When cholesteatoma breaches the dural barrier: A case report of congenital cholesteatoma presenting with seizure and facial paralysis

OpenAlex İngilizce

Congenital cholesteatoma is a rare pathology originating from epithelial cells that may develop in the middle ear, temporal bone, or intracranially. Initially asymptomatic, it can progress to cause hearing loss, vertigo, cerebrospinal fluid leakage, cranial neuropathies, and central nervous system infections. This report describes a unique case of an 8-year-old female who initially presented with severe left-sided otalgia, subsequently developing a generalized tonic-clonic seizure and left facial paralysis. The seizure event prompted urgent diagnostic workup, which revealed pachymeningitis secondary to a congenital cholesteatoma breaching the dural barrier. Surgical intervention was successfully performed on the left ear to excise the cholesteatoma and reconstruct the ossicular chain. Postoperatively, the facial paralysis gradually resolved, and no further seizures occurred during multidisciplinary follow-up. To the best of our knowledge, this is the first documented case of pediatric congenital cholesteatoma presenting with otalgia that progressed to seizures and pachymeningitis. Early diagnosis and prompt surgical intervention are imperative to prevent permanent sequelae and serious intracranial complications associated with this pathology.