Otologic and laryngologic manifestations of mucopolysaccharidoses after bone marrow transplantation.

Abstract

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. 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. 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. Diminution of the progression of hearing loss and voice abnormality probably occurs in patients after treatment by bone marrow transplantation.