What is the Natural Course of

the Disease?

The sensory hearing loss is

attributed to leakage of enzy-

mes from bone into the inner

ear. Sensory hearing loss is

also attributed to atrophy of

the spiral ligament caused by

involvement of lamellar bone

at the inner surface of the co-

chlear capsule. A third cause

is vascular, due to degenera-

tion of the stria vascularis. In

early stages, treatment with

fluoride may be helpful. The

sensory component of the di-

sease may eventually lead to

complete deafness, but fortu-

nately, this is unusual. In most

cases, both ears are affected,

although in about 10 to 15%

of patients, hearing loss oc-

curs on one side only.

What is otosclerosis?

Otosclerosis is a disease of

the bones of the middle and

inner ear

The ossicles (bones) beco-

me knit together into an im-

movable mass, and do not

transmit sound as well as

when they are more flexible.

Otosclerosis can also affect

the other ossicles (malleus

and incus) and the otic cap-

sule.

OTOSCLEROSIS

How is otosclerosis treated?

There are four treatment opti-

ons:

1-Do nothing (conservative

approach)

2-Amplification

Hearing aids are usually effec-

tive for conductive hearing

loss.

3-Medical treatment

4-Surgical treatment

How is otosclerosis diagno-

sed?

Diagnosis is usually made

by a combination of family

history, progressive con-

ductive hearing loss pat-

tern, and exclusion of alter-

natives. Hearing tests may

initially show a sensory pat-

tern and later show the typi-

cal conductive loss pattern.

Acoustic reflexes may even-

tually be absent, but early

on may show the “on-off”

effect. Tympanometry often

shows stiffening of the ossi-

cular chain. A CT scan of

the temporal bone is speci-

fic but insensitive. It may be

the only way to document

otosclerosis early in the di-

sease.

OTOSCLEROSIS

What Causes Barotrauma?

Barotrauma is caused by a

difference in pressure bet-

ween the external environ-

ment and the internal parts of

the ear. Since fluids do not

compress under pressures

experienced during diving or

flying, the fluid-containing

spaces of the ear do not alter

their volume under these

pressure changes. However,

the air-containing spaces of

the ear do compress, resul-

ting in damage to the ear if

the alterations in ambient

pressure cannot be equalized.

What is Barotrauma?

Barotrauma refers to injury

sustained from failure to

equalize the pressure of an

air-containing space with

that of the surrounding envi-

ronment. The most common

examples of barotrauma oc-

cur in air travel and scuba di-

ving. Although the degree of

pressure changes are much

more dramatic during scuba

BAROTRAUMA

How is Barotrauma Treated?

For outer ear barotrauma, the

treatment consists of clearing

the ear canal of the obstruc-

tion, and restricting diving or

flying until the blockage is co-

rrected and the ear canal and

drum return to normal.For

middle ear barotrauma, treat-

ment consists of keeping the

ear dry and free of contamina-

tion that could cause infec-

tion. Topical nasal steroids

and decongestants may be

started in an attempt to de-

congest the eustachian tube

opening. The presence of pus

may prompt the use of appro-

priate antibiotics. For inner

ear barotrauma, treatment

consists of hospitalization

and bed rest with the head

elevated 30 to 40 degrees.

How is Barotrauma Diagno-

sed?

Diagnosis is initially based

on careful history. If the his-

tory indicates ear pain or diz-

ziness that occurs after di-

ving or an airplane flight, ba-

rotrauma should be suspec-

ted. The diagnosis may be

confirmed through ear exa-

mination, as well as hearing

and vestibular testing.

BAROTRAUMA

What Causes a Perilymph Fis-

tula?

Head injury or ear surgery

Pressure trauma

Congenital

Infection

Ear surgery, particu-

larly stapes surgery, often

causes fistula. Some patients

develop symptoms attributed

to fistula following airplane

decent. Fistulae may be pre-

sent from birth (usually in as-

sociation with deafness) or

may result from chronic ear

infections ca-

lled cholesteatomas.

What is a Perilymph Fistula?

A perilymph fistula (PLF) is

an abnormal opening bet-

ween the air-filled middle ear

and the fluid-filled inner ear.

It may occur due to a defect

in one of three locations:

Round window

Oval window

Otic capsuleIn most instan-

ces it is a tear or defect in

one or both of the small, thin

membranes between the

middle and inner ears. These

membranes are called the

“oval window” and the

“round window.” Figure 2

illustrates a round-window

fistula

PERILYMPH FISTULA

Surgery

If you have an otic capsule

fistula, if your symptoms are

significant and have not res-

ponded to the conservative

approach outlined above, or if

you have a progressive hea-

ring loss, surgical repair of

the fistula may be required.

For otic capsule fistulae invol-

ving a canal, surgery genera-

lly involves plugging of the

canal.

How is a Perilymph Fistula

Diagnosed?

Certain rare fistulae due to

tumor or bone defects (such

as superior canal dehiscen-

ce) are relatively easy to

diagnose because they can

be seen on magnetic reso-

nance imaging (MRI) or com-

puterized tomography (CT)

scan. However, for the ot-

hers, there is considerable

controversy about how to

make the diagno-

sis. Ménière’s disease, which

is much more common than

fistula, can have identical

symptoms, including pressu-

re sensitivity. For this

reason, fistula diagnoses

made in patients without ba-

rotrauma are easily questio-

ned.

PERILYMPH FISTULA

Medications

For persons with plugged

up eustachian tubes (such

as due to a cold or

allergy), decongestants,

allergy medication, and

ventilating tubes may be

of use. Medications in the

minor tranquilizer family

such as diazepam

(Valium), klonazepam and

lorazepam help some indi-

viduals. Antivert and phe-

nergan are also medica-

tions which some find hel-

pful.

What Causes Acoustic Neuro-

ma?

Acoustic neuroma occurs in

two forms: a sporadic form

and a form associated with an

inherited syndrome. About

95% of all cases are sporadic.

The cause of the sporadic

form is unclear. Some small

studies have found an asso-

ciation of acoustic neuromas

with cellular phone use or

prolonged exposure to loud

noises, but other studies do

not find this link

The acoustic neuroma, also

known as vestibular schwan-

noma,or an acoustic neuro-

ma, is a nonmalignant tumor

of the 8th cranial nerve. Most

commonly, it arises from the

covering cells (Schwann ce-

lls) of the inferior vestibular

nerve . Acoustic neuromas

comprise about 6% of all in-

tracranial tumors, about 30%

of brainstem tumors, and

about 85% of tumors in the

region of the cerebelloponti-

ne angle. (source: National

Institutes of Health).

ACOUSTIC NEUROMA

Only about 10 tumors are

newly diagnosed each

year per million persons in

the United States, corres-

ponding to between 2,000

and 3,000 new cases each

year. In patients with hea-

ring asymmetry, it is belie-

ved that only about 1 in

1,000 has acoustic neuro-

ma

How is Acoustic Neuroma

treated?

Gamma Knife

This is a method of irradiating

the tumor, invented by Lars

Leksell in 1971. Gamma knife

stereotactic radiosurgery has

become more prevalent re-

cently as it has been demons-

trated to be safe and effective

in the control of acoustic neu-

romas (Likhterov, 2007). Gam-

ma knife does not generally

make tumors go away — Figu-

re 3 is actually that of a pa-

tient who had gamma knife

surgery several years prior.

Instead, gamma knife radia-

tion shrinks the tumor and

prevents future growth in

most patients.

How is Acoustic Neuroma

Diagnosed?

Conventional audiometry is

the most useful diagnostic

test for acoustic neuroma.

The most common abnorma-

lity is an asymmetrical high-

frequency sensorineural

hearing loss. However, recall

that about 1 in 1,000 patients

with hearing asymmetry ha-

ve acoustics. It has been es-

timated that 5% of

persons with senso-

rineural hearing loss

have acoustics.

ACOUSTIC NUEROMA

(Daniels et al 2000), but

this estimate is suspect as

it would imply a much

higher prevalence of

acoustic neuromas than

are commonly accepted.

Electronystagmography

(ENG testing) is frequently

abnormal, and about 50%

of all tumors are associa-

ted with unilateral loss of

What Causes Tinnitus?

Tinnitus is usually static noi-

se in the auditory system

that is associated with loss

of sound from the external

environment. Therefore, tin-

nitus is common and in

most, but not all, cases it is

associated with some degree

of hearing loss. The most

common causes of tinnitus

are damage to the high fre-

quency hearing by exposure

to loud noiseor elevated le-

vels of common drugs that

can be toxic to the inner ear

in high doses.

What is Tinnitus?

Tinnitus (pronounced “tin-it-

us”) is a noise heard by the

patient which is not caused

by any sound in the environ-

ment. Tinnitus is common —

nearly 36 million Americans

have tinnitus and more than

half of the normal population

has intermittent tinnitus.

About 6% of the general po-

pulation has what they con-

sider to be severe tinnitus.

Tinnitus

Tinnitus can come and

go, or be continuous. It

can sound like a low

roar, or a high pitched

ring. Tinnitus may be in

both ears or just in one

ear. Seven million Ame-

ricans are so severely

affected that they can-

not lead normal lives.

How Is Tinnitus Treated?

If a specific cause for tinnitus

is determined, it is possible

that treating the cause will eli-

minate the noise. If a specific

cause is not found, it is unli-

kely that the tinnitus can be

eliminated.

In most cases of tinnitus, the

sound is an abnormal audi-

tory sense perception of a

sound that is really neither in

the body nor coming from the

outside. Our sensory systems

are very adaptable. We get

used to all kinds of smells

that on first exposure seem

intolerable. We also get used

to sensations on the skin and

in the mouth that, at first, bot-

her us. Actually, we appear to

learn to ignore sounds from

our body.

How is Tinnitus Diagnosed?

Because tinnitus is a sym-

ptom rather than a disease, it

is important to evaluate the

underlying cause. Persons

who experience tinnitus

should be seen by a physi-

cian expert in ear disease,

typically an otolaryngologist.

The ears need to be exami-

ned and a hearing test obtai-

ned. Be sure to tell your doc-

tor what medications you ha-

ve been taking. Other tests

may also be indicated. There

are some causes for tinnitus

which need to be treated. For

example, tinnitus may be the

earliest sign of pressure on

an auditory nerve.

Tinnitus