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The objective of this study was to develop a reliable, validated disease-specific score measuring quality of life (QOL) in clinical practice and treatment trials in Neurofibromatosis 2 (NF2) individuals. In NF2 patients, qualitative interviews (n = 15) and a focus group session (n = 30) generated items for a pilot questionnaire. This was tested and refined (n = 20). The final version (NFTI-QOL) was validated (n = 50) with two generic QOL questionnaires (SF-36 and EuroQOL). The NFTI-QOL was also administered to patients with solitary vestibular schwannoma (SVS) (n = 30) and normal controls (n = 30). The participants were NF2 patients, SVS patients, and normal controls. NFTI-QOL score, SF-36 score, and EuroQOL score were the main outcome measures. Mean NFTI-QOL score was 9.4 (range: 0 to 20, maximum possible score = 24). The NFTI-QOL score correlated strongly with EuroQOL (r = 0.71, p < 0.001) and SF-36 (r = 0.81, p < 0.001). NF2 individuals were significantly worse than the SVS patients, who in turn were worse than the controls on the NIFTI-QOL. The NFTI-QOL showed good internal reliability (Cronbach's α = 0.87). We developed an eight-item disease-specific QOL score for NF2 patients, validated against SF-36 and EuroQOL. It correlated strongly with clinician-rated disease severity in NF2, with better correlation than the SF-36 in this regard.

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There are anecdotal reports of neurofibromatosis 2 (NF2) patients and vascular disease, but no previous studies have compared blood pressure (BP) in people with NF2 and in the general population. This study is the first to show that BP is significantly higher in patients with NF2 than in matched control patients. This is important for the management of patients with NF2, and in the differential diagnosis of secondary hypertension.

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With the emergence of three effective management options for vestibular schwannoma and the drastic reduction in mortality rate, the last two decades have seen increasing attention being paid to health-related quality of life. The vast majority of quality of life studies have been retrospective. We prospectively assessed quality of life of vestibular schwannoma patients before and after conservative or microsurgical management. We performed a prospective observational study conducted at a tertiary referral center between October 2001 and October 2003. Patients were divided into two groups: conservative management and microsurgery. Quality of life was assessed using the Medical Outcome Study 36-Item Short Form (SF-36) and Glasgow Benefit Inventory (GBI). The questionnaires were administered at initial assessment, 1 month, 3 months, and 6 months in both groups. Thirty-three patients completed the study, 18 in the conservative group and 15 in the microsurgical group. One month after microsurgery, SF-36 scores were significantly reduced within three of eight domains; however, 3 months after microsurgery, no significant difference existed in patients' scores on any of the SF-36 domains compared with preoperatively, and at 6 months there was a significant improvement in one domain compared with preoperatively. There was no significant difference in overall quality of life alteration (GBI total score) between microsurgery and conservative management. The improved quality of life of patients 6 months after microsurgery (relative to preoperatively, and in comparison with an age- and sex- matched population) is a new finding that has not been previously documented in the literature.

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We compared the use of glycerol and icthammol (G&I) ribbon gauze versus topical antibiotic and steroid drops in the management of otitis externa. Patients presenting with acute otitis externa were systematically recruited from attendance in the ENT emergency clinic. Exclusion criteria were age <18 years, diabetic patients, and those who had been previously treated for otitis externa by the department within the last month. A total of 43 patients were recruited with 48 infected ears, the mean patient age was 47 years, (range 18-80 years). Patients were randomised to either insertion of a G&I ribbon gauze or topical ear drops by means of a ballot with no stratification. All patients were reviewed 5 days later. Patients were asked to comment on their pain using the ten point visual analogue pain score. Oedema of the canal wall, presence of debris and visibility of the tympanic membrane were graded on microscopy. Patient satisfaction and return to work were assessed. There was a statistically significant decrease in the pain score between each visit for the entire group of patients and decrease in canal wall swelling (P < 0.001). There was no significant difference between drops and ribbon gauze in terms of pain relief, canal wall oedema, or aural discharge. Following microbiological analysis, 27.8% of bacteria were resistant to neomycin. The cost of treatment with G&I is less than a third of that for topical antibiotic-steroid drops and requires less patient compliance. Patient concerns with the ribbon gauze included its cosmetic appearance and loss of hearing and with drops included frequency of application. We recommend the use of G&I ribbon gauze as first-line treatment, particularly for those who have had allergies to carriers of drops, those that are poorly compliant or with poor manual dexterity, for example patients with rheumatic hands.

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The Tullio phenomenon is an extremely rare condition in which loud noise induces a brisk vestibular response. Osteomas of the middle ear cleft are also uncommon. We report a patient with an osteoma of the middle ear cleft that became symptomatic with progressive facial palsy and mixed hearing loss. The onset was heralded by the Tullio phenomenon, which she had experienced for 10 years. The differential diagnosis of the Tullio phenomenon and the management of middle ear osteomas are discussed.