Otosclerosis – The Silent Condition Leading to Hearing Loss

Hearing is one of the most valuable senses, essential for communication, orientation, and safety. It allows us to interact with those around us, perceive dangers, and enjoy the beauty of music. While the ear does not require complicated maintenance, there are conditions that can endanger it. Besides infections treatable with medical help, some diseases develop without obvious symptoms and can lead to hearing loss. A notable example is otosclerosis.

What is otosclerosis? Also known as otospongiosis, it is a condition of the inner ear that involves the deformation or remodeling of the tiny bones in the middle ear. This structural change affects the transmission of sounds, which can gradually lead to hearing loss. The main problem is that the disease develops slowly, making it difficult to detect in its early stages and potentially dangerous if ear health does not receive the necessary attention.

The article below highlights the most important things we need to know about otosclerosis. From it, we will learn how and why bones deform, but especially what we can do to prevent hearing loss and what the main preventive measure available is.

Otosclerosis – what it is and what causes it?

The definition of otosclerosis is based on the terms “oto,” which refers to the ear and hearing, and “sclerosis,” meaning abnormal hardening of tissues. This condition involves a process of abnormal remodeling of the small bones in the middle ear. Normally, bone remodeling is a continuous process, essential for replacing old bone tissue with new.

However, in the case of otospongiosis, this process does not occur correctly, which can lead to gradual hearing loss. How does this happen? During the demineralization of the bones, the transmission of sounds from the middle ear to the inner ear is affected, disrupting the auditory process. Thus, hearing degrades progressively.

Can otosclerosis be prevented? There are no direct ways to do this, but research has shown that in countries where the measles vaccine has been administered, the incidence of the disease has decreased. What can we do for this purpose? Besides regular check-ups, we can use various supplements to ensure our bone health.

One of these supplements is Liposomal Vitamin D3 at 4000 IU. As we well know, vitamin D is essential for the health of our bones. Although we can also get it from food and the sun, it is harder to fix in the body from these two natural sources. However, in liposomal form, it is rapidly absorbed into cells and blood by 99%.

Among the benefits of vitamin D3 are support for the skeletal system, the immune system, and even the proper functioning of the heart and brain. Its regular consumption is especially recommended for children, pregnant women, and the elderly.

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Otosclerosis of the ear – how does it affect the complex process of sound perception?

To understand how otosclerosis develops in the ear, it is important to know the process by which sound is perceived. Sound waves captured from the environment are converted into electrochemical signals, which are then picked up by the auditory nerve and transmitted to the brain. The path of these sound waves involves several stages:

• first, they reach the outer ear;
• from there, they travel through the ear canal to the eardrum;
• once they reach the eardrum, they cause vibrations in it;
• these vibrations reach the 3 bones in the middle ear: the malleus, the stapes, and the incus;
• the bones amplify the vibrations, sending them into the inner ear to the cochlea where there is fluid;
• furthermore, that fluid moves, forming and sending a sound wave along the basilar membrane on which the auditory structures of the cochlea are located;
• the membrane is covered by tiny hairs that move when capturing sound waves;
• these movements lead to the opening of pore-like channels, which secrete chemical substances and generate an electrical impulse;
• the auditory nerve picks up this impulse, passes it on to the brain, helping us to recognize the sound.

Otosclerosis occurs in the middle ear, specifically in the 3 bones that renew themselves throughout our lives. When remodeling is defective, the transmission of sound to the inner ear is affected, leading to a decrease in hearing quality.

What are the causes that lead to the appearance of otosclerosis? The main trigger has not been established yet, but in most cases, the condition is genetic.

Otosclerosis and its causes

Most often, otosclerosis is caused by genetic factors. Studies have shown that in almost 50% of cases, heredity was the main cause of the disease. Mutations in the OTSC1, OTSC2, and OTSC3 genes are considered the main culprits for the onset of the condition. However, it can also occur as a result of hormonal changes in women, especially during pregnancy.

Another cause of otosclerosis can be an abnormal immune system response during bone regeneration. This can lead to tissue inflammation and abnormal formation of tissue. However, studies have not revealed the exact way in which it acts as a trigger for the condition.

One of the latest causes that can lead to the onset of otosclerosis is measles virus infection. Although this factor has not been definitively established, several studies have shown that in countries where the vaccine against the disease has been administered to a large part of the population, its incidence has decreased.

Who does otosclerosis affect?

According to data collected from several countries, otosclerosis is often found in young people, between the ages of 20 and 40. At the same time, women are affected to a greater extent, suggesting a possible link with hormonal factors. Other studies have shown that this condition is more prevalent among Caucasian populations.

Based on the triggering causes, we must know that, most of the time, otosclerosis is bilateral. Unilateral otosclerosis is very rarely encountered.

Bilateral otosclerosis – what does it mean?

Otosclerosis is frequently bilateral, meaning it usually affects both ears simultaneously. Due to the slow progression of the disease, it is possible to fool ourselves into believing that we only occasionally experience hearing difficulties. Hearing loss progresses gradually, starting with a decrease in the ability to perceive low-frequency sounds.

A distinctive feature of otosclerosis is that, although it affects both ears, the disease may initially start in only one. This particularity can create the impression that the problem is temporary, like a simple earwax blockage.

Otosclerosis – symptoms and progression

How do we recognize the symptoms of otosclerosis and the signs of a possible hearing problem?

Otosclerosis is a silent condition that progresses slowly, requiring special attention to detect any changes in hearing in time. It is important to know the signs that may indicate hearing loss caused by otospongiosis, so that we can promptly consult an ENT specialist.

People suffering from otosclerosis frequently report a feeling of clogged ears or distorted sounds. When these symptoms occur in both ears, especially at an advanced age, it is recommended to consult a doctor for a hearing evaluation.

Here are the most important aspects to pay attention to if we suspect we might have bilateral otosclerosis:

• although the first symptoms of otosclerosis often appear after the age of 30, there are cases where the disease began in childhood;
• otosclerosis is characterized by 3 signs that appear simultaneously, namely hearing loss, tinnitus or ringing in the ears, and vertigo;
• hearing loss occurs slowly, with bilateral otosclerosis affecting both ears in 70% of cases.

These symptoms appear either due to the degeneration of nerve endings or due to changes in the chemical composition of the perilymph.

It is important to note that people suffering from otosclerosis may exhibit all these symptoms, but their intensity and frequency vary depending on the stage of the disease. This variability is an essential aspect to consider when discussing this condition.

Progression of otosclerosis and its stages

Considering that otosclerosis occurs as a result of the replacement of normal bone tissue with sclerotic or spongy tissue, we must know that it sets in in stages. These are:

• Early phase: In this stage, bone resorption and the formation of sclerotic tissue occur, but symptoms are absent.
• Progressive lesion phase: Connective tissue replaces bone, and hearing loss begins to become evident, especially for low sounds.
• Late phase: The process of forming sclerotic bone in the areas affected by resorption is completed.

In many cases, the lesions caused by otosclerosis occur exclusively at the oval window, which can lead to:

• calcification of the annular ligament;
• blocking of the stapes footplate.

Both these conditions cause conductive hearing loss, perceived as a distorted perception of sounds.

In more severe situations, otosclerosis can affect the cochlea or the entire ossicular chain, resulting in sensorineural hearing loss.

When should you consult a doctor?

It is important to see a specialist at the first signs of hearing loss or if you experience the three characteristic symptoms simultaneously: poor hearing, tinnitus, and vertigo.

Otosclerosis – diagnosis and treatment

The diagnosis of otosclerosis is made by an ENT specialist who will perform a series of tests to establish it. In the first phase, they will talk to the patient to take the medical history. The medical history is very important, as it can indicate the approximate time of the onset of hearing loss. Also, during the initial consultation, the symptoms presented by the person will be determined. A visual examination of the ear is also not excluded.

Subsequently, the following investigations may be performed:

• audiometry;
• impedancemetry.

Audiometry measures the ability to hear sounds at different volumes and tones. Impedancemetry, on the other hand, checks the function of the eardrum and the small bones in the middle ear. The two help in diagnosing otosclerosis of the ears, as well as the severity of hearing loss.

When in doubt about the diagnosis, imaging tests such as computed tomography or magnetic resonance imaging can be performed to confirm otospongiosis.

Treatment of otosclerosis

The treatment protocol for otosclerosis of the ear will take into account the degree of hearing loss and the general health status of the person concerned. This is because there are several treatment methods, namely:

• medication;
• fluorine therapy;
• use of hearing aids;
• stapedectomy, which is a surgical procedure.

Treatments used in the management of otosclerosis include the administration of vasodilators and B-complex vitamins. These, along with fluorine therapy, have the main objective of slowing down the progression of the disease. In cases of significant hearing loss, the use of hearing aids is recommended.

On the other hand, stapedectomy is a surgical option that involves replacing a small bone in the inner ear. This is the only method that offers a considerable success rate in symptom improvement, but it comes with risks, such as the possibility of hearing loss.

The choice of appropriate treatment is made through collaboration between the patient and the doctor, establishing a protocol that ensures optimal quality of life and the best possible hearing function. However, being a progressive condition, otosclerosis requires early diagnosis to be managed with less invasive methods.

In conclusion, otosclerosis is a condition of the middle ear that affects the three small bones within it. Although the bones regenerate throughout life, there is a risk that sclerotic tissue may form instead of normal tissue, leading to the development of otospongiosis. This condition progresses slowly and is often asymptomatic, with its onset sometimes occurring in youth. The main symptom is gradual hearing loss in both ears, frequently accompanied by tinnitus and vertigo.

References:

www.nidcd.nih.gov/health/otosclerosis

www.my.clevelandclinic.org/health/diseases/22033-otosclerosis

https://www.webmd.com/cold-and-flu/ear-infection/otosclerosis-facts

https://www.nhs.uk/conditions/otosclerosis/

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