A case of primary amyloidosis of the external auditory canal is described. To the best of our knowledge this is the first case described in the literature.

An extensive schwannoma in a 54-year-old woman is described. There was extension intracranially to involve the brain stem, laterally to fill the middle ear and external meatus, and inferiorly into the infratemporal fossa. Initially the intracranial portion of the tumour was excised. Later a second operation was undertaken to remove the residual tumour from the temporal bone and infratemporal fossa.

We report a case of persistent epistaxis in a seven-month-old child with Wilms' tumour who had an associated von Willebrand's type bleeding diathesis. Correction of the bleeding diathesis to control the epistaxis required treatment of the tumour with chemotherapy.

The case of a 73-year-old lady with late congenital pharyngo-laryngeal syphilis is presented. Its finding was at a potentially difficult intubation during the induction of general anaesthesia. She was asymptomatic; there was no evidence of active disease on either clinical examination or serology and no treatment was required. However, it is important to differentiate this condition from others which may require treatment.

A case of tuberculous lymphadenitis with a nasopharyngeal tuberculoma is presented where a novel use of the flexible nasendoscope to provide a diagnostic aspiration biopsy avoided an examination and biopsy under general anaesthesia. The use of this approach is discussed.

Immunocytochemistry (ICC) proved to be an essential adjunct in the fine-needle aspiration (FNA) cytological diagnosis of chordoma of the clivus in a 62-year-old woman. The cytological picture in routinely stained smears was not entirely diagnostic for chordoma due to the paucity of typical ‘physalipherous’ cells. To exclude other primary or metastatic neoplasms of the skull base possibly sharing the same cytological picture, additional direct smears were immunostained with antibodies specific for cytokeratin (CK), vimentin (VIM), SI00 protein (SIOOP), carcinoembrionic antigen (CEA), epithelial membrane antigen (EMA), glial fibrillary acidic protein (GFAP), CD68 antigen (KP1) and with the ‘panepithelial’ antibodies B72.3 and Ber-EP4. Chordoma cells showed the following immunoprofile: CK+/VIM−/S100P+/CEA−/EMA+/GFAP−/B72.3−/Ber-EP4−/CD68+. The pattern of immunoreactivity for CK, S100P and CEA confirms previously reported data, while the B72.3−/Ber-EP4−/CD68+ staining profile represents a novel observation. The etection of a CK+/S100+/CEA−/B72.3−/Ber-EP4− immunocytological profile of chordoma cells in aspirates is a basic requirement to exclude pertinent diagnostic differentials, such as metastatic carcinoma, ependymoma and sarcoma, and permits a reliable pre-operative diagnosis of the tumour by aspiration cytology.

Four cases of first branchial arch defects are presented. The literature relating to such defects is reviewed and a possible embyrogenic mechanism is proposed with particular reference to the disposition of the facial nerve. The classification of these defects into Types I and II by Work (1972) is supported

Mucoceles are uncommon and the majority occur in the fronto-ethmoidal complex. We present the first case of a secondarily infected mucocele arising from a concha bullosa of the middle turbinate. It was excised intranasally with complete resolution of symptoms.

Lipomas are common benign connective tissue tumours composed of adult adipose tissue. They are relatively rare in the upper aerodigestive tract, although they occur with considerable frequency in other areas, particularly in the subcutaneous tissues of the neck. Although there are several reports of this tumour occurring in the oropharynx, there is no recorded case of a lipoma of the tonsillar fossa. An 83-year-old man with a left tonsillar fossa lipoma is presented. Clinical presentation, management and a literature review are discussed.

Nasal myiasis is the infestation of the nasal cavities by larvae (maggots) of Diptera flies. Several species of flies deposit their ova in the nose and the larvae feed on the host's tissues. We present a case of nasal myiasis by larvae of Oestrus ovis – Sheep Nasal Bot Fly. The larvae of Oestrus ovis are well known parasites in the nasal cavities and paranasal sinuses of sheep and goat. In Britain very rarely larvae may be deposited in the eye, nostrils or outer ear of man, usually husbandry workers. Reputedly, the larvae never survive beyond the first stage with acute catarrhal symptoms lasting only a few days.

This is the first reported case in the UK of an urban-dwelling patient infected by mature, third instar larvae of O. ovis. His nasal infestation resolved after endoscopic removal of the live maggots.

Chronic inflammation of the skin and mucous membranes following prolonged exposure to high doses of toxic hexavalent chromium is well known in the occupational medical literature. Perforation of the nasal septum is a cardinal clinical feature. Late development of cancer in the respiratory system is established. A recently diagnosed case with an unusually severe affect on the eyes is described. The industrial usage of chromium and its toxiological profile is reviewed.

A 57-year-old man presented with a history of nasal obstruction of five to six years duration. 'Nasal polyps' were removed on several occasions. He had previously had an episode of paraplegia which resolved after the removal of a spinal tumour. Histology from both sites was thought to represent a malignant fibrous histiocytoma. On presentation the patient had computed tomographical (CT) evidence of extensive ethmoidal disease, with threatened intracranial extension. He also had evidence of lung and retroperitoneal disease with pancreas and kidney involvement. The ethmoidal disease was considered potentially lethal and therefore a craniofacial resection was performed. Review of all the histology revealed that the diagnosis was extranodal Rosai–Dorfman disease (sinus histiocytosis). The patient's course is described, and the literature on this disease of unknown aetiology is reviewed.

Angioleiomyoma of the nasal cavity is an extremely rare tumour (Sawada, 1990). We present an unusual case of angioleiomyoma arising from the lateral wall of the nasal cavity without any symptoms of pain or epistaxis. Another unusual feature was that the tumour clinically resembled an inverted papilloma.

Myxomas are rare tumours which may occur in the head and neck region. About 60 cases have been reported in the literature so far. We describe a retropharyngeal myxoma in this paper.

An abnormally large vestibular aqueduct has a well recognized association with inner ear anomalies and it has been assumed previously to be a variant of a Mondini type of deformity (Shuknecht, 1980; Emmett, 1985). The sole radiological finding in some patients with progressive sensorineural loss has been a large vestibular aqueduct (Valvassori and Clemis, 1978; Valvassori, 1983), which is now accepted as a separate clinical entity, i.e. the large vestibular aqueduct syndrome (LVAS).

A case is presented which is believed to be the first reported with unilateral LVAS and stapes fixation and also the first stapes gusher described in association with LVAS.

Prolonged exposure to sulphuric acid fumes is associated with an increased risk of developing laryngeal carinoma. Lead acid batteries are a potential source of these fumes. We present a case of an electric fork lift truck driver who developed laryngeal carcinoma as a result of such exposure.

Monostotic fibrous dysplasia of the temporal bone is a rare disease entity that may lead to progressive stenosis of the external auditory canal with resultant trapping of skin and the development of cholesteatoma. The cholesteatoma may extend widely into the temporal bone. Once cholesteatoma occupies the petrous apex, erosion out of the temporal bone can occur superiorly into the middle fossa, posteriorly into the posterior fossa, inferiorly into the neck, and infero-medially into the parapharyngeal space. We present a case in which minimal symptoms were present despite a massive temporal bone fibrous dysplasia and cholesteatoma. Eventually, cholesteatoma eroded into the parapharyngeal space, leading to airway compromise. Late presentation occurred because the otic capsule was spared, there was no external skull deformity and there was slow inferior and medial growth that was well tolerated, until the development of a parapharyngeal space abscess.

We report, to our knowledge, the 10th recorded case of mesenchymal chondrosarcoma (MC) occurring in the maxilla. Our case is the youngest person reported with a tumour in this location. The prognosis for cure is poor with a high incidence of local recurrence as well as metastases. Treatment is based on radical surgery. Radiotherapy and chemotherapy have a adjuvant role but additional experience with this tumour is required to define the most efficacious treatment.

Wegener's granuloma is a systemic necrotizing vasculitis which predominantly affects the upper and lower respiratory tracts and kidneys. The nose and paranasal sinuses are often the first or main sites to be affected. Equally ear involvement is common and a recognized early manifestation of the disease. Salivary gland involvement is rare and is usually part of more widespread systemic involvement.

We present a 34-year-old female with a six-week history of hearing loss and a unilateral parotid swelling. One year previously she was diagnosed as having myalgic encephalomyelitis following a long period of post-viral malaise. The case points to the difficulties encountered when Wegener's granuloma presents in an unusual and varied way. A high index of suspicion coupled with early diagnosis and treatment is essential. The patient made a good recovery.

The history, examination and operative findings of primary amyloidosis of the larynx are very suggestive of carcinoma, indicating the need for careful histological examination. Staining with Congo red shows a characteristic birefringence. Systemic amyloidosis may be present