Treatment for otosclerosis, the ringing of the division bell had begun - PDF Review

Otosclerosis is usually inherited in an autosomal dominant pattern with variable penetrance. When otosclerosis significantly involves the bone which surrounds the inner ear, called the otic capsule, a sensory type hearing loss occurs. People of African-American descent rarely have otosclerosis — it is usually a condition found in persons of Caucasian or Oriental descent.
Most doctors and scientists believe that otosclerosis is an inherited, an autosomal dominant disease with variable penetrance. Pathologically, otosclerosis occurs only in human temporal bones, and is considered to be a disorder of new bone formation.
Dizziness can occur in otosclerosis and was reported in one study to occur in 15% of patients.
To date, the only proposed medical treatment has been sodium fluoride, which is a dietary supplement (not a drug). In theory, avoidance of estrogens or use of estrogen blockers might be helpful in individual with otosclerosis, since otosclerosis frequently worsens during pregnancy, suggesting hormonal modulation. Otosclerosis is a condition in which the bones in the middle ear become fixed and immobile, leading to hearing loss (usually one-sided). The first involves no treatment and coping with the degree of hearing loss that is present and following the hearing with serial audiograms. Hearing aidshave been found to be useful in otosclerosis provided the clarity is still preserved.
No one should scare you from having a stapedectomy operation for fear of facial paralysis or hearing loss.
Factors Influencing DurationFactors influencing the length of disability for otosclerosis include the general health and fitness of the individual before surgery, pre-existing diseases affecting any of the major body systems that might interfere with the healing process, the individual's general ability to heal and compliance with postoperative orders, and whether or not the individual experiences any degree of depression following surgery.
Otosclerosis causes bilateral (occurring in both ears) hearing loss, with the hearing deficit in one ear usually progressing more rapidly than in the other. Stapedectomy, either partial or total, is the preferred surgery to treat almost all cases of otosclerosis. This means that you have a 50% chance of getting the gene for otosclerosis if one parent has it, but that not everyone with the gene develops symptoms.
We do not know what about these genes or mutation in them causes otosclerosis to develop, although advances in genetic research are beginning to elucidate the matter (Moumoulidis et al 2007, Schrauwen et al 2012). When otosclerosis involves the small bones of the middle ear, a conductive type loss is found.
The conductive type of otosclerosis usually progresses up to a maximum when patients are in their 30s. As there is also evidence of viral influences in otosclerosis, a recent hypothesis is that otosclerosis requires a combination of a specific gene with exposure to a specific virus (for example measles) for it to be expressed and for hearing loss to occur (McGuirt et al, 1998). Viral materials have be found in the nucleic acid of an otosclerotic stapes footplates (Karosi et al 2008) and increased levels of antibodies to the measels virus are present in the inner ears of patient with otosclerosis (Niedermeyer et al 2007).
Histologic otosclerosis, meaning that it is found only on microscopic examination but has no symptoms, is found in one of every 10 Caucasians in the United States.
John Shea invented the procedure of stapedectomy, which produced excellent hearing results, that remain good for many years after the surgery.

Sedentary work involves sitting most of the time, but may involve walking or standing for brief periods of time.
This procedure usually restores at least partial hearing in most individuals with otosclerosis involving only the stapes and can significantly improve the individual's quality of life.
Pharmacologic treatment to relieve symptoms associated with balance disorders (possible nausea and vomiting) might include antihistamines, anticholinergics, and sedative-hypnotics.
Otosclerosis involves the small bones of the middle ear, the malleus (2), the incus (3) and the stapes (4), as well as the bone that surrounds the inner ear, which is called the otic capsule. One family with otosclerosis was found to have a mutation in a gene for collagen and antibodies against collagen, suggesting an autoimmune mechanism (Niedermeyer & Arnold 2002).
A recent study found the rate of otosclerosis to be significantly lower in patients who received the measels vaccination (Arnold et al 2007). Clinical otosclerosis, with symptoms of hearing loss, occurs in one of every 10 patients with histologic otosclerosis (Nadol, 1998).
The mechanism for dizziness is unknown, although there is speculation that it derives from release of enzymes from metabolically active bone into the inner ear. Cochlear implants are more difficult to position in otosclerosis due to sclerosis of the inner ear. The AHRF has supported research on otosclerosis in the past, and expects to do so in the future. Resolution of untoward nonauditory stimulation and cochlear implant performance gain after extraction of a stainless steel dental crown in a patient with cochlear otosclerosis.
Disease-associated novel CD46 splicing variants and pathologic bone remodeling in otosclerosis. No evidence for an association between persistent measles virus infection and otosclerosis among patients with otosclerosis in Japan. Antimeasles immunoglobulin G and virus-neutralizing activity in sera of patients with otosclerosis.
Otosclerosis: incidence of positive findings on high-resolution computed tomography and their correlation to audiological test data. Cochlear implantation in 53 patients with otosclerosis: demographics, computed tomographic scanning, surgery, and complications. New perspectives for middle ear implants: first results in otosclerosis with mixed hearing loss. Surgical findings and long-term hearing results in 3,050 stapedotomies for primary otosclerosis: a prospective study with the otology-neurotology database. Otosclerosis is a condition marked by abnormal bone growth in the middle ear that results in the stapes becoming fixed or immobile. Individuals with hearing loss (temporary or permanent) may require vocational or occupational therapy to help them prepare for a different job. When otosclerosis involves both the small bones and the cochlea, a mixed type hearing loss occurs.
Patients with otosclerosis may have weaker immune responses against the virus (Lolov et al 2007). This results in the rather amazing conclusion that 1% of the population should exhibit clinical otosclerosis.

In our opinion, particularly helpful would be a method of preventing progression of the disease, and also and a method of determining who is susceptible to otosclerosis.Learn more about donating to American Hearing Research Foundation (AHRF) to diagnose Otosclerosis. This tendon is routinely cut by most surgeons when doing stapes surgery to restore hearing in otosclerosis. A recent study found patients treated with fluoride to have smaller otosclerotic foci on CT scanning, also supporting a beneficial effect (Naumann et al 2005).The idea of using fluoride is not unlike that of using it for teeth — fluoride speeds up hardening of bone.
Between 80% and 90% of individuals with otosclerosis have this type of hearing loss (Shohet). Twice as many women as men seek treatment for the disorder, but studies show that the there is no actual biological difference in the frequency of occurrence between sexes (Roland). The individual may be advised to avoid nose blowing, lifting, or straining for 6 weeks and to sneeze with the mouth open. After two years of fluoride treatment, the dose is reduced from three times a day to once a day. Patients with stapedectomy may attain better results with hearing aids because of the need for less amplification. Cochlear implantation has been performed as initial treatment and following stapedectomy with good results.
About 8% of individuals who have otosclerosis have another type of hearing loss called sensorineural hearing loss. The spread of otosclerosis to the inner ear (cochlear otosclerosis) results in a sensorineural hearing impairment.
Airline travel is restricted for about 2 weeks, and water in the ear should be avoided until the ear is healed.
Speech therapists provide suggestions for coping with hearing loss, such as using carpeting, drapes, and upholstered furniture to absorb noise and eliminating extraneous background noise when communication is necessary. Tinnitus appears to improve following surgical intervention in a number of cases, since “the conductive hearing deprivation produced by otosclerosis is associated with limited and reversible modifications in the central auditory pathway that are linked to tinnitus”(Deggouj et al 2009).
Once the otospongiosis phase of otosclerosis is over and there is a clear cut otosclerosis documented by conductive hearing loss, fluoride may be stopped. If surgical repair is not successful and results in hearing loss, treatment consists of developing skills for coping with deafness and includes hearing aids, visual clues, or other technology.
Some individuals with otosclerosis are unaware they have the disorder until hearing loss becomes so severe that communication is difficult. The sensory component of the disease may eventually lead to complete deafness, but fortunately, this is unusual. Some individuals with otosclerosis may prefer a hearing aid (amplification) for a trial period before considering surgical intervention.

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