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​BackgroundThe clinical severity of disease in neurofibromatosis type 2 (NF2) is variable. Patients affected with a constitutional truncating NF2 mutation have severe disease, while missense mutations or mosaic mutations present with a milder attenuated phenotype. Genotype-derived natural history data are important to inform discussions on prognosis and management.MethodsWe have assessed NF2 clinical phenotype in 142 patients in relation to the UK NF2 Genetic Severity Score to validate its use as a clinical and research tool.ResultsThe Genetic Severity Score showed significant correlations across 10 measures, including mean age at diagnosis, proportion of patients with bilateral vestibular schwannomas, presence of intracranial meningioma, spinal meningioma and spinal schwannoma, NF2 eye features, hearing grade, age at first radiotherapy, age at first surgery and age starting bevacizumab. In addition there was moderate but significant correlation with age at loss of useful hearing, and weak but significant correlations for mean age at death, quality of life, last optimum Speech Discrimination Score and total number of major interventions. Patients with severe disease presented at a younger age had a higher disease burden and greater requirement of intervention than patients with mild and moderate disease.ConclusionsThis study validates the UK NF2 Genetic Severity Score to stratify patients with NF2 for both clinical use and natural history studies.

VNS is rarely encountered by otolaryngologists; however, stimulus-related laryngopharyngeal side effects of VNS, such as periodic dysphonia, dysphagia and coughing, are not uncommon. 1 At high stimulation amplitude, spastic contractions of ipsilateral intrinsic laryngeal muscles can be observed at laryngoscopy. 1 Stridor has also been reported in a small number of cases; one patient had sleep-related stridor, and others had episodic stridor. 2 3 In all cases once the VNS settings were decreased, the stridor resolved.

We describe the presentation, investigation and challenging management of a young adult with recurrent aseptic meningitis. The presence of cholesterol and triglycerides in the cerebrospinal fluid sample was recognised as a potential sign of an underlying dermoid or epidermoid cyst. We demonstrate how appropriate imaging of the skull base with diffusion-weighted imaging (DWI) MRI can help differentiate between lesions of the petrous apex, facilitating the diagnosis of a petrous apex congenital cholesteatoma/epidermoid cyst. Awareness of this unusual mode of presentation of a rare disease, as well as knowledge of key diagnostic investigations, may allow for earlier identification, treatment and reduced morbidity/mortality.

Anticoagulation with warfarin is commonly used for prevention of thromboembolic events in patients with atrial fibrillation. Bleeding is the main side effect of anticoagulation. We report the case of a 66-year-old man who developed two spontaneous synchronous upper airway haematomas while on warfarin therapy. To our knowledge, this is the first reported case of a sublingual haematoma presenting simultaneously with supraglottic laryngeal haematomas. Upper airway haematomas are rare in the absence of a history of trauma but need to be urgently assessed due to their life-threatening potential. Clinicians should be aware of the possibility of haematomas involving the upper airway in patients on anticoagulant therapy, particularly if complaining of red flag symptoms such as acute onset dysphonia, odynophagia or airway/breathing difficulties.

With the increase in shift pattern work for junior doctors in the NHS, accurate handover of patient clinical information is of great importance. There is no published method that forms the gold standard of handover and there are large variations in practice. This study aims to compare the reliability of three different handover methods. We observed the handover of 12 simulated patients over five consecutive handover cycles between SHOs on a one-to-one basis. Three handover styles were used and a numerical scoring system assessed clinical information lost per handover cycle. After five handover cycles, only 2.5% of patient information was retained using the verbal-only handover method, 85.5% was retained when using the using the verbal with note taking method and 99% was retained when a printed handout containing all patient information was used. When patient information is handed over by the verbal only method, very few facts are retained; therefore, this method should be avoided whenever possible. Verbal handover with note taking is shown to be an effective method of handover in our study, although we accept that this is an artificial scenario and may not reflect the reality of a busy hospital. Nearly all information is retained by the printed handout method but this relies on the handout being regularly updated.

Epistaxis is a common problem. Most patients presenting to hospital will stop bleeding with simple first-aid measures or with nasal packing. Those who do not stop will usually require surgical management. For persistent posterior epistaxis, the sphenopalatine artery may be ligated as the artery leaves the sphenopalatine foramen to enter the nasal mucosa of the lateral wall of the nose. This may be performed endoscopically. We describe the anatomy of the area and the surgical technique. We also present a brief review of the literature on this technique.

We describe the presentation, investigation and challenging management of a young adult with recurrent aseptic meningitis. The presence of cholesterol and triglycerides in the cerebrospinal fluid sample was recognised as a potential sign of an underlying dermoid or epidermoid cyst. We demonstrate how appropriate imaging of the skull base with diffusion-weighted imaging (DWI) MRI can help differentiate between lesions of the petrous apex, facilitating the diagnosis of a petrous apex congenital cholesteatoma/epidermoid cyst. Awareness of this unusual mode of presentation of a rare disease, as well as knowledge of key diagnostic investigations, may allow for earlier identification, treatment and reduced morbidity/mortality.

Anticoagulation with warfarin is commonly used for prevention of thromboembolic events in patients with atrial fibrillation. Bleeding is the main side effect of anticoagulation. We report the case of a 66-year-old man who developed two spontaneous synchronous upper airway haematomas while on warfarin therapy. To our knowledge, this is the first reported case of a sublingual haematoma presenting simultaneously with supraglottic laryngeal haematomas. Upper airway haematomas are rare in the absence of a history of trauma but need to be urgently assessed due to their life-threatening potential. Clinicians should be aware of the possibility of haematomas involving the upper airway in patients on anticoagulant therapy, particularly if complaining of red flag symptoms such as acute onset dysphonia, odynophagia or airway/breathing difficulties.