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Hearing Disorders
Hearing loss affects 28 million people in the United States. Many things can cause it, from a single traumatic event to chronic or progressive medical conditions. The New York Otolaryngology Group provides the most specialized care for hearing loss and related conditions available in the greater New York region. Dr. Sperling and his team address the full range of illnesses affecting the ear. He has particular expertise in treating and diagnosing otitis media, otosclerosis, cholesteatoma, and autoimmune inner ear disease, described below.
Otosclerosis
Otosclerosis is a condition of the ear caused by abnormal growth. The growth surrounds the tiny vibrating bones of the middle ear, resulting in conductive hearing loss. This typically affects the Stapes bone. Immobilized, the Stapes cannot properly conduct sound energy toward the inner ear, which causes conductive hearing loss.
Approximately 60 percent of otosclerosis cases are hereditary and are most common in Caucasians between 20 and 45 years of age. (It is seen in other ethnic groups, though less often.) Women are twice as likely to be affected than men and, once affected, often experience accelerated hearing loss during pregnancy.
Interestingly, many more people have otosclerotic growth than ever seek treatment for hearing loss. The abnormal bone growth can exist in the ear without interfering with the ossicles' proper function. This is called histologic otosclerosis, and by some measures up to 10 percent of Americans have it. It can and often does lead to clinical otosclerosis, the variant that causes hearing loss and requires treatment. The rate at which this can occur increases with age.
Symptoms
Hearing loss is the primary symptom of clinical otosclerosis. The hearing loss may initially be slight and often increases over time. Patients with otosclerosis may also suffer tinnitus, a ringing or rushing sound in the affected ear. Balance problems, such as dizziness, vertigo and unsteadiness, may also occur but are far less common.
Diagnosis
Patients are first given a hearing test. But hearing loss can be caused by a number of disorders, so a CT scan may be necessary.
Treatment
Conductive hearing loss due to otosclerosis is reversible with surgery in most cases. Dr. Sperling performs laser stapedotomy, a procedure that removes part of the Stapes and replaces it with a prosthesis. With the prosthesis in place, the bones of the middle ear can move properly and restore conductive function to the middle ear.
Dr. Sperling uses a specialized laser stapedotomy technique that includes vein grafting in order to eliminate post-operative vertigo, which is common after more traditional stapedectomy procedures. This technique is 90 - 95% effective at restoring the ability to hear and restores hearing immediately after surgery.
In some cases a sodium fluoride treatment may be prescribed as it slows the progression of the disease. Traditional hearing aids can correct the hearing loss associated with otosclerosis, and are an alternative to surgery.
Cholesteatoma
Cholesteatoma is a benign growth caused by the build-up of skin cells and protein from the ear canal behind the eardrum.
The outer ear is lined with a layer of skin that gives off dead cells containing keratin, a protein. This discharge occurs normally and is a component of earwax. It is removed when the ears are routinely cleaned.
But if a bit of this skin penetrates the eardrum, (which can occur for a number of reasons) the keratin that continues to be given off by the skin becomes trapped in the middle ear and builds-up over time, resulting in a cholesteatoma. If left untreated, the growth can erode the ossicles (the tiny hearing bones of the middle ear) and cause a conductive hearing loss; it can further fill the mastoid and destroy structures of the inner ear, causing permanent, sensorineural hearing loss. Unchecked, this tumor-like growth has the potential to cause facial paralysis, hydrocephalus, meningitis, or a brain abscess.
Symptoms
Although some patients have very mild or no symptoms of cholesteatoma, the most common sign of cholesteatoma is frequent ear infection, possibly with a foul smelling discharge from the ear. Coincident loss of hearing is often present.
Diagnosis
The diagnosis of cholesteatoma is primarily made by microscopic examination of the ear during an office visit. An audiogram, or hearing test, and CT scan of the temporal bone, where the hearing mechanism is stored, are required to determine the amount of hearing loss and the degree to which the cholesteatoma has spread.
Treatment
The primary goal of treatment is to control the infection and bone destruction caused by a growing cholesteatoma. The secondary goal is to restore one's ability to hear. If the tumor is small and hearing loss remains at acceptable levels, then microscopic cleaning may be performed during an office visit to clean the keratin out of the ear.
For a more advanced cholesteatoma, or if the tumor has grown into the inner ear or onto the facial nerve, Dr. Sperling may perform a mastoidectomy, and depending on the circumstances, may accompany it with tympanoplasty and ossicular (hearing) reconstruction.
Mastoidectomy is a surgical procedure designed to remove cholesteatoma and infections from within the mastoid bone, which protects the structures of the middle and inner ear. Tympanoplasty is the surgical reconstruction of the eardrum and hearing apparatus. Ossicular reconstruction may be necessary if the infection has eroded the bones of the middle ear. The damaged bones are replaced by bone or a prosthesis, which restores the proper function of the middle ear.
As cholesteatomas can recur, close post-surgical follow-up is very important.
Autoimmune Inner Ear Disease
Autoimmune inner ear disease is an uncommon disease with which most doctors are unfamiliar. It is caused by a malfunction in the body's immune system, which causes it to attack the structures of the inner ear as it would a virus or bacteria. It is known to cause only one percent of all hearing loss cases.
Symptoms
Autoimmune inner ear disease may present itself similarly to Meniere's disease. Patients usually will notice bilateral progressive or fluctuating loss of hearing. Tinnitus and aural fullness, a feeling of fullness or pressure in the ear, may also occur.
Diagnosis
Currently the best method for diagnosing this disease is by clinical evaluations in the office and blood testing.
Treatment
A steroid called prednisone is most commonly prescribed to suppress the immune system and slow its attack on the inner ear structures. Other steroid medications are now commonly delivered directly to the inner ear via intratympanic injections, as opposed to the more traditional intravenous or oral administration of the drug. By injecting the steroid directly into the ear, the affected region receives a potent dosage of the medicine without concern for potential negative side effects of the steroids on the rest of the body.
Intratympanic steroid delivery is a promising new method of treating diseases of the ear. It is a painless procedure that can be done in the doctor's office under mild anesthesia. Other devices have been developed and are sometimes used to deliver medication to the inner ear.
Dr. Sperling has extensive experience treating this rather rare condition. Significant help to one's hearing can be achieved with treatment.
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