Hearing loss in Hunter's syndrome--mucopolysaccharidosis II.

Hunter's syndrome [mucopolysaccharidosis II (MPS II)] is a genetic, metabolic disease of excessive mucopolysaccharide storage leading to mental and skeletal abnormalities, distinctive facial features, and increased incidence of hearing loss. However, the hearing impairment in MPS II has not been well described in the literature. This paper examines the auditory aspects of MPS II by reviewing the literature and by presenting two affected brothers. Each subject had mixed hearing disorders, fluctuating between moderate and severe degrees associated with recurrent middle ear effusions. Hearing loss appears to be a frequent concomitant of MPS II and is usually of mixed nature. The conductive component may persist after myringotomy. Aggressive audiological and otological management are required to enhance communicative development.