Understanding Cholesteatoma: Symptoms, Causes and Treatment

Dr. K. R. Meghanadh

Introduction | Causes | Symptoms | Diagnosis | Treatment | Home Remedies | Complications | Risk Factors | Prevention

Introduction

Cholesteatoma is a bone-eroding ear disease where the eardrum and ear canal skin move into the middle ear. It’s a type of Otitis media, a middle ear infection caused by negative pressure in the middle ear, leading to the retraction of the eardrum and ear canal skin and forming a sac of dead skin cells from the outer ear canal skin.

The middle ear requires a constant air supply and must maintain equal air pressure to the atmosphere. Any interruption in this air supply can lead to negative pressure buildup in the middle ear.

In cholesteatoma, the negative pressure doesn’t have to affect the entire middle ear. Instead, it may be confined to a pocket or specific section within the middle ear. A dependent cavity with dead skin flakes from the ear canal skin is created in front of pressured areas on the eardrum. The added weight of these flakes increases the volume of the cavity, causing further expansion into the middle ear.

As cholesteatoma grows, it can expand, invading middle ear bones and reaching the inner ear, causing damage. The cavity housing the facial nerve in the middle ear may also be affected, leading to facial paralysis that impacts eyelid closure, mouth deviation, and facial muscles on the particular side.

As cholesteatoma enters the middle ear, it damages the incus, stapes, and malleus bones in that sequence. These bones are crucial in transmitting sound from the ear drum to the inner ear. As they erode, hearing loss can occur.

Once the base of the stapes, the smallest bone that is connected to the inner ear, is damaged, the infection will spread to the inner ear, hence showing the features of inner ear infection like vertigo, tinnitus, and profound hearing loss.

Causes

Cholesteatoma can have various causes, making it challenging to pinpoint a single reason. These factors contribute to the buildup of negative pressure in the middle ear, leading to eardrum retraction and the development of cholesteatoma:

1. Frequent Nose Infections: Repeated nose infections can block the eustachian tube frequently, creating negative pressure in certain areas or the whole of the middle ear.

2. Mucosal Folds in Middle Ear: Some individuals may have mucosal folds in the middle ear from birth, restricting airflow or ventilation to certain areas resulting in negative pressure.

3. Blockage of Isthmus Anticus and Isthmus Posticus: Narrow pathways called Isthmus Anticus and Isthmus Posticus facilitate ventilation and drainage in the middle ear. Blockage of these openings can disrupt middle ear ventilation, leading to negative pressure and cholesteatoma.

4. Allergy: Allergies can contribute to eustachian tube blockage, potentially playing a role in the development of cholesteatoma.

5. Chronic Sinusitis: Persistent sinusitis can lead to eustachian tube blockage, especially when fluids become thick, cutting off ventilation in the middle ear.

6. Enlarged Adenoids or extra growth in the nasopharynx: The eustachian tube or auditory tube that provides airflow to the middle ear has an ending in the nasopharynx, i.e., the back of the nose. Enlarged adenoids or extra growth of tissues in the nasopharynx can obstruct the eustachian tube and restrict the airflow, thereby causing negative pressure in the middle ear.

Symptoms

In some cases, individuals come forward with cholesteatoma symptoms, but a significant number arrive with cholesteatoma complications, often neglecting the initial signs of cholesteatoma.

Rather than addressing symptoms early, people seek medical attention when complications become apparent.

The list below outlines both cholesteatoma symptoms and complications that prompt individuals to consult an ENT specialist.

1. Foul-smelling Ear Discharge: Noticeable watery discharge with a strong, unpleasant odor is a common symptom.

2. Occasional Ear Pain: Individuals may experience intermittent pain in the affected ear.

3. Occasional Blood-stained Ear Discharge: The presence of blood in the ear discharge can occur periodically.

4. Fluctuating Hearing Loss: As the cholesteatoma sac grows, it erodes the bones in the middle ear, making contact with the inner ear and serving as a medium to transmit sound from the ear drum to the inner ear, temporarily improving hearing. This creates a false sense of relief from the ear infection. However, hearing is lost again as the disease clears, whether by gravity or other factors. Therefore, the improvement is only temporary, and in cholesteatoma, hearing loss may appear to fluctuate, but it is technically permanent.

5. Sudden Hearing Loss: Abrupt or unexpected hearing loss in the affected ear. This occurs when the disease reaches eroded stapes and reaches the inner ear.

6. Facial Paralysis: In severe cases, cholesteatoma can lead to facial paralysis, affecting eyelid closure, mouth direction, and muscle use on that side.

7. Tinnitus: Persistent ringing or buzzing sounds in the ear indicating that the disease has spread to the inner ear.

8. Vertigo: A sensation of dizziness or spinning is experienced when the disease spreads to the inner ear part, which is responsible for maintaining balance.

Diagnosis

When an Ear, Nose, and Throat (ENT) specialist examines the ear canal with an otoscope or a diagnostic endoscope, cholesteatoma diagnosis is often confirmed by the eardrum and ear canal skin retraction.

However, the extent, severity, and cause of the cholesteatoma can be only known during the surgery when the middle ear and mastoid are opened.

Treatment

The primary and often only treatment for cholesteatoma is surgery. During the procedure, the disease is removed, and the underlying causes of the negative pressure that leads to cholesteatoma are identified and corrected. The corrective measures for a few causes required are taken in this surgical procedure.

For cases where an allergy is identified as the cause, an ENT specialist may prescribe medicines to address the allergy. In situations where chronic sinusitis is the culprit, surgical intervention for the sinusitis may be necessary to resolve the underlying issue.

Cholesteatoma Surgery

Indeed, the full extent and specific causes of cholesteatoma are often not completely understood until surgical intervention is performed. During the surgical procedure, the middle ear and mastoid are opened by making an incision at the back of the ear. This approach allows the medical team to directly visualize and address various aspects related to cholesteatoma based on the findings obtained during the surgery. Surgical intervention is crucial for accurate diagnosis, the extent of the disease, and appropriate treatment.

1. Clear Mucosal Folds: If present, mucosal folds in the middle ear are cleared during the surgery.

2. Remove Blockage of Isthmus Anticus and Isthmus Posticus: Any blockage in these narrow pathways is identified and removed.

3. Reconstruct the Eardrum: The surgery involves reconstructing a new eardrum to withstand negative pressure.

4. Bone Reconstruction: If any bones are damaged, reconstruction is carried out using cartilage or soft bone harvested from the pinna (outer ear).

The surgery needs to be done as early as possible. As people age, they might not be fit for this surgery.

The surgery for cholesteatoma can cost from 1.5 lakhs to 4 lakhs INR in India.

Cholesteatoma surgery is a significant and intricate procedure, occurring near vital structures such as the facial nerve, brain, and blood vessels supplying blood to the brain and neck. Successful completion requires advanced equipment, including a high-quality microscope and a surgeon with specialized training. Even in skilled hands, the success rate of this surgery is around 99%.

Sometimes, a second-look surgery may be necessary approximately nine months to one year after the initial procedure. The decision for a second-look surgery is contingent upon the findings from the first surgery, and it aims to assess and address the ear's condition post-surgery.

Cholesteatoma surgery is a time-consuming process, typically ranging from 2 to 7 hours. The exact duration cannot be predetermined and depends on the extent of the disease. Certain areas of the middle ear, especially those around critical structures like the stapes and facial nerve, can be challenging to visualize and treat with a microscope.

High magnification is often necessary for these critical areas, requiring a slow and meticulous approach to ensure the best possible results. The surgery's duration is prolonged when dealing with disease proximity to blood vessels carrying blood from the brain to the neck or when it is close to the brain itself.

Cholesteatoma surgery involving the brain

Cholesteatoma can cause damage to the bone between the ear and the brain and may even become attached to the brain. In most cases, an ENT specialist can successfully remove infections near the brain. However, in rare instances, a skull opening may be necessary. In such cases, a collaboration between an ENT specialist and a neurosurgeon becomes essential to address and clear the disease effectively.

Dr. K. R. Meghanadh has extensive experience, having performed around 2000 cholesteatoma surgeries, with only two instances requiring the assistance of neurosurgeons to open the skull and address the disease.

Home Remedies for Cholesteatoma

While cholesteatoma disease doesn't have direct home remedies for treatment, you may be able to slow down the progression of the disease by addressing its underlying causes through home remedies.

Identifying causes, such as mucosal folds or blockages in isthmus anticus and isthmus posticus, may require surgical intervention, as they are not easily discernible without surgical examination.

Therefore, it's essential to consult with a healthcare professional for accurate diagnosis and appropriate treatment.

Allergy Management

For those with allergies, managing them alongside surgery can be beneficial.

1. Taking Steam Inhalation This may help to deal with the inflammation of the mucosa layer that runs into the eustachian, which could be a potential cause of the halt of airflow in the middle ear.

2. Avoiding Allergens Identify and avoid allergens; for example, if allergic, refrain from dry mopping at home.

Chronic Sinusitis

Individuals with chronic sinusitis can use home remedies to address potential eustachian tube blockages. For more details, refer to the "Sinusitis Home Remedies" article.

However, in such scenarios, it is essential to fix the causes immediately or as quickly as possible. So, even if the chances of the sinusitis resolving with home remedies, antibiotics, and other medications are high, they will take months to resolve.

In cases where only home remedies are put to use, the results are unpredictable. So, to fix the issue quickly and prevent recurrence and further complications, sinus surgery is highly recommended to fix the issue quickly.

Unlike cholesteatoma surgery, sinus surgery is not a major tough surgery. Sinus surgery is simpler and the results will be good when the correct technique and technologies are chosen. To know more about sinus surgery, refer the below mentioned article.

Sinusitis Surgery: An Overview of the Different Technologies

Complications of Cholesteatoma

As mentioned earlier in the symptoms section, the number of cholesteatoma patients who reach out to an ENT with complications is higher than the ones who reach out due to symptoms. So, complications in cholesteatoma are common.

1. Erosion of Bones (Stapes, Incus, Malleus): Cholesteatoma can erode the small bones in the middle ear responsible for transmitting sound, leading to hearing loss.

2. Facial Nerve Canal Erosion: The cholesteatoma's progression can lead to erosion of the facial nerve canal, potentially causing paralysis on one side of the face.

3. Inner Ear Involvement: Cholesteatoma can extend into the inner ear, resulting in symptoms such as tinnitus, vertigo, and profound hearing loss. These effects are often irreversible.

4. Spread to the Brain: In a few cases, the infection can spread to the brain. In most scenarios, an ENT surgeon alone will be able to fix the condition, but in 0.1% of cases necessitating the involvement of a neurosurgeon during surgery. This complication is typically detected only during the surgical procedure, and emergency intervention may be required to address it effectively.

Who is at risk of cholesteatoma?

We cannot predict who is at risk of cholesteatoma. We do not have any investigations as of now to predict this exactly.

Prevention

We do not know who is at risk of cholesteatoma, so we cannot prevent cholesteatoma. The only thing we can do is go for immediate surgery once identified and prevent the complications.

Dr. K. Raja Meghanadh