Treatment outcomes of cochlear implantation in pediatric patients with Cockayne syndrome type I: a case series
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Published on: January 9, 2019
Mariana Kostova1, Anirvan Banerjee1
1Department of Otorhinolaryngology, The James Cook University Hospital, Middlesbrough, TS4 3BW, United Kingdom.
Insights
Cochlear implantation benefits pediatric patients with Cockayne syndrome (CS) and hearing loss. Even with developmental delays, the surgery improved quality of life and school performance in two young patients.
Area of Science:
- Otolaryngology
- Genetics
- Pediatrics
Background:
- Cockayne syndrome (CS) is a rare genetic disorder associated with progressive sensorineural hearing loss.
- Cochlear implantation is a potential intervention for hearing loss in pediatric populations.
Observation:
- This retrospective case series examined two pediatric patients with CS type I and bilateral sensorineural hearing loss who underwent cochlear implantation.
- Outcomes were assessed using Visual Reinforcement Audiometry (VRA) and evaluation of cochlear implant tolerance.
Findings:
- One patient showed significant improvements in VRA results and academic performance after bilateral cochlear implantation.
- The second patient experienced enhanced quality of life and environmental awareness following unilateral implantation, despite challenges in obtaining objective VRA data due to developmental delay.
Implications:
- Cochlear implantation can be a beneficial treatment for pediatric patients with Cockayne syndrome and hearing loss.
- The study suggests that patients with CS who are post-lingual or have better cognitive function may particularly benefit from cochlear implants.
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.
