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Hearing loss in patients with mucopolysaccharidoses-1 and -6 after hematopoietic cell transplantation: A longitudinal analysis

Authors :
Peter M. van Hasselt
Brigitte T.A. van den Broek
Jaap Jan Boelens
Adriana L. Smit
Source :
Journal of Inherited Metabolic Disease
Publication Year :
2020

Abstract

Hearing loss is frequently seen in mucopolysaccharidoses (MPS) patients. Although hematopoietic cell transplantation (HCT) increases overall survival, disease progression is observed in certain tissues. This study describes the course of hearing loss (HL) over time in transplanted MPS patients. Transplanted MPS patients between 2003 and 2018 were included and received yearly audiological evaluation, including auditory brainstem response (ABR) or pure tone audiometry (PTA). Twenty‐eight MPS‐1 and four MPS‐6 patients were analyzed with a median follow‐up of 5 years (range 11 months–16 years). Air conduction threshold improved significantly over time (P

Details

ISSN :
15732665
Volume :
43
Issue :
6
Database :
OpenAIRE
Journal :
Journal of inherited metabolic disease
Accession number :
edsair.doi.dedup.....7e63c8821cae1b95aa0590dc145644e8