Tympanomastoidectomy

In general, if it is possible to avoid tympanomastoidectomy in children with CdLS, that is preferable.  Other children who require repeated tubes often benefit from aeration of their mastoid and middle ears, and adults are even more likely to do so. However, mastoidectomy will not address the underlying problem with CdLS.  It has to do with the shape of the skull base and eustachian tube function.  Your otolaryngologist should be advised to think of children with CdLS with ear problems as more like children with cleft palates than like the general population.  However, children with CdLS have the added problem of narrow ear canals.  Most of the time, it is possible to control middle ear fluid through repeated tube placement, despite the small ear canals.  If a child develops granulation tissue and chronic infections that cannot be cleared up through aggressive medical therapy, then mastoidectomy and enlargement of the ear canal may be reasonable.  The procedure should be performed with extra care regarding position of the facial nerve, especially as the ear canal is being widened. 

RS/ TK 7-13-10