Otologic and laryngologic manifestations of mucopolysaccharidoses after bone marrow transplantation.
BACKGROUND
Children with mucopolysaccharidosis have significant functional impairment of a number of systems because of deposition of glycosaminoglycans. The otolaryngologic system is affected, resulting in conductive and sensorineural hearing loss and voice abnormality.


METHODS
Eleven children who had undergone bone marrow transplantation had their hearing and voices assessed. Unmatched data from patients who had not undergone transplantation were collected for nonstatistical comparison.


RESULTS
The hearing loss in children who had undergone bone marrow transplantation was variable but on average was less severe than in the younger children who had not undergone transplantation. The incidence of otitis media with effusion was diminished in the transplanted group. Approximately half had normal voices.


CONCLUSIONS
Diminution of the progression of hearing loss and voice abnormality probably occurs in patients after treatment by bone marrow transplantation.
Papsin
B C
BC
Department of Otolaryngology, Great Ormond Street Hospital for Children, Toronto, Ontario, Canada.
Vellodi
A
A
Bailey
C M
CM
Ratcliffe
P C
PC
Leighton
S E
SE
D016026:Bone Marrow Transplantation; D002648:Child; D002675:Child, Preschool; D005260:Female; D006311:Hearing Disorders / Q000209:etiology*; D006801:Humans; D007223:Infant; D008297:Male; D009083:Mucopolysaccharidoses / Q000150:complications* / Q000628:therapy; D010034:Otitis Media with Effusion / Q000209:etiology; D014832:Voice Disorders / Q000209:etiology*