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

Autor: Peter M. van Hasselt, Brigitte T.A. van den Broek, Jaap Jan Boelens, Adriana L. Smit
Rok vydání: 2020
Předmět:
Male
Pediatrics
medicine.medical_specialty
Adolescent
Hearing loss
Hearing Loss
Sensorineural
03 medical and health sciences
Bone conduction
disease progression
Genetics
medicine
Lysosomal storage disease
Evoked Potentials
Auditory
Brain Stem
Humans
mucopolysaccharidosis 1
Longitudinal Studies
hematopoietic cell transplantation
Hurler syndrome
Child
Genetics (clinical)
030304 developmental biology
0303 health sciences
medicine.diagnostic_test
business.industry
030305 genetics & heredity
Hematopoietic Stem Cell Transplantation
Infant
Auditory Threshold
Original Articles
Mucopolysaccharidoses
Cerebellopontine angle
medicine.disease
Magnetic Resonance Imaging
Transplantation
Auditory brainstem response
surgical procedures
operative
lysosomal storage disease
hearing
Child
Preschool
Audiometry
Pure-Tone
Female
Original Article
Pure tone audiometry
medicine.symptom
business
Zdroj: Journal of Inherited Metabolic Disease
ISSN: 1573-2665
Popis: 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
Databáze: OpenAIRE
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