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Cochlear Implantation in Children With CHARGE Association
Paul W. Bauer, MD;
Franz J. Wippold II, MD;
Jenifer Goldin, MS, CCC-A;
Rodney P. Lusk, MD
Arch Otolaryngol Head Neck Surg. 2002;128:1013-1017.
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.
Design Case series report.
Setting Tertiary referral children's hospital pediatric cochlear implant program.
Patients Six children with CHARGE association and sensorineural hearing loss.
Intervention All patients were evaluated and followed up by the cochlear implant
team. Cochlear implantation was attempted in all 6 children.
Main Outcome Measures 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.
From Washington University in St Louis, School of Medicine (Drs Bauer
and Lusk); the Division of Pediatric Otolaryngology (Drs Bauer and Lusk),
Departments of Radiology (Dr Wippold) and Audiology (Cochlear Implant Program)
(Ms Goldin), St Louis Children's Hospital; and Mallinckrodt Institute of Radiology,
Washington University Medical Center (Dr Wippold), St Louis, Mo; and F. Edward
Hébert School of Medicine, Uniformed Services University of the Health
Sciences, Bethesda, Md (Dr Wippold). Dr Bauer is now with the Division of
Pediatric Otolaryngology, Department of Otolaryngology–Head and Neck
Surgery, The University of Texas Southwestern Medical Center, Dallas.
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