Cochlear implantation in children with cochlear aplasia.
CONCLUSIONS
Children with cochlear aplasia can achieve favorable speech perception abilities from cochlear implantation (CI) by inserting an electrode array into the vestibule if an audiological response is reliably demonstrated before surgery by behavioral audiometry and objective measures.


OBJECTIVE
The purpose of this study was to evaluate the surgical outcome of CI in children with cochlear aplasia.


METHODS
The medical records of 454 children who underwent CI at the Dong-A University Hospital between January 1994 and September 2011 were reviewed. The prevalence of cochlear aplasia and the preoperative evaluations, operative findings, and postoperative speech perception abilities of implanted children with cochlear aplasia were evaluated.


RESULTS
The implanted children with cochlear aplasia accounted for 2% of those with inner ear malformations and 0.4% of all pediatric cochlear implantees. They showed audiological responses in preoperative electrophysiologic tests, and the eighth nerve was present in magnetic resonance imaging. The electrode array was inserted into the vestibule through an oval window after removing the stapes or through a transmastoid labyrinthotomy site. Electrical compound action potential and electrical auditory brainstem response tests showed clear neural responses after implantation. The hearing thresholds of the implanted ear improved to 25 dB HL and the children achieved significant open set speech perception abilities.
Department of Otolaryngology-Head and Neck Surgery, College of Medicine, Dong-A University, Busan, Korea.
Sung-Wook
SW
Jeong
Lee-Suk
LS
Kim
D000200:Action Potentials / Q000502:physiology; D001299:Audiometry / Q000379:methods; D001309:Auditory Threshold / Q000502:physiology; D001933:Brain Stem / Q000473:pathology / Q000503:physiopathology; D002675:Child, Preschool; D003051:Cochlea / Q000002:abnormalities* / Q000473:pathology / Q000503:physiopathology; D019929:Cochlear Implantation / Q000379:methods*; D003056:Cochlear Nerve / Q000002:abnormalities / Q000503:physiopathology; D004567:Electrodes, Implanted; D016057:Evoked Potentials, Auditory, Brain Stem / Q000502:physiology; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007223:Infant; D008279:Magnetic Resonance Imaging; D008297:Male; D017084:Otoacoustic Emissions, Spontaneous / Q000502:physiology; D013067:Speech Perception / Q000502:physiology*; D014057:Tomography, X-Ray Computed; D014722:Vestibule, Labyrinth / Q000473:pathology / Q000503:physiopathology