Özet:

Mucopolysaccharidoses are rare inherited metabolic disorders due to deficiency of specific lysosomal enzymes. Accumulation of these molecules in tissues results in severe health problems. Otolaryngological problems are usually otitis media with effusion, hearing loss and obstructive sleep apnea. Deposition of mucoplysaccharides in the eustachian tube, middle ear and postnasal space may increase the risk for otitis media. Multiple ventilation tube insertions for otitis media with effusion may require because of recurrences. Each operation has anesthetic risks due to airway problems in these patients. In this article, two cases with mucopolysaccaridosis were presented. One had bilateral secondary external auditory canal cholesteatoma due to external ear canal stenosis. Right ear of the case underwent tympanomastoidectomy and canalplasty. The other case had left chronic otitis media and treated by medical therapy. Treatment approaches and signs of these cases were discussed.

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