Otitis media with effusion (OME) is a constant finding in children with muc
opolysaccharidoses (MPS). Affected children may also present the anaestheti
st with a difficult airway. A 7-year retrospective review of the management
of OME in individuals with MPS was carried out. Nine patients were identif
ied. All had a number of short-term ventilation tube insertions (one to fou
r, mean two) before a diagnosis of MPS was made. Following diagnosis three
required repeated short-term ventilation tubes insertions (two to four, mea
n three), four had long-term ventilation tube insertions once only. Five ch
ildren who had residual hearing loss were provided with hearing aids but co
mpliance 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. (C) 2000 Elsevier Science Ireland Ltd.
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