Hearing restoration with auditory brainstem implant in three children with cochlear nerve aplasia.
OBJECTIVE
To verify the possibility of auditory habilitation in children with aplasia and hypoplasia of the cochlear nerve by direct electrical stimulation of the cochlear nuclei with an auditory brainstem implant.


METHODS
Retrospective case review.


METHODS
Study conducted at the Ear, Nose, and Throat Department of the University of Verona, Italy.


METHODS
Three children, aged 4, 3, and 2 years, respectively, with severe bilateral cochlear malformations and cochlear nerve aplasia have received an auditory brainstem implant at this institution in the past 2 years.


METHODS
The classic retrosigmoid approach was used. Correct positioning of the electrodes was evaluated using electric auditory brainstem responses and neural response telemetry. Before the patients were discharged, high-resolution computed tomography with a bone algorithm reconstruction technique was performed to evaluate electrode placement. The auditory brainstem implant was activated 30 to 60 days after implantation.


RESULTS
No postoperative complications were observed. To date, 21, 18, and 8 electrodes, respectively, have been activated in the three children. The first patient, 12 months after activation, had achieved good environmental sound awareness, good speech detection, and some speech recognition. The second child, 8 months after activation, had achieved good environmental sound awareness and moderate speech detection. The third patient, 1 month after activation, had obtained good environmental sound awareness.


CONCLUSIONS
This study indicates that auditory brainstem implantation is technically feasible in children with cochlear nerve aplasia. The early results suggest the possibility of achieving auditory habilitation with auditory brainstem implantation in this population.
Colletti
Vittorio
V
Ear, Nose, and Throat Department, University of Verona, Italy. entdept@libero.it
Carner
Marco
M
Fiorino
Francesco
F
Sacchetto
Luca
L
Miorelli
Veronica
V
Orsi
Alessandro
A
Cilurzo
Francesco
F
Pacini
Lorenzo
L
D000465:Algorithms; D038262:Auditory Brain Stem Implants; D001861:Bone Regeneration / Q000502:physiology; D002675:Child, Preschool; D003056:Cochlear Nerve / Q000002:abnormalities* / Q000000981:diagnostic imaging / Q000601:surgery*; D003638:Deafness / Q000601:surgery*; D005240:Feasibility Studies; D005260:Female; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D013506:Otologic Surgical Procedures / Q000379:methods; D019736:Prostheses and Implants; D012189:Retrospective Studies; D014057:Tomography, X-Ray Computed