Primary external auditory canal cholesteatoma in a young adult: Diagnostic challenges and surgical management of a rare otologic entity

Abstract

External auditory canal cholesteatoma is a rare and potentially destructive otologic condition characterized by the accumulation of keratinizing squamous epithelium within the external auditory canal, leading to progressive bony erosion. Despite being described in the 19th century, EACC remains an uncommon and frequently underrecognized entity, often misdiagnosed as keratosis obturans or chronic otitis externa. Its pathogenesis is multifactorial and incompletely understood, involving impaired epithelial migration, chronic microtrauma, local inflammation, and ischemic changes of the canal skin. Clinical presentation is usually nonspecific and includes otalgia, otorrhea, aural fullness, and conductive hearing loss, contributing to diagnostic delay. Otoscopic examination typically reveals keratin debris associated with focal bone exposure. High-resolution computed tomography of the temporal bone plays a key role in evaluating bony erosion and disease extent; however, it lacks specificity for definitive tissue characterization, and magnetic resonance imaging may be useful in selected cases for differential diagnosis. We report the case of a 25-year-old patient with no relevant medical history who presented with persistent unilateral otorrhea of 3 months' duration, associated with severe otalgia and progressive hearing loss. A comprehensive clinical and radiological assessment suggested the diagnosis of primary external auditory canal cholesteatoma, which was subsequently confirmed by histopathological examination. The patient underwent surgical management with favorable clinical and functional outcomes. This case highlights the importance of a multidisciplinary diagnostic approach and early stage-adapted management to prevent complications and reduce the risk of recurrence.