Deafness & Otosclerosis

MIDHUN J

Hearing loss non organic organic conductive

neural

sensorineural
sensory

(cohclear)

peripheral

central

Conductive hearing loss and its management

Any disease process which interfears with the conduction of sound to reach cochlea causes conductive hearing loss Aetiology of conductive hearing loss Congenital or acquired

Congenital
Meatal atrasia Fixation of stapes foot plate Fixation of mallous head Ossicular discontinuty Congenital cholesteatoma

Acquired

External ear- obstruction in the ear canal Eg:- wax, foreign body Middle ear 1. perforation of tympanic membrane 2. fluid in the middle ear Eg:-acute otitis media serous otitis media or hemotympanum

3. mass in middle ear

Eg:-benign or malignant tumour 4. fixation of ossicle Eg:-otosclerosis 5. disruption of ossicle 6. eustachain tube blockage Eg:- retracted tympanic membrane serous otitis media

Management
1. removal of canal obstruction 2. removal of fluids 3. removal of mass from middle ear 4. stpedectomy 5. tympanoplasty 6. hearing aids

Sensorineural hearing loss&its management

SNHL result from lesion of the cochlea, 8th cranial nerve or central auditory path way it may present at birth (congenital) or start later at life (acquired)

Aetiology of SNHL
Congenital - anomalies of inner ear or damage to hearing apparatus by prenatal or perinatal factors Acquired Infection of labyrinth, Trauma of labyrinth or 8 cranial nerve (Eg fracture of temporal bone) Noise induced hearing loss, Auto toxic drugs, Familial progressive SNHL

Management
Early detection SNHL is important as measures can be taken to stop its progress, reverse it or to start an early rehabilitation program so essential for communication

SPECIFIC FORMS OF HEARING LOSS

A)INFLAMMATION OF LABRYNTH
IT MAY BE VIRAL, BACTERIAL OR

SYPHILITIC 1)VIRAL LABRYNTHITIS viruses usually reach the inner ear by blood stream affecting stria vascularis & then endolymph & organ of corti Measles, mumps, CMV ,rubella

BACTERIAL
BACTERIAL INFECTIONS REACH

LABYRINTH THROUGH THE MIDDLE EAR OR THROUGH CSF

3)SYPHILITIC
SENSORINEURAL HEARING LOSS IS

CAUSED BY BOTH CONGENITAL & ACQUIRED SYPHILIS DIAGNOSIS OF OTOSYPHILIS MADE BY OTHER CLINICAL EVIDENCE OF AQUIRED & CONGENITAL SYPHILIS AND LAB TEST

T/T: PENICILLIN AND STEROIDS

B)FAMILIAL PROGRESSIVE SENSORINEURAL HEARING LOSS

IT IS A GENETIC DISORDER IN WHICH THERE

IS A PROGRESSIVE DEGENERATION OF COCHLEA DEAFNESS IS BILATERAL WITH FLAT OR BASIN SHAPED AUDIOGRAM BUT AN EXCELLENT SPEECH DISCRIMINATION

C)OTOTOXICITY
A)AMINOGLYCOSIDE ANTIBIOTICS Streptomycin Gentamycin B)DIURETICS Furosemide Ethacrynic acid

 C) ANTIMALARIALS Quinine Chloroquine D)CYTOTOXIC DRUGS

Cisplatin
Carboplatin E)ANALGESICS

Salicylates
Indomethacin F)CHEMICALS

Alcohol
Tobaco

 G)MISCELLANEOUS
Erythromycin Ampicillin

D)NOISE TRAUMA
HEARING LOSS ASSOCIATED WITH

EXPOSURE TO NOISE HAS BEEN WELL NOTED IN BOILER MAKERS, IRON AND COPPER SMITHS AND ARTILLARY MEN

Hearing loss associated with execissive noise can be divided into 2 groups

1)Acoustic trauma :permanent

damage to hearing can be caused by a single brief exposure to very high intense sound. Eg- explosion, gun fire or a powerful cracker Sudden loud sound may damage outer hair cells,disrupt the organ of corti and rupture the reissners membrane

2)NOISE INDUCED HEARING LOSS

IN THIS CASE CHRONIC EXPOSURE TO LESS

INTENSE SOUND CAUSE HEARING LOSS A frequency of 2000-3000Hz causes more damage than lower or higher Hz

E)SUDDEN HEARING LOSS

ITS IS DEFINED AS A SENSORINEURAL

HEARING LOSS THAT HAS DEVELOPED OVER A PERIOD OF HRS OR FEW DAYS MOSTLY IT IS UNILATERAL

AETIOLOGY
1)INFECTIONS:mumps, herpes zoster,

meningitis 2)TRAUMA: head injury, ear operations 3) VASCULAR: Hemorrhage, Embolism, Thrombosis of Labyrinthine or cochlear artery 4) Ear(otology): Minieres disease, Cogans syndrome 5) Toxic: Otoxic drugs, Insectisides

 6.NEOPLASTIC-Acoustic

neuroma,carcinomatous neuropathy 7.MISCELLANEOUS-multiple sclerosis,hypothyroidism 8.PSYCHOGENIC

F)PRESBYCUSIS
SENSORINEURAL LOSS ASSOICIATED WITH

PHYSIOLOGICAL AGING PROCESS IN THE EAR IS CALLED PRESBYCUSIS 4 PATHOLOGICAL TYPES OF PRES BYCUSIS HAVE BEEN IDENTIFIED

 1)SENSORY characterised by degeneration of organ of corti Higher frequencies are affected 2)NEURAL characterised by degeneratin of cells of spiral ganglion Higher auditory path ways are also affected 3)STRIAL OR METABOLIC this is characterised by atrophy of stria vascularis in all turns of cochlea.audiogram is flat 4)COCHLEAR CONDUCTIVE this is due to

stiffening of the basillar membrane thus affecting its movements Audiogram is sloping type

Non organic Hearing loss (NOHC)

In this type, there is no organic lesion. Either due to malingering or psychogenic.

Hearing loss and Deafness

Hearing loss :- impairment of hearing and its severity may varies from mild to severe or profound Deafness :- when there is little or no hearing at all

In 1980, WHO recommended that, the term deaf should be applied only to those individuals whose hearing impairment is so severe that they are unable to benefit from any type of amplification. A similar definition is used in our country while extending benefits to the hearing handicapped.

Definition of Deaf (Ministry of social welfare, Government of INDIA Scheme of assistance to hearing handicapped) The deaf are those in whom the sense of hearing is non-functional for ordinary purposes of life They do not hear/understand sounds at all even with amplified speech. The cases included in the category will be those having hearing loss more than 90 dB in the better ear (profound impairment) or total loss of hearing in both ears.

OTOSCLEROSIS

INTRODUCTION
Otosclerosis Hardening of the ear Otosclerosis is a primary & exclusive disease of otic capsule

(bony labrinth) & ossicles charecterised by alternate phases of bone resorption and formation. Mature lamellar bone is removed by osteoclasis & replaced by woven bone of greater thickness cellularity & vascularity Seen exclusively in humans. Clinical otosclerosis-location of bony changes or its secondary effects results in evident clinical manifestations. Histological otosclerosis-bony changes are present, but not clinically manifested.

Most often otosclerosis focus involves the stapes region leading to stapes fixation and conductive deafness. However it may involve certain other areas of the bony labyrinth where it may cause neurosensory loss or no symptoms at all

Aetiology
Exact cause otosclerosis is not known Heriditary- About 50% of otosclerosis have

positive family history.Rest are sporadic Race- Whites are affected more than negros Sex- Females are affected twice as often as males. In our country males are most affected Age of onset-Deafness is usually seen between 20-30 years of age and is rare before 10 and after 40 years

Types of Otosclerosis

Stapedial Otosclerosis Cochlear Otosclerosis Histologic Otosclerosis

 Stapedial Otosclerosis causing stapes fixation and conductive deafness is the most common variety
Cochlear Otosclerosis involves region of round

window or other areas in the otic capsule and may cause sensory neural hearing loss probably due to liberation of toxic materials into the inner ear fluids

Histologic Otosclerosis This type of otosclerosis remains unsymptomatic and causes neither conductive nor sensory neural hearing loss

stapedial otosclerosis

Pathology
Grossly- Lesion appears chalky white

grayish or yellow Microscopically- spongy bone appears in the normally dense enchondral layer of otic capsule

Symptoms
1.

2.

3. 4.

5.

Hearing loss Paacusis Willisii- an otosclerotic patient hears better in noicy than quite surrounding .this is because a normal person will race his voice in noicy surrounding Tinnitus- It is more commonly seen in cochlear otosclerosis and in active lesions Vertigo- It is an uncommon symptom speech Patient has a monotonous well modulated soft speech

Signs
1. Tympanic membrane is quite normal and

mobile .sometimes a reddish hue may be seen on the promontory through the tympanic membrane (Schwartze sign). This is indicative of active focus with increased vascularity 2. Eustachian tube function is normal 3. Tuning fork test show negative Rinne(i.e, BC>AC)

Differential Diagnosis
Serous otitis media Adhesive otitis media Typanosclerosis Attic fixation of head of malleus Ossicular discontinuity or congenital stapes

fixation

Treatment
Medical- No medical treatment Surgical- Stapidectomy with prosthesis

replacement is the treatment of choice . In 90% of patients there is good improvement in hearing after stapidectomy

ThankYou You...... Thank