Audiologic Performance After Cochlear Implantation in Children With X-Linked Deafness: Comparison With Deaf Children With a Normal Inner Ear Structure

Objective: To compare audiologic performances of cochlear implantation (CI) in children with X-linked deafness with those of CI in age-and sex-matched children with normal cochleae. Patients: We identified 4 patients with X-linked deafness and selected 10 age-and sex-matched deaf patients with normal cochleae between April 1999 and April 2012. Main Outcome Measures: Auditory brainstem responses, Categories of Auditory Performance (CAP), and Meaningful Auditory Integration Scale (MAIS). Results: The ages of patients with X-linked deafness at the time of implantation ranged between 1.3 and 13.6 years, with a mean age of 5.3 years. All patients were male subjects and showed severe-to-profound hearing loss. Patient 1 lacked ABR in both ears. Patients 2, 3, and 4 showed ABR in the contralateral ear, at 70, 70, and 90 dB nHL, respectively. All 4 patients had bilateral X-linked cochlear anomaly and experienced cerebrospinal fluid (CSF) gushers. Straight electrodes were used in Patients 1 and 3, with perimodiolar electrodes used in the others. Patients 2 and 3, who were about the same age, had CAP scores of 6 or more at 18 months after activation of the implant despite the use of different electrode arrays. There were no statistically significant differences in MAIS and CAP scores between each patient with X-linked deafness and the control group. Conclusion: The audiologic performances of patient with Xlinked deafness after CI are comparable to those of patients with normal inner ear structure after CI.