meniere’s disease & otosclerosis

8/2/2019 MENIERES DISEASE & OTOSCLEROSIS

1/44

MENIERES DISEASE


2/44

Meniere's disease ( endolymphatic hydrops ) is a

disorder of the inner ear that causes spontaneous

episodes of vertigo a sensation of a spinning

motion along with fluctuating hearing loss,

ringing in the ear (tinnitus), and sometimes a

feeling of fullness or pressure in your ear.

In most cases, Meniere's disease affects only one

ear.


3/44

CAUSES :

Endolymphatic hydrops:

-hydrops is when the inner ear membranes

look distended.

-progressive dilation of the inner ear

structures causes micro-ruptures in the

delicate membranes causing contamination of

the inner ear nerve endings.


4/44


5/44

Autoimmune:

-There is a large body of evidence that the

bodys own immune mechanism may be

responsible for this disorder.

-Researchers have found antibodies toinner ear proteins circulating in the

bloodstreams of many patients with the

disorder.


6/44

Viral: viral particles have been identified

within the inner ear of many Menieres

Patients

Sodium and water retention

Hypothyroidism

Allergy


7/44

SYMPTOMS :

Menieres disease is usually characterized 4symptoms:

Periodic episodes of rotatory vertigo or dizziness. Fluctuating, progressive, low-frequency hearing loss

Tinnitus

A sensation of "fullness" or pressure in the ear


8/44

Tests and diagnosis

Hearing assessment

Electronystagmography (ENG)

Rotary-chair testing

Vestibular evoked myogenic potentials (VEMP)

testing

Magnetic resonance imaging (MRI).Computerized tomography (CT).

Auditory brainstem response audiometry


9/44

Treatments and drugs

Motion sickness medicationsAnti-nausea medications

Hearing aid

Middle ear injections

Vestibular nerve section

Labyrinthectomy


10/44

Benign PositionalVertigo

Stroke, Ischemic

Headache, Migraine SubarachnoidHemorrhage

Hypothyroidism andMyxedema Coma

Temporal Lobe Epilepsy

Labyrinthitis Toxicity, Salicylate

Multiple Sclerosis Transient IschemicAttack

Otitis Media Vestibular Neuronitis

Differential Diagnoses
http://emedicine.medscape.com/article/791414-overviewhttp://emedicine.medscape.com/article/791414-overviewhttp://emedicine.medscape.com/article/793904-overviewhttp://emedicine.medscape.com/article/792267-overviewhttp://emedicine.medscape.com/article/794076-overviewhttp://emedicine.medscape.com/article/794076-overviewhttp://emedicine.medscape.com/article/768053-overviewhttp://emedicine.medscape.com/article/768053-overviewhttp://emedicine.medscape.com/article/1184509-overviewhttp://emedicine.medscape.com/article/226-overviewhttp://emedicine.medscape.com/article/818242-overviewhttp://emedicine.medscape.com/article/793013-overviewhttp://emedicine.medscape.com/article/794281-overviewhttp://emedicine.medscape.com/article/794281-overviewhttp://emedicine.medscape.com/article/764006-overviewhttp://emedicine.medscape.com/article/794489-overviewhttp://emedicine.medscape.com/article/794489-overviewhttp://emedicine.medscape.com/article/794489-overviewhttp://emedicine.medscape.com/article/764006-overviewhttp://emedicine.medscape.com/article/794281-overviewhttp://emedicine.medscape.com/article/794281-overviewhttp://emedicine.medscape.com/article/793013-overviewhttp://emedicine.medscape.com/article/818242-overviewhttp://emedicine.medscape.com/article/226-overviewhttp://emedicine.medscape.com/article/1184509-overviewhttp://emedicine.medscape.com/article/768053-overviewhttp://emedicine.medscape.com/article/768053-overviewhttp://emedicine.medscape.com/article/794076-overviewhttp://emedicine.medscape.com/article/794076-overviewhttp://emedicine.medscape.com/article/792267-overviewhttp://emedicine.medscape.com/article/793904-overviewhttp://emedicine.medscape.com/article/791414-overviewhttp://emedicine.medscape.com/article/791414-overview


11/44

OTOSCLEROSIS


12/44

Otosclerosis is a disease of abnormal sponge-like

bone growth in the middle ear. This growthprevents the ear from vibrating in response to

sound waves.

Otosclerosis is a common cause of hearing

impairment and is hereditary


13/44

Otosclerosis causes abnormal

bone material on the Stapes


14/44

Cochlear Otosclerosis:

When otosclerosis involves the hearingportion of the inner ear a sensorineural

hearing impairment may result due to

interference with the nerve function.

This nerve impairment is called cochlear

otosclerosis and once it develops it may bepermanent.


15/44

Vestibular Otosclerosis:

On occasion the otosclerosis may spread to the

balance canals and may cause episodes ofunsteadiness or spinning sensations.

This may occur in the absence of anymeasurable hearing loss.


16/44

Stapedial Otosclerosis:

Otosclerosis commonly involves the bone around

the stapes or stirrup bone, the final link in the chain

of middle ear bones.

This stapes rests in the small groove, the ovalwindow, in intimate contact with the inner ear

fluids.

Anything that interferes with its motion results in a

conductive hearing impairment.

This type of impairment is called stapedial

otosclerosis and is usually correctable by surgery.


17/44

Epidemiology

Race incidence of microscopic otosclerosis

Caucasian 10%

Asian 5%

African American 1%

Native American 0%


18/44

Epidemiology

Sex variation (M:F=1:2.5)

Women more commonly seek medical attention

for hearing loss secondary to otosclerosis, histologic studies prevalence of otosclerosis show

no difference in men versus women.


19/44

Epidemiology

Age

The incidence of otosclerosis increases with age.

The most common age group presenting withhearing loss from otosclerosis is 15-45 years,

however it has been reported to manifest as early

as 7 years and as late as the mid 50s.


20/44

Pathophysiology

Otosclerosis (otospongiosis) is an osseous

dyscrasia, limited to the temporal bone, and

characterized by resorption and formation ofnew bone in the area of the ossicles and otic

capsule.


21/44

Pathophysiology

otosclerosis has two main forms:

an early of spongiotic phase (otospongiosis)

a late or sclerotic phase


22/44

Pathophysiology

Microscopically, a focus of active otosclerosis

reveals finger projections of disorganized bone,

rich in osteocytes particularly at the leadingedge. In the center of the focus,

multinucleated osteocytes are often present.

In the sclerotic phase


23/44

Hearing loss

Tinnitus

Paracusis

Vertigo

Symptoms


24/44

Diagnosis

of Otosclerosis


25/44

Physical Exam

Otomicroscopy

Most helpful in ruling out other disorders

Middle ear effusions

Tympanosclerosis

Tympanic membrane perforations Cholesteatoma or retraction pockets

Superior semicircular canal dehiscence

Schwartzes sign

Red hue in oval window area

10% of cases

Pneumatic otoscopy

Distinguish from malleus fixation


26/44

Physical Exam

Tuning forks

Audiometry :

- Tympanometry-Impedance Testing

-Acoustic reflexes

Pure tones


27/44

Acoustic Reflexes

Result from a change in the middle ear

compliance in response to a sound stimulus

Change in compliance

Stapedius muscle contraction

Stiffening of the ossicular chain

Reduces the sound transmission to the vestibule


28/44

Acoustic Reflexes

Otosclerosis has a predictable pattern of

abnormal reflexes over time

Reduced reflex amplitude

Elevation of ipsilateral thresholds

Elevation of contralateral thresholds

Absence of reflexes


29/44

Pure Tone Audiometry

Most useful audiometric test for otosclerosis

Characterizes the severity of disease

Frequency specific

Carharts notch

Hallmark audiologic sign of otosclerosis

Decrease in bone conduction thresholds

5 dB at 500 Hz

10 dB at 1000 Hz

15 dB at 2000 Hz

5 dB at 4000 Hz


30/44

Imaging

Computed tomography (CT) of the temporalbone

Proponents of CT for evaluation of otosclerosis

Pre-op

Characterize the extent of otosclerosis

Severe or profound mixed hearing loss

Evaluate for enlarge cochlear aqueduct

Post-op

Recurrent CHL

Re-obliteration vs. prosthesis dislocation

Vertigo


31/44

TREATMENT

Hearing aidsAt first, when the hearing loss is mild, patient may not need

any treatment.

As the disease progresses and hearing loss becomes worse,hearing aids can make a big difference.

However, when the hearing loss is severe, hearing aids may

not be of much help.


32/44

STAPEDECTOMY

Stapedectomy consists of removing a portion of the

sclerotic stapes footplate and replacing it with an

implant that is secured to the incus. This procedurerestores continuity of ossicular movement and allows

transmission of sound waves from the eardrum to

the inner ear.

Surgical Steps


33/44

STAPEDOTOMY

A modern variant of this surgery called a

stapedotomy, is performed by drilling a small

hole in the stapes footplate with a micro-drill

or a laser, and the insertion of a piston-likeprothesis.


34/44

Medical

Sodium Fluoride

Reduces tinnitus, reverses Schwartzes sign, resolution of

otospongiosis seen on CT

Dose 20-120mg Indications

Non-surgical candidates

Patients who do not want surgery

Surgical candidates with + Schwartzes sign

Treat for 6 mo pre-op

Postop if otospongiosis detected intra-op


35/44

Medical

Bisphosphonates

Class of medications that inhibits bone resorption by

inhibiting osteoclastic activity

Often supplement with Vitamin D and Calcium.


36/44

Differential Diagnosis

Ossicular discontinuity

Congenital stapes fixation

Malleus head fixation

Pagets disease

Osteogenesis imperfecta

Superior semicircular canal dehiscence


37/44

SENSORINEURAL

HEARING LOSS

Sensorineural hearing loss can be mild moderate


38/44

Sensorineural hearing loss can be mild, moderate,

or severe, including total deafness.

The great majority of human sensorineural hearing

loss is caused by abnormalities in the hair cells of

the organ of Corti in the cochlea.

There are also very unusual sensorineural hearing

impairments that involve the VIIIth cranial nerve

(the Vestibulocochlear nerve) or the auditoryportions of the brain.


39/44

Sensorineural causes:


40/44

Sensorineural causes:

Sensorineural causes are from damage to the hair

cells or nerves that sense sound waves.

Acoustic trauma - Prolonged exposure to loud

noises causes the hair cells on the cochlea to

become less sensitive.

pressure trauma or ear squeeze - Usually in

divers

Head trauma - A fracture of the temporal

bone can disrupt the nerves of the auditory

system

Ototoxic drugs - Certain drugs can affect hearing by


41/44

Ototoxic drugs - Certain drugs can affect hearing by

damaging the nerves involved in hearing. Usually this

occurs when large or toxic doses are used but may

also occur with lower doses.oAntibiotics including aminoglycosides

(gentamicin, vancomycin), erythromycins, and

minocyclineoDiuretics including furosemide and ethacrynic

acid

oSalicylates (aspirin) and nonsteroidal anti-

inflammatories (NSAIDs) such as ibuprofen and

naproxen

oAntineoplastics (cancer drugs)
http://www.emedicinehealth.com/script/main/art.asp?articlekey=102964http://www.emedicinehealth.com/script/main/art.asp?articlekey=103188http://www.emedicinehealth.com/script/main/art.asp?articlekey=102770http://www.emedicinehealth.com/script/main/art.asp?articlekey=102954http://www.emedicinehealth.com/script/main/art.asp?articlekey=102781http://www.emedicinehealth.com/script/main/art.asp?articlekey=102781http://www.emedicinehealth.com/script/main/art.asp?articlekey=102132http://www.emedicinehealth.com/script/main/art.asp?articlekey=101875http://www.emedicinehealth.com/script/main/art.asp?articlekey=101939http://www.emedicinehealth.com/script/main/art.asp?articlekey=2580http://www.emedicinehealth.com/script/main/art.asp?articlekey=2580http://www.emedicinehealth.com/script/main/art.asp?articlekey=101939http://www.emedicinehealth.com/script/main/art.asp?articlekey=101875http://www.emedicinehealth.com/script/main/art.asp?articlekey=102132http://www.emedicinehealth.com/script/main/art.asp?articlekey=102781http://www.emedicinehealth.com/script/main/art.asp?articlekey=102781http://www.emedicinehealth.com/script/main/art.asp?articlekey=102781http://www.emedicinehealth.com/script/main/art.asp?articlekey=102954http://www.emedicinehealth.com/script/main/art.asp?articlekey=102770http://www.emedicinehealth.com/script/main/art.asp?articlekey=103188http://www.emedicinehealth.com/script/main/art.asp?articlekey=102964


42/44

Vascular diseases (problems with blood vessels)

include sickle cell disease, diabetes, leukemia and

diseases in which excessive blood clotting occurs.

Children and adults with kidney problems are

more susceptible to sensorineural hearing loss

Mnire disease - A disease that affects hearing

and balance. It is usually associated with tinnitus

(ringing in the ears). It has a gradual onset and

often progresses to deafness and severe vertigo.


43/44

Infections

oMumps

oMeaslesoInfluenza

oHerpes simplex

oHerpes zoster

oMononucleosisoSyphilis

oMeningitis

Aging

Acoustic neuroma - A tumor in the auditory nerve. Usually

associated with ringing in the ears.


44/44

TREATMENT

hearing aids

cochlear implants is an electronic device implanted

behind the ear. Unlike a hearing aid that amplifies

sounds, cochlear implants directly stimulate the auditory

nerve fibers in the cochlea

Some research suggests that idebenone alone or

combined with Vitamin E may delay the onset of hearingloss or perhaps reverse it. Use of these agents for this

purpose is considered experimental at this time.

meniere’s disease & otosclerosis

Documents