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Cochlear implantation in children with large vestibular aqueduct syndrome
Insights
Cochlear implants significantly improve hearing in children with large vestibular aqueduct syndrome (LVAS). This study shows substantial gains in auditory performance, enabling better educational outcomes for these children.
Area of Science:
- Otolaryngology
- Pediatric Audiology
- Medical Devices
Background:
- Large vestibular aqueduct syndrome (LVAS) is a common cause of congenital hearing loss in children.
- Profound hearing loss associated with LVAS can significantly impact a child's development and education.
- Cochlear implantation is a potential intervention for hearing restoration in such cases.
Purpose of the Study:
- To evaluate the effectiveness of multielectrode cochlear implant prostheses in children diagnosed with LVAS.
- To assess the impact of cochlear implantation on auditory performance and educational outcomes in this pediatric population.
Main Methods:
- Retrospective study of ten children with profound hearing loss and radiologic evidence of LVAS.
- Children received multielectrode cochlear implants, followed by device programming and auditory habilitation.
- Postoperative audiologic and school performance were assessed at regular intervals.
Main Results:
- All children demonstrated improved postoperative auditory performance.
- Significant increases in BKB, word, and phoneme scores were observed within six months.
- Older children experienced reduced reliance on support staff, facilitating continued education in mainstream settings.
Conclusions:
- Multielectrode cochlear implants are effective in providing hearing to children with deafness due to LVAS.
- Cochlear implantation offers considerable benefits for children experiencing deteriorating hearing loss from LVAS.
- This intervention supports improved communication and educational integration for affected children.
Objective:
This study describes the effectiveness of a multielectrode cochlear implant prosthesis (Cochlear; Cochlear Pty., Lane Cove, Australia) for providing hearing to children with deafness caused by large vestibular aqueduct syndrome (LVAS).
Study Design:
The study design was a retrospective study.
Setting:
All the children attended The Children's Cochlear Implant Center (NSW), which is a specialist center that provides audiologic testing, speech therapy, habilitation, and medical assistance for children with cochlear implants.
Patients:
Ten children were studied who had profound hearing loss and radiologic evidence of a vestibular aqueduct larger than 2 mm in width in its intraosseous portion.
Intervention:
The children received a multielectrode (Cochlear) cochlear implant prosthesis, and the associated programming of the device and habitation were performed postoperatively. No significant problems were encountered at any of the surgeries, although there was an initial gush of perilymph when the otic capsule was opened in 7 ears.
Main Outcome Measures:
Postoperative audiologic performance at six monthly intervals and school performance were assessed.
Results:
The postoperative auditory performance was improved in all children. At 6 months, their average BKB score had increased from 31% to 79%; average word score, from 8% to 43%; and average phoneme score, from 38% to 70%. The older children were able to continue their education in their usual setting with less reliance on hearing support staff.
Conclusion:
Children with a deteriorating hearing loss caused by LVAS can derive considerable benefit from a cochlear implant.