gms | German Medical Science

The Apert syndrome (Acrocephalosyndactyly) is one of the craniosynostotic syndromes that is characterized by acrocephaly and syndactyly of hands and feet. It is often associated with deformations in the temporal bone. We discuss the case of a 13 year old girl with Apert syndrome. She suffered a chronic middle ear effusion on both sides. While performing tympanoplasty Type I we found a high jugular bulb which extended to the ear chanal. The ct-scan of the temporal bones confirmed this observation. It demonstrated a high jugular bulb on both sides, on the right sight it was only seperated by a membrane from the external ear canal.Our recherche did not reveal a higher rate of the presence of a 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 otorhinolaryngical examination in each of these patients and a ct scan of the temporal bone before any operation of the middle ear.Key words: Apert syndrome, craniofacial syndrome, high jugular bulb