact causes unknown, many theories

.

-

Measles virus RNA

is found in

otosclerotic

foci in footplates removed during surgery. Measles virus infection may activate the gene responsible for

otosclerosis

. -

- Hereditary (50% +ve family history) inhereted as autosomal dominant pattern with incomplete penetrance- Incidence: more in white races, female two time more male- Age of onset 20-30 years of age.- Effect of pregnancy accelerate the condition but never cause it (pill have the same effects)

Slide23

Pathology

:

normal bone absorbed and replaced by new spongy

osteoid

bone. The commonest site 80-90% is on the anterior margin of the footplate of the stapes at its attachment to the oval window called (

fissula

ante

fenestram

) and spread via vascular channels leads to

ankylosis

(fixation) of the stapes footplate. It may involve the cochlea and labyrinth in8% so called labyrinthine

otosclerosis

, 2% both stapes and cochlea

.

Slide24

Clinical features:

-

Deafness

is the predominant

symptom

, usually

gradual bilateral

(80%) and it's conductive in

type.

Patients exhibit low volume-speech.(hear their voice louder).

Usually takes many years to

becom

obvious .

Sensorineural

deafness if the cochlea involved. *Paracusis welleci(the patient hears better in a background noise). -Tinnitus --Vertigo symptom appear at any age from 15-45yrs mostly at twenties.

Slide25

On Examination:

-

Normal tympanic membrane. Flamingo-pink tinge (reddish –blue hue) (

Schwartze's

sign)

may be seen through the TM due to hyperemia of the promontory due to hyper vascular immature bone.

-

Conductive deafness by tuning fork tests

Slide26

I

nvestigation

:

-

PTA

show

low frequency conductive

loss

. Then involve high frequency with widening of

airbone

gap

Carhart's

notch = dip at 2 KHz

which is characterized by elevation of bone conduction thresholds of 5 dB at 500 Hz, 10 dB at 1000 Hz, 15 dB at 2000 Hz, and 5 dB at 4000 Hz. A Carhart notch may also be seen in cases of incudostapedial joint detachment and incus or malleus fixation. -Tympanometry =(type A) normal middle ear pressure with reduced compliance and absent stapedial reflex - Tomography may show thickening of the footplate of the stapes. D.Dx 1-Chronic non- suppurative OM 2- Ossicular disconnection or fixation    

Slide27

Slide28

Slide29

How is

Otosclerosis

Treated

1

-Do nothing (conservative approach

2--

Amplification

Hearing aids

are

usually effective for conductive hearing loss

3-

-

Medical treatment

To date, the only proposed medical treatment has been sodium fluoride, which is a dietary supplement (not a drug). This treatment is not widely accepted, and has not been proven to be effective4-surgical treatment:***The stapedotomy operation.. Do opening (fenestra) in footplate of stapes.*** The stapedectomy operation . when thick sclerosed footplate.Complication1-Loss of hearing due to sensory hair cell damage.2-Dizzenes loss of balance3-Taste disturbance chorda tympani damage4-Tinitus5-**Cohlear implant

Slide30

Meniere's Disease

WHAT IS MÉNIÈRE

`

’S DISEASE

?

M

é

ni

è

re

`

’s disease describes a

set of episodic symptoms

including vertigo

, intermittent hearing loss, tinnitus . Episodes of vertigo (spinning sensation) typically last from 20 minutes up to 4 hours. Hearing loss is often intermittent, , lattr

on

becomes permanent

.

Tinnitus

:-

A

roaring,

buzzing, or ringing sound in the ear

),

and a sensation of fullness in the affected ear.

may come and go with changes in hearing, occur during or just before attacks, or be constant.

M

é

ni

è

re

s

disease

is also called idiopathic endolymphatic hydrops and is one of the most common causes of dizziness. 

The disease

most

ly

unilateral

,

only 15% both ears

. 

Slide31

C

ause of Meniere's

disease

isn't well understood

Due to

abnormal volume

or

composition of fluid in the inner ea

r

.

Inner ear consist of bony labyrinth and inside it membranous labyrinth contain endolymph, there is sensory cell response for fluid movement ,pressure ,composition.In order to function properly, the fluid needs to retain a certain volume, pressure and chemical composition. Too much fluid may accumulate either due to excess production or inadequate absorption.. Alteration of the properties of inner ear fluid may help cause Meniere's disease.

Slide32

An

umber

s

of potential causes or triggers,

including

:

Faulty water metabolism

or

Improper fluid dra

i

nage, perhaps because of a blockage or anatomic abnormality

Sodium retentionDisturbance of hormonal control of water and electrolyte transportAbnormal immune response ( autoimmmune disease). Allergies (Histamine sensitivity).Sympathetic over activityViral infection Genetic predisposition Head trauma Migraines Because no single cause has been identified, it's likely that Meniere's disease is caused by a combination of factors

Slide33

Pathology:

-Distension of membranous labyrinth (especially in

scala

media of the cochlea and

saccule

). Distension of the

scala

media causes bulging of

reissner's

membrane into

scala

vestibule. The distended

saccule

may spread over the

stapedial footplate. Rupture of distended parts.-Degeneration of sensory elements.

Slide34

Clinical features

:

Usually unilateral at first. Slightly more common in males. Onset usually between 35-55 years.

-

Vertigo in attacks

, the duration varies from few minutes to two hours. Horizontal

nystagmus

usually to opposite side may be seen.

-

Sensorineural

deafness at first affects the lower frequencies

early

reversible but progress with each attack and the higher frequencies are increasingly involved and become permanent at the end. Recruitment (abnormal rapid growth of loudness, due to cochlear damage) also may be found.

-

Slide35

--

Tinnitus

.

-

Vagal disturbances

: nausea, vomiting even diarrhea, pallor, cold sweat and lowered blood pressure.

-

Headache

(Associated migraine in 7-20%)

-

Anxiety.

Slide36

Diagnosis

1-History

2-•

An audiometric examination

(hearing test PTA) indicates a sensory type of hearing loss in the affected ear. Speech discrimination is often diminished in the affected ear

.

3-Caloric

test

:between

the attacks show canal paresis.

4-For

balance

•An ENG (

electronystagmogram) with caloric test. In about 50 percent of patients, the balance function is reduced in the affected ear. 5-Other tests•Electrocochleography (ECoG) may indicate increased inner ear fluid pressure in some cases of Ménière’s disease.The auditory brain stem response (ABR), , computed tomography (CT), or magnetic resonance imaging (MRI) may be needed to rule out a tumor occurring on the hearing and balance nerve. Such tumors are rare, but they can cause symptoms similar to Ménière’s disease.

Slide37

Differential diagnosis

:

1-Labyrinthitis

2-Cogan's disease

3-Vestibular neuronitis

4-BPPV

5-Acoustic neuroma

6-Disseminated sclerosis

7-Epilepsy

Slide38

T

reatment

:

Conservativ

e

: -

Reassurance

- -

To reduce the

frequency of episodes by

Avoid stress and

excess fluid&

salt ingestion, caffeine, smoking, and alcohol. Get regular sleep and eat properly. Remain physically active, but avoid excessive fatigue.Attacks of vertigo can controlled by-  Sedation (diazepam)-  Labyrinthine sedatives (Cinnarizine, promethazine)-  Vasodilators (Betahistine hydrochloride)-  Removal of toxic foci-  Diuretics (if the attacks are related to menstrual cycle) -  Restriction of salts and fluid intake - Intratympanic injection with either gentamicin) vestibulotoxic)through temporary opening or placing a tube in the eardrum. But gentamycin cause SND -An air pressure pulse generator a mechanical pump that is applied to ear canal for 5 minutes 3 times a day -

Slide39

WHEN IS SURGERY RECOMMENDED

?

Surgery is needed in only a small minority of patients with Meniere

’s disease

.

If vertigo attacks are not controlled by conservative measures and are disabling, one of the following surgical procedures might be recommended:

•

Endolymphatic sac shunt or decompression

relieves attacks of vertigo in one-half

to two-thirds of cases

•

Selective vestibular neurectomy

:-. While vertigo attacks are permanently cured in a high percentage of cases, patients may continue to experience imbalance, hearing function is usually preserved.•Labryrinthectomy and eighth nerve section :. This is considered when the patient with Ménière’-s disease has poor hearing in the affected ear. This procedure will result in the highest rates for control of vertigo attacks.

Slide40

Presbycusis

Presbycusis (also spelled presbyacusis

,

from Greek

presbys

“elder” +

akousis

“hearing”

[1]

), or age-related

hearing loss

,

is the cumulative effect of aging on

hearing

. It is a progressive bilateral symmetrical age-related sensorineural hearing loss. The hearing loss is most marked at higher frequencies. There are four pathological types of presbycusis:Sensory: characterised by degeneration of organ of corti.Neural: characterised by degeneration of cells of spiral ganglion.Strial/metabolic: characterised by atrophy of stria vascularis in all turns of cochlea.

Cochlear conductive

:

due to stiffening of basilar membrane thus affecting its movement

Slide41

Slide42

Presentation

Bilateral high frequency

sensorineural

deafness,

poor speech

discrimination.

Deterioration in hearing has been found to

start

early

,

Age

affects high frequencies

more than low, and

men more frequently than women. The effects of aging can be exacerbated by exposure to environmental noise, This is noise-induced hearing loss (NIHL) and is distinct from presbycusis.Over time, the detection of high-pitched sounds becomes more difficult, and speech perception is affected, particularly of sibilants and fricatives. Both ears tend to be affected .

Slide43

Pathophysiology

Degeneration due to vascular insufficiency: Atrophy of epithelial, neural tissues and

stria

vascularis

in the cochlea.

Slide44

CAUSES

Factors that can cause hearing loss

Heredity

: Factors like early aging of the cochlea and susceptibility of the cochlea for drug insults are genetically determined.

Atherosclerosis

: May diminish vascularity of the cochlea, thereby reducing its oxygen supply.

Dietary habits

: Increased intake of

saturated fat

may accelerate atherosclerotic changes in old age.

Diabetes

: May cause vasculitis and endothelial proliferation in the blood vessels of the cochlea, thereby reducing its blood supply.

Noise trauma

: Exposure to loud noise/music on a continuing basis stresses the already hypoxic cochlea, hastening the presbycusis.Smoking: Is postulated to accentuate atherosclerotic changes in blood vessels aggravating presbycusis.Hypertension: Causes potent vascular changes, like reduction in blood supply to the cochlea, thereby aggravating presbycusis.Ototoxic drugs: Ingestion of ototoxic drugs like aspirin may hasten the process of presbycusis

Slide45

Treatments]

Devices like

hearing aids

and

cochlear implants

already help improve hearing of many elderly

.

Though still in their early stages, several treatments for presbycusis are in development. Included in these are the water-soluble

coenzyme Q

10

formulation,

.

In a study performed in 2010, it was found that the water-soluble formulation of coenzyme Q10 (CoQ10) caused a significant improvement in liminar tonal audiometry of the air and bone thresholds at 1000 Hz, 2000 Hz, 4000 Hz, and 8000 Hz.[6] It is likely that a larger clinical trial will be performed

Slide46

Ototoxicity

 

..

The extent of ototoxicity varies with the drug, the dose, and other conditions. In some cases, there is full recovery after the drug has been discontinued. In other cases, the extent of damage is limited, and may even be too small to be noticed. This may occur in highfrequency hearing loss,, there may be permanent and complete deafness

.

.

[9

]

Symptoms of ototoxicity

includ

e

mild ,

moderat,sever

or profound hearing

loss

.

vertigo

,

and tinnitus

.

[

9]

Definition

:

damage of the cochlear and or the vestibular part of the inner ear and or the

vestibulocochlear

nerve by drugs and chemical agent

Slide47

Causes and symptoms

Many drugs can cause ototoxicity.

Antibiotics

amikacin (Amikin)

streptomycin

neomycin

gentamicin (Garamycin(

erythromycin

.

kanamycin (

tobramycin (

netilmycin (Netromycin)

vancomycin (Vancocin

)

Anti-cancer drugscisplatin (Platinol AQ)

bleomycin (Blenoxane)

vincristine (Oncovin

)

Diuretics

acetazolamide (Diamox)

furosemide (Lasix)

bumetanide (Bumex)

ethacrynic acid (Edecrine

)

.

Aspirin

overdose causes ringing in the ears. The

antimalarial drugs

quinine and chloroquine may also cause ear damage. environmental chemicals that can cause ear damage

are tin, lead, mercury, carbon monoxide, and carbon disulfide.

Slide48

Treatment

There are no current treatments to reverse the effects of

ototoxicity.

Preventive

: Avoid or discontinue

ototoxic

drugs

                 

Monitor treatment.

                  Monitor hearing (How?)

Therapeutic

: no medical or surgical treatment is effective

.

People who suffer permanent hearing loss may elect to use hearing aids, or, when appropriate, receive a cochlear implant. For those who have balance problems, physical therapy may often be helpful. Physical therapists can help people with balance problems learn to rely more on vision and the sensations from muscles to achieve balance.