| Article ID | Journal | Published Year | Pages | File Type |
|---|---|---|---|---|
| 8343044 | Molecular Genetics and Metabolism | 2018 | 7 Pages |
Abstract
The strong correlation between reduced height and hearing loss indicates that patients with severe skeletal dysplasia may be at higher risk of developing more severe hearing loss. More importantly, the spectrum of hearing disorders indicates that MPS IV patients should have annual neurophysiological hearing tests in addition to audiometric testing from an early age regardless of their skeletal severity to more carefully monitor disease progression.
Keywords
GAGMEMRC6SN-acetylgalactosamine-6-sulfate sulfataseADLPTADPOAEABRenzyme replacement therapyElectrophysiologyMR imagesMagnetic resonance imagescomputed tomographyCNSGALNSKeratan sulfateAuditory systemcentral nervous systemActivity of daily livingpure tone averageMucopolysaccharidosisMPsERTauditory brainstem responseChondroitin-6-sulfateGlycosaminoglycan
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Authors
Kyoko Nagao, Thierry Morlet, Elizabeth Haley, Jennifer Padilla, Julianne Nemith, Robert W. Mason, Shunji Tomatsu,