Positive Outcomes and Surgical Strategies for Bilateral Cochlear Implantation in a Child With X-Linked Deafness.
OBJECTIVE
To recognize that bilateral cochlear implantation (CI) in X-linked deafness is safe, describe techniques to maximize successful electrode placement and minimize surgical risks, and recognize that normalization of hearing as well as language acquisition is achievable.


METHODS
A 6-month-old male patient presented with bilateral profound sensorineural hearing loss and was confirmed to have X-linked deafness secondary to POU3F4 gene mutation. Due to lack of benefit from amplification, he underwent bilateral CI in a staged fashion at 12 (right) and 15 months (left) of age. A transmastoid-facial recess approach was used bilaterally utilizing perimodiolar electrodes. High-flow gushers were controlled with muscle plugs. C-arm fluoroscopy was used during insertion of electrodes.


RESULTS
Follow-up data are available for 12 months. No complications were encountered, including no postoperative cerebrospinal fluid (CSF) leakage or facial stimulation. Postoperative audiograms in aided conditions showed hearing thresholds <40 dB. At 11 months following activation of his second CI, he scored in the normal range for his chronological age on standardized language measures.


CONCLUSIONS
With careful preparation and the assistance of intraoperative fluoroscopy, CI in patients with congenital X-linked deafness can be done safely. Performing bilateral CI followed by dedicated auditory-verbal rehabilitation may allow patients to achieve normal language development.
The Ohio State University Wexner Medical Center, Columbus, Ohio, USA Nationwide Children's Hospital, Columbus, Ohio, USA leslie.kim@osumc.edu.
Leslie
L
Kim
The Ohio State University, Columbus, Ohio, USA.
C Ellis
CE
Wisely
Nationwide Children's Hospital, Columbus, Ohio, USA.
Shana
S
Lucius
Nationwide Children's Hospital, Columbus, Ohio, USA.
Jori
J
Weingarten
The Ohio State University, Columbus, Ohio, USA Nationwide Children's Hospital, Columbus, Ohio, USA.
Edward Eugene
EE
Dodson
D019929:Cochlear Implantation / Q000295:instrumentation / Q000379:methods; D003054:Cochlear Implants; D005471:Fluoroscopy / Q000379:methods; D040181:Genetic Diseases, X-Linked / Q000175:diagnosis / Q000601:surgery; D006319:Hearing Loss, Sensorineural / Q000175:diagnosis / Q000601:surgery; D006320:Hearing Tests / Q000379:methods; D006801:Humans; D007223:Infant; D007430:Intraoperative Care / Q000295:instrumentation / Q000379:methods; D008297:Male; D009154:Mutation; D050809:POU Domain Factors / Q000235:genetics*; D017755:Prosthesis Fitting / Q000379:methods; D016896:Treatment Outcome
D050809:POU Domain Factors; C091774:POU3F4 protein, human
cochlear implant; otolaryngology; otology; pediatric; pediatric ear malformations; pediatric hearing loss; pediatric management of hearing loss