The term otosclerosis is derived from the Greek words for "hard" (scler-o) and "ear" (oto). It describes a condition of abnormal growth in the tiny bones of the middle ear, which leads to a fixation of the stapes bone. The stapes bone must move freely for the ear to work properly and hear well.
Hearing is a complex process. In a normal ear, sound vibrations are funneled by the outer ear into the ear canal where they hit the eardrum. These vibrations cause movement of the eardrum that transfers to the three small bones of the middle ear, the malleus (hammer), incus (anvil), and stapes (stirrup). When the stapes bone moves, it sets the inner ear fluids in motion, which, in turn, start the process to stimulate the auditory (hearing) nerve. The hearing nerve then carries sound energy to the brain, resulting in hearing of sound. When any part of this process is compromised, hearing is impaired.
It is estimated that ten percent of the adult Caucasian population is affected by otosclerosis. The condition is less common in people of Japanese and South American decent and is rare in African Americans. Overall, Caucasian, middle-aged women are most at risk. The hallmark symptom of otosclerosis, slowly progressing hearing loss, can begin anytime between the ages of 15 and 45, but it usually starts in the early 20’s. The disease can develop in both women and men, but is particularly troublesome for pregnant women who, for unknown reasons, often experience a rapid decrease in hearing ability. Approximately 60 percent of otosclerosis cases are genetic in origin. On average, a person who has one parent with otosclerosis has a 25 percent chance of developing the disorder. If both parents have otosclerosis, the risk goes up to 50 percent.
Gradual hearing loss is the most frequent symptom of otosclerosis. Often, individuals with otosclerosis will first notice that they cannot hear low-pitched sounds or whispers. Other symptoms of the disorder can include dizziness, balance problems, or a sensation of ringing, roaring, buzzing, or hissing in the ears or head known as tinnitus.
Because many of the symptoms typical of otosclerosis can also be caused by other medical conditions, it is important to be examined by an otolaryngologist (ear, nose and throat doctor) to eliminate other possible causes of the symptoms. After an ear exam, the otolaryngologist may order a hearing test. In a patient with a conductive hearing loss and a normal ear exam, 90% of the time they have otosclerosis. The only way to definitively make the diagnosis is with surgery. If it is discovered that something else is causing the hearing loss, over 75% of the time the hearing can still be repaired.
If the hearing loss is mild, the otolaryngologist may suggest continued observation and a hearing aid to amplify the sound reaching the eardrum. Sodium fluoride has been found to slow the progression of the disease and may also be prescribed, however that will never improve hearing. In most cases of otosclerosis, a surgical procedure called stapedotomy is the most effective method of restoring or improving hearing.
A stapedectomy is an outpatient surgical procedure done under local or general anesthesia through the ear canal with an operating microscope. No external incisions are made. It involves removing the upper part of an immobilized stapes bone, putting a small hole in the remaining footplate, and placing a titanium piston between the hole and the incus. The prosthetic device allows the bones of the middle ear to resume movement, which stimulates fluid in the inner ear and improves or restores hearing The success rate of stapedotomies is over 95%. In rare cases (less than one percent of surgeries), the procedure may worsen hearing. Otosclerosis affects both ears in eight out of ten patients. For these patients, ears are operated on one at a time; the worst hearing ear first.
Most patients return home following surgery. Your doctor may prescribe oral antibiotics. Some patients experience dizziness the first few days after surgery. Taste sensation may also be altered for several weeks or months following surgery, but always returns to normal. Following surgery, patients may be asked to refrain from nose blowing, swimming, or other activities that may get water in the operated ear. Normal activities (including air travel) are usually resumed three weeks after surgery. Since packing is placed in the ear at the time of surgery, hearing improvement will not be noticed until it is removed or is dissolved after surgery.
Notify your otolaryngologist immediately if any of the following occurs:
Complications from stapedectomy are infrequent and seem to be related to the presence of uncommon variations in anatomy. The incidence of total hearing loss reported by most experienced surgeons is about one in 200 cases. Facial nerve paralysis is extremely rare for stapedectomy. Loss of taste on the side of the tongue is common postoperative complaint that usually resolves within two months. Some dizziness after surgery is normal and may last for a few days or weeks, but disabling dizziness may indicate injury to the inner ear, and the surgeon should be informed. Tinnitus (ringing or buzzing in the ear) that was present commonly persists, although following surgery it may disappear. On the other hand, tinnitus may develop as a result of surgery. The incidence is not known but it is uncommon. Hearing loss that does not improve as a result of surgery occurs in about 2% of cases. If it is discovered that a condition other than otosclerosis is causing the hearing loss which is possible to safely repair during surgery, this is also consenting for that repair to be made with an ossiculoplasty. If there is a condition present making the stapedotomy or ossiculoplasty impossible to safely perform such as a congenital anomaly of the facial nerve, incus, round window, or massive obliteration of the stapes footplate, surgery will need to be aborted and there will be no improvement in hearing.
A hearing aid may be a reasonable alternative to surgery and the options between surgery or a hearing aid should be considered. Stapedotomy for otosclerosis is an elective procedure.
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