Treatment outcomes of cochlear implantation in pediatric patients with Cockayne syndrome type I: a case series
Abstract
Abstract This retrospective case series evaluates treatment outcomes post-cochlear implantation in pediatric patients diagnosed with Cockayne syndrome (CS) and bilateral sensorineural hearing loss. Two female pediatric patients with CS type I underwent either bilateral or unilateral cochlear implantation. Visual reinforcement audiometry (VRA) and postoperative cochlear implant tolerance were the main outcome measures. Patient 1 demonstrated notable improvements in VRA results and school performance following bilateral implantation. Patient 2 experienced enhanced quality of life and environmental awareness post-unilateral implantation, despite a lack of objective VRA results due to developmental delay. The study underscores the benefits of cochlear implantation in CS patients, especially in patients who are post-lingual or with better cognitive function.
How strong is the evidence?
Evidence Hub reads this as a case series, done in people. Its evidence level is 5 of 5 (Very low · case / narrative / animal / in vitro / in silico). Case reports, narrative reviews, and animal, cell or computer-model studies give the weakest evidence for human health.
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