Cholesteatoma: Causes, Symptoms & Treatment

If the demarcation between the external auditory canal and the middle ear falls away, there is a risk of cholesteatoma, which then makes surgical treatment inevitable.

What is a cholesteatoma?

Schematic diagram showing the anatomy of the ear with cholesteatoma. Click to enlarge. Cholesteatoma is a disease of the ears. By nature, the ears are divided into different sections, which include the external auditory canal and the middle ear. Both sections are cut off from each other by the eardrum. In the majority of cases with cholesteatoma, this barrier is defective, meaning that the natural partitioning of the middle ear from the external auditory canal is no longer present. As a result, components of the external auditory canal can grow into the middle ear and, in the worst case, even into the inner ear, which can then lead to a cholesteatoma. The growth of squamous epithelia into the middle ear can destroy the structure of the bones there, which then leads to the typical image of the cholesteatoma: dead skin layers that are particularly stable due to their structure. Furthermore, a final, white corneal layer forms over the partially destroyed bone structure of the middle ear. Due to the loss of the natural demarcation barrier, secretions from the middle ear can now flow into the external auditory canal and cause inflammatory reactions there. The biggest problem here is the risk of superinfection. This refers to an infection with different pathogens, each of which promotes the occurrence of the other, which makes therapy much more difficult. In addition to the foul-smelling odor as a result of inflammation in the ear, typical symptoms of cholesteatoma include dizziness and, depending on the severity of the disease, deafness.

Causes

The cause of cholesteatoma, as already mentioned, is the fact that the tympanic membrane is no longer intact, that is, the partition between the external auditory canal and the middle ear is no longer guaranteed. Now, squamous epithelia of the external auditory canal can grow into the middle ear and destroy the bone structure there. Depending on whether the loss of function of the tympanic membrane was caused by inflammation, a distinction is made between primary and secondary cholesteatoma. In addition, although it is rare, there is a variant of cholesteatoma in which a defect of the tympanic membrane, whether inflammatory or non-inflammatory, need not be present in the first place. This is a congenital defect in which the squamous epithelia form behind an (intact) tympanic membrane in the middle ear, leading to cholesteatoma.

Symptoms, complaints, and signs

In cholesteatoma, the affected person suffers from various hearing complaints and, as a result, a significant reduction in quality of life. In severe cases, the affected person may also lose hearing completely, and this loss cannot be restored. First and foremost, however, a cholesteatoma leads to a strong discharge from the ear, which is associated with a very unpleasant odor. This odor also has a negative effect on the social environment of the affected person, so that difficulties can arise in this area. Likewise, it can lead to bullying or teasing in children, so that they develop psychological discomfort and depression as a result. As it progresses, cholesteatoma leads to hearing loss and severe dizziness. The patient may also lose consciousness and faint. Paralysis of the face may also occur, so that the patient can no longer easily take in food and liquids. There is pain in the ears, which can spread to the head. Fever may also occur and be associated with a general feeling of illness. As a rule, however, cholesteatoma does not affect the life expectancy of the affected person.

Diagnosis

Cholesteatoma can be diagnosed relatively easily. For the trained eyes of an otolaryngologist, visual diagnosis using an ear microscope is already sufficient. Based on the relatively clear clinical picture, he can determine the presence of a cholesteatoma. However, in order to determine how advanced the cholesteatoma is, i.e. how far it protrudes into the middle ear, further diagnostic measures are required. Among the most common is computed tomography (CT for short).

Complications

Cholesteatoma usually results in ear complications.Severe ear pain may occur. In many cases, the pain from the ears spreads to the neighboring regions, so most patients also suffer from headaches and toothaches. In the worst cases, paralysis of the face occurs and the affected person suffers hearing loss. The pain is often not permanent, but sporadic. However, loss of hearing is noticeable to the affected person. The face is also affected by paralysis, so that certain muscles cannot be moved. Paralysis of the tongue or mouth may also occur, so that it is no longer possible for the patient to eat normally. Similarly, paralysis of the tongue can lead to speech disorders. The quality of life is severely restricted by the cholesteatoma and everyday life is made considerably more difficult for the patient. The symptoms not infrequently also lead to depression and suicidal thoughts. Unfortunately, restoration of the ossicles is not possible in every case, so that in the worst case the patient has to live with hearing loss for the rest of his or her life. If surgery is possible, inflammation is prevented by antibiotics. Life expectancy is not reduced by cholesteatoma.

When should you see a doctor?

As a rule, a cholesteatoma requires medical examination and treatment in any case. In most cases, those affected require surgical intervention. A doctor should definitely be consulted if there is a sudden hearing loss or severe ear pain. A foul-smelling discharge from the ear may also indicate cholesteatoma and should be examined by a physician. In many cases, patients suffer from dizziness or paralysis of various facial regions. Therefore, if these symptoms also occur, medical treatment is definitely necessary. Similarly, fever with headache may also indicate cholesteatoma. Especially the sudden occurrence of these symptoms and hearing loss indicates cholesteatoma. In emergency cases, hospitalization can also be sought. In general, however, cholesteatoma is diagnosed and treated by an otolaryngologist. With early treatment, this usually results in a positive course of the disease.

Treatment and therapy

The only way to treat a cholesteatoma is surgical removal. This involves cutting open the affected ear so that the cholesteatoma can be cut away using a scalpel. An attempt is then made to restore the tympanic membrane, thus counteracting the possible recurrence of the cholesteatoma by treating the cause. Finally, if it should be possible, an attempt is made to repair the destruction to the bone structure of the middle ear caused by the cholesteatoma. However, before surgery can be performed, it is important to treat any existing superinfection. The administration of antibiotics should kill the bacteria that have settled there, otherwise there is a risk that the bacteria will spread further during surgery.

Outlook and prognosis

In most patients, cholesteatoma has a favorable prognosis. The growth on the ear can be removed in a surgical procedure. In many cases, the growth leads to inflammation, which must also be treated so that freedom from symptoms can ultimately be achieved. People who have a stable immune system experience a regression of the inflammation within a few weeks through drug therapy. The older the patient and the weaker his or her health condition, the longer the healing process will normally be. Nevertheless, there is a good chance of recovery in this case as well. The operation is associated with the usual risks and side effects. These must be taken into account when making the prognosis. With good medical care, there is generally no impairment after the treatment. The usual hearing is restored as the healing process subsides. If cholesteatoma is not detected and treated until a very advanced stage, permanent damage may occur. The risk for hearing loss or changes in the bone structure in the ear increases as the growth in the ear increases. In very rare cases, the growth of the cholesteatoma has already reached brain areas. The patient is then in a life-threatening condition, as a stroke may occur.

Prevention

The trigger of cholesteatoma is thus the removal of the tympanic membrane; this allowed squamous epithelia to grow into the middle ear in the first place, which could then lead to the subsequent inflammations. Prophylaxis of cholesteatoma can thus only be limited to keeping the tympanic membrane intact and preventing damage. Since inflammation can also lead to a defect in the eardrum, careful ear hygiene is recommended. However, this should not be exaggerated, otherwise the natural skin flora of the ears could be permanently damaged, which would not only not prevent the settlement of pathogens and thus a cholesteatoma, but could favor it.

Aftercare

After surgical treatment of cholesteatoma, a tamponade usually remains in the affected person’s ear canal for one to three weeks. Only after removal of this protective device can it be verified how successful the therapy was. It is not always possible to determine the extent to which hearing has been restored on the day of removal. In some patients, another operation is necessary to check the functionality of the auditory canal in a year. In principle, control appointments are necessary only sporadically in the case of surgery without complications. However, if a so-called ear radical cavity was created during the operation, it is recommended to have it medically treated at regular intervals. Pain after cholesteatoma surgery is not expected in uncomplicated procedures. Healing of the wounds in this case is completed superficially after about two weeks. However, wound healing is delayed, especially when an ear radical cavity is used. This may take several weeks to months. In this case, the patient should take care to avoid the penetration of water (especially soapy water) and dirt. Activities of daily living can be resumed about one week after surgery. This may not include sports activities such as swimming and diving. Anyone wishing to engage in these activities should discuss this with the attending physician. How long a patient is unable to work after surgery depends on the extent of the medical intervention on the one hand and on the work content on the other.

What you can do yourself

Treatment of a cholesteatoma focuses primarily on removing the causative narrowing of the ear canal, with concomitant symptom relief. The affected person can take some measures to assist treatment and reduce discomfort. After surgery, the affected ear must be spared. The sufferer is advised to avoid cold, especially drafts. Other influences such as great heat or shocks must also be avoided, otherwise serious complications may develop. In the worst case, the surgical scar will tear open and the patient will have to be operated on again. If unusual symptoms such as ear pain, fever or dizziness occur after the operation, the doctor in charge must be informed. In addition, the physician’s instructions regarding wound care should be followed. A cholesteatoma can usually be treated well, but requires extensive monitoring by the responsible physician. After treatment is complete, the affected ear must be checked regularly by the ear specialist. In some cases, a cholesteatoma forms again, which must be treated surgically. If hearing problems to the point of hearing loss have already developed, a hearing aid must be worn.