Cholesteatoma

Synonyms in a broader sense

Pearly lump, middle ear, inflammation

English: cholesteatoma

definition

A cholesteatoma, also known as a pearly tumor, is a chronic, purulent inflammation of the middle ear with bone destruction.

root cause

Squamous epithelium (superficial layer of skin), which forms the outer skin, grows here Ear canal lined in that Middle ear and is surrounded by an inflammatory envelope.
The outflow of fluid from the middle ear is disturbed by the masses of skin debris and thus there is a risk of bacterial superinfection (bacterial infection that "settles on" an existing infection), often with the pathogen Pseudomonas aeruginosa, large.

The inflammation is caused by the permanent exfoliation of the skin eardrumcells and leads to ear runny ears with a foul (fetid) odor.

The skin overgrowth also destroys existing structures (e.g. ossicles: hammer, anvil, stirrup) and can reach into Inner ear advance.

The bone loss (bone erosion) / bone infection resulting from a cholesteatoma (Osteomyelitis) can lead to an otogenic, i.e. from ear outgoing, Meningitis / meningitis or brain inflammation or attack the adjacent facial canal (the facial nerve is responsible for facial expression).

diagnosis

If cholesteatoma is suspected, computed tomography (CT) should be performed in order to show the extent of the bone destruction, which is usually sharply limited by the proliferating squamous epithelium (keratinizing skin layer).

Classification

One differentiates between one primary cholesteatomawhich occurs when the eardrum is initially closed and secondary bone suppuration (osteomyelitis), in which there is a defect in the eardrum.

The secondary cholesteatoma develops through the encroachment of keratinizing skin layers from the external auditory canal through a peripheral hole in the eardrum onto the middle ear mucosa.

The primary form the disease has various causes:

• Retraction cholesteatoma:
  If there are ventilation disorders in the tubes (connection of the middle ear and throat), a negative pressure is created in the middle ear and the eardrum shows a retraction with the formation of pockets. We know this phenomenon from the landing approach in an airplane, when there is a temporary disturbance of the pressure equalization in the middle ear.
  Cells that detach from these eardrum pockets accumulate and cause the tympanic cavity to be lined with squamous epithelium (keratinizing skin), which physiologically, i.e. in a healthy state, does not occur here.
• Immigration scholesteatoma:
  As part of an actively progressing process, cells of the upper wall of the auditory canal and eardrum cells grow into the tympanic cavity like cones.
• Cholesteatoma in childhood:
  Remaining embryonic (prenatal) mucous membrane can be present as nutrient tissue for the development of bone dilatation (osteomyelitis) and also prevents ventilation of the tympanic cavity and the mastoid process.

Symptoms

At the Cholesteatoma, where in most cases so-called keratinizing squamous epithelium from the external ear canal by the defective eardrum in the Middle ear reaches, there are no characteristic symptoms at the beginning, without an acute inflammatory process.
An additional one inflammationwhich is caused by impaired secretion drainage and bacterial superinfections, ensures regular "Ear runny ears“ (Otorrhea), whereby the ear discharge usually has a putrid, unpleasant smell, which is why one of a fetid otorrhea speaks.

Also one Hearing loss and Earache can be observed in the corresponding ear. In addition, there is often a reduced general condition fever.

An inflammation in the context of a cholesteatoma can have various neighboring structures overlap, destroy them step by step and thus cause complications that can be observed frequently and early, especially with an untreated cholesteatoma.
Examples of structures that can be attacked are:

• the Auditory ossicles
  • hammer
  • anvil
  • stirrup
• the Inner ear

Damage to the inner ear can be a Hearing loss up to Deafness condition. The organ of equilibrium, which is also located in the inner ear, can also be included dizziness, nausea and Vomit than cause more cholesteatoma symptoms.

In addition to these symptoms, a Cholesteatoma often have even more serious consequences. Serious is a attack of nearby bone canalin which the Facial nerve runs (Facial nerve). Involvement of the facial nerve requires a so-called peripheral facial palsy, i.e. a functional disorder of the facial nerve, with one-sided facial paralysis caused by it. This usually progresses slowly, which means that the full clinical picture only comes to light after a few weeks.

The symptoms that then show up include, for example unilateral loss of function of the mimic muscles (Facial muscles) and an inability to die forehead on the affected side too frown. Also the Mouth closure may be impaired and a drooping corner of the mouth can be seen on one side.

Furthermore, a Labyrinthitis, so inflammation of the labyrinth may occur. This is located in human inner earwhere next to the bony and the membranous labyrinth, there is the Balance organ (Vestibular organ), the hearing organ and the cochlea. As the ending “itis” suggests, so-called labyrinthitis is an inflammation of the labyrinth in the inner ear, which can arise in various ways, including a cholesteatoma. Patients with labyrinthitis complain next to one poor general condition over distinct Vertigo in combination with nausea and Vomit. In addition, a Hearing impairment to be added.

A Labyrinth fistula is another complication that can be seen in approximately 7% of people with cholesteatoma. This is a Connection between the inner ear (in 95% of cases starting from the horizontal semicircular canal, which is part of the organ of equilibrium) and Middle earcaused by bone loss. Sometimes those affected feel the labyrinth fistula as a symptom Attacks of dizziness in the course of Ear cleaning or at pressure on the small mass of cartilage on the auricle (tragus).

One is also serious Participation of structures of Brain. If these are attacked by processes in the ear, this is referred to as so-called otogenic endocranial complications. To be mentioned here

• a thrombosis of the venous blood conductor of the brain (sigmoid sinus thrombosis)
• a Epidural- or Subdural abscess
• a Brain abscess (an abscess is an encapsulated collection of pus) or
• a Meningitis (Meningitis)

All of these complications that can arise in the course of a cholesteatoma require as well one peripheral facial palsy, one Labyrinthitis and a Labyrinth fistula an instant one hospitalized in a hospital. Depending on their severity, otogenic endocranial complications result in a greatly reduced general condition with fever. Even one Restriction of consciousness and cramps can afflict those affected. Other symptoms of otogenic endocranial complications may be boring headache and a Neck stiffness come to light.

therapy

Since the cholesteatoma can lead to the above-mentioned serious complications with brain involvement (e.g. meningitis), one is surgical supplies necessary.

First, conservative treatment is used as preparation for the operation antibiotic ear drops (e.g. ciprofloxacin), which act against Pseudomonas aeruginosa, the common pathogen responsible for inflammation.

The goals of surgical treatment are the complete removal of the cholesteatoma, the healing of the bone healing, the prevention of otogenic complications (affecting the ear), the restoration of a functional ossicular chain and the closure of the eardrum defect to close the tympanic cavity in the direction of the external auditory canal.

Cholesteatoma surgery

There is both a closed and an open one Surgical technique.

In the open technology a bone cavity is created that includes the tympanic cavity, the mastoid cavity, and the mastoid cells affected by inflammation. The cholesteatoma is cleared out, i.e. the horn-forming skin cells are removed and a wide connection to the external auditory canal is also established.

In the closed technology a so-called mastoidectomy is carried out, i.e. the mastoid process, the cells of which are aerated and lined with mucous membrane, is completely cleared out, so that only its bony walls remain.

If the ossicles are also attacked by the pearl tumor, one can Tympanoplasty, the reconstruction of the sound conduction apparatus. This procedure is performed after the cholesteatoma has been removed.

A distinction is made between the five basic techniques according to Wullstein of tympanoplasty (restoration of the ossicular chain):

• Type I - myringoplasty (eardrum plastic)
  If there is an eardrum defect and an intact, vibratory ossicular chain, the eardrum defect is covered / closed and a connection with the bones is established.
• Type II - ossiculoplasty
  In the case of a defective ossicular chain, missing parts are replaced or the missing parts are bridged.
• Type III
  If the transmission chain is defective and no longer functional, the operation ensures direct sound transmission from the eardrum or an inserted transplant to the inner ear.
• Type IV
  The sound pressure transmission takes place without the ossicular chain.
• Type V
  Fencing operation: the eardrum and stapes are connected to one another in this method.

A hearing improvement can be achieved through this operation if the ear trumpet (tube) is continuous and the inner ear is functional.

Complications

Due to the destruction of the bones, it is possible that the ossicles are also destroyed and the sound conduction and sound amplification function of the middle ear is considerably restricted:
It can turn out to be a Hearing loss develop.

The attack / inflammation of the bony mastoid process (Mastoiditis) can lead to duct formation in the semicircular canal system (organ of equilibrium), which leads to vertigo attacks (dizziness) can lead.

The inflammation can extend into that Inner ear as well as the Facial canal or even in that Cranial cavity (Meninigitis).