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.