Title - Case of Unilateral Congenital Aural Atresia & Microtia With Cholesteatoma - Title - Case of Unilateral Congenital Aural Atresia & Microtia With Cholesteatoma - Open Access Pub

We report a case of cholesteatoma associated with unilateral congenital aural atresia (CAA) and microtia, resulting in extracranial complication. A 13-year-old girl presented with right ear purulent discharge from pre and post auricular area with mastoid tenderness. On clinical examination, she had right grade II microtia with pre-auricular skin tag and healed scar tissue in periauricular region. On High Resolution Computerized Tomography (HRCT) scan of temporal bones, there was CAA of right ear with extensive cholesteatoma in middle ear cleft. The cholesteatoma was subsequently removed by radical mastoidectomy with uneventful post-operative period. Cholesteatoma associated with CAA may remain undiagnosed in early childhood resulting in life threatening temporal and intracranial complications. DOI10.14302/issn.2379-8572.joa-16-1368 Congenital aural atresia (CAA) refers to a spectrum of ear deformities present at birth that involves some degree of failure of the development of the external auditory canal (EAC). The incidence of congenital aural atresia is 1 in 10,000 to 1 in 20,000 live births1. Microtia has an incidence of 1 in 7000 to 8000 births in the general population 2, 3. Males are affected more commonly, and the reported ratio of right-to-left-to-bilateral is approximately 5:3:1 with bilateral deformity occurring in only 10% of patients4, 5, 6, 7. A 13 year old girl presented in ENT outpatient department with complains of recurrent right ear purulent discharge from pre and post auricular area for last 5 years. She had a history of incision and drainage in right post auricular area six months prior for similar complain. On local examination, there was right Grade II8microtia with atretic External Auditory Canal (EAC) and pre-auricular skin tag (Figure 1). A small fistulous opening was also seen in right cavum conchae region. Left ear examination was normal with intact tympanic membrane. Tuning fork tests using 256 and 512 Hz tuning forks showed moderate to severe conductive hearing loss in right ear with Rinne’s test positive in left ear. HRCT scan of temporal bones showed membranous atresia of right EAC with grade II microtia. Soft tissue density was seen filling the EAC and the entire middle ear cavity (Figure 2A). A CT based fistulogram done four months before our consultation identified multiple fistulous tracts situated in post auricular region and anterior to stylomastoid foramen in inframastoid region. These fistulous tracts were communicating through middle ear cavity (Figure 2B). There was associated erosion of posteroinferior and