Synonyms in a broader sense
Pearl tumour, middle ear, inflammation English: cholesteatom
Definition
A cholesteatoma, also known as a pearl tumour, is a chronic purulent inflammation of the middle ear with bone destruction.
Cause
Squamous epithelium (superficial layer of skin), which lines the external auditory canal, grows into the middle ear and is surrounded by an inflammatory envelope. The outflow of fluid from the middle ear is disturbed by the masses of skin residue and thus the risk of a bacterial superinfection (bacterial infection that “builds up” on an existing infection), often with the pathogen Pseudomonas aeruginosa, is high. The inflammation is maintained by the permanent exfoliation of eardrum cells and leads to ear-running with a stinky (foetal) smell. The skin growth also destroys existing structures (e.g. ossicles: hammer, anvil, stirrup) and can penetrate to the inner ear. The bone resorption (bone erosion) / bone infection (osteomyelitis) resulting from a cholesteatoma can lead to an otogenic, i.e. from the ear, meningitis / meningitis or inflammation of the brain or attack the adjacent facial canal (the facial nerve is responsible for facial expression).
Diagnosis
If cholesteatoma is suspected, a computer tomography (CT) should be performed to show the extent of bone destruction, which is usually sharply defined, by the proliferating squamous epithelium (keratinizing skin layer).
Classification
A distinction is made between a primary cholesteatoma, which occurs when the eardrum is initially closed, and a secondary bone suppuration (osteomyelitis), which involves a defect in the eardrum. The secondary cholesteatoma develops when keratinizing skin layers from the external auditory canal spread through a peripheral hole in the eardrum to the mucosa of the middle ear. The primary form of the disease has various causes:
- Retraction cholesteatoma: If there are ventilation disorders in the tubes (connection between the middle ear and throat), a negative pressure develops in the middle ear and the eardrum shows a retraction (retraction) with pocket formation.
We know this phenomenon from the landing approach in an airplane when there is a temporary disturbance of the pressure balance in the middle ear. Cells that detach from these eardrum pockets accumulate and cause a lining of the tympanic cavity with squamous epithelium (keratinizing skin), which physiologically, i.e. in a healthy state, does not occur here. – Immigration cholesteatoma:In the course of an actively progressing process, cells of the upper auditory canal wall and eardrum cells grow cone-like into the tympanic cavity. – Cholesteatoma in childhood:Remaining embryonic (prenatal) mucosa may be present as nutritive tissue for the development of bone ulceration (osteomyelitis) and also prevents aeration of the tympanic cavity and mastoid process.
Symptoms
In the case of the cholesteatoma, where in most cases so-called cornified squamous epithelium from the external auditory canal passes through the defective eardrum into the middle ear, there are no characteristic symptoms at the beginning, without an acute inflammatory process. An additional inflammation caused by impaired drainage of secretions and bacterial superinfections causes regular “otorrhoea” (otorrhea), whereby the ear discharge usually has a putrid, unpleasant odour, which is why it is called fetal otorrhoea. Hearing loss and earaches can also be observed in the corresponding ear.
In addition, there is often a reduced general condition with fever. Inflammation within the scope of a cholesteatoma can spread to various neighbouring structures, destroying them step by step and thus causing complications which can be observed frequently and early on, especially in the case of an untreated cholesteatoma. As structures that can be attacked are for example Damage to the inner ear can cause hearing loss or even deafness.
In addition, the inclusion of the organ of equilibrium, which is also located in the inner ear, can cause dizziness, nausea and vomiting as further cholesteatom symptoms. – The ossicles HammerAnvil Stirrups
- Hammer
- Anvil
- Stirrups
- The inner ear
- Hammer
- Anvil
- Stirrups
Besides these symptoms, a cholesteatoma can often have even more serious consequences. The most serious consequence is an attack of the nearby bone canal in which the facial nerve runs (facial nerve).
An involvement of the facial nerve causes a so-called peripheral facial nerve paresis, i.e. a functional disorder of the facial nerve, with a resulting unilateral facial paralysis. This usually progresses slowly, with the result that the full clinical picture is only revealed after a few weeks. The symptoms that then appear include, for example, a one-sided loss of function of the mimic muscles (facial muscles) and an inability to frown on the affected side.
The closure of the mouth can also be affected and a drooping corner of the mouth can be observed on one side. Furthermore, labyrinthitis, i.e. an inflammation of the labyrinth, can occur. This is located in the human inner ear, where, in addition to the bony and membranous labyrinth, the organ of equilibrium (vestibular organ), the hearing organ and the cochlea are also found.
As the suffix “itis” suggests, so-called labyrinthitis is an inflammation of the labyrinth in the inner ear, which can occur in various ways, including through a cholesteatoma. Patients with labyrinthitis complain of a poor general condition, as well as of pronounced rotational vertigo in combination with nausea and vomiting. In addition, a hearing disorder may also be present.
A labyrinthine fistula is another complication that can be observed in about 7% of people with a cholesteatoma. It is a connection between the inner ear (in 95% of cases starting from the horizontal arcade, which is part of the organ of equilibrium) and the middle ear, caused by bone resorption. Sometimes, as a symptom of labyrinthine fistula, patients experience dizzy spells as a result of cleaning the ear or pressure on the small cartilage mass on the auricle (tragus).
The involvement of brain structures is also serious. If these are attacked by processes in the ear, this is known as otogenic endocranial complications. All these complications, which can occur in the course of a cholesteatoma, require immediate hospitalization, as do peripheral facial paresis, labyrinthitis and labyrinth fistula.
According to their severity, otogenic endocranial complications cause a strongly reduced general condition with fever. Even a reduction in consciousness and convulsions can affect those affected. Other symptoms of otogenic endocranial complications may include boring headaches and neck stiffness. – a thrombosis of the venous blood vessels of the brain (thrombosis of the sigmoid sinus)
- An epidural or subdural abscess
- A brain abscess (an abscess is an encapsulated accumulation of pus) or
- An inflammation of the meninges (meningitis)
