Otosclerosis (Abnormal Remodeling Disease of The Middle Ear Bones)

Otosclerosis - Abnormal Bone Growth and Sclerosis in The Middle Ear

A Brief Looking at the Anatomy of The Ear

SNHL,Sensorineural hearing loss,Conductive hearing loss, CHL, Ear anatomy

In our ears, there are anatomical structures with different functions in the outer, middle and inner ears. The sound waves transmitted through the outer ear canal vibrate the eardrum (black arrow in the picture on the side) and vibrate to the ossicles in the middle ear immediately adjacent to the membrane. The oscillation system that acts as a lever and the vibration generated by the sound waves are transmitted to our inner ear (the red arrow in the picture on the right). In other words, in transmitting a sound wave to our inner ear, the outer and middle ear should work in a healthy way. Therefore, in diseases and other conditions affecting our outer and middle ears, the sound cannot be transmitted to the inner ear. This type of hearing loss is called conductive hearing loss. In the diseases and conditions related to the inner ear, although the sound transmission; This type of hearing loss is called neural (sensorineural) hearing loss because there will be no detection of vibrations transmitted to the inner ear.

What is Otosclerosis? What Are The Risk Factors? Which is Seen in Age?

Otosclerosis is characterized by the involvement of the middle ear ossicles, the stapes bone that is closest to the inner ear, and sometimes the inner ear; is a disease caused by a disorder related to bone formation and destruction. Calcification focus on the stirrup prevents the ossicles from vibrating freely and prevents sound from being transmitted to the inner ear. Otosclerosis is more common in white and young to middle-aged women. The disease can progress rapidly during pregnancy with the effect of estrogen. It is a genetic disease of otosclerosis, with an autosomal dominant inheritance. Children with family otosclerosis are more likely to develop otosclerosis than other individuals in the community. Flour malnutrition and measles infection are also possible factors. The exact cause is unknown.

Symptoms of Otosclerosis

Otosclerosis can be seen in some of the following symptoms:

Hearing loss

In otosclerosis, symptoms such as hearing loss and ringing or dizziness may occur in the foreground depending on the affected areas. 80% of both ears are affected and 90% of people with eotosclerotic foci have no symptoms. It usually occurs in a 30-40-year-old female patient and there is increasing hearing loss. Between the ages of 50 and 70, the loss of my work is usually fixed up to a certain level. Hearing loss may increase especially with the effect of estrogen hormone during pregnancy. Some of the patients express better hearing in noisy environments. This is called Willis Parakuzisi.


Approximately 2/3 of patients with otosclerosis complain of tinnitus. It is more common especially in patients with cochlear involvement. Especially in the form of engine sounds or water sounds and other forms of ringing complaints may occur. It may disturb the patient, especially in a quiet environment and falling asleep at night. The amount of tinnitus may increase if there is damage due to a pre-existing tympanic hearing loss or drug use.


Imbalance and dizziness may be seen in 25-30% of the patients. Patients with otosclerosis may also experience dizziness when the inner ear is affected; however, there is controversy about the causes of dizziness in patients with otosclerosis. Likewise, after the surgical treatment of otosclerosis, dizziness may be seen in the first few days.

Diagnosis of Otosclerosis

In otosclerosis, the diagnosis is made after physical examination, audiometric tests, tuning fork tests and sometimes together with the desired imaging methods.

Classically expected examination and test results in patients with otosclerosis can be summarized as follows:

◊ Normal eardrum examination
◊ Schwartze (Flamingo Red) Symptom: A pink remnant of hyperemia due to the promontorium on the inner ear of the eardrum (occurs during the active phase of the disease).
◊ Tuning fork tests: Rinne (-) lateralizes the Weber pathological ear. Gelle test result: pathological.
Omet Audiometry result: Conduction type, Mixed type or Sinal type hearing loss.
Ped Impedancemetry: In addition to the normal (sometimes "As" type) tympanogram curve, ipsilateral and contralateral stapes reflex may not be obtained in the probe ear.
◊ X-ray: Normal mastoid ventilation is observed.

Audiometry may show a specific finding for otosclerosis. In the audiogram, a notch that becomes evident due to the increase in bone thresholds at 2000 Hz can be observed, which is called the h Carhart Notch ”.

In pathological examinations, autoskerotic foci are stained basophilic in HE staining. This view is called Mant Blue Mantle ”. It is one of the early signs of otosclerosis.

Diseases and conditions that can be confused with otosclerosis (differential diagnosis)

◊ Ossicular chain break
◊ Congenital stirrup fixation
◊ Fixation of the incuse head
◊ Paget's Disease
◊ Osteogenesis Imperfecta Disease

Treatment of Otosclerosis

Separate treatment is planned for each patient. Especially in the 1960s, although the use of sodium fluoride as a preservative; sodium fluoride is not recommended by current sources. Therefore, there is no preventive measure against the disease; Medical treatment and follow-up is recommended unless hearing loss is very severe. The results of treatment options other than surgical intervention are insufficient. It is appropriate to perform the surgical procedure after the patient reaches adulthood, before complete deafness occurs, and during a period when the disease stops. In terms of long-term outcome, there is no reliable treatment option other than surgery.

Medical Treatment, Follow-up and Use of Hearing Aids in Patients with Mild Hearing Loss (Amplification)

Patients with mild hearing loss can be followed up and 6-month cures can be planned for soybean fluoride treatment in patients with active disease. Since it is controversial that otosclerosis is caused by a defect in bone formation and destruction, some sources argue that vitamin D and calcium carbonate may also be used in treatment. Sodium fluoride is replaced by the hydroxyl portion of the hydroxyapatite in the bone, resulting in the formation of fluoroapateite and increases bone resilience by increasing calcification in the bone. Sodium fluoride treatment is recommended especially for patients with Schwartze (Flamingo Redness) symptoms during the examination. Surgical treatment should be avoided in this importance which is thought to be active. The use of hearing aids (amplification) may be an appropriate option in patients whose hearing loss becomes more prominent and refuses surgical treatment.

Surgery is the most effective treatment for otosclerosis. Surgical procedure has a higher chance of success in the presence of the following factors:

Factors Related to Hospital and Physician

◊ The ENT specialist has mastered the ear anatomy and has performed a sufficient number of ossicular surgical procedures
Yeterli Adequate technical equipment of the hospital

Patient Related Factors

◊  Patient's willingness to surgery
◊ Good health of the patient
Kulak Having previously unoperated ear
◊ At least 25-40 dB hearing loss in audiometry and air bone gap is high.
◊ Good speech discrimination score
◊ Rinne test is negative

Surgical Treatment of Otosclerosis

After total stapedectomy surgery, first described by John Shea in 1956 for the treatment of otosclerosis, stapedotomy operations were described with developing technology and medical experience. I wanted to share the names of the surgical techniques that were published to you. In fact, all of these surgical procedures consist of removing the areas affected by the disease and replacing the replacement ossicles or prostheses that serve as ossicles. As the stirrup bone is mostly held, removal of this region and replacement of the prosthesis in the form of pistons are performed.

A technique is determined based on the experience of the surgeon and the equipment of the hospital. Particularly in recent years, the popularization of the stirrup bone with laser or the use of different prosthesis applications in the form of attention. Each method has its own potential complications and risks. I prefer stapedectomy or stapedomi surgery with the help of microture, especially after making a single incision in the external auditory canal.

After entering the middle ear during the surgical procedure, the joint of the stirrup with the hammer bone is cut, the tendou is cut and the base is removed or pierced. If the base is removed, the procedure is called "stapedectomy" and the base is called "stapedotomy". After the base has been removed or punctured, it is placed between the denture and the base of the stirrup.

In fact, this is the last stage of surgery. Too much insertion of the prosthesis may cause the patient's complaint of increased dizziness with postoperative head movements; Blood leakage into the inner ear before implantation can result in increased postoperative hearing loss or dizziness. In addition, if the arm of the prosthesis is over-tightened, the long arm of the hammer bone may break, while over-inserting the ear tampon may cause the eardrum to stick directly to the prosthesis. No doubt; The experience of the surgeon and the planning of the surgical procedure according to the patient is a very important factor in surgical success.

It is normal for dizziness that lasts for the first few days after surgery. After a well-selected patient and a good surgical intervention, many patients complain of hearing loss.

Murat Enoz, MD, Otorhinolaryngology, Head and Neck Surgeon - ENT Doctor in Istanbul

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