[Otologic findings in the Apert syndrome].
The Apert syndrome (Acrocephalosyndactyly) is one of the craniosynostotic syndromes, which is often associated with congenial malformation in the temporal bone. We present a case of a 13 year old girl with Apert syndrome. By planning a tympanoplasty we arranged a CT-scan of the temporal bones. It demonstrated next to some other malformations a high jugular bulb on both sides, on the right side just with a membraneous bound to the external ear canal. In a recherche we did not find a higher incidence of high jugular bulb in Apert-syndrome and other craniosynostotic syndromes. However as we expect different malformations of the temporal bone in Apert syndrome we recommend an otorhinolaryngeal examination in each of these patients and a ct scan of the temporal bone before any operation of the middle ear.
Lücke
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Klinik für Hals-Nasen-Ohrenheilkunde, Charité, Campus Wirchow-Klinikum. Augustenburger Platz 1 13353 Berlin. dorothee.luecke@charite.de
Stroszczynski
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C
Gartenschläger
S
S
Olze
H
H
D000168:Acrocephalosyndactylia / Q000150:complications / Q000175:diagnosis / Q000000981:diagnostic imaging; D000293:Adolescent; D006311:Hearing Disorders / Q000209:etiology*; D006801:Humans; D007601:Jugular Veins / Q000002:abnormalities* / Q000000981:diagnostic imaging; D008297:Male; D010033:Otitis Media / Q000209:etiology*; D013701:Temporal Bone / Q000002:abnormalities* / Q000000981:diagnostic imaging; D014057:Tomography, X-Ray Computed; D014432:Tympanic Membrane; D014433:Tympanoplasty