Sleep apnea in hurler syndrome: Looking beyond the upper airway

Sumera Shaikh Solaiman, Daniel Scott Rifkin, Harish Rao

Research output: Contribution to journalArticlepeer-review

1 Scopus citations


This case involves a 13-month-old male with Hurler syndrome. Due to oxygen desaturations during sleep, this patient was referred for polysomnography, which revealed severe mixed sleep apnea (apnea-hypopnea index [AHI] 72 events/h). Because sleep apnea in patients with Hurler syndrome is frequently attributed to upper airway obstruction, he was referred to otolaryngology. Prior to his evaluation by otolaryngology, he underwent ventriculoperitoneal (VP) shunt placement, which had been scheduled due to hydrocephalus on brain magnetic resonance imaging (MRI). After VP shunt placement, his oxygen desaturations during sleep resolved. Repeat polysomnogram revealed mild sleep apnea (AHI 1.9). The etiology of his sleep apnea was likely his hydrocephalus. This is the first report of a patient with Hurler syndrome with sleep apnea which markedly improved with ventriculostomy and VP shunt placement. This highlights the importance of considering neurological etiologies for sleep apnea in Hurler's patients, despite their predisposition for airway obstruction.

Original languageEnglish (US)
Pages (from-to)1423-1424
Number of pages2
JournalJournal of Clinical Sleep Medicine
Issue number10
StatePublished - 2016

All Science Journal Classification (ASJC) codes

  • Pulmonary and Respiratory Medicine
  • Neurology
  • Clinical Neurology


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