We describe a case of palato-pharyngo-laryngeal myoclonus, an unusual variant of palatal myoclonus, which presented with dysphagia and dysarthna. The aetiology and presenting features of myoclonus are discussed. Various treatment options are considered.

A six-year-old girl with a rare bone dysplasia (Melnick-Needles Syndrome) presented with a five month history of severe sleep apnoea, weight loss and failure of thrive. The syndrome is associated with craniofacial abnormalities, including micrognathia. Following a multi-disciplinary assessment an elective tracheostomy was considered the most appropriate treatment. The patient developed severe respiratory distress 10 days prior to the arranged date of surgery and required an emergency tracheostomy. This resulted in a dramatic return to health. The recognition of severe sleep apnoea in patients with craniofacial abnormalities and the role of initial tracheostomy are discussed.

After a short survey on the diagnostic work-up for acoustic neuroma, the authors underline the importance of magnetic resonance (MRI), enhanced by gadolinium, for otoneurological pathology. Through the presentation of a clinical case, the pros and cons for the routine application of MRI in suspect cerebellopontine angle (CPA) tumours in particular are reviewed. The possibility of false-positive MRI findings is discussed, although in this specific case a proper therapeutic approach was eventually carried out in order to resolve the associated Vllth nerve disturbance.

We describe three cases of metastatic carcinoma in the parapharyngeal space associated with the Carotid Sinus Syndrome. This has not been previously described.

A case of nasal T-cell lymphoma in a Caucasian male is reported, in which the presence of Epstein-Barr virus within tumour cells was demonstrated by in situ hybridization to the Epstein-Barr virus encoded RNAs EBER-1 and EBER-2.

A case of persistent pneumonia in a depressed adult woman occurring as a complication of the repeated insertion of foreign bodies into the trachea via a tracheostomy is presented. This is an unusual complication of tracheostomy but should be considered in cases of persistent or unusual pulmonary infection in tracheostomy patients.

A rare case of a 71 -year-old man with malignant oncocytoma is presented. The tumour was found in the left parotid region with enlarged lymph nodes in the neck. The resected parotid mass consisted of three discrete nodules, each histologically characterized by a uniform proliferation of oncocytic tumour cells. The patient underwent repeated operations for recurrences and metastases, and eventually died of acute pneumonia 18 months after the first admission. Metastases to the lymph nodes, ribs, spine and liver had been clinically pointed out. Immunohistochemically, the tumour cells were positive for a-1-antitrypsin, a-1-antichymotrypsin, lactoferrin, secretory component and carcinoembryonic antigen (CEA), while they were negative for S-100 protein and HHF35 (muscle specific actin). Ultrastructural pictures disclosed numerous mitochondria in the cytoplasm of the tumour cells, revealing neither myofibrils nor secretory granules. These findings therefore support the concept that this tumour is of a glandular epithelial origin.

A Hurthle cell tumour is a variant of follicular thyroid cell carcinoma. This is a report of metastatic Hurthle cell tumour causing central airway obstruction, emphasizing the difficulty in differentiating benign from malignant lesions.

Spontaneous perforation of the oesophagus is extremely rare in children, as is perforation due to vomiting in pregnancy. We report the case of a 15-year-old in whom vomiting inearly pregnancy resulted in oesophageal perforation with subcutaneous emphysema causing marked facial swelling in the absence of other signs. The more common clinical presentation of spontaneous oesophageal rupture (Boerhaave's syndrome) is discussed.

Carcinoma in the thyroglossal duct remnant is relatively uncommon. Since the first report by Uchermann (1915), more than 150 cases of carcinoma have been reported, and the majority have been papillary thyroid carcinomas (Li Volsi etal., 1974; McNicol etal., 1988). In this report, we present a case of papillary carcinoma in the thyroglossal duct with a normal thyroid gland

Retropharyngeal haemorrhage is a rare condition. The classical picture is described as a triad of features; superior mediastinal obstruction, anterior displacement of the trachea on a plain X-ray of the neck and subcutaneous bruising appearing on the neck and spreading on to the chest wall (Sandor and Cooke, 1964). Two cases are reported here, neither of which had mediastinal compression. The available literature is summarized.

The first case of pigmented villonodular synovitis of the temporomandibular joint in a Chinese patient is reported. The dinicopathological features are described and the presentation as a parotid mass is emphasized. This rare tumour requires a high clinical suspicion for diagnosis. For removal, meticulous dissection of tumour and facial nerve is necessary.

We present a patient with established histiocytosis who developed dysphagia, retching, regurgitation, hoarseness and stridor. These symptoms were managed with carbon dioxide laser vaporization, electively on three occasions, and once as an urgent procedure, while awaiting radiotherapy, to control her airway.

Histiocytosis is a rare cause of a number of otolaryngological syndromes, but there has been no previous record of this disease causing laryngopharyngeal symptoms. This paper discusses the classification of histiocytosis, and describes our management of this rare and intriguing case.

Ossifying fibromyxoid tumour (OFT) is a recently described, mesenchymal neoplasm originally defined as a borderline or low-grade malignant lesion. Prior reports of OFT characterize it as a slow growing lesion with a propensity to occur in both the upper and lower extremities. Most OFTs have occurred within the deep subcutis or skeletal muscle. We report nine cases which arose in the head and neck region. Six of the nine tumours were classified as ossifying variants of OFT while two were non-ossifying variants that lacked a discernable shell of lamellar bone. One tumour was classified as a malignant OFT. Seven lesions occurred in a subcutaneous site while two lesions occurred intraorally beneath the gingival and palatal mucosa. The OFTs occurred in six men and three women (age range of 29–75 years). The tumours had histological features compatible with previously described OFTs and consisted of lobulated nests of small, cytologically bland round cells (with the exception of one malignant OFT), with a myxoid to hyalinized stroma and were surrounded in part by dense fibrous connective tissue. Six cases had an incomplete rim of lamellar bone with occasional perpendicularly orientated spicules of bone. Five lesions were immunostained. S-100 protein, neuron specific enolase, and Leu-7 were found in three out of five tumours. Glial fibrillary acidic protein, smooth muscle actin (SMA), and muscle specific actin (MSA) were detected in two out of five lesions, although staining for SMA and MSA was weak in reactivity. Staining for vimentin was strongly positive in all five cases tested. The tumours were not reactive with antibodies directed against cytokeratin, epithelial membrance antigen or neurofilament protein. Follow-up information, available in eight cases, revealed multiple local recurrences in the one tumour believed to be a malignant OFT. The histogenesis of these tumours is uncertain, although the preponderance of evidence suggests a Schwann cell origin.

The ultrastructure of the mucosa of the eustachian tube was studied in four temporal bones showing tympanosclerosis, cholesteatoma, otitic meningitis and a grafted tympanic membrane (tympanoplasty). The mucosa of tube was abnormal in the four cases confirming the relationship between the state of the eustachian tube and the inflammatory process in the middle ear. The observed abnormalities included: ciliary loss, abnormal ciliary morphology and motility, oedema of the microvilli, hyperplasia of the goblet cells and the seromucinous acini, desquamation of the non-ciliated cells and appearance of mast cells in the lamina propria of the tube. Ciliary changes were the most frequent abnormalities and the morphological changes, in general, were fewest in the case of healed tympanoplasty. The pathophysiology of the morphological changes was discussed and correlated with the disease in the middle ear.