Journal article
Cochlear implantation in a patient with mucopolysaccharidosis IVA
SAGE open medical case reports, v 7, p2050313X19873791
2019
PMID: 31516706
Featured in Collection : UN Sustainable Development Goals @ Drexel
Abstract
Mucopolysaccharidosis IVA (OMIM 253000; also known as Morquio A syndrome) is associated with skeletal, airway, and hearing abnormalities. Cochlear implantation is an effective intervention for patients with severe-to-profound hearing loss. Patients can gain substantial improvement in auditory performance, speech perception, and their quality of life from cochlear implantation. Although severe progressive sensorineural hearing loss is a common feature of mucopolysaccharidosis IVA, no detailed description of cochlear implantation for mucopolysaccharidosis IVA has been reported. To review the effectiveness and special considerations associated with cochlear implantation in patients with mucopolysaccharidosis IVA, we here report the case of cochlear implantation in mucopolysaccharidosis IVA by a multidisciplinary team. A retrospective chart review was conducted on a 34-year-old female with mucopolysaccharidosis IVA, who received a cochlear implant. Audiometric thresholds, speech perception scores, and cochlear implant processor mapping information were reviewed during the first 12 months following cochlear implantation. The results of audiological tests indicate improved hearing thresholds as well as remarkable enhancement of speech perception skills over 12 months of cochlear implant use. Cochlear implantation improved auditory performance in a mucopolysaccharidosis IVA patient with postlingually severe-to-profound sensorineural hearing loss. The benefits of cochlear implantation could be meaningful for other Morquio patients with progressive hearing loss, although the risks of surgery and anesthesia should be carefully considered by a multidisciplinary team of experts during the cochlear implant candidacy process.
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Details
- Title
- Cochlear implantation in a patient with mucopolysaccharidosis IVA
- Creators
- Kyoko Nagao - Alfred I. duPont Hospital for ChildrenCassidy Walter - University of DelawareWilliam J Parkes - Thomas Jefferson UniversityMichael Teixido - Christiana Care Health SystemMary C Theroux - Alfred I. duPont Hospital for ChildrenStacy Szymkowski - Alfred I. duPont Hospital for ChildrenThierry Morlet - Salus UniversityShunji Tomatsu - Alfred I. duPont Hospital for Children
- Publication Details
- SAGE open medical case reports, v 7, p2050313X19873791
- Publisher
- SAGE Publications
- Grant note
- P30GM114736 / ;
- Resource Type
- Journal article
- Language
- English
- Academic Unit
- Audiology - Distance
- Web of Science ID
- WOS:000483535100001
- Other Identifier
- 991022169838704721
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- Collaboration types
- Domestic collaboration
- Web of Science research areas
- Medicine, General & Internal