Overview

A cholesteatoma is an unusual, non-cancerous growth of skin-like tissue that develops in the middle ear. The middle ear is the air-filled space behind your eardrum. While it is rare, this condition can be present from birth (known as a congenital cholesteatoma) or, more commonly, it can develop over time (an acquired cholesteatoma) as a complication of long-standing changes in the pressure within your ear.

Essentially, a cholesteatoma forms when skin cells from the lining of your ear canal become trapped in the middle ear, an area where they do not normally belong. These trapped skin cells continue to multiply and shed, much like skin anywhere else on your body. Over several months, these dead skin cells gradually accumulate to form a growing sac or cyst. This growth is significant because it is locally invasive and destructive. This means it can slowly erode and damage the delicate bones of the middle ear, called the ossicles (tiny bones that help you hear), and can even extend into the mastoid bone, which is the air-filled bony lump located behind your ear.

Although the name 'cholesteatoma' might sound like it refers to a tumour or cholesterol, it is neither. It is a collection of epidermal (skin) and connective tissues that grows independently and can cause a range of problems, from a persistent ear discharge to, in very rare cases, serious complications affecting the brain. It usually affects only one ear, but can occasionally affect both. In the UK, it is estimated to affect around 3,000 adults and 1,000 children.

Symptoms and Causes

Understanding why a cholesteatoma occurs and how it shows itself is key to early diagnosis and treatment.

Symptoms

The symptoms of a cholesteatoma often begin subtly, and very small growths might not cause any noticeable problems at first. However, as the growth gets larger, typical symptoms usually appear:

• Persistent Ear Discharge: This is a very common symptom. You might notice a watery discharge from the affected ear that often has a foul smell. This discharge, known as otorrhoea, can be persistent and may not clear up even with antibiotic ear drops.
• Gradual Hearing Loss: Many people experience a slow loss of hearing in the affected ear. This is often a conductive hearing loss, meaning there's a problem with how sound waves are conducted through the outer or middle ear.
• Feeling of Fullness or Pressure: You might feel a sense of pressure or a blocked sensation inside your ear.
• Recurrent Ear Infections: Frequent ear infections that keep coming back are a common sign.
• Dizziness (Vertigo): Some people experience vertigo, which is a sensation of spinning or feeling off-balance.
• Tinnitus: You might hear ringing, buzzing, or other noises in your ear, known as tinnitus.
• Ear Pain: In more advanced cases, or if an infection is present, you might experience pain in the affected ear.
• Facial Weakness or Paralysis: Rarely, if the cholesteatoma grows large enough to damage the facial nerve (the nerve that controls your facial muscles), it can lead to weakness or drooping on one side of your face.
• Headache: In very advanced cases, a headache can occur.
• Pearly White Mass or Retraction Pocket: During an examination, an ENT specialist might see a characteristic pearly white mass behind the eardrum or a retraction pocket (where the eardrum is pulled inwards) with trapped skin debris.

In children, common symptoms include a persistent leak or discharge from the ear, a gradual loss of hearing, and a feeling of ear pain or pressure.

Causes

The root causes of cholesteatoma are mainly divided into two types: congenital and acquired.

• Congenital Cholesteatoma: This type is rare and means you are born with the condition. It happens when tiny skin cells (squamous epithelium) become trapped within the temporal bone (the bone on the side of your skull that contains the ear) during your development as an embryo. These usually appear in childhood, often between 6 months and 5 years of age, as a pearly white lump behind an eardrum that looks otherwise healthy. Children with congenital cholesteatoma typically don't have a history of ear infections or eardrum perforations.
• Acquired Cholesteatoma: This is the more common type, accounting for about 80% of cases. It usually develops due to long-standing problems with the Eustachian tube, which is the small tube connecting your middle ear to the back of your throat. If this tube doesn't work properly, it can lead to chronic negative pressure in the middle ear. This negative pressure can cause a part of your eardrum to be sucked inwards, forming a pocket. This pocket then traps skin cells that normally line the ear canal, allowing them to build up and form the cholesteatoma.
• Secondary Acquired Cholesteatoma: This can happen if your eardrum has been damaged, for example, by an injury, a severe infection, or previous ear surgery. This damage can create an opening that allows skin cells from the outer ear canal to migrate into the middle ear space, where they accumulate and form the cholesteatoma sac. Repeated ear infections are a common cause in children.

Diagnosis and Investigations

If you experience symptoms that suggest a cholesteatoma, it's important to see your doctor. The diagnosis process typically involves several steps, starting with your GP and then moving to a specialist.

Diagnosis

Your journey to diagnosis usually begins with your GP. They will listen to your symptoms and examine the inside of your ear. If they suspect an infection, they might initially prescribe ear drops. However, if your symptoms persist despite antibiotics, or if your GP strongly suspects a cholesteatoma, they will refer you to an Ear, Nose, and Throat (ENT) specialist.

The ENT specialist will conduct a thorough clinical assessment. This often involves an otoscopic examination, where they use a special instrument (an otoscope, or often a microscope for a more detailed view) to look deep inside your ear. During this examination, the specialist will look for characteristic signs of a cholesteatoma, such as:

• A retraction pocket: This is where a part of your eardrum is pulled inwards.
• A pearly white mass behind your eardrum.
• Squamous debris (dead skin cells) or a crusty discharge in the attic (the upper part of the middle ear) or the ear canal.
• Granulation tissue, which is a type of inflamed tissue. If a granular polyp (a small, fleshy growth) is seen in the ear canal, it will be treated as a cholesteatoma until proven otherwise.
• Any twitching or weakness in your facial muscles, which could indicate that the facial nerve is involved.

They will also take a detailed history of your ear problems.

Investigations

To confirm the diagnosis and understand the full extent of the cholesteatoma, your ENT specialist will arrange further investigations:

• CT Scan: A Computed Tomography (CT) scan is a crucial imaging test. It uses X-rays to create detailed cross-sectional images of your ear and surrounding bones. This scan helps the specialist to see the exact size and location of the cholesteatoma, identify any bone erosion (damage to the ossicles, mastoid bone, or the bony covering of the inner ear and facial nerve), and plan the best surgical approach.
• MRI Scan: In some cases, an Magnetic Resonance Imaging (MRI) scan may be needed. This is particularly true if there's a suspicion that the cholesteatoma might have spread into the cranial cavity (the area inside your skull where your brain is located), or to help differentiate the cholesteatoma from other conditions in the middle ear.
• Hearing Evaluations (Audiometry): These tests are performed to measure your hearing loss and determine its type and severity.
• Balance Tests: If you've been experiencing dizziness or vertigo, balance tests may be conducted to assess the impact on your balance system.
• Microscopic Suctioning: Sometimes, the ENT specialist may use a microscope to carefully suction away any discharge or debris from your ear. This can provide important information about the condition of your eardrum, the integrity of the ossicular chain (your hearing bones), and any destruction of the mastoid bone.

Management and Treatment

The management of cholesteatoma is primarily surgical, as medical treatments alone cannot cure the condition.

Medical Management:

Medical treatments are generally limited to managing any associated infections. If you have an infection causing discharge or pain, your GP or ENT specialist may prescribe:

• Topical and/or Oral Antibiotics: These are used to clear up the infection, reduce discharge, and ease pain.

It is very important to understand that while antibiotics can treat the infection, they do not remove the cholesteatoma itself. Regular ear cleaning with suction at an ENT clinic and using antibiotic drops when necessary can help to manage symptoms and stop the disease from spreading further, but this approach will not cure the cholesteatoma and still carries the risk of serious complications.

Surgical Management:

Surgery is the main and most effective treatment for cholesteatoma. The primary goal of the operation is to completely remove the cholesteatoma to create a safe, dry ear and prevent serious complications. The operation is usually performed under general anaesthetic, meaning you will be asleep, and typically takes between one and three hours.

During the surgery, incisions (cuts) are usually made either behind the ear, above the opening of the ear, and/or within the auditory meatus (ear canal). Depending on the size and extent of the cholesteatoma, the surgeon may perform different types of mastoidectomy. This involves carefully removing infected cells and bone from the mastoid bone. For very limited disease, some surgeons may use an endoscope (a thin, lighted tube with a camera) through the ear canal.

In addition to removing the cholesteatoma, the surgeon may also perform:

• Tympanoplasty: This procedure involves repairing the eardrum, often using a graft (a small piece of tissue taken from another part of your body).
• Ossiculoplasty: This is performed to repair or reconstruct the delicate hearing bones (ossicles) if they have been damaged by the cholesteatoma.

After the Operation:

After surgery, you may have stitches and packing inside your ear, which are usually removed after a few weeks. You might also have a head bandage, which is typically removed the morning after your operation. You will likely be given topical antibiotics (ear drops) and possibly steroids to aid healing.

Recovery usually takes about 6 to 8 weeks. During this time:

• You will need to rest for about two weeks.
• Pain around the ear is normal and can be managed with prescribed pain relief.
• Some blood-stained discharge from the ear is common initially.
• Your hearing will likely be muffled due to the packing in your ear, and any tinnitus you experience might temporarily worsen.
• Your balance may be disturbed for a few days.

It is crucial to follow all post-operative instructions carefully to avoid infection and aid healing. This includes keeping your ear and the surgical scar dry when washing (using cotton wool smeared in Vaseline as an ear plug), completing any course of antibiotics you are given, and avoiding activities that cause pressure changes in your ear, such as vigorous nose blowing or sneezing with your mouth open. Flying should be avoided for at least 4 weeks after surgery. If you have a mastoid cavity (a space created in the bone behind your ear during surgery), you will need regular cleaning of wax and to keep the ear dry long-term.

Prevention

Preventing cholesteatoma can be challenging because its development is often linked to underlying ear conditions or congenital factors. However, there are steps you can take to reduce your risk of developing an acquired cholesteatoma and to prevent its progression:

• Prompt Management of Ear Infections: Seek medical attention for any persistent ear discharge or recurrent ear infections. Early and effective treatment of chronic ear infections (chronic otitis media) can help prevent the conditions that lead to cholesteatoma.
• Address Eustachian Tube Dysfunction: If you experience ongoing problems with your Eustachian tube (the tube that equalises pressure in your middle ear), such as a feeling of fullness or pressure, discuss this with your doctor. Proper management of this condition may reduce the risk of eardrum retraction and subsequent cholesteatoma formation.
• Maintain Good Ear Hygiene: While not a direct preventative measure for cholesteatoma itself, maintaining good ear hygiene and avoiding inserting objects into your ear can help prevent injury or infection that could potentially lead to secondary acquired cholesteatoma.
• Early Recognition of Symptoms: Be aware of the symptoms of cholesteatoma, such as persistent, foul-smelling ear discharge, gradual hearing loss, or recurrent ear infections. Seeking prompt medical attention for these symptoms can lead to an earlier diagnosis and treatment, potentially preventing the disease from causing significant damage.
• Regular Follow-up: If you have a history of chronic ear problems or eardrum retraction pockets, regular check-ups with an ENT specialist may be beneficial to monitor your ear health and detect any changes early.

Outlook / Prognosis

The long-term outlook after cholesteatoma surgery typically involves a recovery period of 6 to 8 weeks. However, long-term follow-up care is absolutely essential to monitor for any problems, especially the possibility of the cholesteatoma returning.

After Surgery:

• Hearing: Surgery aims to prevent complications and may improve your hearing. However, it is not guaranteed, and your hearing could remain the same or even worsen. The risk of total and permanent hearing loss is higher if the disease has already damaged the inner ear or balance system. Some children may require hearing aids or implants after surgery.
• Recurrence: Cholesteatoma can recur, either as a residual (left behind) or recurrent (new growth) cholesteatoma. This is a significant concern, and you will need regular follow-up appointments, which may include clinical examinations, MRI scans at certain intervals, or even further 'second-look' surgery to check for any regrowth. Regular ear cleaning visits may also be necessary to prevent reinfection.
• Surgical Risks: While surgery is generally safe, there are potential risks. These include temporary or, rarely, permanent facial muscle weakness, prolonged dizziness (especially if the disease has affected the balance system), worsening or permanent hearing loss, persistent eardrum perforation, ongoing discharge, infection, and a temporary loss of taste on the front one-third of the tongue on the same side as the operation.

Potential Complications if Untreated:

If a cholesteatoma is left untreated, it can lead to very serious complications due to its destructive nature:

• Permanent Deafness: The cholesteatoma can destroy the ossicles (hearing bones) and damage inner ear structures, leading to permanent hearing loss.
• Damage to Inner Ear Structures: This can cause permanent deafness, severe dizziness, and ongoing balance problems.
• Facial Nerve Damage: The growth can damage the facial nerve, leading to permanent facial weakness or paralysis.
• Spread of Infection: In rare but severe cases, the infection associated with a cholesteatoma can spread to the brain, causing life-threatening conditions such as meningitis (inflammation of the membranes surrounding the brain and spinal cord) or a brain abscess (a collection of pus in the brain).
• Mastoid Bone Damage: The cholesteatoma can erode and damage the mastoid bone behind the ear.

Early detection and management of cholesteatoma are crucial for preventing these serious complications and improving your long-term outcome.