Otosclerosis is a disease associated with abnormal bone growth as a result of a violation of its metabolism. The process takes place in the middle ear and can lead to a significant deterioration and even loss of hearing. It usually begins with a one-sided decrease in hearing acuity, gradually covering both ears. When audiometry in the early stages, as a rule, a loss of response to low- frequency sounds is noticeable.
| Otosclerosis | |
|---|---|
| ICD-10 | H 80. |
| ICD-10-KM | and |
| ICD-9 | 387 |
| ICD-9-KM | |
| OMIM | , , , , , , and |
| DiseasesDB | |
| MedlinePlus | |
| Mesh | |
In almost all cases chronic conductive hearing loss is noted. Neurosensory hearing loss is also sometimes noted, usually in the high frequency range, in the later stages of the disease.
Content
Reasons for Otosclerosis
- Currently, the causes of otosclerosis have not been studied. The disease is more common in women, during puberty, menstruation, pregnancy, breastfeeding and menopause.
- Genetic causes are inherited by an autosomal dominant type with incomplete penetrance from 20% to 40%. Monozygous twins have almost 100% concordance in otosclerosis. To date, eight loci associated with otosclerosis, which are designated as OTSCI-8, have been identified.
- One study suggests that the disease may be due in part to variations in the RELN gene . [2]
- Measles virus ( RNA virus was detected in the study of archival and fresh samples of the stirrup plate)
- In addition, one of the alleged causes of otosclerosis may be an increased production of the hormone estradiol
Risk factors for otosclerosis
Among the risk factors for otosclerosis are the following conditions:
- The long course of chronic inflammation of the middle ear, in which there is a partial or complete necrosis of the auditory ossicles. In case of necrosis of the anvil and stirrup junction, fibrosis can form, which limits the transmission of sound vibrations through the auditory ossicles.
- Congenital stirrup fixation.
- Paget's Disease.
- Congenital malformations of the hearing organs.
Manifestations of otosclerosis
Otosclerosis is usually manifested by a conductive type of hearing loss. In the beginning, one ear is most often affected, but gradually the process of otosclerosis affects the second ear. In the study during audiometry, there is a defeat in the transmission of low frequencies of sound, and later of high frequencies. In late stages of the disease, sensorineural hearing loss also joins.
Otosclerosis occurs in about 0.5% of the population. Modern studies of the problem of otosclerosis have shown that this condition can occur in 10% of people, but it is not always manifested.
When otosclerosis observed foci of sclerosis in the middle ear. This means that the patient has a decrease in the mobility of the auditory ossicles, namely, in the place of attachment of the stirrup to the oval window, as a result of which the transmission of sound vibrations is disturbed.
Diagnosis of otosclerosis
For the diagnosis of otosclerosis using a method such as audiometry . In addition, tympanometry is conducted.
Otosclerosis Treatment
Treatment of otosclerosis is carried out in two ways: this is the use of hearing aids or surgery. Hearing aids can help to effectively deal with hearing impairment only in the later stages of the disease, when surgical treatment is not indicated. In the surgical treatment of otosclerosis, stapedoplasty surgery is performed . This intervention is performed mainly under local anesthesia and is performed on a stirrup . It is performed when there is a fixation of the stirrup in the oval window. The operation consists of removing the stirrup legs and replacing it with a prosthesis. A small hole is made in the bottom plate of the stirrup and a thin prosthesis is inserted into it that works like a piston (piston stapedoplasty).
These operations give a good result in patients suffering from otosclerosis - a violation of the mobility of the auditory ossicles in the middle ear. Positive results are observed in nine out of ten patients who have undergone this operation; in less than 1% of the operated patients, the operation was ineffective.
Contraindications to stapedectomy
- Severe general condition of the patient.
- Low reserve snail function.
- Tinnitus or dizziness.
- The presence of active foci of otosclerosis.
Complications of stapedectomy operation
- Paralysis of half of the face on the side of the affected ear due to damage to the branches of the facial nerve.
- Dizziness in the postoperative period.
- Vomiting.
- Isolation of perilymph from the ear.
- Rupture of the eardrum.
- Damage to the maze.
- Labyrinthitis (inflammation of the structures of the inner ear).
- Gusher syndrome .
Note that even despite the excellently performed operation, after it there can be such complications as:
- Complete deafness of the operated ear.
- Prolonged dizziness.
- Constant sensation of noise or buzz in the operated ear.
- Paralysis of half of the face due to damage to the branches of the facial nerve.
Links
Palchun V.T. and Sakhalinskas M.A. Clinic and surgery of otosclerosis, Vilnius, 1976;
Preobrazhensky N.A. and Patyakina O.K. Stapedectomy and stapedoplasty with otosclerosis, M., 1973, bibliogr .;
Soldatov I.B. and others. Functional diagnostics and issues of modern surgery of otosclerosis, M., 1974, bibliogr .;
Hearing loss, ed. ON. Transfiguration, M., 1978.
See also
- Samuel Rosen .
Notes
- β Disease Ontology release 2019-05-13 - 2019-05-13 - 2019.
- β Schrauwen I., Ealy M., Huentelman MJ, Thys M., Homer N., Vanderstraeten K., Fransen E., Corneveaux JJ, Craig DW, Claustres M., Cremers CW, Dhooge I., Van de Heyning P. , Vincent R., Offeciers E., Smith RJ, Van Camp G. A Genome-wide Analysis Identifies Genetic Variants in the RELN Gene Associated with Otosclerosis (Eng.) // Am. J. Hum. Genet. : journal. - 2009. - February. - DOI : 10.1016 / j.ajhg.2009.01.023 . - PMID 19230858 .
Literature
- Khilov K. L. Otosclerosis. - L .: Medgiz . Leningrad Department, 1958. - 124 p.
- Khilov K. L. , Preobrazhensky N. A. Otosclerosis. - 2nd ed., Corr. and add. - L .: Medicine . Leningrad Department, 1965. - 240 p. - 5000 copies