The diagnosis of otoclerosis has been established as the cause of your hearing impairment. Otosclerosis means hardening of one of the bones of the ear. Approximately one-third of all persons with impaired hearing have this condition. Since otosclerosis is hereditary, someone in your family had this condition and passed it down to you. You may not be aware of the presence of the hearing loss in your family because it may date back many generations. The fact that you have otosclerosis does not necessarily mean that your children will be affected. Because otosclerosis is hereditary, diseases such as scarlet fever, ear infections, measles, influenza, etc., have no relationship to its development.

In your case, even before you knew you had a hearing impairment, had we been able to examine your inner ear under a microscope, we would have seen minute areas of hardening (sclerosis). When this hardening process spreads to involve the stapes bone, a conductive (mechanical) hearing loss results, due to impairment of its vibrations.

Appropriately identified adults as well as profoundly deaf children (starting at age 12 months) can be implanted. Research demonstrates that the earlier a deaf child is implanted, the better the long term result will be with respect to speech and language development. Following surgery, rehabilitation is necessary, as the child must learn to associate the sound signals with normal sounds. Regarding deaf adults, research suggests that adults who receive cochlear implants are less lonely, have less social anxiety, are more independent, have increased social and interpersonal skills, and of course, they hear better with the cochlear implant!

FUNCTION OF THE NORMAL EAR
The ear is divided into three parts:

• External ear (the outer ear canal)
• Middle ear
• Inner ear

Sound vibrations enter the external ear canal and cause the ear drum membrane (tympanic membrane) to vibrate. Movements of the tympanic membrane are transmitted across the three middle ear bones (hammer or malleus, anvil or incus and stirrup or stapes). These middle ear bones act as a transformer, changing sound vibrations in air into fluid waves in the inner ear. The fluid waves bend delicate nerve endings (hair cells) in the cochlea and electrical impulses occur. These electrical impulses then are transmitted by the hearing nerve (cochlear nerve) to the brain, where they are interpreted as understandable sounds.

CAUSES OF HEARING IMPAIRMENT
If there is some difficulty in the external (outer) or middle ear, a conductive hearing impairment occurs. If the hearing loss is secondary to problems in the inner ear, a sensorineural hearing impairment occurs (as a result of the loss of hair cells). When there is difficulty in both the middle and inner ear, a mixed or combined impairment exists. Mixed impairments are common in otosclerosis because of stapes fixation and also because of the bony changes which occur in the cochlea.

HEARING IMPAIRMENT FROM OTOSCLEROSIS

Stapedial Otosclerosis
Usually otosclerosis spreads to the stapes or stirrup bone. This stapes bone is positioned in a small groove called the oval window and is in intimate contact with the inner ear fluids. As a result of the inability of the stapes bone to vibrate the inner ear fluid, a conductive hearing impairment occurs. This type of impairment is called stapedial otosclerosis and is usually correctable by surgery.

The amount of hearing loss due to involvement of the stapes (stapedial otosclerosis), and the degree of hair cell loss (cochlear otosclerosis) present, can be determined only by a careful hearing test.

Cochlear Otosclerosis
When otosclerosis spreads to the inner ear, a sensorineural hearing impairment (hair cell loss) may result due to interference with the inner ear function. This hearing impairment is called cochlear otosclerosis and once it develops it is permanent. This otosclerosis disease process affects the cochlea because the disease releases enzymes which are toxic to the hair cells and leads to cochlear deterioration. In addition, there are some other connective tissue changes which result in bone destruction. This process, however, is limited to the ear and involves no other organs. When one has otosclerosis it may affect only the stapes, the inner ear or both areas. In selected cases, medication may be prescribed in an attempt to prevent further hair cell loss and subsequently prevent further inner ear damage. This medication is sodium fluoride. On occasion the otosclerosis may spread to the balance canals and may cause episodes of unsteadiness or dizziness.

SYMPTOMS OF OTOSCLEROSIS
Progressive hearing loss is the most typical symptom of the stapedial type of otosclerosis. In pure cochlear otosclerosis a sensorineural hearing loss is the main finding. The hearing loss appears to be accelerated by pregnancy. In addition to a hearing loss, some patients experience dizziness or unsteadiness. It is more like dysequilibrium and not usually vertigo. Often the dizziness or dysequilibrium disappears after a stapes operation. Most patients with otosclerosis notice tinnitus (ear noise) to some degree. Tinnitus develops due to changes in the hearing pathway. It is usually more apparent when the patient is fatigued, nervous, or in a quiet environment. The amount of tinnitus is not necessarily related to the degree of hearing impairment. Following successful stapes surgery, the tinnitus is often decreased.

TREATMENT OF OTOSCLEROSIS

Medical
There is no local treatment to the ear itself or any medication that will improve the hearing in persons with otosclerosis. In some cases medication (sodium fluoride) is used to prevent further loss of hearing. This is given to those patients who have evidence of having hair cell loss, as a result of involvement of the cochlea.

Surgical
The stapes operations are recommended for patients with otosclerosis who are candidates for surgery. This operation is performed under local or general anesthesia and requires a short period of hospitalization and convalescence. Over 90 percent of these operations are successful in restoring hearing permanently.

For the stapes operation to be successful in restoring hearing in otosclerosis, the hearing nerve must be able to receive and transmit sound to the brain. Careful and thorough hearing tests are necessary to determine the hearing nerve function.

YOUR HEARING
Hearing is measured in decibels (dB). A hearing level of 0 to 25 dB is considered normal hearing for conversational purposes.

Our hearing tests reveal your hearing level to be:

Right ear ____________________________ decibels

Left ear ____________________________ decibels

(conversion to degree of handicap)

25dB ................................. 0%

55dB (Moderate) ..............45%

30dB (Mild) ...................... 8%

65dB (Severe) ...................60%

35dB (Mild) ......................15%

75dB (Severe) ...................75%

45dB (Moderate) ..............30%

85dB (Severe) ...................90%

SURGERY RECOMMENDATIONS

You have a minor degree of stapedial otosclerosis. We do not advise surgery at this time.

You have unilateral (one ear) stapedial otosclerosis. If the stapes operation is successful you will have improved hearing from the involved side, less difficulty determining the direction of sound, and should hear well in difficult listening situations.

You have good cochlear (hair cell) function and are a very suitable candidate for the stapes operation.

You have a slight amount of hair cell loss due to cochlear otosclerosis. If the stapes operation is successful, serviceable hearing will be restored to you.

You have a hair cell loss due to cochlear otosclerosis to some degree. If the stapes operation is successful, you should be able to hear in many situations without a hearing aid, but may need an aid for distant hearing.

You have a hair cell loss due to cochlear otosclerosis to a significant degree. If the stapes operation is successful, you will gain more benefit from the use of a hearing aid.

You have a hair cell loss due to cochlear otosclerosis to a severe degree. For this reason the chances of surgery improving your hearing are reduced. If surgery should prove successful, your hearing should be improved to the extent that you may be able to use a hearing aid.

You have profound hearing loss due to inner ear (hair cell loss) involvement. As such, stapes surgery would not be of benefit to you at this time.

Whenever an ear is operated upon, there is a chance that the hearing could be made worse or lost altogether. The possibility of this happening differs from one operation to another and may occur even if the surgery goes well and there are no complications.

THE STAPES OPERATION (Small Fenestra Stapedotomy)
Small fenestra stapedotomy is performed through the ear canal under local or general anesthesia. At necessary times a very small incision may be made above the ear to obtain some (areolar) tissue for covering the opening to the inner ear.

Under high-power magnification the tympanic membrane (ear drum) is turned forward and an opening is made into the fixed stapes bone usually with a laser. An artificial prosthesis is inserted to replace the non-mobile bone. The ear drum is then returned to its previous normal position.

The artificial prosthesis allows sound vibrations to again pass from the ear drum to the inner ear fluids. The hearing improvement obtained is usually permanent.

The person having stapes surgery is ordinarily hospitalized overnight after surgery and may return to work several days, depending upon occupational requirements.

There may be a slight dizziness on sudden head motion for several weeks. There may be a taste disturbance immediately following surgery. This subsides within two or three weeks in most cases.

One should not plan to drive a car home from the hospital. Air travel is permissible two days following surgery and is preferred to automobile or train travel for trips of over 200 miles.

HEARING IMPROVEMENT FOLLOWING STAPES SURGERY
Hearing improvement may or may not be noticeable at surgery depending upon the degree of hearing loss that the patient had prior to the surgery date. If the hearing improves at the time of surgery, it usually decreases in a few hours due to swelling in the ear and the presence of packing and blood. Improvement in hearing is usually apparent within 2-3 weeks after surgery. Maximum hearing, however, is obtained in approximately 4-6 weeks.

The degree of hearing improvement depends on how the ear heals. In the majority of patients the ear heals perfectly and they can expect good hearing improvement. In some, the hearing improvement is only partial or temporary. In these cases the ear usually may be re-operated upon with a good chance of successfully improving the hearing permanently.

In 2% of the cases, the hearing may be further impaired due to development of scar tissue, infection, blood vessel spasm, irritation of the inner ear or a leak of inner ear fluid (fistula).

In 1% of the cases, complications in the healing process may be so great that there is a severe loss of hearing in the operated ear, sometimes to the extent that one may not be able to benefit with an aid in that ear. For this reason the poorer-hearing ear is usually selected for surgery.

When further loss of hearing occurs in the operated ear, head noise may be more pronounced. Unsteadiness may persist for some time.

RISKS AND COMPLICATIONS OF STAPES SURGERY
Dizziness is normal for a few hours following stapes surgery and may result in nausea and vomiting. Some unsteadiness is common during the first few postoperative days. Dizziness with sudden head motion may persist for several weeks. On rare occasions the dizziness may be prolonged.

Taste Disturbance and Mouth Dryness
Taste Disturbance and mouth dryness is not uncommon for a few weeks following surgery. In 5% of the patients, this disturbance may be prolonged.

Loss of Hearing
Further loss of hearing develops in 2% of the patients due to some complications in the healing process. In 1% this hearing loss is severe and may prevent the use of an aid in the operated ear.

Tinnitus
Should the hearing be worse following surgery, tinnitus (ear noises) likewise may be more apparent.

Eardrum Perforation
A perforation (hole) in the tympanic membrane (ear drum) is an unusual complication. It develops in less than 1 % and is usually due to an infection. Fortunately, should this complication occur, the membrane may heal spontaneously. If healing does not occur, surgical repair (myringoplasty) may be required.

Weakness of the Face
A very rare complication of a stapedectomy is a temporary weakness of the face. This may occur as a result of an injury or swelling of the facial nerve.

HEARING AIDS
If you are a suitable candidate for surgery, you are also suitable to benefit from a properly fitted hearing aid. If you have otosclerosis and are not suitable for stapes surgery, you still may benefit from a properly fitted hearing aid.

If you are suitable candidate for surgery, and do not wish to have the stapes operation at this time, it is advisable to have a careful hearing test repeated at least once a year.

Fortunately, patients with otosclerosis very seldom go "totally deaf." Some will be able to hear with an electronic aid.

OTHER SURGICAL PROCEDURES
In the event that your previous stapes surgery has not given you any benefit, or if you have been unable to use a hearing aid because of a profound hair cell loss, due to inner ear involvement (cochlear otosclerosis), you may be a candidate for a cochlear implant. This is a procedure in which electrodes are placed into the inner ear and stimulate the hearing nerve directly, while bypassing the hair cells. This is used in selected cases, but can be an alternative when stapes surgery or a hearing aid cannot be utilized.

GENERAL COMMENTS
Patients should be reminded that in spite of having had successful stapes surgery they should have their hearing checked in the years following to monitor the possible development of cochlear otosclerosis.

If they were to develop some hair cell loss (inner ear involvement), it would be possible in some patients to use medication (sodium fluoride) to control it.