Wydawnictwo Medyczne Mediton

Under the scientific patronage of

the Polish Society of Audiology and Phoniatrics


Case study

« return
Multiple extratemporal, intratemporal and intracranial complications of middle ear congenital cholesteatoma in a 10 year-old child
Jerzy Kuczkowski 1/, Waldemar Narożny 1/, Karolina Markiet 2/, Wojciech Biernat 3/, Czesław Stankiewicz 1/
  • 1/ Katedra i Klinika Otolaryngologii, Gdański Uniwersytet Medyczny, Gdańsk
  • 2/ II Zakład Radiologii, Gdański Uniwersytet Medyczny, Gdańsk
  • 3/ Katedra Patomorfologii, Gdański Uniwersytet Medyczny, Gdańsk

Congenital cholesteatoma is a rare disease. The most common presentation is a pearly white mass, medial to an intact tympanic
membrane, with no previous history of ear discharge, perforation or any ear surgery. It can lead to various complications, both intratemporal and extratemporal, some of which may be fatal.

Here we present a rare case of a 10 year-old child with congenital cholesteatoma, complicating to acute mastoiditis, Bezold’s abscess and sigmoid sinus thrombophlebitis. After necessary examinations (CT, MRI scans), the patient underwent surgery for complete removal of cholesteatoma (canal wall down tympanoplasty), removal of a thrombus from sigmoid sinus with its luminal occlusion, ligation of internal jugular vein and Bezold’s abscess drainage. To our knowledge, this is the first report accessible in the literature worldwide on the subject of intracranial complications of congenital cholesteatoma.

Otorynolaryngologia vol 14. no 1. Marchpages: from 58to 64
full version