Description
Case I
Case II
Case III


Description 

Congenital cholesteatomas are rare lesions of the temporal bone. Main diagnostic criterion includes the presence of white mass below intact tympanic membrane. The diagnosis of congenital cholesteatoma requires normal tympanic membrane and no history of perforation, discharge or previous surgery (e.g. tympanotomy). Congenital cholesteatomas grow slowly, their course is often asymptomatic for years. Growing, they can cause destruction of the ossicles, temporal bone and also tympanic membrane. In this stage one cannot differentiate their origin (congenital or acquired). Symptoms depend on the destruction of individual structures and include most commonly 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, and rarely can be found also in adult patients. Its course is more aggressive in children and more often causes serious complications.


Case I

The photo below shows congenital cholesteatoma of the middle ear, right ear, 5-year-old child. Whitish mass fills the middle ear, cholesteatoma is located in front of the handle of the malleus, in the anterior and superior quadrants, in a typical position, most frequently reported in the literature. All other anatomical structures, including the handle of the malleus are normal, undamaged. Cholesteatoma of this location is asymptomatic for a long time. It does not destroy the ossicles, which are located it the posterior quadrants. It can cause hearing loss and otitis media with effusion if spread in the direction of the eustachian tube. Otitis media with effusion can therefore obscure the presence of a tumor in the middle ear (fig. 1 and 1a).

In this case, cholesteatoma showed no symptoms, the diagnosis was raised during a routine examination. The diagnosis of congenital cholesteatoma of such placement sometimes may be difficult, especially in young children, where the anterior quadrant of the tympanic membrane is poorly visible.

Congenital cholesteatoma of the middle ear
Fig. 1 Congenital cholesteatoma of the middle ear
Congenital cholesteatoma - scheme
Congenital cholesteatoma – scheme
  1. lateral process of malleus
  2. handle of malleus
  3. umbo
  4. cholesteatoma in antero – superior quadrant of tympanic cavity
  • Range of surgical intervention: explorative tympanotomy with cholesteatoma removal.
  • Ossiculoplasty was unnecessary, the ossicular chain was intact (incus and stapes are located in posterior quadrants of the tympanic cavity).
  • Second – look operation was unnecessary because site of removal of the cholesteatoma was very well visible in videootoscopy.
  • It was the early stage of uncomplicated congenital cholesteatoma.
  • There were no signs of recurrence in two years follow-up.

Case II

The photo shows congenital cholesteatoma of the middle ear, left ear, 6-year-old child. Whitish mass fills the middle ear. Cholesteatoma is located in the posterior quadrants of the tympanic cavity. The tympanic cavity is filled with a serous fluid. Typical air-fluid level is also visible. This is large cholesteatoma that grows in the direction of the eustachian tube causing otitis media with effusion. It also destroys the ossicles which are located just it the posterior quadrants. Note that sometimes chronic otitis media with effusion may obscure the presence of a tumor / cholesteatoma in the middle ear (fig. 2).

In this case the symptoms included aural fullness, plugged ear, moderate hearing loss which was connected to the presence of the mass, effusion in the middle ear and destruction of the ossicles (long process of the incus and stapes). Despite the destruction of the ossicles, hearing loss may not be pronounced because cholesteatoma itself is a sound conductive medium. The diagnosis was raised during a routine examination. The diagnosis of congenital cholesteatoma of such placement is rather easy in otoscopy.

Congenital cholesteatoma
Fig. 2 Congenital cholesteatoma
  • Range of surgical intervention: explorative tympanotomy with cholesteatoma removal, antromastoidectomy, posterior tympanotomy, myringoplasty with temporal fascia and ossiculoplasty with artificial titanium ossicles.
  • The cholesteatoma spread in the tympanic cavity as well as in the mastoid causing destruction of the ossicles (incus and stapes superstructure). That is why surgical intervention was wide and included broad opening of the tympanic cavity and mastoid.
  • Removing the cholesteatoma in children is much more difficult than in adults because the mastoid is well pneumatized and there are thousands of air cells that can store microscopic cholesteatoma remnants.
  • Because of this there is a need to perform second – look operations, usually 6 months after initial procedure. CT scans can be the option to assess the presence of the cholesteatoma in mastoid.
  • Ossiculoplasty with autogenous ossicles was impossible due to their extensive destruction. Ossicular reconstruction was performed using titanium prosthesis. The prosthesis was placed on the stapes footplate and under handle of the malleus.
  • It was the late, complicated stage of congenital cholesteatoma.

Case III

The photo shows congenital cholesteatoma of the middle ear. Right ear, 8-year-old child. Whitish mass fills completely the middle ear. The middle ear structures are invisible. The tympanic membrane is bulging and opaque. This is large advanced cholesteatoma that grows in the direction of the eustachian tube and mastoid cavity causing almost complete destruction of the ossicles. The diagnosis of congenital cholesteatoma of such placement is easy using an otoscope. Differential diagnosis should include advanced tympanosclerosis – but this condition in young children without a history of chronic otitis is practically non-existent (fig. 3).

In this case the symptoms included hearing loss which was connected to the presence of the mass and destruction of the ossicles, aural fullness, plugged ear. Despite the destruction of the ossicles, hearing loss may not be pronounced because cholesteatoma itself is a sound conductive medium (in cases where inner ear is intact). The cholesteatoma in this case was diagnosed during examination by otolaryngologist.

Congenital cholesteatoma
Fig. 3 Congenital cholesteatoma
  • Range of surgical intervention: explorative tympanotomy with cholesteatoma removal, antromastoidectomy, posterior tympanotomy, myringoplasty with temporal fascia and ossiculoplasty with artificial titanium ossicles.
  • Tympanic membrane was partialy removed because cholesteatoma adjoined firmly to its inner surface and it could not be separated.
  • The cholesteatoma spread in the tympanic cavity as well as in the mastoid causing destruction of the ossicles (incus and stapes superstructure).
  • That is why surgical intervention was wide and included broad opening of the tympanic cavity and mastoid.
  • Removing the cholesteatoma in children is much more difficult than in adults because the mastoid is well pneumatized and there are thousands of air cells that can store microscopic cholesteatoma remnants.
  • Because of this there is a need to perform second – look operations, usually 6 months after initial procedure.
  • CT scans can be the option to assess the presence of the cholesteatoma in mastoid.
  • Ossiculoplasty with autogenous ossicles was impossible due to their extensive destruction.
  • Ossicular reconstruction was performed using titanium prosthesis.
  • The prosthesis was placed on the stapes footplate and under handle of the malleus.
  • It was the late, complicated stage of congenital cholesteatoma.