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You start to notice that your hearing is getting worse in one ear, or both, but without having been exposed to loud noises, without recent infections, and without any apparent cause. Perhaps you’re in your thirties or forties. Perhaps someone in your family had the same problem. And you’ve been attributing it to tiredness or distraction for a while now.
Otosclerosis is a common but little-known cause of progressive hearing loss in young adults. It is painless, has no noticeable symptoms at first, and develops so gradually that many people wait years before seeking an evaluation.
The good news is that it can be diagnosed with simple tests and has effective treatments, both surgical and non-surgical. Early detection is key.
In this article we explain what otosclerosis is, how it manifests, whether it can be hereditary, and what treatment options exist.
Do you have progressive hearing loss with no apparent cause or a family history of hearing problems?
At Clinicaudio we perform a complete and free hearing evaluation to identify the origin and guide you towards the most suitable options for your case.
What is otosclerosis?
Otosclerosis, also called otospongiosis, is a disease of the temporal bone that causes abnormal bone growth in the middle ear, especially around the stapes, the smallest of the three ossicles of the ear.
The stapes acts as a mechanical transmitter; when sound vibrates the eardrum, it picks up that vibration and transmits it to the inner ear. In otosclerosis, the growth of new bone tissue around the stapes gradually fixes it to the oval window—the opening that connects the middle and inner ear—preventing it from vibrating normally.
The result is conductive hearing loss; sound reaches the ear, but it cannot be transmitted properly because the mechanism that carries it is blocked. In some cases, when the process also affects the cochlea, the loss can be both conductive and sensorineural, which complicates the situation.
Otosclerosis most often affects young adults, usually between the ages of 15 and 45, and is more common in women than in men. It is usually bilateral, affecting both ears, although not always to the same degree or at the same time. In many cases, it begins in one ear and spreads to the other years later.
At Clinicaudio, we frequently see people who come in believing their hearing loss is unexplained. When there is a hereditary component and the loss began before age 45, otosclerosis is always a possibility that should be ruled out with objective tests.
Why it happens: the stapes and abnormal bone growth
Under normal conditions, the bone of the otic capsule is one of the most stable tissues in the body. Otosclerosis disrupts this balance; normal bone is resorbed and replaced by spongy, vascularized bone tissue that, over time, hardens and fixes the stapes.
The exact cause is not fully established, but several influencing factors are known.
Genetic factors: It has a clear hereditary basis. It is transmitted in an autosomal dominant manner with variable penetrance, which means that it can skip generations or manifest with different intensity in different members of the same family.
Hormonal factors: The disease appears or worsens more frequently during pregnancy, suggesting that female sex hormones may influence its activation or progression.
Viral infections: A possible relationship with the measles virus has been studied, although the evidence is not conclusive.
What is clear is that otosclerosis is not caused by noise exposure, ear infections, or headphone use. It is a bone disease with a significant genetic component.
Symptoms: how otosclerosis manifests
The most important characteristic of otosclerosis is that its progression is slow and silent. Symptoms appear gradually and, in the initial stages, may go completely unnoticed.
Progressive hearing loss
This is the main symptom. It begins as difficulty hearing soft voices or conversations in quiet environments and gradually worsens. Unlike other forms of hearing loss, otosclerosis primarily affects low frequencies in its initial stages.
Paracusia of Willis
It’s one of the most characteristic and paradoxical signs: the person hears better in noisy environments than in silence. This happens because in noisy settings, people tend to raise their voices, which partially compensates for the hearing loss. If you notice that you understand better in a restaurant than at home, it could be a sign.
Tinnitus
Tinnitus, or ringing or buzzing in the ear, often accompanies otosclerosis, especially when the condition is bilateral or involves a cochlear component. It may appear before hearing loss is noticeable or develop as the disease progresses.
Sensation of ear fullness
Some people describe a feeling of pressure or blockage, even though the canal is completely clear and there is no earwax or infection.
Vertigo or instability
In cases where otosclerosis affects the inner ear, episodes of instability or vertigo may occur. This is less common than hearing loss, but when it does occur, it should be carefully evaluated.
Is otosclerosis hereditary?
Yes. Otosclerosis has a significant genetic component. It is estimated that between 25% and 40% of people with otosclerosis have a family history of the disease.
The inheritance pattern is autosomal dominant with variable penetrance, which means that:
- A parent with otosclerosis can pass on the genetic predisposition to their children.
- No todos los portadores del gen desarrollan la enfermedad de la misma forma ni con la misma intensidad.
- It can skip generations or manifest itself very mildly in some family members.
If you have first-degree relatives (parents, siblings) with diagnosed or undiagnosed progressive hearing loss, and you yourself begin to notice hearing difficulties in young adulthood, otosclerosis is a possibility worth exploring.
How it is diagnosed
The diagnosis of otosclerosis is based on medical history, symptoms, and the results of various hearing tests. There is no single test that confirms it; it is the combination of findings that guides the diagnosis.
Pure-tone audiometry: Otosclerosis exhibits a characteristic pattern. Air conduction shows significant hearing loss, while bone conduction is relatively preserved, at least in the initial stages, confirming the conductive component. If you want to understand what audiometry results mean, our article on degrees of hearing loss explains it in detail.
Tympanometry: Analyzes the function of the middle ear. In otosclerosis, the eardrum moves with rigidity, producing a characteristic tympanometric pattern that points toward the diagnosis.
Stapedial reflex: In otosclerosis it is usually absent or altered, because the fixed stapes cannot contract normally in response to sound.
Carhart’s notch: A specific audiometric sign of otosclerosis that appears in bone conduction audiometry around 2,000 Hz. Its presence is highly indicative of the diagnosis.
The definitive diagnosis is established by the otolaryngologist, who may also request a high-resolution computed tomography scan to visualize bone growth and plan surgical treatment if necessary.
At Clinicaudio we perform a complete hearing evaluation (audiometry, tympanometry and stapedial reflex) and we guide referral to a specialist when the findings point to otosclerosis.
Have you noticed that your hearing is progressively getting worse and you’ve never had a complete hearing evaluation?
At Clinicaudio, the first examination is free. We evaluate both ears separately and determine if the pattern is consistent with otosclerosis or other causes of conductive hearing loss.
Tratamiento de la otosclerosis
Otosclerosis has two main treatment options: surgery and hearing aids. The choice depends on the degree of hearing loss, the specific characteristics of the case, the patient’s age, and their preferences.
Cirugía: estapedotomía y estapedectomía
Surgical intervention is the treatment with the best long-term results for cases of otosclerosis with a significant conductive component.
The most common technique is stapedotomy, in which the surgeon makes a small opening in the footplate of the fixed stapes and inserts a prosthesis that replaces its function, restoring sound transmission to the inner ear. Stapedectomy is an older variant in which the entire stapes is removed.
The success rate of the surgery is very high in expert hands, exceeding 90%, and can result in significant hearing recovery. However, like any surgical procedure, it carries risks that the otolaryngologist must assess on a case-by-case basis.
Surgery does not cure the underlying disease; otosclerosis can continue to progress in the inner ear even after the stapes has been operated on.
Earphones
When surgery is not indicated, is not desirable for the patient, or there is an added neurosensory component that surgery cannot resolve, hearing aids are an effective alternative.
In conductive otosclerosis, hearing aids compensate for the hearing loss by amplifying the sounds that the stapes bone can no longer transmit normally. Current models allow for very precise fitting to each person’s hearing profile.
Otosclerosis and hearing aids: when are they the best option
The question of whether to opt for surgery or hearing aids doesn’t have a universal answer. There are situations in which hearing aids are the first or only recommended option:
- When hearing loss is mild or moderate and the person prefers to avoid surgery.
- When there is a significant neurosensory component that surgery cannot correct.
- When the person is not a surgical candidate due to age, health status, or other reasons.
- While awaiting surgery, to improve hearing quality in the meantime.
- When surgery has been performed on one ear and the other requires compensation.
Many people with otosclerosis use hearing aids for years with very good results. Current models have very advanced technology to compensate for conductive hearing loss, sound processing adapted to each frequency, ambient noise reduction, and Bluetooth connectivity, in discreet or virtually invisible designs.
If you want to know the differences between types of hearing aids and what options are available in each range, in our article on hearing aids vs. amplifiers you will find all the information to guide you before your consultation.
When to consult a specialist
Otosclerosis is a progressive disease: without treatment, hearing loss tends to worsen over time. Therefore, the earlier it is diagnosed and treated, the better the outcome.
Consult a specialist if:
- You notice that your hearing is getting progressively worse for no apparent reason.
- You have a family history of early hearing loss.
- You hear better in noisy environments than in silence (Willis’ paracusis).
- There is a persistent ringing or buzzing in one or both ears.
- Hearing loss affects one ear more than the other.
- You already have a diagnosis of otosclerosis and you haven’t had a check-up in the last year.
At Clinicaudio, we perform a complete hearing evaluation and determine if the pattern is consistent with conductive hearing loss, such as that caused by otosclerosis. If the findings indicate it, we refer the patient to an otolaryngologist for surgical evaluation.
Preguntas frecuentes sobre la otosclerosis
What exactly is otosclerosis?
It is a disease of the temporal bone that causes abnormal bone growth in the middle ear, progressively fixing the stapes. As the stapes loses mobility, it can no longer transmit sound normally, resulting in conductive hearing loss.
Is otosclerosis hereditary?
Yes, it has a significant genetic component. Between 25% and 40% of cases have a family history. It is inherited in an autosomal dominant pattern with variable penetrance, meaning it can manifest with varying severity or skip generations.
Is there a cure for otosclerosis?
Stapedotomy or stapedectomy surgery can significantly restore hearing lost due to conductive hearing loss in a high percentage of cases. However, it does not eliminate the underlying disease: otosclerosis can continue to progress in the inner ear. Hearing aids are an effective alternative when surgery is not indicated.
What is the difference between otosclerosis and presbycusis?
Presbycusis is age-related sensorineural hearing loss that primarily affects people over 60-65 years of age. Otosclerosis is a conductive bone disease of the middle ear that typically appears between the ages of 15 and 45. The audiometric pattern and treatment differ.
Does pregnancy worsen otosclerosis?
In some women, pregnancy can accelerate the progression of otosclerosis or trigger the first symptoms. It is believed that female sex hormones may influence the abnormal bone remodeling process. This is not a universal rule, but it is worth considering if there is a family history of the condition.
Does otosclerosis affect both ears?
It is usually bilateral in 70-80% of cases, although not always with the same intensity or at the same time. It commonly begins in one ear and the second ear is affected years later.
Otosclerosis is treatable, but the first step is to find out what's happening.
Otosclerosis is one of the most frequent causes of progressive hearing loss in young adults, and also one of the slowest to be diagnosed because its symptoms are gradual and painless.
Diagnosis is straightforward with the right tests, and treatment with surgery or hearing aids can effectively restore or compensate for lost hearing in most cases.
If you have a family history of hearing loss or have noticed your hearing getting worse for some time without an apparent cause, don’t ignore it. At Clinicaudio, we offer a complete and free hearing evaluation and will advise you on the most appropriate steps for your situation.
Does your hearing loss affect only one ear, or is it clearly worse in one than the other?
Otosclerosis usually begins in one ear before spreading to the other. In our article on hearing loss in one ear, we explain the signs that many people ignore when only one ear is affected and why it’s important to act promptly.
Is there ringing in the ear in addition to hearing loss?
Tinnitus is a common symptom of otosclerosis, especially when it is bilateral. In our article on tinnitus, we explain why the ringing occurs, what types exist, and what can be done to reduce its impact on daily life.
