Microtia is often accompanied by congenital aural atresia (CAA). A cholesteatoma can occur with CAA without the atretic plate; thus, if CAA with otalgia or ear cellulitis is present, an infection related to a cholesteatoma should be considered. We report herein two cases of unilateral CAA with a cholesteatoma requiring external auditory canal reconstruction. Case 1, a 10-year-old, male patient with Grade 2 right microtia, presented right CAA without the atretic plate (Jahrsdoerfer scale 8) on computed tomography (CT) at age 3 years. Hearing in the right ear was 80 dB on an auditory brainstem response (ABR) test while the unaffected side was normal. The patient did not require a hearing aid and was followed up yearly. He later presented otalgia and bloody otorrhea, and CT revealed an extensive abscess in the right external auditory canal. After cholesteatoma debridement and external auditory canal reconstruction, his hearing improved. The external ear canal is currently open without lateral healing. Case 2, a 3-year-old, female patient with Grade 3 left microtia, presented left CAA without the atretic plate (Jahrsdoerfer scale 7) on CT at age 1 year. Left ear hearing was 50 dB on an ABR test. She presented with otalgia and bloody otorrhea, and CT revealed an extensive abscess in the left external auditory canal. After cholesteatoma debridement and external auditory canal reconstruction, her hearing improved. As in case one, the external ear canal is open without lateral healing. These cases demonstrate that a cholesteatoma may co-occur with CAA without the atretic plate. Although auricular reconstruction is commonly performed at age 10 years when sufficient costal cartilage is available for the procedure, the present cases suggest that screening for a cholesteatoma and surgery may be considered in younger patients.
View full abstract