The Hurler and Hunter syndromes are two forms of mucopolysaccharidosis. Although the diseases are rare, those afflicted commonly require otolaryngologic consultation. Upper airway obstruction is often severe, progressive, and not infrequently the suspected cause of death in these patients. Four patients with these problems are presented. In all of the children, obstructive sleep apnea was a major management problem. This and other upper airway difficulties are detailed with clinical and pathological correlates.

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