Cholesteatoma is a lesion of the temporal bone and the middle ear. It is formed from the proliferating squamous epithelial cells within the area of the temporal bone. In other words – it is abnormal skin growth in the middle ear. Usually it grows slowly, causing initially destruction of the tympanic membrane, then ossicles and temporal bone. Symptoms depend on the destruction of individual structures and include most commonly discharge from the ear and hearing loss. Untreated may cause intratemporal or intracranial complications, even life-threatening. They include paresis and paralysis of the facial nerve, dizziness, vestibular malfunction, meningitis, abscesses of the temporal lobe of the brain and cerebellum. These complications occur even in the era of ubiquitous antibiotic. As mentioned, cholesteatoma generally grows slowly, but note that compared to adults, in children its course is more aggressive and more often causes serious complications. Cholesteatoma may be infected and thus results in chronic discharge from ear.
The photo below shows the middle ear cholesteatoma, right ear, 14-year old patient. This is an image that can be confused with the presence of wax on the tympanic membrane. Whitish – yellow mass is located in the rear quadrants, i.e. over the ossicles and incudostapedial joint. This mass invaginates into the tympanic cavity. The handle of the malleus can be seen, the remainder of the tympanic membrane is intact, which further may hinder diagnosis. Cholesteatoma in this location partially destroyed the ossicles – the long process of the incus was absent. The cholesteatoma was limited to the tympanic cavity without going to the mastoid (fig. 1 and 1a).
In this case cholesteatoma symptoms were moderate, there was no discharge from ear nor hearing loss. Sound transmittion was almost perfect (through the mass of the cholesteatoma itself). Cholesteatoma suspicion of such a placement and extent is often accidental, during routine examination with an otoscope.
- dotted line – posterior quadrants of the tympanic membrane (anterior quadrants invisible on this photo)
- Range of surgical intervention: explorative tympanotomy, removing of the cholesteatoma from the middle ear, myringoplasty using tragal perichondrium, ossiculoplasty with incus interposition, adenoidectomy and tonsillectomy.
- Ossiculoplasty with incus interposition: removing the incus which long process was destroyed by cholesteatoma, remodelling using diamond burr, putting it on the head of the stapes under handle of the malleus to create new modified ossicular chain.
- Ossicular reconstruction should be performed using autogenous materias like remnants of the ossicles. If it is imposible due to their extensive destruction, the surgeon can use artificial prosthesis made of titanium or hydroxyapatite.
- Very often cholesteatomas require antromastoidectomy and posterior tympanotomy but in this case the condition was restricted only to the tympanic cavity, so opening of the mastoid was unnecessary.
- It was the initial, early stage of cholesteatoma.
This is a picture of the advanced stage with destruction of the tympanic membrane and cholesteatoma, left ear, 10-year-old patient with cleft palate. You may notice yellowish fluid in the tympanic cavity in anterior quadrants. You may also notice an epidermis in posterior quadrants. The tympanic membrane is atrophic, pulled in the pars tensa as a result of chronic negative pressure in the tympanic cavity. The epithelium (atrophic tympanic membrane) lies directly on the promontory, and goes to the facial recess creating the uncontrolled retraction pocket. In pars flaccida (locus minoris resistentiae of the tympanic membrane) there is also uncontrolled retraction pocket. This is the initial stage of cholesteatoma, it is located mainly in facial recess (fig. 2 and 2a).
In this stage symptoms may include aural fullness and plugged ear. Hearing loss is not common, because atrophic tympanic membrane lies directly on the incudostapedial joint; if it is undamaged, sound can be transmitted unhindered to the inner ear. There can be symptoms associated with adenoid or tonsils hypertrophy, upper respiratory tract infections and other conditions leading to the significant eustachian tube problems like cleft palate.
- malleus handle
- epidermis in posterior quadrants
- In this case one should make a decision to refer to a specialist and perform surgical treatment, because this is the initial stage of cholesteatoma.
- Range of surgical intervention: explorative tympanotomy, removal of the epithelium from tympanic cavity, antromastoidectomy and posterior tympanotomy with removal of the epithelium from the facial recess, myringoplasty with periostium, ventilation tube insertion.
- The effusion and destruction of the tympanic membrane was a consequence of chronic eustachian tube problems due to cleft palate.
- Nonpharmacological methods e.g. nasal balloons were ineffective.
- Nasal sprays with steroids were used ocassionally during upper respiratory tract infection with nasal obstruction.
- Because hearing was deteriorating and the evident destruction of the tympanic membrane was present, the decision of surgical treatment was made.
- Note that there were only moderate symptoms incuding aural fullness and plugged ear, without discharge and hearing loss – but the surgical intervention was wide, including opening the temporal bone with facial recess.
The picture shows the destruction of the lateral attic wall (top). It takes the form of a large uncontrolled retraction pocket. The left ear, 13 – year old child. The thin layer of epidermis lies directly on the neck and the head of the malleus. In the upper part of the pocket there are deposits of the epidermis which invaginates into the mastoid and forms cholesteatoma. The light bounces off the head of the malleus. At the bottom of the picture one can see a part of the tympanic membrane which is pulled and opaque. The tympanic cavity is filled with a dense fluid. Ossicular chain was intact in this case. This is a picture of the late stage of untreated chronic otitis media, complicated by the cholesteatoma of the temporal bone (fig. 3).
In this stage symptoms included aural fullness, plugged ear and noticeable hearing loss. It was associated with a chronic obstruction of the Eustachian tube as a result of structural abnormalities of the nasopharynx in a child with congenital craniofacial deformation. Such chronic otitis media with temporal bone destruction and cholesteatoma lasted several years.
- handle of malleus
- lateral process of malleus
- head of malleus (invisible in normal ears)
- epithelium forming the cholesteatoma
- part of the tympanic membrane which is pulled, opaque and filled with a fluid
- In this case one should make a decision to refer to a specialist and perform surgical treatment, because this is the late stage of cholesteatoma.
- Range of surgical intervention: explorative tympanotomy, removal of the epithelium and retraction pocket, antromastoidectomy, posterior tympanotomy with removal of the epithelium from the facial recess and ossicles, attic wall reconstruction and myringoplasty with temporal muscle fascia and cartilage, ventilation tube insertion.
- The destruction of the temporal bone was a consequence of chronic obstruction of the Eustachian tube as a result of congenital craniofacial deformation.
- Nonpharmacological methods and conservative treatment were ineffective.
- Note that there were only moderate symptoms incuding aural fullness, hearing loss and plugged ear, without discharge – but the surgical intervention was wide, including opening the temporal bone with facial recess.
- Second – look operation was performed 6 months after initial surgery.