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Cholesteatoma

Cholesteatoma is an ear disorder that begins in childhood. In its more rare form of congenital cholesteatoma it begins during the fetal phase of life. The underlying cause of acquired cholesteatoma is considered to be the dysfunction of the Eustachian tube which leads to lack of ventilation of the tympanic cavity. Lack of ventilation causes a retraction of the tympanic membrane, where dermal epithelium is enclosed giving rise to the development of cholesteatoma. Congenital cholesteatoma is caused by cells that remain into the tympanic cavity and turn into cells of the cutaneous epithelium during fetus development.

 

Thus, cholesteatoma is the enclosure of cutaneous epithelium into the tympanic cavity. The epithelium grows continuously and the dead cells unable to come out are accumulated in the cholesteatoma bag which inflates at a variable pace and progressively corrodes the bone walls and the ear structure. This process seems to be accelerated by toxic substances production during an ear inflammation. The erosion caused by the cholesteatoma may cause a malfunction of the ear and also further complications to the organs that are near the ear or pass by the ear.

 

One of the first and more common effects of cholesteatoma is the progressive hearing loss that may lead to practical deafness.  In more advanced forms of the disease, vertigo episodes may occur as a result of erosion in the posterior part of the labyrinth. Moreover, in unattended cases paralysis of the facial nerve (responsible for the movements of the face), meningitis and cerebral abscesses may occur thus making obvious the importance of timely identification and proper treatment of cholesteatoma. Proper monitoring of children is even more important in order for the situations which may lead to cholesteatoma to be diagnosed in time and to be properly treated before it result in the forms previously described.


The patient normally sees the doctor during an ear suppuration. Purulent otorrhea has an intense bad odour. The patient also complaints for hearing loss and ear congestion. Before proceeding with the final diagnosis we must wait until the acute phase is over because pus and edema of the tissues distort the image of the ear. During this phase, ear cleaning is the first thing to do because local care of the ear is more important than any antibiotic treatment. Thus, pus must be removed, possible polyps as a result of the inflammation must also be removed or cauterized and finally local antiseptics must be placed. Patient and doctor must have quite a lot of patience as they may have to repeat the above procedure several times.

After a period of approximately two weeks, when the inflammation is in the phase of recession and the ear is “dry”, the image of the ear changes radically. Only then is the doctor able to talk about a cholesteatoma diagnosis with great certainty. It is advisable to complete the examination with a computed tomography which will show us the size of the cholesteatoma sac and the extension of erosion in several ear structures such as the posterior labyrinth, enabling us to know what to expect during an eventual surgical intervention.

The computed tomography as well as the diagnosis must be done during the recession of the inflammation’s acute phase, because pus in the external auditory canal, polyps or fluid in the mastoid cavity  are depicted in the same way as the cholesteatoma and may lead to wrong conclusions. Cortisone administration before the tomography would also be another proper choice so that fluid can be removed of the tympanic cavity and the edema of the tissues can improve.

The only treatment for the cholesteatoma is its surgical removal. If it remains in the ear, this may prove to be quite dangerous for the patient’s health and he/ she must be absolutely aware of this. Before intervention we need to control ear hearing ability. The surgeon will do his/her best to improve or at least maintain the hearing ability. However, the removal of the cholesteatoma should be the main goal of the intervention even if this entails hearing loss.

Some patients are likely to suffer from cholesteatoma in both ears. In this case, hearing level, the size of cholesteatoma and further threatening complications determine which ear will be operated first. Other patients may have lost the sense of hearing in one ear and suffer from cholesteatoma in the other. In this case also, the same factors must be taken into consideration before proceeding to surgical intervention. Nevertheless, here the decision may be more conservative. Last but not least, as in every operative treatment, it is necessary to evaluate the patient’s overall health.

The surgical operation is performed under general anesthesia and the patient must remain in hospital for at least one day after surgery. We make the incision behind the ear pinna. Nevertheless, nowadays the scientific community has not yet come to a conclusion regarding the best technique for the removal of the cholesteatoma. Our team uses the technique according to which the cholesteatoma bag is dissected and subsequently removed. Today there is an increase in the doctor’s awareness towards the diagnosis of cholesteatoma, patients have easier access to doctors and general anesthesia has become safer.  As a result, the decision to perform or undergo the surgical intervention has become more direct, the cholesteatoma is diagnosed in time and the surgical cavity necessary for its removal is usually smaller. Therefore, this technique of following the cholesteatoma bag seems quite acknowledged.

After the cholesteatoma removal, the surgeon must restore the anatomy of the ear as much as possible. Therefore, areas protecting important anatomical structures such as the posterior labyrinth and the facial nerve must be strengthened. Furthermore, the ear ossicular chain must also be restored and the doctor must try to perform tympanoplasty using temporalis fascia and cartilage-perichondrium graft. Tympanic cavity is covered with absorbable material.

Diagnosis and surgery of congenital cholesteatoma may prove to be particularly demanding. This type of disease develops behind the normal tympanic membrane and it is likely to cause loss of hearing, fluid accumulation in the ear and complications resulting from the erosion of the bone coverage of many anatomic elements. Its insidious course may allow cholesteatoma to grow considerably in the cranial base bones before being detected through its complications.

Its surgical treatment may prove quite demanding and therefore a thorough preoperative review with computed tomography and magnetic imaging of the area is considered necessary. Navigation systems may be exceptionally useful in this kind of intervention. Surgical results regarding the ear functionality may be very poor or there may be no functionality at all with obliteration of the ear cavity. However, after pondering eventual complications which are often a threat for the patient’s life, complete and radical removal of cholesteatoma is, beyond all doubts, our first and principal goal. After the operation, the cavity of the ear is filled with absorbable material and patient’s head is bandaged. The patient leaves the hospital with instructions regarding the first post-operative days and the subsequent ear protection.