Medical Information - Muenke Syndrome
MUENKE syndrome is usually initially diagnosed because of craniosynostosis affecting the coronal sutures. This may affect only one side (unicoronal synostosis) or both sides (bicoronal synostosis). Apart from the resulting distortion in skull shape, the appearance is otherwise essentially normal. There are no other physical features that are characteristic of the diagnosis (as in most other craniofacial syndromes), although the fingers may sometimes be short, slightly crooked or webbed, but this does not affect their function. Muenke syndrome can only be confirmed by genetic testing.
It has been estimated that about 5% of people with unicoronal synostosis and up to 30% of people with bicoronal synostosis have Muenke syndrome. It is unlikely that anyone where the coronal sutures are not affected has Muenke syndrome, although it is possible to have Muenke syndrome without having craniosynostosis at all - some have a large head size and others have essentially normal skulls.
Muenke syndrome is generally more severe in girls than in boys and may require more than a single operation in particular individuals. There may also be associated hearing loss and learning difficulties in 10%-30% of cases and it is important for affected individuals to have hearing tests to check on the possibility of a problem.For further information: