Asstprof drFALIH ALANBAKY Hearing impairment deafness or hearing loss refers to the inability to hear or understand things either totally or partially Symptoms may be mild moderate ID: 911465
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Slide1
DEAFNESS,,,,,HEARING LOSS
Asst.prof dr.FALIH AL-ANBAKY
Slide2Hearing 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
.
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.
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.
Slide5How do we hear things?
Slide6Three 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.
Slide7Conductive 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.
Slide8Treatments 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
Slide9Sensorineural 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
Slide10Treatment 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.
Slide11Treatment 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
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, .
Slide13Degree 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
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
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.
Slide16The 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
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
Slide21Otosclerosis
Definition:
a localized disease of the
otic
capsule in which new spongy bone causes
ankylosis
of the footplate of stapes or invades the cochlea.
.
Slide22Aetiology
:
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)
Slide23Pathology
:
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
.
Slide24Clinical 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.
Slide25On 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
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
Slide27Slide28Slide29How 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
Slide30Meniere'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
.
Slide31C
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.
Slide32An
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
Slide33Pathology:
-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.
Slide34Clinical 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.
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 testsElectrocochleography (ECoG) may indicate increased inner ear fluid pressure in some cases of Ménières 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 Ménières disease.
Slide37Differential diagnosis
:
1-Labyrinthitis
2-Cogan's disease
3-Vestibular neuronitis
4-BPPV
5-Acoustic neuroma
6-Disseminated sclerosis
7-Epilepsy
Slide38T
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 -
Slide39WHEN 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.
Slide40Presbycusis
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
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 .
Slide43Pathophysiology
Degeneration due to vascular insufficiency: Atrophy of epithelial, neural tissues and
stria
vascularis
in the cochlea.
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
Slide45Treatments]
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
Slide46Ototoxicity
..
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
Slide47Causes 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.
Slide48Treatment
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.