
Otosclerosis is one specific condition in which new and unwanted bony tissue is formed in the bone which surrounds the inner ear. This new bone contains many blood vessels and therefore it is much less hard than normal bone. Problem is that, gradually, the new bone matures and becomes hard, during the process called sclerosis. In otosclerosis, the consistency of the sound-conducting bones of the ear changes from hard, mineralized bone to spongy, immature bone tissue. Big problem is that the lack of vibration prevents sound from being conducted to the inner, leading to a conductive hearing loss.
Ear anatomy
Normally, the ear conducting system is divided into three parts, the outer ear, the middle ear and the inner ear. The outer ear collects the sound via the ear drum that vibrates. These vibrations are transmitted to the inner ear by three small bones:
- the malleus (hammer),
- the incus (anvil),
- the stapes (stirrup)
These are three smallest bones in our body! They work by increasing the sound pressure and transmitting the sound to the inner ear. In the inner ear, the vibrations stimulate the hair cells which transform the vibrations into electrical impulses that are carried by the hearing nerve to the brain.
Pathophysiology of otosclerosis
Of course that if there is any difficulty in the outer or middle ear, a conductive hearing impairment occurs. Depending on the localization of damage- we have different impairments!
- If the trouble is in the inner ear, it will lead to some nerve hearing impairment
- When there is difficulty in both the middle and inner ear, it leads to the mixed impairment.
The fact is that these mixed impairments are very common in otosclerosis but the conductive component predominates in most cases.
Why is this stapes bone so important?
It is the stapes which connect to the inner ear in a bony gap called the oval window. The biggest problem is that, when we talk about otosclerosis, the formation of new bone around the oval window stops the stapes from moving which normally lead to complete deafness.
Incidence of the condition
When this condition is present, both ears are usually involved and several studies have proven that the condition occurs in men and women with almost equal frequency and usually begins in the teens or early twenties. Less than 1% of the population is affected by this condition. Although otosclerosis tends to run in families, it does so irregularly; parents with otosclerosis do not necessarily transmit it to their offspring. Between the ages of 30 and 50, some studies have shown that about 10 percent to as high as 18 percent of all white women and 7-9 percent of all white men have histological signs of otosclerosis. Less than 10 percent of these people will actually seek help for hearing loss.
Types of hearing impairment from otosclerosis
In generally, there are two types of otosclerosis and they are:
During this process, otosclerosis can spread to the inner ear a sensory-neural hearing impairment may result due to interference with the nerve function. This nerve impairment is called cochlear otosclerosis and once it develops it is permanent and there is no cure for it.
In some cases- otosclerosis can spread to the stapes or stirrup bone, the final link in the middle ear transformer chain which leads to the conductive hearing impairment. This type of impairment is called Stapedial otosclerosis and is usually correctable by surgery.
Possible cause of otosclerosis
Many experts believe that only heredity could be the cause! Otosclerosis runs in families in many cases, but the truth is that genetic cause is not clear. It is very possible that there are a number of different genetic types of otosclerosis but they are still not so clear! The most common theory has been that there is a dominant gene that can cause otosclerosis.
It is also difficult to find the underlying cause of otosclerosis, since it may not be the same in everyone.
It some studies, it is proven that women are more likely to develop otosclerosis than men.
It is proven that susceptible women may develop otosclerosis during pregnancy.
Caucasians (white people) tend to be most commonly affected.
It is one genetic disorder is characterized by abnormally brittle bones
Some evidence suggests that drinking non-fluoridated water may increase the risk of otosclerosis in susceptible people.
Some people develop otosclerosis even though they don't have any of the known risk factors.
Symptoms of otosclerosis
Deafness is the main symptom and is usually present in both ears. Most patients notice the deafness before the age of 50. Noises in the ear (tinnitus) are present in most cases. Several patients have also notices:
- slight unsteadiness
- Pain
- discharge from the ears
- Hearing may improve in noisy conditions
- Dizziness
Diagnosis of otosclerosis
Diagnosis of otosclerosis is based on a combination of the:
- patient's family history
- the patient's symptoms
- the results of hearing tests
Every doctor should first determine whether the patient's hearing loss is conductive or sensory-neural. Two tests that are commonly used to distinguish conductive hearing loss from sensory-neural are:
A computed tomography (CT) scan or x ray study of the head may also be done to determine whether the patient's hearing loss is conductive or sensorineural.
Treatment of otosclerosis – Stapedectomy
The best possible treatment of otosclerosis is Stapedectomy! Stapedectomy is a surgical treatment for otosclerosis in which the immobilized stapes is removed, and a tiny platinum or stainless steel prosthesis is inserted in the middle ear to replace it. It is very good and effective procedure! The fact is that the artificial stainless steel prosthesis is less than 1/8 of an inch long. Of course, it is logical that more extensive damage may require removal of the entire stapes footplate, while a small focus of disease allows for removal of less tissue. In Stapedectomy, the ear drum is turned forward and the fixed stapes is removed. After this, tissue is placed over the opening to the inner ear and a prosthesis is inserted and attached to the anvil.
Stapedectomy effectiveness
The operation is done for years now and the fact is that this surgery is extremely effective, and usually restores normal hearing in patients with conductive hearing loss. Patients can usually return to work in about a week! Procedure is usually performed on an outpatient basis. Either local or general anesthesia may be used, depending on the comfort of the surgeon and the patient. Not many people know that a small tissue graft, usually taken from a vein in the patient’s hand, is taken and used to seal the oval window after the prosthesis is inserted.
Possible complications of Stapedectomy
It isn't difficult to assume that every manipulation of the stapes always produces a reaction in the inner ear which can sometimes lead to giddiness and possibly hearing loss. What's so good about this operation -in 90% of cases the results of stapedectomy are very good. In a small percentage of cases there may be severe or total loss of hearing in the operated ear.
Important is that this may occur immediately after surgery or be delayed for several years.
In about 10% of cases the hearing is only slightly improved or there is no improvement.
- Damage of the facial nerve
The fact is that, because the facial nerve which supplies the muscles of the face runs through the middle ear, it is occasionally damaged, producing a lop-sided weakness of the face.
Nerve called the chorda tympani supplies the sense of taste and it is frequently damaged. This results in a temporary metallic taste which does not usually cause any significant problem.
Giddiness with loss of hearing and tinnitus may occur some time after surgery which can be due to a leakage of fluid around the artificial stapes, infections of the middle ear and other conditions!
After operation tips
The evening after the operation patient should lie quietly on the un-operated ear. The surgery usually takes two to three hours. If all goes well, you can go home when you are fully awake and determined ready for discharge by your doctor. Be sure to bring someone to drive you home. It is not recommended to:
- Blow your nose
- Lie flat in bed
- Remove any packing from your ear
Other tips:
- Do not get water in the ear
- No strenuous exercise
- Do not remove any packing
- Notify your doctor of fever greater than 100 degrees F, excessive pain, excessive drainage, or drainage that has an odor.
Alternative to surgery
In some cases, surgery is not possible and a hearing aid may be used instead. People who should not have stapedectomy include those who experience frequent changes in barometric pressure (pilots and divers), elderly patients with a baseline imbalance, people whose vocations demand excellent balance, and anyone with known Meniere’s disease (stapedectomy often causes permanent profound hearing loss in patients with Meniere’s disease). If a person has a perforated tympanic membrane due to middle ear infection, the infection must be cleared and the membrane healed before stapedectomy. Patients with sensorineural hearing loss or mixed hearing loss may not improve after stapedectomy, and would not be likely to benefit from surgery.