Cholesteatoma

Cholesteatoma is a serious ear condition that can significantly impact hearing and overall health. This abnormal cell growth in the middle ear or mastoid bone develops slowly over time, often without early warning signs. Left untreated, cholesteatoma disease can lead to hearing loss and other complications, making early detection and proper management crucial. Let's explore its causes, symptoms, and available treatment options. 

What is Cholesteatoma? 

Cholesteatoma ear is a rare but potentially serious condition affecting the middle ear. Despite its name, which suggests cholesterol or fat presence, it involves an abnormal collection of skin cells deep inside the ear, particularly in the mastoid bone or behind the tympanic membrane. This benign growth can cause significant damage to the surrounding structures if left untreated. 

The following are three main types of cholesteatoma: 

• Congenital Cholesteatomas: These congenital growths are extremely rare and arise from squamous epithelium trapped within the temporal bone during development. 
• Primary-acquired Cholesteatomas: These cholesteatomas result from pressure changes in the middle ear, making the eardrum prone to collapse and creating a pocket where dead skin cells accumulate. 
• Secondary-acquired Cholesteatomas: They develop due to direct injury to the eardrum, usually from infection or trauma. 

Causes and Risk Factors of Cholesteatoma 

The following are some common cholesteatoma causes: 

• Problems Associated With the Eustachian Tube: This tube connects the middle ear to the throat. When this tube fails to function correctly, it can build negative pressure in the middle ear, causing the eardrum to retract and form a pocket. This pocket can then accumulate dead skin cells, resulting in a cholesteatoma. 
• Congenital Factor: Rarely children are born with cholesteatoma. 
• Infection: Acute otitis media or chronic ear infections can lead to abnormal growth development in the ear. 

Several factors can increase the possibility of developing this condition. These include: 

• Congenital defects in the ear structure 
• Individuals with a history of repeated ear infections 
• Previous ear surgery 
• Trauma to the ear 
• Cleft palate 
• People with weakened immune systems 

Symptoms of Cholesteatoma 

Cholesteatoma symptoms can vary from person to person and depend on whether the condition is congenital or acquired. Congenital cholesteatomas, present at birth, may not cause noticeable symptoms until they grow larger. In contrast, acquired cholesteatomas, which develop over time, often produce more apparent signs. Here are some common cholesteatoma symptoms: 

• Foul-smelling Discharge From the Affected Ear: This discharge may resemble pus and can be a sign of infection. 
• Fullness or Pressure in the Ear: These sensations can be uncomfortable. 
• Tinnitus: Some people may also notice tinnitus, a persistent buzzing or ringing sound in the ear. 
• Dizziness & Loss of Balance: These symptoms may worsen as the condition advances, affecting the inner ear structures responsible for maintaining balance. 
• Hearing Loss: As the condition worsens, it can damage the tiny bones of the middle ear. This results in a gradual decline in hearing ability. 

Complications 

Cholesteatoma can lead to serious complications if left untreated. The condition can potentially damage delicate structures within the ear that are crucial for hearing and balance. As the cholesteatoma grows, it can cause persistent ear infections, resulting in discharge from the ear. Some of the complications are: 

• Hearing loss 
• Vertigo (a sensation of spinning) 
• Tinnitus 
• Damage to the facial nerve or facial palsy that leads to weakness on one side of the face. 
• Mastoiditis (an infection of the mastoid bone) 
• Infection extends to the brain, resulting in a brain abscess or meningitis. 

Diagnosis 

• Physical Examination: ENT doctors use an otoscope to inspect the eardrum and ear canal, looking for signs of cholesteatoma, such as a white mass behind the eardrum or a retraction pocket. 
• Computed Tomography (CT) Scans: CT scans of the temporal bone are the primary imaging methods used to evaluate the extent of the condition and guide surgical planning. 
• Magnetic Resonance Imaging (MRI): It is used when there are concerns about complications such as dural involvement or brain abscesses. 
• Audiometric Tests: These tests assess hearing loss, which is a common symptom of cholesteatoma. 
• Tympanometry: It evaluates middle ear function, although it cannot definitively diagnose cholesteatoma. 

Treatment for Cholesteatoma 

The primary cholesteatoma treatment is surgical intervention. This is necessary to remove the abnormal skin growth and prevent serious complications, such as: 

Tympanomastoidectomy: 

• It is the most common surgical procedure and involves both a tympanoplasty to repair the eardrum and a mastoidectomy to remove the disease from the mastoid bone. 
• During the operation, which is performed under general anaesthesia, the surgeon makes an incision either behind or in front of the ear. They carefully remove the cholesteatoma and any affected bone. Sometimes, they may need to remove part of the ear canal, creating a cavity that opens into the ear. 
• Recovery typically involves wearing a head bandage for 24 to 48 hours post-surgery. Patients usually need about 1-2 weeks of rest, depending on the extent of the surgery. It's crucial to keep the ear dry during the healing process. 

When to See a Doctor 

Immediately seek medical attention if you: 

• Experience persistent ear problems 
• Notice changes in your hearing 
• Have a watery discharge from your ear 

Prevention 

Preventing cholesteatoma is crucial for maintaining ear health. While some cases may be unavoidable, you can take several steps to diminish risk. Some preventive techniques are: 

• Early intervention for chronic ear infections as untreated infections may contribute to cholesteatoma formation. 
• Regular follow-ups, especially for those with a history of chronic otitis media, can aid in early detection and management of cholesteatoma. 
• Maintaining proper ear hygiene, which includes avoiding water exposure and adhering to prescribed treatments. 

Conclusion 

Dealing with cholesteatoma can be challenging, but with the proper care and follow-up, many people can have good outcomes. Regular check-ups and following doctor's advice are key to keeping your ears healthy. If you notice any unusual changes in your hearing or have persistent ear problems, don't hesitate to seek medical help. Taking care of the health of your ear is crucial for your overall well-being. 

FAQS 

1. Is cholesteatoma life-threatening? 

While cholesteatoma is not typically life-threatening, it can lead to serious complications if left untreated. In rare cases, the infection can spread to the inner ear and brain, potentially causing meningitis or brain abscesses. 

2. What causes cholesteatoma to grow? 

Cholesteatoma grows due to the accumulation of dead skin cells in a pocket formed by a collapsed eardrum. Chronic ear infections, eustachian tube dysfunction, and congenital factors can contribute to its development. 

3. What are the stages of cholesteatoma? 

The EAONO/JOS working group developed a staging system for cholesteatoma: 

• Stage I: Localised cholesteatoma 
• Stage II: Cholesteatoma involving two or more sites 
• Stage III: Cholesteatoma with extracranial complications 
• Stage IV: Cholesteatoma with intracranial complications.

4. Can cholesteatoma spread? 

Yes, cholesteatoma can spread and damage surrounding structures in the middle ear, including the ossicles, mastoid bone, and facial nerve. It can also erode the bone, separating the ear from the brain. 

5. Is cholesteatoma a birth defect? 

Congenital cholesteatoma is present at birth, but it's rare. Most cholesteatomas are acquired and develop later in life due to chronic ear problems or eardrum damage. 

6. Can cholesteatoma grow back? 

Yes, cholesteatoma can recur even after surgical removal. The risk of recurrence is higher in children than adults, necessitating regular follow-up appointments. 

7. Can cholesteatoma affect eyesight? 

In rare cases, cholesteatoma can impact vision. It may result in asymmetric cupping of the optic nerves, potentially leading to visual field defects and other ocular issues. 

Dr. Minal Gupta

CARE Medical Team