Cholesteatoma: Symptoms, Causes & Surgical Treatment

What Is a Cholesteatoma?

A cholesteatoma is an abnormal collection of skin cells that forms a cyst-like growth in the middle ear behind the eardrum. Despite containing "chole" (bile) and "steatoma" (fat tumor) in its name, it contains neither bile nor fat and is not cancerous. The growth consists of layers of dead skin cells (keratin) that accumulate and expand over time, potentially destroying the structures of the middle ear.

The middle ear is a small, air-filled space behind the eardrum that contains three tiny bones called ossicles (the malleus, incus, and stapes). These bones transmit sound vibrations from the eardrum to the inner ear, enabling us to hear. A cholesteatoma develops when skin cells that normally line the ear canal become trapped in the middle ear and begin to accumulate.

Unlike normal skin that sheds its dead cells outward, the skin cells trapped in the middle ear have nowhere to go. They pile up layer upon layer, forming a pearl-like mass that gradually enlarges. This growth releases enzymes and proteins that can erode bone and damage the delicate structures of the middle ear, including the ossicles, the facial nerve, and even the bone separating the ear from the brain.

The term "cholesteatoma" was coined in the 19th century when physicians mistakenly believed the growths contained cholesterol and fat. Although we now understand its true nature, the historical name persists. Other terms sometimes used include "keratoma" (describing its keratin content) or "pearl cyst of the ear" (describing its appearance).

Types of Cholesteatoma

Cholesteatomas are classified into two main types based on how they develop:

Acquired cholesteatoma is by far the most common type, accounting for approximately 98% of cases. It typically develops in one of two ways:

• Primary acquired: Results from a retraction pocket in the eardrum, usually caused by Eustachian tube dysfunction. Negative pressure in the middle ear pulls part of the eardrum inward, creating a pocket where skin cells accumulate.
• Secondary acquired: Develops when skin grows through an existing hole (perforation) in the eardrum, migrating into the middle ear space. This can occur after ear infections, trauma, or ear tube placement.

Congenital cholesteatoma is rare, accounting for only about 2% of cases. It occurs when skin cells become trapped behind an intact eardrum during fetal development. Congenital cholesteatoma is typically discovered in young children who have no history of ear infections or eardrum problems.

What Are the Symptoms of Cholesteatoma?

The most common symptoms of cholesteatoma include persistent ear drainage (often foul-smelling), progressive hearing loss, a sensation of fullness or pressure in the ear, and sometimes ear pain or dizziness. Symptoms typically develop gradually and may initially be mistaken for a chronic ear infection.

Cholesteatoma symptoms often begin subtly and worsen over months to years as the growth enlarges and causes more damage. Many people initially dismiss their symptoms as minor ear problems, which can delay diagnosis and treatment. Recognizing the warning signs early is crucial for preventing serious complications.

Early Symptoms

In the early stages, cholesteatoma may cause few or no noticeable symptoms. When symptoms do appear, they often include:

• Ear drainage (otorrhea): Persistent discharge from the ear is often the first symptom. The drainage may be watery, mucoid, or purulent, and frequently has a distinctive foul odor due to bacterial colonization of the dead skin cells.
• Hearing loss: Gradual reduction in hearing ability, typically affecting one ear. The hearing loss is usually conductive (caused by mechanical obstruction or damage to the ossicles) but can become mixed or sensorineural if the inner ear is affected.
• Ear fullness: A sensation of pressure or blockage in the affected ear, similar to what you might feel during an airplane descent.
• Recurrent ear infections: Repeated episodes of ear infections that don't fully resolve with antibiotic treatment may indicate an underlying cholesteatoma.

Advanced Symptoms

As cholesteatoma progresses and causes more extensive damage, additional symptoms may develop:

• Severe hearing loss: Significant or complete hearing loss in the affected ear if the ossicles are destroyed or the inner ear is damaged.
• Ear pain (otalgia): While not always present, pain can occur, especially if there is secondary infection or if the cholesteatoma is pressing on sensitive structures.
• Dizziness or vertigo: If the cholesteatoma erodes into the inner ear or affects the balance organs, patients may experience episodes of dizziness, imbalance, or spinning sensations.
• Tinnitus: Ringing, buzzing, or other sounds in the affected ear.

What Causes Cholesteatoma?

Cholesteatoma most commonly develops from Eustachian tube dysfunction, which creates negative pressure that pulls the eardrum inward to form a retraction pocket where skin cells accumulate. It can also develop when skin grows through an existing eardrum perforation. Congenital cholesteatoma occurs when skin cells become trapped during fetal development.

Understanding the causes of cholesteatoma helps explain why certain people are more susceptible and how the condition might be prevented. The vast majority of cholesteatomas are acquired during life, while a small percentage are present from birth.

Eustachian Tube Dysfunction

The Eustachian tube connects the middle ear to the back of the nose and throat. Its primary function is to equalize pressure between the middle ear and the outside environment (such as when you "pop" your ears during altitude changes) and to drain fluid from the middle ear.

When the Eustachian tube doesn't function properly, negative pressure develops in the middle ear. This vacuum effect can pull a portion of the eardrum inward, creating a pocket or pouch. Over time, this retraction pocket deepens and skin cells that would normally migrate outward become trapped, accumulating and forming a cholesteatoma.

Factors that contribute to Eustachian tube dysfunction include:

• Chronic nasal congestion from allergies, sinusitis, or structural abnormalities
• Enlarged adenoids in children, which can block the Eustachian tube opening
• Cleft palate or other craniofacial abnormalities affecting Eustachian tube function
• Repeated upper respiratory infections causing inflammation and swelling

Eardrum Perforation

A hole in the eardrum can allow skin from the ear canal to grow through into the middle ear space. This can occur after:

• Acute ear infections that rupture the eardrum
• Trauma to the ear (such as from a cotton swab or barotrauma)
• Previous ear surgery or ear tube placement
• Chronic ear disease that erodes the eardrum

Congenital Factors

Congenital cholesteatoma is believed to result from epithelial (skin) cell rests that become trapped during fetal development. These cells are present behind an intact eardrum from birth and gradually grow over the child's early years. The exact reason why these cell rests form is not fully understood, but it may relate to incomplete development of the middle ear structures.

Risk Factors

Several factors increase the likelihood of developing cholesteatoma:

• History of ear infections: Repeated middle ear infections (otitis media), especially in childhood
• Previous ear surgery: Any procedure involving the eardrum or middle ear
• Allergies and sinus problems: Conditions affecting Eustachian tube function
• Male sex: Cholesteatoma is slightly more common in males
• Caucasian ethnicity: Higher incidence in white populations compared to other groups
• Poor socioeconomic conditions: Limited access to healthcare for ear infections

How Is Cholesteatoma Diagnosed?

Cholesteatoma is diagnosed through a combination of medical history, physical examination using an otoscope, and imaging studies. CT scan of the temporal bone is the primary imaging modality to assess the extent of disease. Audiometry (hearing test) evaluates the degree of hearing loss. MRI may be used to differentiate cholesteatoma from other conditions.

Accurate diagnosis of cholesteatoma is essential for planning appropriate treatment and preventing complications. The diagnostic process typically involves several steps, beginning with a thorough clinical evaluation and often including advanced imaging studies.

Physical Examination

The initial evaluation begins with a detailed medical history and physical examination. Your doctor will ask about:

• Duration and nature of symptoms (ear drainage, hearing loss, dizziness)
• History of ear infections or ear surgeries
• Previous hearing problems
• Any neurological symptoms (facial weakness, severe vertigo)

Using an otoscope (a lighted instrument for examining the ear), the doctor can often see characteristic signs of cholesteatoma:

• White, pearly mass visible behind or through the eardrum
• Retraction pocket in the eardrum, especially in the upper portion (attic region)
• Eardrum perforation with skin or debris visible in the middle ear
• Crusting or drainage from the ear canal
• Granulation tissue (abnormal healing tissue) in the ear

Hearing Tests (Audiometry)

A comprehensive hearing evaluation is an essential part of cholesteatoma diagnosis. Audiometry measures the degree and type of hearing loss, providing valuable information about which structures are affected:

• Conductive hearing loss: Indicates mechanical obstruction or damage to the eardrum or ossicles
• Sensorineural hearing loss: Suggests inner ear involvement, which carries a worse prognosis for hearing recovery
• Mixed hearing loss: Combination of both types, often seen in advanced cases

CT Scan of the Temporal Bone

High-resolution computed tomography (CT) scanning is the primary imaging study for cholesteatoma. CT provides detailed images of the bony structures of the ear and can reveal:

• The size and location of the cholesteatoma
• Erosion of the ossicles (hearing bones)
• Involvement of the mastoid bone
• Proximity to important structures (facial nerve, inner ear, brain)
• Complications such as bone erosion near the brain or major blood vessels

MRI Scan

Magnetic resonance imaging (MRI), particularly diffusion-weighted imaging (DWI), has become increasingly important in cholesteatoma evaluation. MRI is particularly useful for:

• Differentiating cholesteatoma from other conditions: MRI can distinguish cholesteatoma from granulation tissue, fluid, or other masses
• Detecting recurrence after surgery: Non-invasive method to identify residual or recurrent disease
• Evaluating intracranial complications: Better visualization of brain involvement than CT

How Is Cholesteatoma Treated?

Cholesteatoma requires surgical treatment; there is no medication that can cure it. Surgery aims to completely remove the cholesteatoma, prevent complications, create a safe ear, and when possible, restore hearing. The main surgical approaches are mastoidectomy (removal of disease with the mastoid bone) combined with tympanoplasty (eardrum reconstruction). Surgery has a success rate of 85-95%.

Unlike many ear conditions that can be treated with medications, cholesteatoma requires surgical intervention. The disease will continue to grow and cause damage if left untreated, making surgery the only option for definitive management. However, certain non-surgical measures may be used to prepare for surgery or manage symptoms temporarily.

Non-Surgical Management

While surgery is ultimately necessary, the following treatments may be used before or alongside surgical intervention:

Ear cleaning (aural toilet): Regular removal of debris, discharge, and loose skin cells from the ear canal by a specialist. This can help control infection and drainage but does not stop cholesteatoma growth.

Antibiotic ear drops: Topical antibiotics (such as ciprofloxacin or ofloxacin drops) treat bacterial infections associated with cholesteatoma. They help reduce drainage and odor but cannot eliminate the underlying problem.

Oral antibiotics: May be prescribed for active infections, especially before surgery, to reduce inflammation and infection risk during the procedure.

Surgical Treatment

Surgery for cholesteatoma has three main goals, in order of priority:

1. Complete removal of the cholesteatoma to eliminate the disease
2. Prevention of complications by creating a safe, disease-free ear
3. Preservation or restoration of hearing when possible

The specific surgical approach depends on the size and location of the cholesteatoma, the extent of damage, and patient factors. Common procedures include:

Mastoidectomy

Mastoidectomy involves surgical access through the mastoid bone (the bony prominence behind the ear) to remove the cholesteatoma and any affected tissue. There are several types:

• Canal wall up (CWU) mastoidectomy: The posterior canal wall (back wall of the ear canal) is preserved. This maintains a more normal ear anatomy but has a higher recurrence rate. A "second-look" surgery may be needed 9-12 months later to check for recurrence.
• Canal wall down (CWD) mastoidectomy: The posterior canal wall is removed, creating a larger open cavity (mastoid bowl). This approach has lower recurrence rates but requires lifelong care to keep the cavity dry and may have some limitations for hearing aid use.

Tympanoplasty

Tympanoplasty repairs or reconstructs the eardrum. This may involve:

• Grafting tissue (usually from the patient's own temporalis fascia or cartilage) to close a perforation
• Reinforcing weakened areas of the eardrum
• Reconstructing the eardrum after cholesteatoma removal

Ossiculoplasty

If the tiny hearing bones (ossicles) have been damaged by the cholesteatoma, ossiculoplasty may be performed to restore the sound-conducting mechanism. This can involve:

• Repositioning the patient's own ossicles
• Using prosthetic devices made of titanium or other materials
• Placing cartilage grafts to support the reconstruction

Hearing reconstruction may be performed during the initial surgery or as a staged procedure several months later, depending on the extent of disease and surgeon preference.

Surgical Outcomes

Modern cholesteatoma surgery has excellent success rates:

• Disease control: 85-95% of surgeries successfully remove all cholesteatoma
• Recurrence rates: 5-15% depending on surgical technique and disease extent
• Hearing improvement: Many patients experience improved hearing, though results depend on the degree of pre-existing damage

What Is Recovery Like After Cholesteatoma Surgery?

Recovery after cholesteatoma surgery typically takes 2-6 weeks for initial healing. Patients must keep the ear dry, avoid strenuous activity, and follow up regularly with their surgeon. Complete healing may take 2-3 months. A second surgery may be recommended 6-12 months later to check for recurrence and potentially improve hearing.

Understanding what to expect during recovery helps patients prepare for the post-operative period and optimize their healing. Recovery varies depending on the extent of surgery but follows a general timeline.

Immediate Post-Operative Period (First 1-2 Weeks)

In the days immediately following surgery, patients may experience:

• Ear discomfort or pain: Usually manageable with prescribed pain medications
• Temporary dizziness: Common and typically improves within a few days
• Altered taste: The chorda tympani nerve (which runs through the middle ear) may be affected, causing temporary changes in taste sensation
• Ear packing: Material placed in the ear canal to promote healing; removed at follow-up appointments
• Hearing changes: Hearing may be decreased due to packing and swelling; improvement occurs as healing progresses

Post-Operative Care Instructions

Following your surgeon's instructions is crucial for successful healing:

• Keep the ear dry: Avoid getting water in the operated ear for 4-6 weeks. Use cotton balls coated with petroleum jelly or special ear plugs during showers.
• Use ear drops as prescribed: Antibiotic drops help prevent infection and promote healing.
• Avoid blowing your nose forcefully: Sniff gently or dab with tissue to avoid pressure changes in the ear.
• No flying or diving: Avoid air travel and scuba diving for 4-6 weeks to prevent barotrauma.
• Limit physical activity: Avoid strenuous exercise, heavy lifting, and contact sports for several weeks.
• Attend all follow-up appointments: Regular check-ups allow your surgeon to monitor healing and address any concerns.

Long-Term Follow-Up

Because cholesteatoma can recur, long-term monitoring is essential:

• Second-look surgery: For CWU procedures, a second surgery 6-12 months after the initial operation may be recommended to check for residual or recurrent disease and potentially perform hearing reconstruction.
• MRI surveillance: Non-echo planar diffusion-weighted MRI can detect cholesteatoma recurrence without surgery, reducing the need for second-look procedures in some cases.
• Annual ear examinations: Regular check-ups with an ENT specialist are recommended, often lifelong, as recurrence can occur years after surgery.

What Are the Potential Complications of Cholesteatoma?

Untreated cholesteatoma can cause serious complications including permanent hearing loss, facial nerve paralysis, inner ear damage causing vertigo, and potentially life-threatening spread of infection to the brain (meningitis or brain abscess). Early diagnosis and treatment significantly reduce these risks.

Cholesteatoma is a progressive disease that will continue to grow and cause damage if left untreated. Understanding potential complications underscores the importance of timely diagnosis and surgical intervention.

Hearing-Related Complications

The most common complication of cholesteatoma is hearing loss:

• Conductive hearing loss: Occurs when cholesteatoma damages or destroys the ossicles (hearing bones). This type of hearing loss can often be improved with surgery.
• Sensorineural hearing loss: Results from damage to the inner ear (cochlea) or auditory nerve. This type is usually permanent and may not be correctable with surgery.
• Complete deafness: In severe cases, all hearing in the affected ear may be lost.

Facial Nerve Complications

The facial nerve passes through the middle ear, making it vulnerable to cholesteatoma damage:

• Facial paralysis: Weakness or complete paralysis of the face on the affected side. This can affect eye closure, smiling, and other facial movements.
• Facial numbness: Reduced sensation in parts of the face.

Balance-Related Complications

Erosion into the inner ear structures can affect balance:

• Labyrinthitis: Infection or inflammation of the inner ear causing severe vertigo, nausea, and hearing loss.
• Labyrinthine fistula: Erosion creates an abnormal opening into the inner ear, causing vertigo with certain movements or pressure changes.

Intracranial Complications

The most serious complications occur when cholesteatoma or associated infection spreads beyond the ear:

• Meningitis: Infection of the membranes covering the brain, causing fever, headache, neck stiffness, and potentially life-threatening illness.
• Brain abscess: Collection of pus within the brain tissue, which can cause neurological symptoms and requires urgent treatment.
• Sigmoid sinus thrombosis: Blood clot in a major vein near the ear, which can lead to serious complications.
• Epidural or subdural abscess: Pus collection between the skull and brain.

Can Cholesteatoma Be Prevented?

While not all cholesteatomas can be prevented, risk can be reduced by promptly treating ear infections, managing allergies and sinus problems, avoiding insertion of objects into the ear canal, and seeking early evaluation for persistent ear symptoms. Regular ear check-ups are important for people with risk factors.

Although there is no guaranteed way to prevent cholesteatoma, certain measures may reduce the risk of developing this condition or help detect it early when treatment is most effective.

Preventive Measures

• Treat ear infections promptly: Seek medical care for ear pain, drainage, or hearing changes. Complete the full course of any prescribed antibiotics.
• Manage allergies and sinus problems: Conditions that affect Eustachian tube function should be properly treated to maintain healthy middle ear pressure.
• Don't insert objects into the ear: Avoid using cotton swabs or other objects in the ear canal, which can cause trauma and increase infection risk.
• Protect ears from water: If you have a history of ear problems or a perforated eardrum, keep ears dry during bathing and swimming.
• Avoid smoking and secondhand smoke: Smoking impairs Eustachian tube function and increases ear infection risk.

Early Detection

Since early-stage cholesteatoma may cause minimal symptoms, regular ear examinations are important for people at higher risk:

• Those with a history of recurrent ear infections
• People with previous ear surgery or ear tube placement
• Individuals with known eardrum perforations
• Anyone with persistent ear symptoms such as drainage or hearing changes

If you notice any warning signs such as persistent ear drainage (especially if foul-smelling), gradual hearing loss, or a sensation of ear fullness, seek evaluation by an ENT specialist promptly.