Congenital aural stenosis with first branchial cleft fistula, presenting with recurrent facial nerve palsy, in an elderly patient

Abstract

Both duplication anomalies and external auditory canal stenosis can result in cholesteatoma due to retained epithelium. Despite their common origins, the coexistence of these anatomical abnormalities is quite unusual. This cholesteatoma may go unnoticed till adulthood and present with complications. Moreover, first branchial cleft malformations are often unrecognized or misdiagnosed for other inflammatory lesions in the periauricular region. We report a case of middle-ear cholesteatoma in a 68 year old male patient with grade II microtia with canal stenosis and first branchial cleft fistula in supra-auricular region presenting with recurrent infranuclear facial nerve palsy. The patient was managed surgically by excision of fistulous tract, canalplasty, auricular reconstruction and middle ear and mastoid exploration for cholesteatoma. This may probably be the oldest person to be managed surgically for cholesteatoma due to congenital ear anomalies and first such reported case.