Objective. The aim of the present study was to review the literature on ENT disorders associated with craniosynostosis (CS), focusing on symptoms, diagnostic work-up, treatment and outcome.

Methods. Publications were retrieved by consulting the PubMed® free search engine of the US National Library of Medicine. The term “craniosynostosis” was combined with the following key-words: ENT, apneas, OSAS, sleep-disordered breathing, tonsillectomy, deafness, hearing loss.

Results. The main ENT disorders associated with CS are upper airway obstruction, chronic otitis and hearing loss. Obstructive sleep apnea-hypopnea syndrome (OSAS) is present in 7% to 67% of children suffering from CS and mainly results from midface stenosis with narrow nasal and rhinopharyngeal cavities. OSAS is diagnosed on polysomnography and airway obstruction levels are determined on wake or drug-induced sleep endoscopy and on CT or MRI. OSAS treatment can be surgical (mainly midface advancement, adenoidectomy and tonsillectomy, tracheostomy) or non-surgical (non-invasive ventilation, nasopharyngeal airway). Hearing impairment is frequently associated with CS. Its main cause is otitis media with effusion (OME) but ossicular malformations and sensorineural hearing loss (SNHL) are sometimes observed. SNHL is mostly found in Muenke syndrome. In view of the frequency and potential severity of these disorders into account, yearly ENT visits are recommended in children presenting with CS.