Rare cause of bilateral sudden deafness.

In this paper, we describe the case of a 62-year-old female with recurring episodes of sudden deafness with vertigo and facial paresis. Within a month's time, this resulted in bilateral deafness and vestibular areflexia. Erroneously, the patient was diagnosed with sudden deafness of unknown origin and subsequently with neuroborreliosis (Lyme disease). The true diagnosis of relapsing polychondritis (RP) was revealed 9 months after initial presentation. The diagnostic delay is in part explained by the fact that, by definition, the disease has to relapse before the diagnosis can be made, but also by its pluriform clinical presentation. Timely identification of RP as the cause of this profound sensorineural hearing loss proved to be important. It was key in instigating adequate follow-up, and allowed for cochlear implantation before total cochlear obliteration, which might have hampered optimal hearing rehabilitation.

Neuroendocrine carcinoma of the sphenoid sinus: a case report.

The presentation of a neuroendocrine carcinoma in the paranasal sinuses is extremely rare. Until now only 25 cases have been reported in the literature. We report a case of a 65-year-old male with an atypical carcinoid of the sphenoid sinus which seemed to be associated with multiple endocrine neoplasia type 1 (MEN 1). To the best of our knowledge this is the first report on an atypical carcinoid in the sphenoid sinus.

[Rupture of larynx or trachea resulting from injuries sustained at birth]. / Ruptuur van de larynx of de trachea als geboortetrauma.

Four newborn boys developed respiratory insufficiency and pneumothorax, pneumomediastinum or subcutaneous emphysema as the result of a laryngeal or tracheal rupture. These ruptures were due to birth injuries after difficult labour resulting from shoulder dystocia or a large lymphangioma and to a birth weight of at least 4500 g. The three children with shoulder dystocia also had a clavicular fracture, a Horner's syndrome, Erb paralysis or phrenic nerve paresis. Treatment consisted of surgical repair followed by a few days' intubation. The children with a shoulder dystocia recovered well, although in one of them a tracheal stenosis had to be resected a few months later. The child with the lymphangioma died from a bifurcation embolus. In newborns with respiratory insufficiency and pneumomediastinum or subcutaneous emphysema after a difficult delivery an emergency laryngotracheoscopy has to be performed to exclude rupture of larynx or trachea.

Localization and morphology of antigen-presenting cells in the adenoid of children with otitis media with effusion.

This study describes the localization of antigen-presenting cells (APC) in the different compartments in adenoids of children with otitis media with effusion (OME) and "healthy" children and adults. It is shown that the adenoid of children with OME contains a relatively high number of OKT6 and RFD1 positive cells. Moreover, accumulations of these cells are present in the extrafollicular areas of these adenoids. Very occasionally dendritic cells in the epithelial microenvironment contain Birbeck granules, indicating characteristic Langerhans cells. These OKT6 positive cells are not seen in the adenoids of the control group. Our results clearly show a relation between the presence of dendritic cells and the occurrence of OME. No differences are seen in localization and morphology of the APC in the studied adenoids.

Isolation and characterization of dendritic cells from adenoids of children with otitis media with effusion.

Dendritic cells were enriched from adenoids of children with otitis media with effusion (OME) by density gradient centrifugation and culture techniques. An enrichment of 40-140-fold was obtained for dendritic cells. These cells were identified using morphology, enzyme cytochemistry, immunocytochemistry and functional criteria. Dendritic cells could be easily distinguished from macrophages. It appeared that the MoAb EBM11 (CD68) discriminated between dendritic cells and macrophages; in dendritic cells this activity was localized in a spot, whereas in macrophages it was found throughout the whole cytoplasm. The fractions enriched with dendritic cells showed a strong stimulatory effect on allogeneic T cells. These responses were MHC class II dependent since they could be blocked by anti-HLA-DR/DQ MoAbs. The data clearly show that dendritic cells from adenoids of children with OME still have functional capacities.

Lymphoid and non-lymphoid cells in the adenoid of children with otitis media with effusion: a comparative study.

We characterized on immuno- and enzymecytochemical level the lymphoid and non-lymphoid cells in the adenoid of children with upper respiratory tract infections (URI) and otitis media with effusion (OME) and compared these with the adenoid of children with URI without OME and with the adenoid of 'healthy' children and adults. Besides macrophages and dendritic cells we also showed the presence of MHC class II positive, ciliated, epithelial cells. These non-lymphoid cells were present in all adenoids. However, their number was less than 1% of all cells. We found no difference in lymphocyte subsets from children with URI + OME compared with those from children with URI alone. These two groups showed a significant decrease of CD8-positive (suppressor/cytotoxic) cells and a slight increase in CD22-positive B cells in comparison to 'healthy' children. No difference was found in percentages of CD4-positive (helper/inducer) cells. The localization of the lymphoid subsets in adenoids of children with URI and/or OME did not differ from those of 'healthy' children and adults.