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Tympanomastoidectomy


Question

Our five-year-old has had seven sets of ear tubes. He has a history of both ears having granulation tissue and/or polyps. He also has small ear canals and moderate hearing loss. Any suggestions we should pass on to the ENT regarding children with CdLS needing this type of surgery?

Answer of our experts

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

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Recommendation(s)

Ears and hearing

R39
Hearing should be assessed in individuals with CdLS at an early age and should be followed up over time. Those with severe sensorineural hearing loss should be assessed for auditory neuropathy.
R40
Regular eye (ophthalmologic) and ear, nose and throat (otolaryngologic) evaluations are recommended in adults with CdLS.
R41
Otitis media (middle ear infections) with fluid build up and sinusitis in individuals with CdLS should be considered and treated according to the national guidelines for the general population.

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Please take note that the Ask the Expert service is comprised of volunteer professionals in various areas of focus. Answers are not considered a medical, behavioral, or educational consultation. Ask the Expert is not a substitute for the care and attention your child’s personal physician, psychologist, educational consultant, or social worker can deliver.

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