DOI: https://doi.org/10.36347/sjmcr.2026.v14i07.017

Pages: 1680-1682

Introduction: Middle ear cholesteatoma is a locally destructive disease that may lead to serious complications, including facial nerve palsy. Its association with congenital inner ear malformations such as Michel aplasia is extremely rare. Case Presentation: We report the case of a 50-year-old patient presenting with chronic right-sided otorrhea complicated by a peripheral facial nerve palsy graded II according to the House–Brackmann classification. Temporal bone CT scan confirmed an extensive middle ear cholesteatoma with facial canal erosion and revealed an ipsilateral Michel aplasia. Management and Outcome: The patient underwent right canal wall down mastoidectomy with complete removal of the cholesteatoma. The facial nerve was carefully identified and preserved. Postoperative evolution was uneventful, with stable facial nerve function and good clinical recovery. Discussion: This rare association presents both diagnostic and surgical challenges due to the absence of inner ear anatomical landmarks and the potential for aberrant facial nerve course. Conclusion: Careful preoperative radiological assessment and meticulous surgical dissection are essential to achieve disease control while minimizing the risk of facial nerve injury.