Shakeel Saeed

Incidence of vestibular schwannoma and neurofibromatosis 2 in the North West of England over a 10-year period: higher incidence than previously thought.

Objective: To determine the incidence of vestibular schwannoma (VS) in sporadic, neurofibromatosis type 2 (NF2) germ-line and mosaic form in a 10-year period. Study Design: Review of all incident cases of VS presenting to the four main neurosurgical centers for a population of 4.1 million from 1990 to 1999 and cross-referencing with the regional cancer registry. Setting: Population based. Patients: All patients presenting with VS detected on magnetic resonance imaging scan. Results: A total of 419 sporadic and 64 NF2-related VS were identified over the study period. This represented an incidence of 10.4 per million per year for sporadic VS and 11.8 per million per year including NF2-related tumors. The incidence rose to 14 per million per year in the latter 5 years. The NF2 patient diagnoses represent an estimated birth incidence of 1 in 25,000, and 7% of the patients with VS had NF2, which is higher than previous estimates. Conclusions: The incidence of VS is rising almost certainly due to increasing diagnosis in the magnetic resonance imaging era. At current rates, 1 per 1,000 individuals will be diagnosed with VS in their lifetime. More VS than previously thought are due to NF2, which may be because of recognition of mosaic forms of the disease.

Management of the patient and family with neurofibromatosis 2: a consensus conference statement.

A consensus conference on neurofibromatosis 2 (NF2) was held in 2002 at the request of the United Kingdom (UK) Neurofibromatosis Association, with particular emphasis on vestibular schwannoma (VS) surgery. NF2 patients should be managed at specialty treatment centres, whose staff has extensive experience with the disease. All NF2 patients and their families should have access to genetic testing because presymptomatic diagnosis improves the clinical management of the disease. Some clinical manifestations of NF2, such as ocular abnormalities, can be detected in infancy; therefore, clinical screening for at-risk members of NF2 families can start at birth, with the first magnetic resonance (MRI) scan at 10 – 12 years of age. Minimal interference, maintenance of quality of life, and conservation of function or auditory rehabilitation are the cornerstones of NF2 management, and the decision points to achieve these goals for patients with different clinical presentations are discussed.

Expression of the leptin receptor in rat and human nodose ganglion neurones

There is evidence for interactions between leptin and cholecystokinin in controlling food intake. Since cholecystokinin acts on vagal afferent neurones, we asked whether the leptin receptor was also expressed by these neurones. Primers for different forms of the leptin receptor were used in reverse transcriptase-polymerase chain reaction (RT-PCR) of rat and human nodose ganglia. RT-PCR yielded products corresponding to the long (functional) form as well as short forms of the rat leptin receptor. Moreover, RT-PCR revealed the long form of the leptin receptor in a human nodose ganglion. The identities of RT-PCR products were confirmed by sequencing. Primers corresponding to leptin itself did not give RT-PCR products in nodose ganglia. Immunocytochemical studies revealed leptin-receptor immunoreactivity in neuronal cell bodies. Many neurones co-expressed the leptin and cholecystokinin type A receptors, or leptin receptor and cocaine- and amphetamine-related transcript. We conclude that vagal afferent neurones that express the cholecystokinin type A receptor and cocaine- and amphetamine-related transcript, may also express the long form of the leptin receptor providing a neurochemical basis for observations of interactions between cholecystokinin and leptin.

Cochlear implant outcomes and quality of life in the elderly: Manchester experience over 13 years.

Objectives:  To objectively evaluate the clinical and functional outcomes of cochlear implantation in an elderly population.

Cochlear implantation in 53 patients with otosclerosis: demographics, computed tomographic scanning, surgery, and complications.

Objectives: To collect data from a large number of cochlear implant recipients with otosclerosis and to make an assessment of these patients’ clinical characteristics, computed tomographic scans, surgical findings, and complications, and to quantify the occurrence of postoperative facial nerve stimulation. Study Design: Retrospective multicenter study. Patients: Fifty-three patients with otosclerosis from four cochlear implant centers in the United Kingdom and The Netherlands were reviewed. Sixty surgical procedures were performed in these patients: 57 devices were placed in 56 ears. Results: The computed tomographic imaging demonstrated retrofenestral (cochlear) otosclerotic lesions in the majority of patients. Although not statistically significant, the extent of otosclerotic lesions on the computed tomographic scan as categorized in three types tends to be greater in patients with rapidly progressive hearing loss, in patients in whom there is surgically problematic insertion of the electrode array, and in patients with facial nerve stimulation. In four patients, revision surgery had to be performed. Twenty of 53 (38%) patients experienced facial nerve stimulation at various periods postoperatively. Conclusion: Cochlear implant surgery in patients with otosclerosis can be challenging, with a relatively high number of partial insertions and misplacements of the electrode array demanding revision surgery. A very high proportion of patients experienced facial nerve stimulation mainly caused by the distal electrodes. This must be discussed with patients preoperatively.

Predictors of audiological outcome following cochlear implantation in adults.

Abstract The objective of this study was to examine variables that may predict open set speech discrimination following cochlear implantation. It consisted of a retrospective case review conducted in a tertiary referral centre with a cochlear implant programme. The patients were 117 postlingually deafened adult cochlear implant recipients. The main outcome measures were Bench, Kowal, Bamford (BKB) sentence scores recorded nine months following implant activation. The variables studied were age at the time of surgery, sex, duration of hearing loss, aetiology of hearing loss, residual hearing, implant type, speech processor strategy, number of active electrodes inserted. Variables found to have a significant effect on BKB following univariate analysis were entered into a multivariate analysis to determine independent predictors. Multivariate ordinal regression analysis gave an odds ration of 1.09 for each additional year of deafness prior to implantation (confidence interval 1.06–1.13; p<0.001). Duration of deafness prior to implantation is an independent predictor of implant outcome. It accounted for 9% of the variability. Other factors must influence implant performance. Copyright

Botulinum toxin is effective and safe for palatal tremor

Palatal tremor (formerly palatal myoclonus) is an extremely rare, but potentially treatable cause, of objective tinnitus. The tinnitus is thought to be secondary to rhythmic involuntary movements of the soft palate. Its aetiology is variable and it remains difficult to treat. Many different medical and surgical remedies have been tried but none have demonstrated reproducible success. Botulinum toxin has been used in sporadic cases and seems to produce good results. Ten patients with palatal tremor have presented to this department over the last three years. After discussion with the patients with regard to the management of this condition and possible complications, five opted for botulinum toxin therapy and five declined further intervention. Clinical diagnosis was made on the confirmation of soft palate movements synchronous with an audible clicking noise. Five patients underwent botulinum toxin injection into the insertion of the levator and tensor veli palatini muscles. Of the five that were treated with toxin, four showed complete resolution of symptoms after a course of treatment. Only one patient reported transient side effects. This would suggest that botulinum toxin is a safe and effective first line treatment for palatal tremor.

Complications following adult cochlear implantation: experience in Manchester

Cochlear implantation is regarded as a safe and effective treatment for the profoundly deaf. However, a proportion of patients suffer complications after implant surgery. This paper examines the complications encountered in 240 adult cochlear implant operations performed in Manchester between June 1988 and June 2002. Minor complications were defined as those that either settled spontaneously or with conservative management. The total number of minor complications was 61 (25.4 per cent of cases). Non-auditory stimulation, which resolved with implant reprogramming, was present in 53 cases (22.1 per cent). Major complications were defined as those requiring further surgery, explantation or causing a significant medical problem, and occurred in 15 patients (6.25 per cent). These included implant extrusion, implant sepsis, electrode migration, flap-related problems, and persistent non-auditory stimulation. Nine of the 15 patients suffering a major complication required explantation. There were no post-operative deaths, cases of meningitis, nor persistent facial palsies in the series.

Survival times of Provox™ valves

This study was performed to assess the survival times of the Provox valve in the Manchester area. Thirty-nine patients from four hospitals, representing 81 valve failures, were studied. The effects of the timing of the tracheo-oesophageal puncture, previous radiotherapy, and the presence and timing of cricopharyngeal myotomy on valve life were analysed. Regression analysis using an extension of the Cox model to allow strata showed that the lifetime of the first valve only is adversely affected by previous radiotherapy. The other covariates do not have a statistically significant effect on valve survival. The median valve survival is 4.5 months, (range one to 12 months). A small percentage of valve users with particularly frequent valve failures may require additional support and prolonged anti-fungal therapy.

The use of clostridium botulinum toxin in palatal myclonus. A preliminary report

Palatal myoclonus is a rare syndrome characterized by involuntary rhythmical movements of the soft palate giving rise to clicking objective tinnitus. The intrusive nature of the tinnitus prompts patients to seek medical advice but to date no single treatment modality has been shown to be consistently effective. We present three cases in whom various management regimens were unsuccessful and in whom botulinum toxin injection to the palatal muscles was undertaken. All three cases were rendered free of their tinnitus with complete abolition of the myoclonus. The questions of optimum dosage as well as frequency of injection will be answered as greater numbers are treated by this method.