[Hyperostosis cranialis interna; a new syndrome with autosomal dominant inheritance]. / Hyperostosis cranialis interna; een nieuw syndroom met autosomaal dominante overerving.

A family is described which currently comprises nine individuals, spanning three generations, who are affected with a bone disorder which is confined to the skull and is accompanied by impaired function of the cranial nerves. Radiological examination showed intracranial hyperostosis and osteosclerosis of the calvaria and the base of the skull, without involvement of the mandible or other skeletal bones. Invariably, the main presenting symptom was recurrent facial nerve paralysis from late childhood onwards, but concurrent and variable involvement of the olfactory, optic, vestibular and acoustic nerves was seen; this could be attributed to nerve compression by the bony encroachment into the cranial foramina. Morphological investigations revealed increased formation of bone tissue with a normal structure. The pedigree suggests an autosomal dominant mode of heredity. A review of the literature did not disclose any previous reports on this disorder.

PACS in practice: on-line communications in daily routine.

After a preparatory phase in which a software link was realized, on-line communications between a diagnostic reporting console and a computed tomography (CT) apparatus were set-up. Two specific groups of CT investigations were selected to be diagnosed in a 'digital' as well as a 'conventional' manner. The 'digital' diagnosis implies medical as well as organizational aspects. The latter will be illustrated by an organization study based on activity diagrams performed to set-up a prototype digital archive (PDA). The PDA was developed to determine the information to be stored in a digital archive and the requirements that users have in accessing image data and corresponding attributes. First experiences with respect to the organizational aspects of this clinical picture archiving and communication systems (PACS) project, in which on-line communications are used in daily routine, will be presented.

X-linked progressive mixed deafness with perilymphatic gusher during stapes surgery.

Stapes gusher is a rare and usually unexpected complication of stapes surgery. This complication will inevitably be encountered during stapes surgery in all affected males with the X-linked, progressive mixed deafness syndrome. The opportunity of studying eight affected males in a large Dutch family with audiometry, vestibulometry, and polytomography was used to identify specific features. Awareness of these features will assist the otologist in recognizing new cases preoperatively.

Perforation of the intra-articular disc diagnosed by arthro-tomography of the temporomandibular joint.

Arthrography was first used almost 40 years ago for evaluation of the non-osseous parts of the TMJ. It did not find wide application and only during the last decade have certain authors re-introduced its use as a diagnostic approach in TMJ pathology. Among the other pathological conditions which can be diagnosed, is a perforation of the intra-articular disc. Four such cases are presented and the value of this method is shown.