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  Patronage of the Polish Society of Audiology and Phoniatrics

vol 10. no 2. June 2011  

 Original articles
Cholesteatoma in a child with Prader-Willi syndrome – A case report
Rafał Zieliński, Anna Zakrzewska

Prader-Willi syndrome is a rare genetically related condition
presenting hypotony, obesity, short stature, deformations of
extremities, mental and motor retardation, hypogonadism and
cranial malformations with almond shaped eyes. Chronic otitis
media in children with this syndrome has not been reported in
the accessible literature. The behaviour typical for the Prader
Willi syndrome children and their lower pain treshold may
make it difficult to diagnose acute otitis media. Microtoscopy,
which is necessary for decision of surgery in the cases of chronic
otitis media is also very difficult in the children.Authors present
a case of a child with Prader-Willi syndrome and middle ear
cholesteatoma. They also propose a diagnostic and therapeutic
procedure for use in Prader-Willi children with suspected
cholesteatoma chronic otitis.

keywords: perlak, zespół Pradera-Willego, cholesteatoma, Prader-Willi syndrome

pages: from 99 to 102

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