Thirty families in which there were 149 individuals affected by the Waardenburg syndrome (WS) were investigated for penetrance of hearing loss and pigmentary abnormalities. Twenty two families contained 89 individuals affected by WS Type II and eight families with 60 individuals affected by Type I. A bilateral symmetrical sensorineural hearing loss was found to be the most common type of hearing loss. The most frequent degree of hearing loss category was a hearing loss of > 100dB HL with no difference between syndrome types.

Although there was no significant difference in the frequency of pigmentation disorders between Type I and II, considering these abnormalities as a whole, heterochromia irides was more common in Type II than Type I and other pigmentation disorders were more frequent in Type I: 62 per cent of Type I patients had more than one pigmentary defect, but only 28 per cent of Type II. Pigmentary disturbances were not significantly more frequent in the hearing impaired group than in the normally hearing group in either Type I or II when taking into account the occurrence of only one pigmentation disorder. There was a significantly higher proportion of pigmentary defects in the hearing impaired group with Type I when only those having more than one pigmentary abnormality were compared: 93.3 per cent of Type I patients and 88.5 per cent of Type II patients with a profound hearing loss had pigmentary defects. The frequency of pigmentation disorders was not greater when the hearing loss was more severe in either type. Penetrance for hearing loss and pigmentary abnormalities showed marked intrafamilial and interfamilial variation.

Otitis externa and chronic otitis media often present to the otolaryngologist with a discharging ear. The conventional method of treatment is to perform regular aural toilet and insert medicated dressings into the external auditory canal. This treatment is either performed by trained nurses or medical staff, but in either case is time consuming.

This study compares the efficacy of the above standard regimen with a novel treatment where a single aural toilet is carried out and medicated ointment instilled into the ear. Both regimens were evaluated at three weeks.

Our results shows that there was no significant difference between the two treatment regimens with regard to the resolution of either of the conditions studied or the improvement in the symptom status of the patients. On the basis of this it would seem that a single aural toilet and instillation of medicated ointment is a valid treatment option, cuts down hospital attendance and could be performed in the community by general medical practitioners or trained practice nurses.

Occupational rhinitis has been a prescribed industrial disease in the UK since 1907. It has only relatively recently received significant attention from otorhinolaryngologists although numerous studies have been performed in the past by occupational and industrial health physicians. At the present time the precise mechanisms of pathogenesis are unclear and would appear to be multiple.

Recently interest has arisen because of compensation claims. Diagnosis made on the basis of the clinical history is subject to two problems: firstly, there is difficulty in differentiating between occupational and nonoccupational rhinitis, and secondly, clinical histories can easily be feigned. Physical signs would be a more reliable indicator of occupational damage to the nasal mucosa if they differ from the signs normally found in allergic or vasomotor rhinitis. In a series of 100 shipyard workers dry atrophic nasal mucosa was found in 66and septal ulceration in two. From their clinical histories 78 individuals complained of nasal obstruction, 28 of epistaxis, 42 of hyposmia, 10 of anosmia and 90 of rhinorrhoea. Possible pathogenesis is described

The use of tissue autofluorescence for the detection and localization of cancer of the larynx is described. In this pilot study, eight patients with probable carcinoma of the vocal folds underwent laryngoscopy in which the tissue autofluorescence spectra of normal and pathologically confirmed tumour tissue were acquired in vivo. Fluorescence images of the suspect areas were also acquired using the LIFE system (Xillix Technologies Corp.). The results suggest that the autofluorescence properties of laryngeal tissue, under 442 nm illumination, are similar to those of bronchial tissue and that the LIFE system has the potential to increase the accuracy of staging of cancer of the larynx and also to allow earlier diagnosis of tumours and theirrecurrence

A retrospective study was undertaken to assess the outcome of 19 consecutive patients, who underwent endolaryngeal resection with a CO2-laser for laryngeal carcinomas. Sixteen of these patients had recurrences after primary curative radiotherapy. Seventeen of the patients had a curative operation carried out. So far, four CO2-laser treatment failures have been observed (median observation time 35 months). None of the patients needed tracheostomy in connection with the operation. Voice quality was in all cases acceptable.

It is recommended, that small recurrences (after radiotherapy of laryngeal squamous cell carcinomas)should be considered for a partial laryngectomy with the aid of a laser.

This study includes 155 patients with T1–4N0 carcinoma of the postcricoid region seen between 1963 and 1993. Sixty-seven were treated by primary surgery, 50 by primary irradiation therapy, 36 were unsuitable for curative treatment and two patients were lost to follow-up. Reasons for deciding against curative therapy were: advanced age, poor general condition and advanced disease at the primary site. This study included only those patients who had no neck node metastases at presentation.

Patients receiving surgery tended to be in better general physical condition and tended to have more advanced disease than those treated by irradiation in this series. The tumour-specific five-year survival rate for those treated by surgery was 43 per cent (95 per cent confidence interval (CI) 23–60 per cent). For those patients treated by irradiation the five-year survival rate was 48 per cent (95 per cent CI 27–66 per cent) and for those receiving no treatment the median survival rate was three months (95 per cent CI two-six months). The observed survival for the surgery group was only 18 per cent and for the radiotherapy group 25 per cent at five years.

Multiple logistic regression showed no significant difference in proportions of host and tumour factors between the group receiving radiotherapy and the group receiving surgery. Recurrence at the primary site and the appearance of neck node metastases were not predicted by any host or tumour factor.

Twenty-one patients out of 67 receiving primary surgery had recurrence at the primary site compared with 26 patients out of 50 receiving primary irradiation. Neck node metastases occurred in 16 out of 67 patients receiving surgery and in eight out of 50 receiving radiotherapy. The difference was statistically significant for recurrence at the primary site (X21 = 4.261; p = 0.039) but not significant for neck node metastases (X 21= 0.661;p = 0.416). The data were further analysed using Cox's proportional hazards model for survival and no host or tumour factors were found to be predictive of eventual outcome apart from poorly differentiated histology. This adversely affected survival (X21 = 6.4444; p = 0.011). If patients not treated were included in the model, treatment became a significant factor in improving the survival (X21 = 4.4197; p = 0.034).

Radiotherapy appears to be at least as good as surgery for treating patients with an early carcinoma of the postcricoid region. We would recommend radiotherapy is used in patients with no detectable neck node metastases and in tumours <5 cm long. The complication rate from radiotherapy was reduced when compared with that of surgery.

Squamous cell carcinoma of the posterior oro-and hypopharyngeal wall (SCCPPW) is a relatively rare tumour. A retrospective investigation of 63 patients with SCCPPW and 449 patients with carcinoma of the lateral oro- and hypopharyngeal wall, treated between 1964and 1992, has been carried out. Most SCCPPW were asymptomatic, macroscopically superficial and at early stages. They were usually detected by chance during an examination for a different type of malignancy. Fifty-seven per cent of SCCPPW patients had multiple tumours; however this occurrence did not alter the survival rate. The crude five-year survival rate for SCCPPW was 22 per cent and was not significantly different from that of patients with lateral wall tumours. Moreover, both local control and recurrences also were not statistically different.

Soft tissue sarcomas in the head and neck are rare. Aetiological factors relating to these tumours have not yet been identified. The association with von Recklinghausen's disease andwith irradiation is however well recognized. In the literature it has been speculated that trauma may also play a role in the development of soft tissue sarcomas. In this article we present five patients with a history of surgical trauma at the site where a sarcoma later developed. Although we cannot prove a causal relationship, the relatively high incidence of possibly trauma-related soft tissue sarcomas in a series of 60 patients we have seen over a 30-year period, suggests that such a relationship could exist.

The management of acute otitis externa is variable, despite the fact that it is one of the commonest otological emergencies. We formed the impression that many patients attending our ENT casualty clinic with otitis externa were being treated and followed up inappropriately. To test this hypothesis we performed a retrospective pilot study on our practice which revealed a lack of uniformity in the management and follow-up of these patients. And as a result of this, guidelines were developed to improve our management of otitis externa and decrease the number of unnecessary review visits. Our practice was then audited prospectively over a six-month period with the guidelines in place. An improvement in the overall management and a rationalization of follow-up for otitis externa was seen by applying basic audit principles to this common clinical problem.

We present four cases where a brow incision was indicated for access to the anterior skull base and paranasal sinuses. We discuss the indications for this incision and present a modification of the incision, to improve cosmesis over the nasion.

A simply manufactured whistle for the use of laryngectomees is described. It is useful for attracting attention in an emergency.

We present a rare branchial cyst of the nasopharynx in a 57-year-old man. The cyst was located at the higher level between the roof and the left lateral band of the nasopharynx, and was removed by the endonasal approach following a preparatory septoplasty. While several cases of nasopharyngeal cysts have previously been reported, this appears to be the first case of the resection of such a cyst via the endonasal approach, and is a useful, and potentially less injurious, alternative to the transpalatal approach.

A pseudotumour represents a lesion that masquerades as a malignant neoplasm. We present an unusual case where the appearance of a tumour was the result of chronic inflammation stimulated by the presence of food matter. This inflammatory reaction resulted in upper airway obstruction associated with a mass lesion and vocal fold palsy.

Penetrating injury to the larynx is uncommon. We present two cases resulting from the recent Bosnian conflict. It is possible to achieve good laryngeal function by appropriate conservative management once the airway is secured. Progress should be monitored by repeated endoscopic examination and imaging techniques.

A review of extracardial rhabdomyomas of the larynx reported in the literature is presented. A new case is added (the largest described yet). The diagnosis was based on routine histological and immunohistological staining, and electron microscopy. The extracardial rhabdomyomas were divided into three types according to histopathological findings: (i) adult; (ii) foetal cellular type; and (iii) foetal myxoid. There are 23 well-documented cases (including this case) of extracardial rhabdomyomas of the larynx; 15 of the adult type, four of the foetal myxoid type and four of the foetal cellular type. The differential diagnosis and the requisite diagnostic procedure is discussed. The tumour is benign. The treatment is surgical excision. Although rare, its existence should be kept in mind in the differential diagnosis of laryngeal tumours.

A rare case of actinomycosis presenting primarily as a mass in the vallecula is described. The patient was a healthy, 65-year-old Saudi, lady who was not immunocompromised and had no other primary pathology in the oral cavity. The clinical presentation and management of the case are discussed and the relevant literature reviewed.

A rare case of a squamous cell carcinoma of the left pyriform fossa is presented. It was found as a pedunculated polypoidal mass in a 17-year-old Hindu girl. A review of literature is included.

A case of intraoperative tracheostomy tube obstruction is reported. The clinical features and the chain of events leading to the diagnosis of cuff herniation are presented. The different mechanisms of herniation are discussed. In the present case we speculate that a manufacturing defect together with nitrous oxide diffusion into the cuff caused dilatation and herniation of the latter which led to obstruction of the distal tube lumen. We draw attention to this rare but life-threatening complication.

Tracheo-innominate artery fistual is usually fatal complication of tracheostomy. The trachea and innominate artery were successfully reconstructed in an 11-year-old girl who presented with this complication. The thymus was interposed betweenn these two structres in order to prevent further long-term complications.