MENIERES DISEASE

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.

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.

Autoimmune: -There is a large body of evidence that the bodys own immune mechanism may be responsible for this disorder.
-Researchers have found antibodies to inner ear proteins circulating in the bloodstreams of many patients with the disorder.

Viral: viral particles have been identified within the inner ear of many Menieres Patients
Sodium and water retention Hypothyroidism Allergy

SYMPTOMS :

Menieres disease is usually characterized 4 symptoms: Periodic episodes of rotatory vertigo or dizziness. Fluctuating, progressive, low-frequency hearing loss Tinnitus A sensation of "fullness" or pressure in the ear

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

Treatments and drugs

Differential Diagnoses
Benign Positional Vertigo Headache, Migraine Stroke, Ischemic

Subarachnoid Hemorrhage Temporal Lobe Epilepsy

Hypothyroidism and Myxedema Coma Labyrinthitis Multiple Sclerosis

Toxicity, Salicylate Transient Ischemic Attack Vestibular Neuronitis

Otitis Media

OTOSCLEROSIS

Otosclerosis is a disease of abnormal sponge-like bone growth in the middle ear. This growth prevents the ear from vibrating in response to sound waves. Otosclerosis is a common cause of hearing impairment and is hereditary

Otosclerosis causes abnormal bone material on the Stapes

Cochlear Otosclerosis: When otosclerosis involves the hearing portion 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 be permanent.

Vestibular Otosclerosis: On occasion the otosclerosis may spread to the balance canals and may cause episodes of unsteadiness or spinning sensations. This may occur in the absence of any measurable hearing loss.

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 oval window, 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.

Epidemiology
Race incidence of microscopic otosclerosis Caucasian 10% Asian 5% African American 1% Native American 0%

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.

Epidemiology
Age
The incidence of otosclerosis increases with age. The most common age group presenting with hearing 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.

Pathophysiology
Otosclerosis (otospongiosis) is an osseous dyscrasia, limited to the temporal bone, and characterized by resorption and formation of new bone in the area of the ossicles and otic capsule.

Pathophysiology
otosclerosis has two main forms:
an early of spongiotic phase (otospongiosis) a late or sclerotic phase

Pathophysiology
Microscopically, a focus of active otosclerosis reveals finger projections of disorganized bone, rich in osteocytes particularly at the leading edge. In the center of the focus, multinucleated osteocytes are often present. In the sclerotic phase

Symptoms
Hearing loss Tinnitus

Paracusis
Vertigo

Diagnosis of Otosclerosis

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

Physical Exam
Tuning forks Audiometry : - Tympanometry -Impedance Testing
-Acoustic reflexes

Pure tones

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

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

Pure Tone Audiometry

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

Imaging
Computed tomography (CT) of the temporal bone
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

TREATMENT Hearing aids At 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.

Surgical Steps
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 procedure restores continuity of ossicular movement and allows transmission of sound waves from the eardrum to the inner ear.

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-like prothesis.

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

Medical
Bisphosphonates
Class of medications that inhibits bone resorption by inhibiting osteoclastic activity Often supplement with Vitamin D and Calcium.

Differential Diagnosis
Ossicular discontinuity Congenital stapes fixation Malleus head fixation Pagets disease Osteogenesis imperfecta Superior semicircular canal dehiscence

SENSORINEURAL HEARING LOSS

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 auditory portions of the brain.

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 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 minocycline oDiuretics including furosemide and ethacrynic acid oSalicylates (aspirin) and nonsteroidal antiinflammatories (NSAIDs) such as ibuprofen and naproxen oAntineoplastics (cancer drugs)

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.

Infections oMumps oMeasles oInfluenza oHerpes simplex oHerpes zoster oMononucleosis oSyphilis oMeningitis Aging

Acoustic neuroma - A tumor in the auditory nerve. Usually associated with ringing in the ears.

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 hearing loss or perhaps reverse it. Use of these agents for this purpose is considered experimental at this time.