Abstract
Objectives:
(1) Recognize that bilateral cochlear implantation (CI) in X-linked deafness is surgically feasible and safe. (2) Recognize that improvement in hearing as well as language acquisition is an achievable goal.
Methods:
This is a retrospective case review at a tertiary care center of one patient who presented with bilateral profound sensorineural hearing loss at age 6 months. He lacked auditory brainstem responses in both ears and was confirmed to have congenital X-linked deafness secondary to POU3F4 gene mutation. Because of lack of benefit from amplification, he underwent bilateral CI in a staged fashion at 12 (right ear) and 15 months (left ear) of age.
Results:
The patient underwent transmastoid-facial recess approach and received Nucleus Freedom implants (Cochlear Ltd) in both ears, utilizing perimodiolar electrodes. High-flow gushers were encountered bilaterally and resolved with temporalis muscle plugs. C-arm fluoroscopy was used during insertion of electrodes in both ears. Follow-up data were available for 12 months. No complications were encountered, including no postoperative 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, the Receptive Expressive Emergent Language Test-3 and the Preschool Language Scale-5.
Conclusions:
With careful preparation and the assistance of intraoperative fluoroscopy, cochlear implantation in patients with congenital X-linked deafness can be done safely. Performing bilateral CI followed by dedicated auditory-verbal rehabilitation may allow patients to trend toward normal language development.
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