Otitis media with effusion (OME) is a constant finding in children with mucopolysaccharidoses (MPS). Affected children may also present the anaesthetist with a difficult airway. A 7-year retrospective review of the management of OME in individuals with MPS was carried out. Nine patients were identified. All had a number of short-term ventilation tube insertions (one to four, mean two) before a diagnosis of MPS was made. Following diagnosis three required repeated short-term ventilation tubes insertions (two to four, mean three), four had long-term ventilation tube insertions once only. Five children who had residual hearing loss were provided with hearing aids but compliance was poor in two. Once a diagnosis of MPS has been made, a hearing aid, if compliant, or a long-term ventilation tube would be a better option than a short term one in order to minimise the anaesthetic risk. A 'watch and wait' policy is not recommended.
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