Otosclerosis Causes Symptoms And Treatment

Otosclerosis is a disease caused by abnormal growth of the osteal capsule of the labyrinth, which leads to a change in the mobility of the auditory ossicles of the middle ear.

 The main symptom of otosclerosis is a gradual decrease in hearing, mainly at low frequencies. The incidence of otosclerosis is 1% of the entire population, in 80% of cases, the sick are women. 

 

There is a frequent and significant deterioration in the condition or appearance of the first signs of otosclerosis due to pregnancy, as well as during other hormonal changes (puberty, menopause).

Causes of Otosclerosis

To date, there are several theories of otosclerosis, but the hereditary nature of the disease is beyond doubt. These or other genetic modifications were detected in almost half of the patients. The family character of the disease serves as a confirmation of the heredity of otosclerosis.
How exactly the genes predisposed in the genome are realised, the scientists so far only speculate. There are versions of the metabolic, endocrine nature of the disease. The role of some trigger factors, the impact of which is necessary to trigger pathological changes, is proved. 

These are infections, trauma, blood supply disorders of the labyrinth and other causes. In rare cases, otosclerosis is preceded by congenital ear pathology (for example, congenital fixation of stapes).

Pathogenesis of otosclerosis

To understand what happens with otosclerosis, you need to understand the structure of the organ of hearing and the mechanism of perception of sound. 

The ear consists of the outer part (the auricle and the outer ear canal), the middle ear, limited from the external eardrum, and from the inner one by the oval window, and the labyrinth (inner ear) filled with liquid and which is directly the receptor part of the auditory analyser.

 The sound is picked up by the outer ear, transmitted on average through the auditory ossicles (malleus, anvil, stapes) to the inner, where the hair cells transmit a nerve impulse along the peripheral nerves to the brain.
Although the capsule of the labyrinth is considered to be a bone structure, in reality, it is not generally subjected to secondary ossification. Activation of osteogenesis leads to the development of otosclerosis.

 First, areas of active spongization (with intensive blood supply) are formed, in which mature bone tissue is gradually formed. If the oval window zone and the base of the stapes are involved in the process with the development of its ankylosis, the sound transmission function is violated-conductive hearing loss occurs.

 When bone tissue is formed in the spinal apparatus (cochlear otosclerosis), the process of perception of impulses in the acoustic analyser is disrupted – neurosensory hearing loss develops.


In almost all cases, otosclerosis is bilateral, although initially, the process manifests itself on the one hand. Unilateral otosclerosis remains no more than in 8% of cases.

Symptoms of otosclerosis

At the initial stage, when the foci of osteogenesis have already appeared, but the perception of sound has not yet been violated, otosclerosis may not manifest itself in any way.

There are cases of rapid development of the disease, but more often from its onset to the appearance of the first symptoms takes 2-3 years. The early signs are usually tinnitus, and hearing loss can be detected only when carrying out audiometry.

The initial stage passes through the period of unfolded clinical manifestations. The patient has the following symptoms:

    • Hearing loss develops slowly and initially affects only low frequencies, usually has a one-sided nature. Gradually, the hearing loss begins to spread to high rates, and the second ear is involved in the process. This happens 1-2 years after the appearance of the first symptoms. Hearing loss is steadily progressing, that is, there is no improvement. At the same time, there is never complete deafness, maximum 3 degree of hearing loss.
    • Noise in the ears appears before the hearing loss and asosiate with it regarding intensity. The sound is caused by vascular and metabolic changes in the cochlea. It can be high or low, but usually not very strong. Noise in the ears is noted not in all cases, but quite often, in about 80% of patients.
    • Dizziness associated with changes in the labyrinth are quite rare. They are non-intensive and pass quickly.
  • Pain in the ear can accompany the active growth of bone tissue. It is bursting and localised in the region of the mastoid process. Given the cause of pain, often after an attack, hearing deterioration is noted.

Diagnosis of otosclerosis

It is not easy to diagnose otosclerosis. This requires an instrumental determination.

The purpose of which is to exclude all other diseases accompanied by a decrease in hearing and noise in the ears (chronic adhesive otitis, neuritis of the spinal nerve, labyrinthitis, etc.)
Upon examination, the doctor carefully collects an anamnesis and conducts an otoscopy. Which allows you to assess the condition of the tympanic membrane and external auditory canal. With otosclerosis, the Holmgren triad is revealed:

  • the absence of earwax;
  • dryness and flaking of the skin of the auditory canal;
  • a decrease in the sensitivity of the skin of the ear canal to irritations.

The tympanic membrane is usually not altered, but it may have a site of atrophy (Schwartz spot). Otoscopy allows to differentiate otosclerosis from chronic otitis media, in which the membrane is deformed and retracted, and maybe perforated.

 

To visualize foci of otosclerosis, radiography or computed tomography (CT) scan of the skull or only the temporal bone is used. CT is more informative and recommended for diagnosis in difficult cases.

Functional methods for diagnosing otosclerosis

It is very important to assess the functional abilities of the hearing organ when suspected of otosclerosis. In the early stages, the changes do not manifest themselves clinically but can be detected by careful examination.

  • Audiometry reveals a decrease in perception of low, and then all other frequencies, as well as whisper speech;
  • A study with a tuning fork reveals normal or increased conduct of sound through tissues, but reduced through the air;
  • The threshold sensitivity to ultrasound is determined for differential diagnosis with cochlear neuritis;
  • Acoustic impedance measurement reveals a decrease in the mobility of the auditory ossicles;
  • The vestibular function is examined with indirect otolithometry, sialography, vestibulometry, but these techniques are of secondary importance since vestibular disorders accompany otosclerosis are not always and are not a pathognomonic symptom.

Treatment of Otosclerosis

Conservative therapy of otosclerosis is possible, but more often than not, surgical intervention is required to restore the hearing. 

Treatment of otosclerosis without surgery is reduced to the appointment of drugs that regulate the exchange of calcium (Xenophon, Fosamax), as well as the calcium in combination with vitamin D3. 

These medicines are prescribed by courses from 3 to 6 months and are held annually. They help to slow the process of hearing loss, in some cases help to get rid of noise in the ears or make it less pronounced.

 Also in the treatment schemes often include preparations of phosphorus, calcium, iodine, bromine. Sometimes subperiosteal injections are administered in the region of the mastoid process of a 2% solution of sodium nitrite.

Surgical treatment of otosclerosis

Most often, with otosclerosis, septolasty is performed, in which partial or complete prosthesis of the sternum is performed. It is carried out sequentially, first on one, then on the other ear with an interval of at least six months.

The prosthesis can be made from the patient’s cartilage or bone tissue or Teflon, titanium, and ceramics. Stapedoplasty does not stop osteogenesis in otosclerosis, so it is not 100% effective.
Another option for otosclerosis is the mobilization of the stapes. During the intervention, the stapes are released from all bone-cartilaginous fissures that prevent it from moving normally. 

Sometimes it is performed fenestration of the labyrinth (the very first of the developed methods of surgical treatment of otosclerosis) – the creation of a new window in the wall of the vestibule of the labyrinth.

The effect of both operations is unstable; the hearing is restored for several years, and then rapidly deteriorates.


Each of the operations is aimed at restoring sound conduction, that is, it will be effective first of all with the conductive hearing loss, that is, when the process of ossification affects the membrane of the labyrinth and the stapes.

Prophylaxis of otosclerosis

Given the hereditary predisposition to the disease, it is necessary to prevent its manifestation and further progression. It has been proven that overwork, stress, bad habits (alcohol, smoking) significantly worsen the course of otosclerosis, speeding up the hearing loss. The same action has strong noises, adverse living conditions, regular hypothermia.

Prognosis of Otosclerosis

Quite often the only way to restore the normal life of patients with otosclerosis is the use of hearing aids (hearing aid). Disability with otosclerosis usually does not give, even with a bilateral hearing loss of grade 3.

Acquire effective drugs to treat this disease

 

Can Otosclerosis help in Hearing Aid

 

  • Otosclerosis is a very unpleasant disease, as a result of it develops deafness and the person is deprived of the opportunity to communicate normally with the surrounding world. Such a person needs help.
  • An effective remedy for otosclerosis is the compensation of hearing loss with the help of special electronic-acoustic devices – hearing aids.
    In 90% of cases, the hearing aid rehabilitates this disease in the early stages, returning the person to the World of sounds.
     To get a patient with hearing deficiencies from the hearing aid? First of all, those inaudible sounds are available to his ears, so that all words of speech become clear, and music is legible.
  •  
  •  So that the hearing aid does not create any discomfort – it did not have its noise, so that the surrounding noise environment did not reproduce to the level of irritation, but suppressed it to fit comfortably in the ear without unpleasant sensations. Finally,
  •  I would like to satisfy the aesthetic desires so that the hearing aid is elegant and invisible.
    For many years scientists, engineers, acousticians, medical specialists of the advanced countries of the World have been busy creating and improving the hearing aid. 
  • Today, the highest level of this technique has been achieved, capable of satisfying the greater part of people who are hard of hearing.

  • Sometimes there are fears that the hearing will worsen when using a hearing aid. Fears are causeless. To overcome the lack of hearing without a hearing aid, you have to strain the auditory, and the general nervous system, destroying it.
  •  
  •  And the hearing aid will relieve this tension and keep the hearing. Moreover, at the same time, the auditory nerves are stimulated, and the brain regions responsible for the hearing are activated.

How Otosclerosis Develops?

 Otosclerosis is understood as dystrophic changes in the bone capsule of the labyrinth. These changes are of a focal character and are localized most often in the area of the oval window; spreading, they capture the footplate of the stirrup, which leads to a violation of its mobility.
 If the mobility of the stapes is disturbed, signals from the middle ear cease to flow to the inner ear, which leads to complete deafness.
Usually, otosclerosis begins at a young age (16 to 20 years), more often in women than in men.
The causes of the disease have not been clarified to the present day.

Symptoms of Otosclerosis

The main and permanent symptom of otosclerosis is a decrease in hearing. The nature of the loss of auditory function depends mainly on the location of otosclerotic foci.
If only the stapes are affected, the sound reduction is reduced mainly, the bone conduction remains normal. If the pathological process affected the cochlea, then the sound perception is also affected.
Another important symptom of otosclerosis is tinnitus. Its appearance is associated with irritation of sensitive auditory nerves with a growing bone. Often, noise is the main and first sign of the disease. Patients even note that they hear better in a noisy environment than in silence.
The main characteristic feature of otosclerosis is progressive deafness. In some cases, deafness develops quickly and leads to complete deafness within 3 to 5 years. In other cases, the disease progresses slowly and deafness occurs only after twenty to thirty years from the onset of the disease.
Deterioration in the state of health of patients often comes after infectious diseases, intoxications, in women after childbirth.
Treatment – surgical
Since in otosclerosis the cause of the disease is bony changes (and they do not lend themselves to therapeutic methods of action), one of the most effective methods of treatment is surgical intervention, in which the stapes are removed and the transmission of excitations from the middle to the inner ear is partially restored.
Based on the article “What is otosclerosis”.

Otosclerosis: save our ears

 

Treatment of otosclerosis is carried out by two methods: the use of hearing aids or surgical intervention – stapedectomy. Hearing aids can help effectively cope with hearing loss only in the early stages of the disease.
Among the surgical methods of treatment of otosclerosis, there is a stapedectomy. This intervention is carried out on the stapes. It is performed when there is a fixation of the stapes to other auditory ossicles or to the oval window. The operation consists in removing the stapes and replacing it with a prosthesis, or a small hole is made in the stapes and a thin prosthesis like a valve is inserted into it.
The operation gives 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 underwent this operation, and only in less than 1% of operated patients, the operation was ineffective.

Contraindications to surgery

Contraindications to stapedectomy are:

  • The severe general condition of the patient.
  • One hearing ear.
  • Low reserve function of the cochlea.
  • Tinnitus or dizziness.
  • Presence of active foci of otosclerosis.

Stapedectomy: during and after surgery

The operation is performed under general anaesthesia. To perform the operation, a special microscope and miniature instruments are inserted into the ear canal. A circular incision is made along the edge of the tympanic membrane, after which the flap of the membrane is raised.
Then the stapes are removed and a plastic bone prosthesis is put instead. After the auditory ossicles are connected again, the flap of the tympanic membrane is put in its place, and a swab moistened with antibiotics is left in the ear canal. Sometimes the surgeon makes a small incision in the earlobe and takes from there a small piece of fat tissue that is installed in the middle ear, which contributes to better healing. After the operation, the patient is usually in the hospital for 4 to 5 days.
After the operation, at first, the patient can note some discomfort and pain. In order to stop the pain syndrome after the operation, pain medications are usually prescribed. In addition, it is strongly recommended not to blow your nose and do not make sharp retractions through your nose.
This is due to the fact that on the back wall of the nasopharynx there are holes of auditory (eustachian) tubes that connect the nasopharynx cavity with the middle ear. The role of these pipes is in equalizing the pressure in the tympanum. With sudden movements of air in the nasopharynx, the pressure in the tympanic cavity can increase, which leads to the movement of the tympanic membrane, and this, in turn, is fraught with a dislocation of the flap and a violation of its engraftment.
Any cold in the postoperative period should be avoided, as this can lead to the development of infectious inflammation in the middle ear. If you notice any discharge from the ear, you should see a doctor.

Possible complications

It should be noted that even in spite of the excellent operation, after it there may be such complications as:

  • Paralysis of half of the face on the side of the affected ear due to damage to the branches of the facial nerve.
  • Vertigo in the postoperative period.
  • Vomiting.
  • Isolation of perilymph from the ear.
  • Rupture of the tympanic membrane.
  • Damage to the labyrinth.
  • Labyrinthitis (inflammation of the structures of the inner ear).
  • Complete deafness of the operated ear.
  • Constant sensation of noise or buzz in the operated ear.

Based on the article “Treatment of otosclerosis. Stapedectomy is the technique of surgery. “

How can I help myself with otosclerosis

 

  1. With such a disease as otosclerosis, the conditions of a patient’s home life and his profession are very important. The room in which a person lives, sick with otosclerosis, must be completely dry, well ventilated and of sufficient warmth; smoking should be prohibited in the room, as well as the burning of the stoves, ironing, in short, everything that pollutes the air and adversely affects the condition of the upper respiratory tract.
  2. The food of the patient should be free from irritating and causing a rush of blood to the head of substances; for the same reason, the ill person is contraindicated in the use of alcoholic beverages, smoking tobacco, as well as cold wiping of the head and neck, walking on warm days with uncovered head, visiting the beach (sea and even river).
    The work of the patient with otosclerosis occupies an important place; he should avoid any fatigue – physical or mental – and should not engage in a profession that causes flushes of blood to the head or upper respiratory tract or inflammatory changes on the part of the latter.
  3. In the case of exacerbation of otosclerosis or the strengthening of any of its individual manifestations, it is useful to completely eliminate a person from his usual daily activities, which drain the body physically and mentally. In view of the harmful influence on the course of otosclerosis of all kinds of emotional unrest and emotions, it is worth paying special attention to people close to the sick person, and they should diligently follow this. Indeed, one often sees how, at first glance, a slight disturbance causes an unexpectedly sharp exacerbation of the course of otosclerosis or, at least, an increase in ear noise.
  4. It is also important: in cases of abnormal abnormalities in the nasal cavity and in the nasopharynx, care must be taken to carefully treat these organs, since otherwise, the diseases of the latter can adversely affect the course of otosclerosis.
Mud cure with otosclerosis: will it help or not?
A favourable effect on otosclerosis of mud treatment was first reported at the 1st All-Russian Congress of Otolaryngologists.
Those wishing to recover and cure otosclerosis several times performed mud treatment procedures in various places. What has come of this?
In the Caucasian mineral waters, 27 patients with otosclerosis were treated with mud, and in the majority of patients (20 of 27) after the fourth to the fifth procedure, there was a significant decrease in noise and cheerfulness increased, and some even improved hearing.
Of the 38 cases of otosclerosis treated with Tambukan mud, there was a complete disappearance of the ear noise in six cases, in 27 – weakening of them, and only in five cases the force and nature of the noise remained unchanged.
Thus, the therapeutic effect of mud therapy seems to exist, but it should be taken into consideration that applying mud cakes directly to the area of the hearing organ causes there phenomena of blood tide and venous congestion, i. E. just those factors that are called among the causes of the onset and development of otosclerosis.
Therefore, if you decided to try the mud treatment method to ease your suffering, then instead of putting mud cakes directly on your ear, put mud compresses on your neck in the form of a collar. And be healthy.
Try a massage
One of the methods for alleviating the suffering of a patient with otosclerosis is considered pneumomassage, especially when using a hand-held device Delstanch’a, Noebel’ya, etc .; In this way, it is sometimes possible to reduce the subjective ear noise or even achieve some improvement in hearing.
Also, it is recommended to perform manual massage with the index finger: with a finger placed on a tragus, the patient makes vibrating movements up to 100 – 150 times per minute; such a massage can be repeated several times a day. It is also possible, especially with strong noises, to massage the area in front of the auricle and behind it; this method contributes to the outflow of venous blood and lymph from the organ of hearing.
Based on the article “Otosclerosis therapy”.

Three forms of otosclerosis

Otosclerosis is a process, which is based on the focal lesion of the osteal capsule of the ear maze.
The essence of the disease: a healthy bone in the lesion is replaced by a newly formed porous, spongy – spongy bone, rich in blood vessels. The more correct is the name “otosposgio”. Usually, the otosclerotic focus is located in the window area of the vestibule, often at the anterior pole of it. Less often – in the field of the window of the cochlea, internal auditory canal, semicircular canals.
Stages of otosclerosis:

  • histological;
  • clinical.

While the changes are localized only in the bone, the process is not clinically manifested. With the transition of the process to the annular bunch of the stirrup, the mobility of the stapes is limited, and gradually the transmission of sounds through the middle ear worsens. There is a progressive deafness and a sense of noise in the ears.

Otosclerosis is a hereditary monogenic disease with an autosomal dominant type of inheritance. There is an opinion on the hereditary inferiority of the ear maze, expressed in an increased sensitivity to ultrasounds. Various parts of the auditory analyzer, including its cortical part, are affected.

It is more correct to treat otosclerosis as a dystrophic process that develops throughout the auditory analyzer, and not just in the capsule of the labyrinth. In the development of otosclerosis, an important role is given to inorganic components. A decrease in the content of many bioactive elements in the bone substance of the stapes has been revealed.

Otoskleroz Multifilament

  • Otosclerosis affects 1 – 2% of the world’s population. More often this disease is observed in people of young and middle age. The first symptoms appear usually at the age of 18 – 30 years, are possible and in early childhood. The process, as a rule, is two-sided. The difference in ear acuity on both ears is usually insignificant.
  • Hearing loss has the property of gradually progressing. There are cases of a fulminant, malignant, transient form of the disease, in which within a few months, almost complete deafness can develop due to the involvement of the inner ear in the process. Significant influence is made by noise and vibration, which allows us to consider otosclerosis as a disease of sound life.
    There are tympanic, cochlear and mixed forms of otosclerosis.
    Timpanal form – the root of the stirrup is immured in the window of the vestibule, its mobility is disturbed.
    The cochlear form (more rare) is the spreading of the process to the cochlea, sometimes to the vestibule and semicircular canals, as well as to the window of the cochlea and internal auditory canal.
  • With otosclerosis, there is a progressive decrease in hearing and a sense of noise in the ears, which is much more difficult for patients than with other forms of hearing loss. When describing the nature of noise in the ears, patients compare it with various phenomena of nature and the everyday environment (surf noise, the rustle of leaves, the buzz of wires).
    Noise in the ears with otosclerosis is divided into three degrees:
    I degree – the sensation of the ear noise of the patient is almost not disturbed, and the presence of this symptom is revealed during active interrogation;
    II degree – complaints about tinnitus along with other complaints;
    III degree – a sense of subjective noise is the leading complaint of the patient.
  • Several symptoms characteristic of otosclerosis – improving hearing acuity when the patient is in a noisy environment, reducing speech intelligibility when swallowing and chewing, the simultaneous conversion of several persons and with intense attention.
  • Often disturbed by dizziness and balance disorders. As a rule, these are short-term dizziness, arising during movement, rapid tilting or tilting of the head. There may be attacks of nausea and vomiting. The reason for dizziness is ithe ntoxication of nerve endings when otosclerosis spreads to semicircular canals and internal auditory canal. Other symptoms are ear pain, tingling and stuffiness in them, headache, memory loss, sleep disturbance.
    When the external examination of the ear is observed:

    • wide external auditory canals;
    • reduction or absence of sulfur secretion;
    • lowering the sensitivity of the skin of the external auditory canal and tympanic membrane;
    • increased mobility of the tympanic membrane;
    • thinning of the tympanic membrane.

    Diagnosis of otosclerosis

     

    otosclerosisThe diagnosis of otosclerosis is based on anamnesis, symptoms, disease course and hearing test data. These hearing tests depend on the form of the disease and on the stage.
    The tympanic form is a significant decrease in the severity of the hearing. When hearing the hearing, the damage to the sound-conducting apparatus is detected. After a trial blowing of the ears, the hearing does not improve.
    Cochlear form – a progressive hearing loss not only in low but also in high tones.
    In most patients, the nature of vestibular reactions is disturbed in experimental samples.
    To diagnose otosclerosis tone, tonal metrics are used – this is a measure of the severity of the hearing, that is, the sensitivity of the auditory organ to sounds of different heights.
    With the x-ray method of diagnosis, the smallest details of the morphological structure of the temporal bone are considered, characteristic changes are observed in the area of the vestibule and snail windows, semicircular canals and internal auditory canal.
    Treatment of otosclerosis
    In order to influence the metabolism, calcium, phosphorus (phytin), bromine, sodium fluoride 20 mg twice daily for 1-2 years, vitamin B, A, E, hormones in time of menopause; electrophoresis with calcium or iodine, darsonvalization (with a slight decrease in tinnitus).
    Improvements in hearing can be expected mainly in tympanic form.
    The most effective treatment is a surgical operation on the stapes. Basic methods of operations:

      • sparing – indirect, direct mobilization, perforation and fragmentation of the base of the stapes, isolation of the otosclerotic focus;
    • Radical – stapedectomy and septoplasty. At present, mainly septoplasty is produced – part of the stapes is removed and replaced with a prosthesis made of synthetic material (Teflon). The efficiency of these operations is very high – 95%.
    Prophylaxis of otosclerosis

    Preventive measures (aimed at preventing the deterioration of otosclerosis) – a quiet lifestyle, avoiding noisy conditions, a diet with a limited content of vitamin D; Avoiding the proximity of the sea, staying in the sun. With a significant decrease in hearing, you are assigned to wear a device that enhances the sounds of speech.

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