Otosclerosis

Otoscleros (synonym topogeos) is a disease of the ear, which consists in the growth of bone tissue in the area oval window, which connects the middle ear to the inner. The result of this process is hindered or completely stops the transmission of sound vibrations through the system of auditory ossicles to the inner ear. In some cases, the abnormal growth of bone extends to the region round Windows and channel snails.
The cause of otosclerosis is not known. In occurrence and course of the disease, apparently, play the role of endocrine factors. There are indications of known value in the etiology of otosclerosis prolonged exposure to noise.
Pathological changes in otosclerotic hearth consist of different stages of development of the newly formed bone. Most often, the focus has the appearance of loose spongy bone, and rare lesions consisting of a dense, Mature bone.
Otosclerosis - disease almost always bilateral, begins in adolescence (15-17 years), sometimes in children (8-10 years), women more often than men. The disease is progressive decline of hearing and noise in the ears. Tinnitus is often the main complaint of patients. The process develops slowly and gradually, beginning of the disease usually goes unnoticed, and patients go to the doctor already under significant decrease in hearing. Pregnancy and childbirth often speed up the process. Often the otosclerosis leads to a sudden loss of hearing, sometimes to complete deafness.
The diagnosis. Characteristic of otosclerosis is the early start, the progressive decline of hearing, expressed tinnitus. When otoscopy (see) pathological changes in the ears do not detect. At hearing research tuning forks and audiometer bone conduction is normal or close to normal, lowering the air conductivity is more pronounced in low tones (see Audiometry). Characteristic of otosclerosis is the improvement of speech perception in noise and concussion (tram, subway).
Treatment. In the early stages of the disease, the recommended drugs phosphorus, calcium, iodine, bromine (phytin 0.25 g three times a day, glycyrrhizinate calcium 0.3 g 2-3 times a day, potassium iodide, sodium bromide in 3% solution for 1 table. L. 3 times a day), subperiosteal injections in the area of the mastoid process of 2% solution of sodium nitrite (Natrii nitrosi) and 1 ml every other day. All these funds designated for 1 - 1,5 months, repeating such courses every 2-3 months. With a significant decrease in hearing can be used hearing AIDS.
Recently when otosclerosis widely used surgical intervention, reducing the mobility of the stirrup by loosening his (mobilization stapes) or the creation of artificial holes in the lower plate; resort to completely remove the stapes (stapedectomy). The hole close transplant (often venous), and remote parts of the stapes replace plastic prosthesis. This operation was named stapedoplastiki. With a full commit the stapes in the oval window and resort to the creation of artificial oval window in the outer semicircular canal (fenestration of the maze).

Otosclerosis [otosclerosis; from the Greek. ous, otos - ear and sclerosis - hardening; synonym oospores (otospongiosis)] - focal lesion of the bone capsules labyrinth, manifested in the form of progressive hearing loss, usually on both ears and tinnitus. Otosclerosis usually occurs during the years of puberty and slowly progresses throughout life. The cumulative fall hearing loss is often the result of fixing otosclerotic hearth pasture plates stapes in the oval window. Localization otosclerotic hearth in the area round window or in the cochlea of the inner ear canal also lead to hearing loss.
Women get sick more often. Otosclerosis is one of the most frequent reasons for the decrease in hearing when nagnoenia lesions ear.
Otosclerotic changes histological examination of the temporal bone find not in all cases. Otosclerosis with clinical manifestations occurs in 5-10 times less frequently than Acting, histologically determined.
The term "sclerosis" in relation to the ear disease meant sclerotic changes of the mucous membrane, leading to commit the stirrup. The Politzer (A. Politzer) proposed to use the term "otosclerosis", as it revealed that the basis of this disease are not pathological changes in the mucous membrane of the middle ear, and a kind of pathological process in the bone capsule of the maze. These data Politzer were confirmed by histological research of Bezold (F. Bezold), Sibomana (F. Siebenmann), and other
Ziberman coined the term "topogeos", as in otosclerotic the hearth there are areas not only seal bones, but its resorption.
The etiology and pathogenesis of otosclerosis is not clear. Some authors give importance to the factor of heredity (according to C. F. Andrica, exogenous factors can reveal hidden forms of inherited otosclerosis), others anomalies of the Constitution. Many authors believe that the changes capsules maze when Acting are an expression of the inferiority of the mesenchyme of the body. This opinion is confirmed by clinical observations. Eder-Dayton (Adair-Dighton) noted syndrome simultaneous existence Acting in combination with blue sclera and osteopsathyrosis. This syndrome is inherited, but also possible partial transfer of signs of inferiority of mesenchyme, for example, only one O. Other signs may be in a hidden state.
Noted that on the occurrence and course of otosclerosis influence neuro-endocrine factors. A connection is established onset of the disease and strengthen its manifestations with puberty, pregnancy, childbirth. Some authors have observed when Acting dysfunction of endocrine glands. Great importance dysfunction parathyroid glands. By N. Century Belogolovov, the main reason for the occurrence and development of O. is in action on bone capsule labyrinth sound injury, which is confirmed by experiments on white mice. Effects on the ear of ultrasonic waves contributes to the proliferation of new bone in the capsule of the maze and is an important factor for the emergence and development O. the Number of authors considers that the reason of occurrence of O. is in violation of the circulation of blood in the bone capsule of the maze.
K. L. Chilov suggests that otosclerosis is a defeat not only bone capsules of the maze, but just auditory analyzer as a whole, including its cortical Department.
One of the predisposing factors in the development O. the author considers a violation of vitamin balance, in particular deficiency of vitamin D.

Fig. 1. Otoscleros (histological pattern): 1 - common osteosclerotic hearth in the lower plate of the stirrup.


Pathological anatomy. Macroscopically otosclerosis hearth more clearly distinguished from the capsule of the maze. It is usually clearly you can see when 2-10-fold-s ' increase during the operation. Most often the focus is located between the front edge of the oval window, and the snail, at least - at the outer surface of the membrane round Windows.
Often in the process involves promontorium. Sometimes isolated otosclerosis hearth is formed in the inner ear canal, in a small percentage of the foci of scattered to the capsule of the maze, but never replace the whole capsule.
Histologically otosclerosis hearth is a clearly distinguished from dense capsules labyrinth area of the newly formed incorrectly built bone with numerous vascular spaces. This newly formed bone is painted haematoxylin-eosin much brighter than the surrounding normal bone capsules labyrinth (Fig. 1). Histological examination otosclerotic focus you can meet the different stages in the formation of bones.
Focus can be loose spongy bone formed incorrectly located plates young bones are separated from each other by wide sections of the bone marrow in which find osteoblasts and osteoclasts, or as much denser Mature bone with a relatively small areas of the bone marrow.
Assume that the spongy bone characteristic actively increasing otosclerotic hearth, and dense for inactive, Mature home.
Advanced vessels hearth give mucosa pink color. Clinically it is sometimes a symptom of x-ray bright pink promotorului wall through the ear drum (symptom Schwartze). This symptom indicates active otosclerotic process.
Over and symptoms. The main signs of otosclerosis are progressive hearing loss (see) and the perception of tinnitus (see). Usually these symptoms occur in both ears. The disease usually develops very slowly. In some cases, the process progresses rapidly - with the disease earlier than usual, age, deterioration in the perception of high tones through the air and bone at a young age, the presence of patients with otosclerosis in the family. The decrease in hearing may be suspended temporarily and then under the influence of exogenous or endogenous adverse factors progressing rapidly.
The feeling of tinnitus in the intensity and nature can be very diverse. Sometimes it is so considerable that is a major symptom of concern to the patient.
Diagnosis of otosclerosis in apparent cases does not represent serious difficulties, sometimes - when uncharacteristic anamnestic data, with mixed type of hearing loss, some of the changes from the eardrum is to establish an accurate diagnosis can be difficult. For diagnosis is essential history. Characterized by the absence of any specific reasons that caused the decrease in hearing, the gradual development of the disease, the progressive loss of hearing, the coincidence of the onset of the disease with puberty or with the first years after this period, hearing loss during pregnancy or after birth, the presence of patients with otosclerosis in the family.
The permanent objective sign of otosclerosis is hearing loss by type of violation of the conductive function of the device (Fig. 2). Typical About. even decrease the perception of all tones air conductivity in normal or near normal bone conduction. Often there is a mixed type of hearing loss, i.e. a violation of the functions not only of conductive, but zvukooperatora system. In many cases, it is expressed paradoxical symptom paracusis Willisii (see), namely, that patients better hear speech in noisy environments - on the street, in the subway, tram, train, etc.
Negative experiences Gellee or M. F. Tsytovich testify to the frets the stirrup. Valuable diagnostic value also have other audiological tests (see Deafness). Otosclerosis must be differentiated from other diseases that cause hearing loss by type of dysfunction of conductive. Because when Acting may be affected function not only of conductive, but zvukooperatora system, require differential diagnosis between the appropriate form O. and defeat the cochlear nerve or the Central Department of the auditory analyzer. This is primarily achieved audiological examination of the patient (see And biometrics).

Figure 2. Typical audiogram with otosclerosis.