Bilateral microtia, canal atresia and aplasia of cochleovestibular nerve

• Published in: Medical journal of Malaysia Vol. 72; no. 2; pp. 135 - 137
• Main Authors: Asma, A, Roslenda, A R, Fadzilah, I, Mazita, A, Marina, M B, Ab Aziz, A
• Format: Journal Article
• Language: English
• Published: Malaysia 01-04-2017
• Subjects: Congenital Microtia - complications; Ductus Arteriosus, Patent - complications; Ear Canal - abnormalities; Ear Canal - diagnostic imaging; Female; Hearing Loss, Sensorineural - congenital; Humans; Infant; Tomography, X-Ray Computed; Vestibulocochlear Nerve - abnormalities; Vestibulocochlear Nerve - diagnostic imaging
• ISSN: 0300-5283

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.