Cochlear implantation in children with CHARGE association.
OBJECTIVE
To explore the anomalies of the temporal bone found on radiologic examination, technical challenges in cochlear implantation, and audiologic benefit derived from cochlear implantation in a series of children with CHARGE association.


METHODS
Case series report.


METHODS
Tertiary referral children's hospital pediatric cochlear implant program.


METHODS
Six children with CHARGE association and sensorineural hearing loss.


METHODS
All patients were evaluated and followed up by the cochlear implant team. Cochlear implantation was attempted in all 6 children.


METHODS
Computed tomographic scans and cochlear implantation operative records were reviewed, and their findings were correlated. Audiometric and speech perception data before and after cochlear implantation were compared.


RESULTS
Five children with CHARGE association received implants. A sixth child did not because of an aberrant course to the facial nerve. The 5 children receiving implants obtained varying degrees of measurable benefit from their implants. All 6 children had temporal bone abnormalities seen on their computed tomographic scans and documented at the time of surgery.


CONCLUSIONS
Variations in the temporal bone anatomy of patients with CHARGE association can lead to increased technical challenges and risk to the facial nerve during cochlear implantation. Individual outcomes after implantation may vary; our patients receiving implants obtained benefit. Parents should be counseled thoroughly and have appropriate expectations before proceeding with implantation.
Bauer
Paul W
PW
Washington University in St Louis, School of Medicine, USA. paul.bauer@utsouthwestern.edu
Wippold
Franz J
FJ
Goldin
Jenifer
J
Lusk
Rodney P
RP
D000015:Abnormalities, Multiple / Q000000981:diagnostic imaging / Q000601:surgery*; D001299:Audiometry; D002648:Child; D002675:Child, Preschool; D019929:Cochlear Implantation; D005240:Feasibility Studies; D005260:Female; D005500:Follow-Up Studies; D006319:Hearing Loss, Sensorineural / Q000151:congenital* / Q000000981:diagnostic imaging / Q000601:surgery*; D006801:Humans; D007223:Infant; D008297:Male; D017063:Outcome Assessment, Health Care; D013577:Syndrome; D013701:Temporal Bone / Q000002:abnormalities* / Q000000981:diagnostic imaging / Q000601:surgery*; D013997:Time Factors; D014057:Tomography, X-Ray Computed