A six-month-old baby with congenital patent ductus arteriosus (PDA), bilateral microtia and canal atresia was referred for hearing assessment. The audiology assessment revealed bilateral profound hearing loss, which is atypical for a case of pure canal atresia. Imaging was performed much earlier than usual and, as suspected, the patient also had bilateral severe inner ear anomaly. It is extremely rare for a person to have both external and inner ear anomaly because of the different embryological origin. The only suitable hearing rehabilitation option for this kind of patients is brainstem implant. However, the parents had opted for sign language as a form of communication.
The purpose of this study was to review the results of our patients with congenital canal atresia after implantation of bone-anchored hearing aids (BAHA). The occurrence of complications was also reviewed.
To review cases of congenital external auditory canal anomaly with cholesteatoma, documenting clinical presentation, cholesteatoma site and extent, complications, and surgery.