Congenital cholesteatoma of the middle ear in children: a hidden pathology

Abstract

Introduction. Congenital cholesteatoma is a rare middle ear pathology in children. An abnormality can be classified as congenital cholesteatoma if it meets certain clinical criteria. Aim. To present patients with congenital cholesteatoma hospitalised at the Department of Paediatric Otolaryngology of the Medical University of Warsaw in 2018-2019 in whom the abnormality was detected incidentally during paediatric and ENT examination. The disease was hidden behind an intact tympanic membrane with no clinical symptoms. Material and methods. A thorough ENT history was taken and microotoscopy and a hearing test were conducted. Preoperative computed tomography of the temporal bone was performed to assess the extent of the pathology in the middle ear. A single-stage surgical procedure was performed in all the patients to remove the pathological growths from the middle ear. Subsequently the patients’ hearing and surgical outcomes were evaluated. Results. During surgery, in two patients cholesteatoma masses were found in the anterosuperior part of the tympanic cavity and in the Eustachian tube area; in one patient, the growth filled the whole mesotympanum. No inflammatory lesions were found in the mastoid cells and antrum. No cholesteatoma recurrence has been found found in follow-up to date. Conclusions. 1. Congenital cholesteatoma in children is most often detected accidentally and localized in the antero-upper quadrant of the tympanic cavity. 2. Congenital cholesteatoma is a slow-growing lesion and develops behind the unchanged eardrum.3. Diagnosis is based on microotoscopy. 4. The only method of treatment is surgical removal of the lesion.