Cholesteatoma - Definition, Symptoms, Complications and Treatment

Cholesteatoma Operation in Istanbul

Cholesteatoma Definition

Cholesteatoma Definition
Cholesteatoma - - An Abnormal, Non-cancerous  (Destructive and Expanding) Skin Growth (Keratinizing Squamous Epithelium) in The Ear
Inflammation focus in the middle ear and surrounding tissues, which is trapped, airless, can cause recurrent inflammation and inflammation, and can cause destruction in the surrounding tissues

Cholesteatoma is  foci of inflammation arising from skin cells in the middle ear behind the eardrum (destructive and expanding growth consisting of keratinizing squamous epithelium). Normal skin cell debris and the ear wax which excreted from outer ear  moved towards outward. Sometimes there is a problem in this process (hole in the eardrum is seen as a barrier, the fact that the negative pressure in the middle ear, chronic or recurrent middle ear infection) can cause for developing of cholesteatoma.

Cholesteatoma can lead to more serious problems, such as chronic ear infections, hearing loss is permanent and  dizziness. More rarely, meningitis, brain abscess, can cause facial paralysis. Cholesteatoma can be found in the middle ear, years before symptoms occur. It's an inflammation, although the structures that make up the contents of a benign, which acts as malignant tumors which can dissolve the bone tissue structures.

There are two types of cholesteatoma: Acquired and congenital cholesteatoma cholesteatoma.
The most common type of acquired cholesteatoma cholesteatoma. It occurs after a hole in the eardrum, or tympanic membrane in a slump pocket (retraction pockets) . After the formation of holes in the eardrum, healthy skin cells towards by moving the the outer ear to the middle ear  rear side of the membrane, initiate the formation of cholesteatoma.

Congenital kolesteaom is much more rare and it occurs behind the intact eardrum. These cholesteatomas often go unnoticed for a long time. Congenital cholesteatoma thought to arise from "misguided" cells during fetal development.

Aural epidermosis, aural keratosis, cholesteatoma auris, cholesteatoma tympani, cholesteatosis, epidermoid cholesteatoma, epidermosis, keratoma, margaritoma, margaroid tumour, mother-of-pearl tumour, pearl tumour, pearly tumour, tumeur perlée

How does cholesteatoma occur?

Cholesteatomas are divided into "Acquired Cholesteatoma" and "Congenital Cholesteatoma" according to their appearance. Acquired cholestetoma is the most common type of cholesteatoma and occurs after collapse or perforation of the eardrum. After the hole in the eardrum has emerged, the skin cells that move from the outer ear canal to the middle ear initiate the formation of cholesteatoma. Congenital cholesteatomas are rare and have a congenital healthy eardrum with a cholesteatoma ball.

Cholesteatoma Symptoms

Cholesteatoma Symptoms
Cholesteatoma Symptoms

Cholesteatomas grow in a long time without any symptoms. Cholesteatomas often cause the following symptoms:

- Hearing loss
- Intermittent or persistent ear infections
- Foul-smelling ear discharge
- In rare cases, the pain and crusting
- Very advanced cases, facial weakness / paralysis of the face, dizziness, full deafness and neurological symptoms may occur.

Interesting Symptoms of Cholesteatoma

The following symptoms may occur in patients with cholesteatoma, unlike patients with simple tympanic membrane perforation:

- Ear discharge with a foul odor, which may disturb the patient, sometimes flowing to the pillow of the patients while they are sleeping (the smell of the inflammatory discharge coming out of the inflammatory focus, which is trapped in the middle ear and mastoid leukemic, which has become airless)
- Spontaneous inflammatory ear discharge without water contact to the ear (cholesteatoma may cause spontaneous inflammatory discharge)
- The presence of inflammatory ear discharge that does not improve or decrease with local or systemic medical treatment (usually the cholesteatoma focus is collected in a focus that does not receive air and does not have sufficient drug effect)
- There are patients with cholesteatoma, which can be detected after complications such as facial paralysis, brain or neck abscess, meningitis, petrositis occur (some patients do not consult a doctor for a long time, considering the current ear discharge as normal. can be placed)
- In patients with cholesteatoma, even if the middle ear ossicles are damaged, sometimes severe hearing loss may not occur due to the transmission of sound energy by the cholesteatoma sac. Hearing loss may occur in patients after surgical removal of the cholesteatoma. The primary aim of the operation (mastoidectomy operation) in these patients is to protect the patient from infection and related complications.

Do patients with cholesteatoma have ear pain?

Patients with cholesteatoma may not have ear pain for many years. Usually, ear pain may be a sign of complications, or it may be due to the enlargement of the cholesteatoma sac into the bone, and may mean the spread of infection to the mastoid bone or meninges. In patients with chronic otitis media with cholesteatoma, ear discharge and progressive hearing loss are the most prominent symptoms. Inflammatory discharge in the ear may begin without contact with water or an upper respiratory tract infection, and this discharge may not decrease with medical treatment.

Is a cholesteatoma a tumor?

Cholesteatoma is a chronic inflammation focus that contains a fat-rich structure, in which the skin epithelium that should not be in the middle ear cavity grows. Although it is not a tumor itself; It can cause pressure and destruction in the surrounding tissue such as a tumoral tissue. It can be considered as a benign, locally destructive tumor.

Diagnosis of Cholesteatoma

Diagnosis of cholesteatoma is made by microscopy of ear ear, thin section tomography and ear MRI. Imaging methods are used to assess the size, boundaries, and status of adjacent structures of the cholesteatoma.

While the skin cells present in the cholesteatoma consistently produce new ones; old dead cells cannot be removed and accumulate in a closed space. As a result, the destruction and pressure of the surrounding tissue constantly occurs. Cholesteatomas provide a favorable environment for bacteria, fungi and other microorganisms in the middle ear and can cause continuous infection.

The condition of the eardrum and middle ear is evaluated during the otoscopic ear examination performed in patients with typical odor, resistant and chronic inflammatory ear discharge and hearing loss complaints. The granulation tissues in the middle ear and the scutum region in the attic region are evaluated whether there is an eroded area in the bone. In some patients, the cholesteatoma sac can be seen directly during ear microscopy. Thin-section temporal bone tomography and audiometry test may be requested as standard preoperative examinations. In the tomography, the presence and location of the costatoma, the condition of the middle ear ossicles, the condition of the facial nerve can be evaluated and the operation can be planned. In this way, the diagnosis is made and the type of treatment is planned. In the selection of the patient to be operated, it is important to choose the hospitals with a quality ear microsurgery instruments and a quality microscope. In patients, whether the bone tissue that normally covers the facial nerve is melted by cholesteatoma can be evaluated in advance on tomography images. In this case, the surgeon must be very careful when cleaning the cholesteatoma sac.

Is there a special laboratory test for the diagnosis of cholesteatoma?

There is no laboratory test performed by blood that enables the diagnosis of cholesteatoma. Anyway, since the diagnosis of cholesteatoma is largely based on clinical history, physical examination, and radiographic findings, no laboratory test or biopsy is usually necessary. However, when there is a mass lesion (such as a tumor) involving the external auditory canal or middle ear and may cause recurrent infections, the diagnosis may need to be confirmed by biopsy. Thin-section temporal bone CT scanning is the diagnostic imaging modality of choice for choleteatomas in the middle ear and/or mastoid bone. CT is also useful in assessing the damage of the cholestetaoma to the surrounding tissue and evaluating the size and location of the cholestetaoma sac.

Cholesteatoma Complications

Cholesteatoma Complications
Cholesteatoma Complications
 Depending on the surrounding tissues destroyed the health complications of cholesteatoma can be seen in the following:

- Labyrinthitis (inner ear infection)
- Permanent hearing loss (see hearing impairment)
- Headache
- Vestibular problems
- Nystagmus
- Facial Paralysis
- Chronic inflammation of the middle ear
- Meningoencephalitis
- Ear pain
- Tinnitus (ringing in the ears)
- Intracranial abscess / granuloma

Patients with cholesteatoma may sometimes apply directly to the doctor's examination with the above-mentioned complications.

Can cholesteatoma cause death?

Depending on the cholesteatoma, the surrounding tissue destruction, recurrent infections and the spread of these infections can lead to serious and dangerous inflammation of the brain, meninges, and veins. In my professional life, I have seen many patients with cholesteatoma who have developed complications associated with it, and so far I have not seen a patient death associated with cholesteatoma. If left untreated, deafness, brain and neck abscess, facial paralysis, dizziness (vertigo), meningitis and very rarely death can occur. Mortality rate for cholesteatoma has decreased considerably thanks to the development of modern diagnosis and tests, early medical treatment and patient follow-up, and reduced to 0.04-0.15% of the cases (source >> Description of 34 Patients with Complicated Cholesteatomatous Chronic Otitis Media). But some scientific articles still report the mortality rate for cholestatoma may reach 34% (source >> Otogenic intracranial complications). The rate at this end is controversial and seems a little higher than normal. In societies where the sociocultural level is high and health services are good, early diagnosis and treatment provide a serious decrease in these rates.

Cholesteatoma Treatment

Cholesteatoma Treatment

Antibiotics may reduce associated infections, but no drug therapy to treat cholesteatomas are available. Surgical treatment of cholesteatoma removal and restoration of hearing to ensure that the most appropriate treatment option. Cholesteatoma in the middle ear and mastoid air cells are often foci of inflammation residues mastoidectomy surgeries are performed in order to clean. This is the eardrum surgery cataract operations (tympanoplasty), and middle ear bones repair (ossiküloplasti) surgery can be performed. Cholesteatoma for patients with large amount, two-stage surgical procedure can be recommended. Focus of the first procedure and any associated cholesteatoma is to clear chronic infections. 6-12 months after the second surgery, the damaged bones in the middle ear can be repaired during the repair or titanium ossicular prosthesis (PORPs or TORPs) can be used.

Mastoidectomy operation video - 1

Mastoidectomy operation video - 2

Bad Smell Inflammatory Ear Discharge May Be a Symptom of "Cholesteatoma" Video

Cholesteatoma Removal Surgery Risks - Facial Paralysis

In the treatment of cholesteatoma, mastoidectomy operations are performed and as seen above, microdrill systems and various metal ear instruments are used during these operations. When the middle ear structures are damaged due to cholesteatoma, especially when the bone protective area on the facial paralysis is destroyed, when the cholesteatoma sac is removed, the facial nerve is compressed or cut, or if the bone tissue is heated with a micromotor, it may be destroyed. Sometimes, even if the cholesteatoma sac is pushed with the help of cotton, temporary facial paralysis may occur after the operation. In my career, I have never had a patient with permanent facial paralysis after a mastoidectomy, and I hope I never will! In other words, altered ear anatomy due to cholesteatoma may increase the risk of complications. Apart from this, there are classical surgical risks such as damage to the anatomical structures adjacent to the middle ear, bleeding and infection during the operation. There is also a risk of facial paralysis due to costeatoma itself. In other words, patients with untreated cholesteatoma are at risk of developing various effects such as facial palsy, memenitis, brain and neck apnea.

Facial paralysis and meningitis in patients with cholesteatoma are conditions that require immediate treatment!

In patients with cholesteatoma who are not aware of it or are not followed up by an otolaryngologist, a serious infection such as facial nerve damage and peripheral facial paralysis or meningitis may occur due to the destructive effect of cholesteatoma over time. These patients may need urgent medical and surgical treatment. Patients may require mastoidectomy and cholestatoma clearance, antibiotics and corticosteroid treatment. In patients presenting with complications, treatment for cholesteatoma should be done as soon as possible.

Does cholesteatoma surgery increase hearing thresholds?

The primary goal in cholesteatoma removal procedures is to protect the patient from possible complications related to cholesteatoma. Hearing gain of patients and restoration of hearing are secondary goals. Sometimes, after the removal of the cholesteatoma focus, the ossicles that play a role in sound transmission may be damaged and replaced by the cholesteatoma, and conductive hearing loss may increase after the removal of the cholesteatoma. In some patients, hearing thresholds may not change after cholesteatoma surgery, while in others they may decrease. 

Cholesteatoma Treatment Cost in Istanbul

Generally, mastoidectomy operations are performed in the treatment of cholesteatoma. During these operations, tympanic membrane operations and ossicular chain reconstructions are sometimes performed together. The operation can be planned as radical mastoidectomy and modified radical mastoidectomy. Mastoidectomy surgery price in Turkey varies between 4000-6000 US Dollars, depending on the hospitals and the procedures to be performed.

Patients with cholesteatoma may require complicated ear surgeries!

In patients with untreated and untreated cornic suppurative otitis media with cholesteatoma, destruction of the middle ear ossicles, spread to the mastoid bone and destruction of the cell tissue may occur over time. In these patients, planned surgical procedure, eardrum operations, ossicular chain operations, mastoidectomy operations may be required. In fact, sometimes these operations can be planned secondarily, that is, they include ossicular operations performed for the purpose of cleaning the first cholesteatotome and inflammatory remnants and then restoring hearing. In other words, the patient may need to plan more than one operation. Since the ossicles called malleus, incus and stapes in the middle ear, which articulate with each other and are responsible for the transmission of the energy created by the sound wave to the inner ear, are destroyed due to cholesteatoma, the patient may need to perform ossiculoplasty using total or partial ossicular replacement prostheses (TORP and PORP). It is appropriate to perform these surgeries in hospitals with well-equipped and high-quality microscopes. While these operations are planned for the treatment of cholesteatoma, the patient's otoscopic examination, audiometric tests and thin-section temporal bone tomography should be evaluated in detail.

Conditions that increase the risk of facial paralysis during cholesteatoma surgery!

In patients who are scheduled for ear operations (mastoidectomy and tympanoplasty operations) for the treatment of cholesteatoma, the condition of the ossicular chain in the middle ear and whether there are eroded areas in the bone above the facial nerve can be evaluated in thin-section ear tomography sections before the operation. The patient may be asked whether he has had facial paralysis before. The risk of facial paralysis may increase in mastoidectomy operations for the treatment of cholesteatoma in the following situations:

- If the cholesteatoma is observed to cause damage to the bone structure over the facial nerve and in the middle ear with imaging methods (due to the cholesteatoma eroding the bone structure, the tmpanic segment of the facial nerve may be exposed), partial or temporary damage to the facial nerve may occur even if there is a simple pressure during the operation.

I would like to share my experience about this with you: "About 8 years ago, a patient who had eardrum surgery in a different clinic before and had ear pain and ear discharge complaints came for examination. We detected an appearance compatible with cholesteatoma in the mastoid cells.We planned a modified radical mastoidectomy operation for the patient and during the operation, when I gently lifted the cholesteatoma ball using cotton, I saw that the tymapnic segment of the facial nerve was open and there was no bone structure on it. We did not detect any facial contractions or irritative fascial muscle activity. After an uneventful operation, a few hours after the patient woke up, when I was seen in the patient's room, I was not very upset and had a heartbeat "only partial slowdown in the left eye. There was movement and other facial muscle examination was normal". Although no metal instrument hit the patient's facial nerve, this partial paralysis what was the reason? Indeed, even just pushing with a cotton ball could cause temporary fascial paralysis, and copious water irrigation was used to protect it from heat damage, especially during the shaping of the bone structure with the help of micro-dirt. We recommended oral cortisone, vitamin B12 and drops with local cortisone to the patient. Within about 3 weeks, the eyelid and surrounding muscle movements had completely returned to normal. In my professional life, I have never had a mastoidectomy operation that resulted in permanent facial paralysis, but really, even if you do the procedure correctly; The risk of facial paralysis should be known by the patients.

- The surgeon's low command of the ear anatomy and less experience in surgery

- Inadequate microscope and other otological surgery set in the hospital where the operation was performed.

- Preventing the recognition of facial contractions caused by stimulation of the facial nerve by administering muscle relaxants during the surgery

- Altered ear anatomy as a result of repeated ear surgeries

- Having facial nerve palsy after previous ear surgery on the same ear

- If the patient presents with facial paralysis as a complication of cholesteatoma, since the facial nerve has already been damaged, it cannot be guaranteed that the cholesteatoma will be completely cleared and the nerve will recover completely with oral medical treatment.

As it is written above, patients who have undergone mastoidectomy before and whose bone structure erodes on the fascial bone should be careful while cleaning their ears in the future; The information letter should be carried with the patient and shown to the physician who will perform ear aspiration. 

Cholesteatoma surgery consists of procedures that require experience and knowledge.

How many days do you need to stay in Istanbul for cholesteatoma treatment?

When you come to Istanbul for the treatment of cholesteatoma, you can come for examinations 1 day before the operation, stay in the hospital for 1 day on the day of the operation and stay here for at least 7 days afterward. I usually prefer to remove the tampons placed in the external ear canal after 2-3 weeks, and some of our patients may prefer to stay in Turkey during this period.

Does the shape of the auricle change after cholesteatoma treatment?

Mastoidectomy operations in the treatment of cholesteatoma are usually performed with a "postauricular incision" made behind the auricle. After the operation is completed, the possibility of "pronounced ear" is reduced thanks to the pressure dressing and various sewing techniques applied to the ear. There are also precautions such as putting pressure dressing on the operated ear for 5-7 days and lying on the ear if the patient notices that the auricle is angling outward. So far, I have not seen a patient with serious deformation of the auricle after mastoidectomy. However, after merging the dorsal ear cavity with the mastoid cavity, the outer ear canal is reshaped by widening, and when meatoplasty is also performed, it can only be noticed that the outer ear canal hole is larger than normal when viewed from the side. This procedure is performed to allow air to enter the middle ear and re-epithelialize the newly formed large cavity. Meatoplasty is performed as a standard procedure in Canal Wall-Down Mastoidectomy Operations, in which the posterior wall of the external auditory canal between the newly formed mastoid cavity and the middle ear cavity is lowered by drilling with the help of a drill. 

What bacteria can cause cholesteatoma?

This issue is controversial and the most frequently detected bacteria in cholesteatoma are Pseudomonas aeruginosa, Staphylococcus aureus, Proteus and anaerobes like Bacteroides and anaerobic gram-positive cocci (source >> Notes on the microbiology of cholesteatoma: clinical diagnosis and treatment). Detection of these bacteria and initiation of medical treatment for them is not sufficient for the permanent treatment of cholesteatoma. The cholesteatoma, which is a focus of inflammation that is usually trapped and remains in the surrounding bone structure, should be surgically removed and this area should be brought into contact with air. Culture of bacteria is not a standard procedure prior to ear surgery. It can be done in the presence of complications or widespread infection.

How long does cholesteatoma surgery take? 

Depending on the extent of the cholesteatoma and the middle ear changes it causes, the duration of ear surgery may take between 2-4 hours. The success of the surgery may vary depending on the patient's preoperative preparation and the surgeon's experience.

Hearing after cholesteatoma surgery

There are 3 possibilities for hearing that may occur to patients after cholesteatoma surgery:

1. the level of hearing may not change
The level of hearing may be reduced (if the cholesteatoma itself contributes to the ossicular chain conduction or if, together with the cholesteatoma, the remaining part of the ossicular chain and eardrum in the middle ear has to be removed)
3. the level of hearing may increase (during the procedure, tympanic membrane repair or ossicular chain reconstruction can be performed)

The aim of surgery in the treatment of cholesteatoma is to protect the patient from cholesteatoma and its associated risks, to clear the cholesteatoma and to reduce the possibility of its recurrence. Hearing preservation or improvement is not the first goal in surgical treatment.

What happens if I don't have cholesteatoma surgery?

Cholesteatoma, which behaves like a tumor, can grow in the middle ear, destroying the ear ossicles, surrounding bones and structures that provide hearing in the middle ear. It can cause a focus of inflammation that spreads to the surrounding tissues. If not treated in time, it can lead to very serious consequences in patients such as facial paralysis, meningitis, brain abscess, neck abscess, neck vein inflammation or cerebellum abscess, inner ear damage and permanent hearing loss. Inflammatory secretion draining from the middle ear to the outer ear may occur hundreds or thousands of times in patients, but the fact that no complications have arisen in previous infections cannot guarantee that these complications will not be seen in the final infection.

Surgery is the only treatment for patients with cholesteatoma!

The effectiveness of medical treatment in cholesteatoma is limited, and in some patients, no response to medical treatment can be obtained. There is no permanent cure for cholesteatoma other than surgery. The aim of the operation is to completely remove the cholesteatoma sac, to eliminate the chronic infection in the middle ear and to prevent possible complications. The second purpose is to preserve or restore hearing function.

Why is the efficacy of medical therapy limited in patients with cholesteatoma?

The distinguishing symptom of cholesteatoma from other simple ear infections is a painless ear discharge that is persistent or frequently recurrent, with limited or no response to medical therapy. The reason for inadequate local or systemic antibiotic therapy in these infections can be explained as follows:

"Clesteatoma sac is an inflammation focus that is self-limited and trapped in a bony cavity. This causes the blood flow to the focus to be limited and almost no blood circulation reaches the center of the focus. In other words, oral or intravenous antibiotics enter the systemic circulation and reach the cholesteatoma focus. Topical antibiotics can often surround the cholesteatoma, suppress the infection in the periphery of the cholesteatoma, and penetrate the center of the cholesteatoma sac in a very limited amount by a few millimeters. Often there may be little or no effect on existing ear discharge. It may be resistant to any antimicrobial therapy due to inadequate topical therapy.As a result, otorrhea persists or tends to recur despite frequent and aggressive treatment with antibiotics.Some of the patients do not have persistent inflammatory auricular discharge. After consulting an otolaryngologist for a reason, they may realize that they have a cholesteatoma".

Source links >>

  • Cholesteatoma
  • Cholesteatoma: MedlinePlus Medical Encyclopedia
  • Cholesteatoma | Health |
  • Cholesteatoma | Vestibular Disorders Association
  • Cholesteatoma - NHS Choices
  • Cholesteatoma - eMedicine World Medical Library - Medscape
  • Cholesteatoma Causes, Diagnosis & Treatments - Clinical Key
  • Cholesteatoma - Cause of Hearing Loss and Deafness

  • Murat Enoz, MD, Otorhinolaryngology, Head and Neck Surgeon - ENT Doctor in Istanbul

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