Abstract
Objective: 1) Describe important points of consideration when planning cochlear implant surgery for patients at risk for cerebrospinal fluid (CSF) gusher on cochleostomy. 2) Report successful cochlear implantation in patients with X-linked mixed deafness.
Method: Fraternal twins with X-linked mixed deafness were enrolled in an observational study at a tertiary academic medical center from 2010 to 2011. Subjects underwent cochlear implantation and were followed during recovery and auditory rehabilitation. Main outcome measures included successful placement of electrode arrays into the cochlea, adequate control of leaking CSF, incidence of postoperative complications, and development of listening and speaking communication skills in children with implants.
Results: Electrode arrays were successfully placed despite challenging middle and inner ear abnormalities. Leaking CSF was controlled without the use of lumbar drains. No perioperative or postoperative complications occurred. Both patients progressed rapidly in the development of hearing, listening, and speaking skills after successful cochlear implantation. A new surgical instrument was designed and used to aid in electrode insertion into gushing CSF.
Conclusion: Cochlear implantation in patients with X-linked mixed deafness requires careful planning and execution, but may result in excellent communicative outcomes.
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