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Otosclerosis

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Otosclerosis is a common cause of medium or severe hearing loss which progressively gets worse. It seems to be hereditary. It is more common among women and usually affects both ears. Practically, there is new bone formation in the ear which may cause anchylosis to the small ear bones (ossicles) or affect the deeper part of the ear, the cochlea. Symptoms begin at a young age, however, very rarely they may also appear at late childhood (juvenile otosclerosis). The patient complains about hearing loss and/or ringing in ears (tinnitus). On rare occasions the patient may feel ear congestion and vertigo. As previously mentioned, hearing loss gets progressively worse with age and acute deterioration is observed in women during pregnancy or after hormone therapy. It seems that the pace of deterioration is reduced with age and at the beginning of the golden age the disease is not progressing any more and is considered stable.

Symptoms in the first stages of the disease are mild and therefore they may not be diagnosed. Thus, in case we suspect that a young person has the disease we must not make him feel worried about it but instead we must keep him informed and suggest that he occasionally undergo hearing tests (audiograms). Normally the patient sees the doctor complaining about hearing loss and tinnitus. If hearing loss gets worse with age, and the patient does not have it congenitally or after an injury or after taking ototoxic medication in these cases we can suspect otosclerosis. We can always investigate the family medical history but it is rather unclear especially for an elderly person.

During the clinical examination there are no signs of abnormalities. Evidence of chronic otitis, calcium depositions in the tympanic membrane, malformations of the pinna or of the external auditory canal may rule out the diagnosis of otosclerosis.

The diagnosis in question is practically made by exclusion of other diseases which may probably cause similar symptoms. When hearing loss affects only the one ear we must be cautious regarding the diagnosis of otosclerosis because, as previously mentioned, this disease usually affects both ears.

Next, a tuning fork test and an audiogram are performed to help us determine the degree of hearing loss and decide if the patient can undergo a surgical procedure in case he / she chooses to do it. A tympanogram test and auditory reflex test may support the diagnosis. If there is a suspicion of a different diagnosis, a medical imaging method such as the CT should be performed. It is considered that through detailed axial tomography otosclerosis signs can be detected but this is not yet confirmed. Thus, if there is a diagnosis or even strong suspicion of otosclerosis we must inform our patient about possible solutions.

The first option is to follow no treatment at all because otosclerosis is not so much of a health problem but rather a matter of quality of life. Thus, its treatment is not considered necessary. However, by doing so, the patient chooses a form of social isolation and therefore this choice should be avoided.

A hearing aid would be the second option. Patients who feel that their hearing level is getting weaker but they cannot or they do not want to undergo any surgery may choose this solution. A hearing aid may be used also after successful surgical interventions in order to improve hearing quality.

The use of florical (sodium fluoride and calcium carbonate) is the third option. This substance matures the otosclerotic foci and therefore impedes the progress of the disorder. Nevertheless, treatment may be long lasting and perhaps may cause gastrointestinal disorders or even worse, possibly osteoporosis. In addition, many health professionals doubt on the effectiveness of this kind of treatment. However, in case of increasingly deteriorating otosclerosis in which the cochlea is also affected such as the case of juvenile otosclerosis, florical treatment may be the only option.

Finally there is the alternative of surgical intervention recommended for the ear with the higher level of hearing loss. In order for a patient to be a candidate for the intervention, the air - bone gap must be greater than 15dB at low frequencies.

To give the patient an idea of what to expect, we can compare the estimated gain in the hearing level of the ear to be operated with the hearing level of the other ear. Thus, in case that the damage in the other ear caused by otosclerosis is not so severe, we must inform the patient that the subjective improvement will be quite little despite the successful result of the intervention.

As previously mentioned the diagnosis of otosclerosis is made by exclusion of other disorders. Therefore when entering the middle ear the surgeon must be able to deal with whatever situation he may encounter apart from the fixation of the stapes. In addition, the surgeon must have the skills to deal with possible anatomic variations in the tympanic cavity.

The operation is called stapedotomy and is the evolution of the older technique called stapedectomy. Generally speaking, stapedotomy involves the removing of the stapes, the ossicle affected by the mineralization process of the otosclerosis, and placing a piston-like prosthesis in a hole opened in the base of the stapes. The treatment is performed through a very narrow space, the ear canal, and the successful outcome is sometimes a question of millimeters. Therefore, it is easy to understand that the patient must remain completely immobile and this is the reason why it is preferable to perform this intervention under general anesthesia. In special cases, when we cannot be sure about the stability of the stapes, the use of the laser may help significantly.

The patient is hospitalized for one or two days and is subsequently discharged after being given the necessary instructions. Feeling dizzy after the intervention is quite usual but it is reduced progressively after a week. Certain taste disorders may be more appear but they also improve with time.

The most common causes of surgery failure are bad placement or non-appropriate piston size. Very rarely acute hearing loss may appear, with until now unknown etiology. There is a failure probability in every intervention, even a low one, which is the reason why we always perform the operation on the worse ear and we do not proceed with the intervention of the other ear before a time period from the first surgery has passed, in order to stabilize the result.

Otosclerosis is a common disorder in Greece and we are greatly experienced in stapedotomy. It must be stressed, however, that the intervention does not cure the disease. It just deals with its effects, the hearing loss. Certain patients are not as bothered by the hearing loss as by tinnitus. In these cases the surgeon must not promise a complete elimination of the effects but rather an improvement.