Positive Outcomes and Surgical Strategies for Bilateral Cochlear Implantation in a Child With X-Linked Deafness

Objectives: 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. Conclusion: 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.