This study reviews 57 papers dealing with the issue of hearing preservation in vestibular schwannoma surgery published in otolaryngologic and neurosurgical literature between theyears 1977 and 1994. The authors', in this review, have made an attempt to verify whether the claims of hearing preservation are real, whether there is a price to be paid in terms of morbidity and whether there are univocal criteria for reporting results. The review shows that there is a wide disarray in reporting hearing results and the claims of hearing preservation are often unreal and misleading. On retabulating the results of a few series according to the minimal prerequisites for normal hearing (PTA ≤3O dB and SDS ≥70 per cent) and according to other various commonly reported criteria, it became evident that rates of hearing preservation differeda lot depending upon criteria. While any measurable hearing could be preserved in many cases, only a few had normal hearing preserved.

Vestibular schwannoma occurs both as a sporadic tumour and in the dominantly inherited familial cancer syndrome neurofibromatosis type 2 (NF2). The gene for NF2 has recently beenisolated on chromosome 22, and the demonstration of inactivating germline mutations in NF2 patients and NF2 associated tumours suggests that it acts as a tumour suppressor. The results of recent research in Cambridge suggest that somatic mutations of the NF2 tumour suppressor gene are a critical step in the pathogenesis of both familial and indeed non-familial unilateral sporadic vestibular schwannoma and that the mechanism of tumourigenesis complies with the ‘two-hit’ model. This paper represents a brief review of the current status of molecular biology in relation to vestibular schwannoma in particular and is discussed in relation to the molecular pathology of skull base tumours as a whole.

Central electrical stimulation of the auditory pathway can allow hearing in patients sufferingfrom deafness localized in the auditory nerve. Developments in a multi-channel auditory brainstem implant based on the Nucleus Mini 22 Cochlear implant with transcutaneous signal transmission is discussed. The devices have been implanted in nine European patients suffering from Neurofibromatosis Type 2. Preliminary speech perception results and patient satisfaction are encouraging, and the data presented include some limited open speech recognition.

There is a paucity of studies investigating middle ear pressures (MEPs) in patients with nasopharyngeal carcinoma (NPC). This paper prospectively examines MEPs in patients with nasopharyngeal carcinoma before and after radiotherapy and discusses their clinical significance. Newly diagnosed patients with nasopharyngeal carcinoma were studied before and at three to 12 months (mean 7.5 months) after radiotherapy. MEPs were measured by tympanometry. Thirty-three patients completed the study. The mean MEP before and after radiotherapy was −55.2 mm water(range −250 to 45 mm water) and −73.1 mm water (range −215 to 35 mm water) respectively. About two-thirds of assessable ears had an increase in negative MEPs after irradiation and the rest had less negative MEPs after irradiation. Those ears which developed post-irradiation middle ear effusions were found to have pre-irradiation negative middle ear pressures of at least −45 mm water. These findings provide possible explanations for some unexplained audiometric observations previously made in patients with nasopharyngeal carcinoma after radiotherapy. Tympanometry before radiotherapy may prove to be useful in identifying ears with a high risk of developing post-irradiation middle ear effusion.

Facial motor function was assessed in 61 patients 18 months after surgical resection, by the translabyrinthine approach, of vestibular schwannoma involving both the cerebellopontine angle (CPA) and the auditory canal.

Pre-operative magnetic resonance imaging (MRI) was performed to measure the maximum extracanalicular diameter of the tumour between the porus and the farthest extension in the CPA on tranverse slices and to calculate extracanalicular tumour volume. Post-operative facial motor function was graded according to the House and Brackmann system.

There was a statistically significant relationship between late facial motor function and extracanalicular diameter. The best cut-off point for good and poor results was 20mm. There was no relationship between the tumour volume and the late post-operative facial motor function grade.

In this study the best pre-operative radiological predictor of the late facial motor function in patients operated on by the translabyrinthine approach was the maximum diameter measured byMRI.

To study the efficacy of peak nasal inspiration flow (PNIF) as a means of recording change in nasal patency 20 subjects were given increasing doses of intranasal 5tometazo1ine. Nasal resistance (NR) and peak nasal inspiratory flow (PNIF) were measured in the resting stateand after each xylometazoline administration. Successive increases in dose caused a progressive decrease in nasal resistance and an increase in PNIF but the change in peak nasal inspiratory flow (PNIF) was much less. Peak nasal inspiratory flow shows a plateau effect as nasal resistance decreases. The reasons for this plateau are discussed in terms of respiratory flow mechanics

Larynges from 24 victims of Sudden Infant Death Syndrome (.S.I.D.S.) and 10 controls, with ages ranging from two days to 24 weeks in the controls and from two toN 16 weeks in theS.1.D.S. victims, were transversely, step-serially sectioned and then stained to show acid, neutral and mixed mucus glycoproteins

The proportion of sulphated mucus glycoprotein and sialylated mucus glycoprotein to total acid mucus glycoprotein was determined by the use of various staining techniques and a comparison was made for each type between S.I.D.S. and controls. The differences were significant with a mean of 27 per cent in the controls compared to 59 per cent in the S.I.D.S. for sulphomucin (difference 32 per cent; standard error of difference 6 per cent; p<0.01) and 73 per cent in the controls compared to 41 per cent in the S.I.D.S. larynges (difference 32 per cent; standard error of difference six per cent; p<0.01) for sialomucin. The results suggest that sulphated mucus glycoprotein is secreted in excess in some victims of Sudden Infant Death Syndrome.

The evolution of voice and speech parameters following supracricoid partial laryngectomy (SCPL) has been evaluated in a prospective fashion over an 18-month period of time in three patients using the Computerized Speech Lab (from Kay Elemetrics). Preliminary results demonstrate the post-operative instability of voice parameters. Speech parameters remain stable with time. Our data stresses that voice is attained post-operatively even if one arytenoid cartilage is disarticulated at time of resection.

A new multi-transducer probe system for measuring pharyngeal pressures was compared with an established open catheter system. Pharyngeal pressure measurements were made at the same time, and site, in subjects awake, at unmodified and with artificially increased nasal airway resistances, and during sleep documented by polysomnography. The two systems yielded almostidentical results. It is anticipated that the multi-transducer probe system will prove of clinical value.

A report of the first 100 patients treated in the multidisciplinary Dysphagia Clinic in Salisbury District Hospital is presented. It was established in January 1992 and involves the Departments of ENT Surgery, Clinical Radiology and Speech and Language Therapy. In the first 18 months, 100 patients have been assessed and treated. These included 46 males and 54 females, between the ages of four and 93 years. Neurological problems accounted for 39 cases. The management comprised the assessment clinic, videofluoroscopy in 83 patients, and finally the appropriate treatment. Forty-four patients were treated primarily by swallowing therapy. Seventy-three patients showed complete recovery, resolution of their symptoms or did not require treatment. Two patients were still under treatment at the time of writing this paper.

A Cochlear Mini System 22 Channel cochlear implant extruded through the skin of a young girl. The implant was saved by use of a transposition flap. Extrusion appeared to be due to pressure necrosis from the implant on the overlying tissues. It is believed that this problem can be prevented by angulation of the implant before insertion so that it conforms to the curvature of the skull, so avoiding pressure on the tissues. The method of angulation is described.

A case report of a thrombosed posterior-inferior cerebellar artery aneurysm is described. The tumour masqueraded as a vestibular schwannoma on imaging but the presenting featureswere unusual, the facial pain and weakness predominating despite well preserved hearing. This is an example of a rare cerebellopontine angle tumour and highlights the need for suspicion when lesions present atypically.

Verbal auditory agnosia implies the failure to recognize sounds in a patient who is nevertheless not deaf. We present a child referred to the ENT outpatients for suspected hearing loss investigation. At one point she had grommets inserted on the basis of a flat tympanogram but with no effect. All hearing tests, including electrocochleography and distraction tests, revealed normal hearing thresholds. At the age of three years and three months, and on direct questioning of the parents, we discovered that the child responded remarkably well to music. Paediatric neurologists made the diagnosis of verbal auditory agnosia. The condition is very similar to another acquired language disorder called Landau-Kleffner syndrome. It is unusual for such a case to present primarily to an ENT surgeon.

Four cases of the rare disorder, inherited degenerative chondropathy have been previously reported (Kurien et al., 1989). A five-year follow-up of these patients and two additional cases are presented in this report. The progress of this disease appears to be arrested after regulardapsone therapy and there was no other organ involvement noted during the follow-up period.

A case is reported of a HIV–positive patient with severe von Willebrand's disease describing the bleeding complications during and after tonsillectomy. This patient underwent surgery for asymmetrical tonsillar hypertrophy. The tonsils were spontaneously haemorrhaging and there therefore was a suspicion of neoplasia. Despite close cooperation between the ENT Department and the Haemophilia Centre, involving per–operative Factor VIII monitoring and replacement, the patient suffered both protracted primary and secondary haemorrhages. We report this as a cautionary tale as our previous experience with mild to moderate haemophilia has been uncomplicated, but on this occcasion there was massive haemorrhage. We feel that tonsillectomy should not be undertaken in a patient with a severe bleeding disorder without an absolute indicate.

Pneumoperitoneum as a result of pneumomediastinum has been described already. Amongst the causes pneumoperitoneum due to a complication of adenotonsillectomy, has not previously been described.

We describe a five-year-old girl with tension pneumoperitoneum with respiratory and circulatory insufficiency half an hour after adenotonsillectomy. Removal of the intra-abdominal air witha 14 gauge needle restored normal respiration and circulation.

A case of severe macroglossia. following intraoral surgery, causing respiratory distress is described. Because conventional therapy appeared inadequate, leeches were used and proved an efficient method of reducing this life-threatening swelling of the tongue.

Cervical heterotopic salivary tissues are rare and are predominantly found in the anterior triangle of the neck especially at the anterior border of the sternocleidomastoid muscl. We report a case of pleomorphic adenoma arising in the left posterior triangle of the neck.