Fraser Syndrome is a rare autosomal recessive disorder (Gupta and Saxena, 1962; Smith, 1982). The most consistent feature is cryptophthalmos (hidden eye), but frequently abnormalities of the ears (meatal stenosis, dysplastic pinna), nose (hypoplastic notched nares, choanal stenosis or atresia), and larynx (glottic web, subglottic stenosis), as well as numerous other anomalies are encountered. We present four cases that have been treated at the Hospital for Sick Children in the last ten years, and describe the various ENT anomalies characteristic of this syndrome.

Malignant external otitis is a progressive pseudomonal infection of the external auditory canal and adjacent structures. In the literature there is no unified policy regarding the management of malignant external otitis. The development of an effective nuclear scanning method and antibiotics active against Pseudomonas aeruginosa have helped in formulating our management policy. A review of four years personal experience with this condition is presented. All of our cases were cured from the disease with no fatality. Gallium67 citrate scans showed that antipseudomonal treatment should continue for up to three months.

Deviations in facial form are discussed. On the basis of previously published hypotheses, it is suggested that oropharyngeal development may be influenced by the posture, and function of the tongue, lips, and jaws. Attention is drawn to the relationship between a firm tongue to palate swallow, and the pump action of the palatine aponeurosis, which it is suggested is necessary for the aeration of the Eustachian tube. The pump action appears to be reduced in long faced adenoidal children, but clinical evidence suggests that it can be regenerated by early Orthotropics (growth guidance) aimed at directing facial growth forward, instead of downward. Any reduction in the height of the face, facilitates an improved lip seal, without which the downward pattern of facial growth is likely to continue. On occasions when these objectives are achieved it would seem that sufficient room is created to align all 32 teeth, with excellent facial form, and little otolaryngological pathology.

Surgical closure of the anteriorly located tympanic membrane perforation can present a problem. The lack of anterior support for the graft frequently leads to graft failure if an underlay method is used whereas anterior blunting is a complication of onlay techniques in this situation. The experience with the Kerr flap, an underlay graft fashioned to include a tab of fascia which is placed laterally under the annulus and the anterior meatal skin, is presented. This method gave a 97.5 per cent closure rate with no cases of anterior marginal blunting and a mean auditory threshold gain of 8.5 dB (95 per cent confidence limits 5 to 11.9 dB, P<O.O1) was achieved at the frequencies tested. The use of the Kerr flap is recommended when repairing the anteriorly placed tympanic membrane perforation

Controversy still exists concerning the ultimate fate of transposted or transplanted ossicles in tympanoplasty. Histological studies on autologous and homologous ossicles removed at revision operations, or from animal studies, vary in their conclusions. The viability, of such materials used in the long-term is still uncertain. Whether this has any functional significance is also uncertain.

This report contains a histological review of 10 ossicle remnants stored in the ‘closed’ mastoid cavity for 12 months, following closed cavity mastoidectomy (combined approach tympanoplasty)

Forty-nine children for tonsillectomy were entered in a prospective controlled single blind study to compare the post-tonsillectomy morbidity of bipolar diathermy as opposed to ligation for haemostasis. We found no significant difference in post-operative discomfort nor reactionary or secondary haemorrhage in the two methods used. This is the first controlled trial where bipolar, rather than unipolar diathermy is used, and compared with ligation in the operation of tonsillectomy

Forty-seven mucocoeles of the ethmoid and/or sphenoid sinuses (33 males, 14 females) were operated on during the 10-years period from 1980 through 1989. Thirty-seven cases were post-operative mucocoele, while the remaining 10 were so-called primary mucocoeles. None of the cases had a history of facial trauma. In the majority of post-operative cases, the mucocoele develops 15–24 years after initial surgery. Paranasal sinus surgery in young patients (teenagers) may lead to a mucocoele due to post-operative scarring in the surgical wound. The principal symptoms include globe displacement, double vision, headache, deep orbital pain, a mass in the supero-medial quadrant of the orbit, visual disturbance, etc. Sufficient opening of the mucocoele wall by the endonasal approach is recommended for surgical treatment of ethmoidal and sphenoidal mucocoeles

We report our experience of using the laryngeal mask airway (LMA) over a period of 18 months in 217 patients undergoing a variety of otorhinolaryngological operations. Advantages over conventional intubation for both patient and surgeon are suggested in both safety, speed and economy. An inadequate airway, necessitating replacement of the LMA, only occurred on two occasions whilst two known cases of difficult intubation easily had their airways secured by use of the LMA. Protection of the lower airways from secretions, fluid or blood, arising above the LMA, would appear to be confirmed

Different techniques were compared in management of 36 patients with bilateral abductor paralysis of the vocal cords. Ten patients were treated by microsurgical arytenoidectomy through a mid-line thyrotomy, with successful decannulation in only three. Fifteen patients were treated by endolaryngeal microsurgical arytenoidectomy, with failure to decannulate four cases.

The procedure of endoscopic laterofixation of the vocal cord was used to treat 11 patients. Ten patients had an adequate long-lasting airway with a socially acceptable voice function. One patient had a revision surgery and was successfully decannulated. The technique was found to be a reliable option in the management of bilateral abductor paralysis. It is a modifiable procedure with a feasibility to adjust the position of the vocal cord under endoscopic control. It can be combined with endolaryngeal arytenoidectomy if the gain in the airway size produced by laterofixation is found insufficient.

Using Ki-67, a monoclonal antibody, the proliferating capacity of 15 salivary gland tumours, including nine pleomorphic adenomas, four adenoid cystic carcinomas, one mucoepidermoid carcinoma and one acinic cell carcinoma was determined immunohistochemically, using normal salivary gland tissue as a control. The frequency of Ki-67 positive cells was 4.7 per cent in the normal salivary gland and one per cent in pleomorphic adenomas, whereas the average frequency in malignant tumours was 18.3 per cent. Among adenoid cystic carcinomas, the frequency was related to the morphological type; the solid sub-type had the highest frequency of Ki-67-positive cells. As this sub-type is recognized as the most aggressive of these tumours, this technique has the potential of providing an early indication of the clinical behaviour of a tumour.

Fifty patients were included in a single blind study to evaluate the effectiveness of ephedrine-naphazoline (0.5 and 0.125 per cent respectively) nasal spray as against nasal packing with lignocaine (4 per cent) and adrenaline solution (1:50,000).

We describe a method for continuous measurement of end-tidal carbon dioxide concentration during jet insufflation anaesthesia for microlaryngeal surgery. This increases the safety of the technique by allowing closer monitoring of inspired and expired gases during anaesthesia

Tinnitus due to muscular causes is rare. The commonest pathological muscular cause of tinnitus is palatal myoclonus.

A case of bilateral tinnitus, related to eyelid blinking, is presented. This is the first case reported in the literature. Previous reported cases of tinnitus related to eyelid blinking have all been unilateral and associated with recovery from a VII nerve palsy. The treatment of the condition is discussed.

A case of primary gingival leiomyosarcoma in a 64-year-old woman is presented. The treatment modalities are discussed and the previous literature is reviewed.

Rhinocerebral mucormycosis is a rapidly fatal fungal disease which involves the nose, paranasal sinuses, orbit and central nervous system. The fungal infection is usually secondary to immunosuppression, diabetic acidosis, or antibiotic, steroid or cytotoxic therapy. It can also occur in patients suffering from burns, malignancy and haematological disorders. Current treatment consists of correction of the underlying disorder, repeated debridement of the wound in combination with intravenous amphotericin B.

This paper describes our experience with a case of rhinocerebral mucormycosis. This is an unusual case in which mucormycosis was seen in a young female where no underlying cause was found. She responded to surgical debridement in combination with intravenous amphotericin B.

An unusual case of pleomorphic adenoma with exuberant bone formation, occurring in the maxillary antrum of a 21-year-old male and showing repeated recurrence, is reported. In contrast to the endochondral ossification in the previous reported cases of pleomorphic adenoma, direct deposition of osteoid by metaplastic myoepithehal cells is suggested to explain the bone formation. The difficulties of distinguishing this tumour from osteosarcoma during intraoperative diagnosis are discussed.

A patient was referred to us with a mass in her upper jaw. This was diagnosed to be a chondrosarcoma of the maxilla. The mass was removed surgically. We present the case here and discuss the salient features.

A previously unreported association between bilateral abductor paralysis and hypokalaemia is reported in a patient presenting with stridor. Both the stridor and the bilateral abductor paralysis gradually resolved as the serum potassium was corrected. A causal link is suggested though the mechanism is unclear.

A case of carcinosarcoma of the larynx and pyriform sinus with sarcomatous and epithelial malignant components is described in a 68-year-old male. The primary site was composed of spindle-shaped malignant mesenchymal cells with osteosarcomatous and chondrosarcomatous differentiation intermingled with areas of well differentiated squamous cell carcinoma, tubular and papillary adenocarcinoma, and trabecular neuroendocrine carcinoma. The metastatic lymph node showed squamous cell carcinoma and chondrosarcoma. Immunohistochemical studies showed that keratin was present in epithelial cells and negative in sarcoma, whereas vimentin and S-100 protein were present in sarcoma, but negative in carcinoma. Neuron-specific enolase was present in the neuroendocrine part of the tumour.

A case of subglottic narrowing caused by an extranodal deposit of sinus histiocytosis in an eight-year-old child with Rosai-Dorfman disease is presented. The airway obstruction was initially managed with a tracheostomy, and then on confirmation of the diagnosis by lymph node biopsy, by excision of the extranodal deposit with a CO2 bronchoscopic laser.