Congenital Cholesteatoma: The Silent Pathology

Background: Congenital cholesteatomas (CC) arise from epithelial remnants around the petrous bone. They enlarge gradually causing progressive destruction and functional damage to the ear and surrounding structures. Because of their insidious course, most patients are misdiagnosed and present late with complications. Methods: This is a retrospective study of 41 cases diagnosed as CC and followed up for 2 years. All patients underwent full audiological evaluation and a radiological study (CT, MRI). Results: The male:female ratio was 2.3: 1, and the mean age was 30.89 years. The mean delay before presentation was 13 months. The commonest presentations in order of frequency were: discharge (60.9%), hearing loss (51.2%), facial paralysis (31.7%) and intracranial complications (31.7%). Forty patients were treated surgically by a variety of approaches depending on the extensions and functional status. The facial nerve was decompressed in 9 cases and cable grafted in 4. Two cases underwent VII-XII anastomosis later on. Conclusion: CC present with a myriad of clinical manifestations and should be suspected in all patients with unexplained otological signs and symptoms. They must be completely excised and the functional status of the ear rehabilitated either immediately or later. Follow-up must be indefinite as they have a high recurrence rate.