Cholesteatoma

Overview

Cholesteatoma is an abnormal non-cancerous skin growth or skin cyst that develops behind the eardrum, in the middle section of the year. Recurring middle ear infections causes the dead skin to accumulate and damage the sensitive bones behind the ear.

Cholesteatoma affects hearing, balance and the working of facial muscles. It starts as a build-up of earwax and skin causing a cyst on the eardrum. Overtime, this can spread to the bone behind the ear, known as a mastoid bone.

In certain cases, cholesteatoma is due to a birth defect. Congenital cholesteatoma form in the middle ear or in other areas of the ear. In such cases, children develop cholesteatoma from a very young age. Most often, it occurs due to repeated infections in the middle ear. The good news is cholesteatoma is not cancerous but if it is not treated on time, it causes problems like hearing loss, which is unfortunately irreversible.

Who are at risk of cholesteatoma?

• Men are more prone to cholesteatoma compared to women.
• Children with undiagnosed cholesteatoma are at risk of hearing loss by early adulthood. Late childhood or early adolescence is the appropriate time for surgery.
• People with recurrent ear infections causing fluid discharge are at risk of developing cholesteatoma.

What Causes cholesteatoma?

Medical experts have pointed out the factors that cause cholesteatoma which leads to hearing loss if not treated in time. Some of the causes are as follows:

• Blockage of the Eustachian tube: Eustachian tube extends from the back of the nose to the middle of the ear. It allows air to flow through, thereby balancing ear pressure. This conditioncausesa partial vacuum in the middle ear creating a cyst. This can grow considerably as it starts collecting old skin cells, fluids, and waste materials and turns to cholesteatoma. The Eustachian tube cannot function well due to the blockage.
• Sinus infections: Nasal cavities get infected, swollen and inflamed.
• Chronic ear infections: Fluid discharge and inflammation.
• Colds: Infection of the nose and throat
• Allergies: Similar to cold with irritation in the throat
• Birth defects: In rare cases,a cholesteatoma may form in the developmental stage of the fetus.Part of the lining of the ear is trapped as the bone grows. This condition can be detected during childhood.

Symptoms of Cholesteatoma

Patients experiencing cholesteatoma observe the following symptoms, which are mild initially, but increase as the cysts becomes large. Cysts are diagnosed at an advanced stage leaving the doctors with no choice but to remove it surgically.

• Foul-smelling fluid from the infected ear
• Enlarging cyst creates a sense of pressure causing discomfort
• Severe pain and swelling in the ear
• Hearing loss
• Dizziness
• Vertigo, facial muscle paralysis occurs as the cyst grows unchecked
• Recurring ear infections

You need to consult an ENT specialist or Otolaryngologist immediately if any of the above symptoms are present.

Types of cholesteatoma

 Cholesteatoma is categorized under the following three types.

• Congenital cholesteatoma: Is rare, and occurs during the development of the embryo when squamous epithelium residue remains in the middle ear. The tympanic (middle ear) membrane is intact.
• Primary acquired cholesteatoma or retraction cholesteatoma: This happens when there is a malfunction in the middle ear due to low pressure in the middle ear cells. The tympanic membrane creates what is known as a retraction pocket. This occurs in the pars flaccid region of the tympanic membrane, which is very limp. Squamous epithelium collects in this retraction pocket leading to the formation of cholesteatoma after a few years.
• Secondary acquired cholesteatoma: This is caused because of a pre-existing peripheral defect in the tympanicmembrane that allows the epithelium to penetrate into the middle ear.

Complications of cholesteatoma

If untreated it can lead to a lot of complications, which can adversely affect victims ‘quality of life.

• Chronic infection can lead to hearing loss and deafness. This is because of damage to the ossicular chain, so the sound is no longer transmitted to the inner ear.
• Cholesteatoma can erode bone, in which case the infection spreads to the inner ear or brain causing more complications like meningitis, brain abscess, dizziness (vertigo), facial paralysis and even death
• The cysts if untreated spread into the face as it continues to grow to cause facial weakness
• In some cases cholesteatoma continues to grow even after surgery, this is usually with respect to large cysts
• Congenital aural stenosis carries a much greater risk of cholesteatoma as compared to congenital aural atresia

Diagnosis

An audiologist and an ENT specialist diagnose cholesteatoma using any or if required all of the following methods.

• Ear Microscopy:This method helps find out the defect in the tympanic membrane, and the extent of bone erosion of the wall of the ear canal, close to the eardrum.
• CT Scan (computer tomography): The extent of bone loss is determined accurately, this can aid the course of treatment. It helps locate the size and placement of the cholesteatoma.
• Hearing Test (audiogram): Since hearing loss is the major issue in cholesteatoma, this test will help determine the extent of hearing
• MRI (magnetic resonance imaging): If the CT scan reports recommend a closer view, MRI is performed to investigate if the infection has spread to the brain. The growth traits of cholesteatoma are clearly visible in an MRI.

Treatment for cholesteatoma

Most often, treatment for cholesteatoma is surgical intervention. The infection gets resistant to antibiotics leaving surgery as the only option.

• As a first step, the ENT specialist evaluates the extent of damage and prescribes medication to reduce the infection and inflammation. The drain from the ear has to stop and it must dry completely. Surgery can be performed only after the inflammation and infection has reduced
• The non-surgicaloption is possible only in the early stages but most often cholesteatoma is diagnosed at a later stage, leaving surgery as the only method of treatment
• Type of surgery depends on the part of the ear that is infected. Sometimes clinical examination helps detect the location of cholesteatoma, whereas at other times an MRI or a CT scan is performed to correctly evaluate the extent of damage
• The main purpose of the surgery is to remove the dead skin, clear the infection and make the area dry before any surgical intervention
• Surgery involves reconstructing the eardrum, removing the bone behind the ear. In certain cases, a second surgery is needed to make sure the entire cholesteatoma is removed before reconstructing the bones
• The second surgery is performed in six to twelve months after the first surgery. Sometimes there is a temporary hearing loss if the reconstruction of the hearing bone is delayed
• In general, this surgery is performed as an outpatient setting. In rare cases of severe infection, prolonged hospitalization is needed with antibiotic treatment
• Cholesteatoma surgery is performed under general anesthesia. At times it is done with intravenous sedation and local anesthesia
• Follow up visits are mandatory to clean the ear and recheck hearing
• Patients can get back to work typically in one or two weeks. If the work involves strenuous activity then aperiod of rest for three to four weeks after surgery may be necessary
• Cholesteatoma requires monitoring and follow up to check for any recurrence of infection

Are antibiotics a cure for cholesteatoma?

• In most cases, surgery remains the main solution to cure cholesteatoma
• Antibiotics can reduce the infection but cannot remove or stop the formation of cysts

 Risks involved with surgery

Patients and family must know the risks of cholesteatoma surgery.

• Facial paralysis the nerve that passes through the ear may get affected. This causes restricted movements in the face that can affectthe closing of the eye, smile, and raising the forehead. This is either partial or complete which happens soon after surgery or after some period. Recovery can be partial or complete
• Tinnitus (noises in the ear) can occur after the surgery, though it is an uncommon problem. Some patients complain of hearing noises and discomfort in the ear post-surgery
• Dizziness is a complaint from a few patients, which usually resolves within a day of surgery. It is unlikely to prevail as a persistent problem. This is due to the effect of anesthesia
• Hearing loss: there is a slight chance of hearing loss which can be permanent
• Taste abnormalities a small nerve that is responsible for the taste and salivary function passes through the ear. After surgery, some patients experience an abnormal taste and dryness of the mouth. This improves over time.
• Infection is a common risk associated with any surgery. Anesthesia-related complications, along with otological risks.

However, these risks are minimized if the surgery is performed by an experienced otolaryngologist. The success of the surgery depends also on postoperative care. Following the instructions after surgery is crucial in the healing process.

Prognosis of cholesteatoma

With successful removal of the cholesteatoma, chances of recovery are good. Many patients are completely treated with cholesteatoma. Prognosis also depends on the extent of complications over the course of the condition. Successful surgery can completely eliminate the large cysts too. Postoperative follow up medical consultation is very crucial to evaluate the condition

Prevention

Cholesteatoma cannot be completely prevented but its intensity can be reduced by seeking timely medical assistance

• Awareness among parents about cholesteatoma reduces the risk of hearing the loss in children
• Treating ear infections quickly and thoroughly helps monitor the formation of cysts
• The occurrence of cholesteatoma is reduced with local treatment
• Vitamin A has proven to be very effective in treating hyperkeratosis, this can be a therapeutic option for cholesteatoma as well
• Research has shown that Cortisporin and Vitamin A is considered effective as local treatment
• Always consult an ENT specialist for better diagnosis and treatment.

References