A direct electron detector for time-resolved MeV electron microscopy.

The introduction of direct electron detectors enabled the structural biology revolution of cryogenic electron microscopy. Direct electron detectors are now expected to have a similarly dramatic impact on time-resolved MeV electron microscopy, particularly by enabling both spatial and temporal jitter correction. Here we report on the commissioning of a direct electron detector for time-resolved MeV electron microscopy. The direct electron detector demonstrated MeV single electron sensitivity and is capable of recording megapixel images at 180 Hz. The detector has a 15-bit dynamic range, better than 30-µm spatial resolution and less than 20 analogue-to-digital converter count RMS pixel noise. The unique capabilities of the direct electron detector and the data analysis required to take advantage of these capabilities are presented. The technical challenges associated with generating and processing large amounts of data are also discussed.

The first reported treatment of Nager syndrome associated hearing loss with bone-anchored hearing aids: case report.

OBJECTIVE: To report the first case of treatment of Nager syndrome associated conductive hearing loss with bone-anchored hearing aids, in a three-year-old boy. METHOD: Clinical case report and current literature review regarding the use of bone-anchored hearing aids in the treatment of conductive hearing loss in children. RESULTS: A three year eight month old boy with Nager syndrome was successfully treated for conductive hearing loss using bilateral bone-anchored hearing aids. CONCLUSION: This is the first case report of the use of bone-anchored hearing aids to treat Nager syndrome associated conductive hearing loss. Treatment was safe and successful in this case.

Bone-anchored hearing aids in children and young adults: the Freeman Hospital experience.

OBJECTIVE: To investigate the utilisation of bone-anchored hearing aids and Softband, as well as the effects on quality of life, amongst the paediatric and young adult population of Freeman Hospital, Newcastle Upon Tyne, UK. METHOD: Retrospective, anonymised, cross-sectional survey using the Glasgow Benefit Inventory and Listening Situation Questionnaire (parent version), administered at least three months following the start of bone-anchored hearing aid or Softband use. RESULTS: One hundred and nine patients were included, of whom syndromic children made up a significant proportion (22 of 109). Patients using bone-anchored hearing aids obtained significant educational and social benefit from their aids. The mean Listening Situation Questionnaire difficulty score was 17 (15 patients), which is below the trigger score of 22+ at which further reassessment and rehabilitation is required. 87% (of 15 patients) did not require further intervention. The overall mean GBI score for the 22 patients (syndromic and non-syndromic) was +29. CONCLUSION: The use of bone-anchored hearing aids and Softband results in significant improvements in quality of life for children and young adults with hearing impairment. There is significant under-utilisation of bone-anchored hearing aids in children with skull and congenital abnormalities, and we would advocate bone-anchored hearing aid implantation for these patients.

Bone-anchored hearing aid modified with directional microphone: do patients benefit?

BACKGROUND: Bone-anchored hearing aids are well established in the treatment of patients with a conductive or mixed hearing loss. However, one of the main problems is that of sound localisation. This can be improved with a directional microphone. This study compared the quality of life of bone-anchored hearing aid wearers before and after the use of a directional microphone. METHOD: Eleven patients were included. They were required to wear the directional microphone for 12 weeks. Quality of life was measured using the Glasgow benefit inventory questionnaire, before and after the study period. RESULTS: The response rate was 82 per cent. The total benefit from the directional microphone was +49.7. The three components of the Glasgow benefit inventory were analysed separately, as follows: general subscale +57.4; physical health +42.6; and social scale +25.9. CONCLUSION: This is the first study to demonstrate a significant improvement in quality of life from a directional microphone fitted to a bone-anchored hearing aid.

Magnetic resonance imaging: is a single scan ever enough for the diagnosis of acoustic neuroma?

A patient presented with unilateral, right-sided hearing loss and tinnitus and underwent gadolinium-enhanced magnetic resonance imaging (MRI). A pure tone audiogram showed a right-sided sensorineural hearing loss. The MRI scan was initially negative but when repeated seven years later, following a further deterioration of symptoms, it showed a 2 mm, right-sided acoustic neuroma. This case has great potential significance for the diagnosis of acoustic neuroma, and it may raise medico-legal issues regarding the exclusion of this diagnosis. The case illustrates that a single negative scan may not be adequate if pure tone audiograms show deterioration in hearing loss.

Intratympanic gentamicin for Ménière's disease: effect on quality of life as assessed by Glasgow benefit inventory.

OBJECTIVES: To evaluate patients' quality of life after receiving intratympanic gentamicin as treatment for Ménière's disease. DESIGN: Retrospective study using the Glasgow benefit inventory scale (GBI) questionnaire. PARTICIPANTS: Patients diagnosed with Ménière's disease, according to the American Academy of Otolaryngology-Head and Neck Surgery (AAO-HNS) diagnostic criteria (refractory to medical management), who were treated with intratympanic gentamicin injection. MATERIALS AND METHODS: This retrospective study included all patients diagnosed with Ménière's disease (according to AAO-HNS criteria; refractory to medical management) undergoing gentamicin labyrinthectomy in our unit over the preceding three years. Other causes of hearing loss were ruled out as all patients underwent magnetic resonance imaging, electronystagmography and sway magnetometry. Patients underwent gentamicin infiltration at a concentration of 2 ml of 30 mg/ml and were reviewed six weeks later and received a repeat injection if no benefit was evident. Six months after their last follow up, they were sent a GBI questionnaire. Twenty-one questionnaires were posted, and the response rate was 81 per cent. RESULTS: As per the responses received, the total benefit of intratympanic gentamicin injection was found to be +30.3. The three components of GBI were analysed separately; it was found that general benefit was greatest (+33.3), followed by physical benefit (+28.1) and social benefit (+21.6). CONCLUSION: This study suggests that gentamicin definitely improves quality of life in patients with Ménière's disease and should be the first line of treatment if medical management fails.

Cochlear implantation in patients with MELAS syndrome.

MELAS syndrome (mitochondrial myopathy, encephalopathy, lactic acidosis and stroke-like episodes) is a rare congenital disorder of mitochondrial DNA (mt-DNA). Patients with this syndrome may present to the otolaryngologist with sensorineural hearing loss (SNHL) that is genetic in origin. Mitochondrial cytopathies can present with a variety of symptoms, but they occasionally present with SNHL as their first manifestation. Two cases of MELAS patients who responded well to cochlear implantation are presented. A review of the literature is also carried out focusing mainly on diagnosis, anesthetic considerations and management of these patients.

The effect of otitis media with effusions on balance in children.

There is increasing interest in the effect, otitis media with effusions (OME) has on the balance in children. The aim of our investigation was to determine whether a universal effect on balance could be demonstrated in children with OME by using sway posturography. Assessment was made in 20 children with proven OME before and after the insertion of bilateral ventilation tubes. Sway posturography was performed on each occasion in each of the four recording conditions in the presence and absence of both reduced optic fixation and reduced proprioception. The pathlength traversed during the recording interval was measured and analysed by a split-unit anova. The results demonstrate an overall improvement in mean pathlength of 20% following treatment of the effusions (P < 0.001) (95% CI 14-25%). The effect of reduced optic fixation and of reduced proprioception were similar when increasing the mean pathlength by 22% (P < 0.001). These results demonstrate that OME has a universal effect on balance in all recording conditions.

The MELAS syndrome. Review of the literature: the role of the otologist.

The mitochondrial myopathy, encephalopathy, lactic acidosis and stroke-like episodes (MELAS) syndrome is a rare congenital disorder of mitochondrial DNA (mt-DNA). Patients with this syndrome may present to the otolaryngologist with sensorineural hearing loss (SNHL), which is genetic in origin. A high index of suspicion is required because this hearing loss is part of a syndrome for which early diagnosis and intervention is required.

Acute epiglottitis: a presentation of haemophagocytic lymphohistiocytosis.

The incidence of acute epiglottitis in children has declined with the introduction of the Haemophilus influenzae b vaccine in 1992. We report a case of acute epiglottitis in a child secondary to an immunocompromised state. We suggest that when acute epiglottitis is diagnosed in a child we should ensure there is no underlying predisposing condition.

Bone-anchored hearing aid quality of life assessed by Glasgow Benefit Inventory.

OBJECTIVE/HYPOTHESIS: The bone-anchored hearing aid (BAHA) is a well established mode of treatment and many studies show the audiological benefit, but none has assessed the benefit to the quality of life of patients. This study uses the validated Glasgow Benefit Inventory to quantify the changes in quality of life. STUDY DESIGN: Retrospective questionnaire study. METHODS: Sixty consecutive patients receiving treatment with BAHA were enrolled in the study. The male/female ratio was 1.26 to 1; mean patient age was 45 years. The most common indication was hearing loss secondary to mastoid disease and surgery followed by congenital atresia and chronic discharge from the ear. RESULTS: The response rate was 85%, which is high and adds weight to the results. The general benefit score was +34 (range, +27-+48), which is comparable to middle ear surgery but just below benefit from cochlear implantation. The social benefit was +21 (range, +12-+37) with only +10 (range, +2-+26) for the physical score. This pattern mirrors that reported for other ear interventions. Maximum benefit was noted in patients with congenital atresias followed by discharging mastoid cavities. CONCLUSION: This study is the first to demonstrate significant quality of life benefit from BAHA surgical intervention as measured by the Glasgow Benefit Inventory.

Gustatory sweating of the external auditory canal.

Gustatory sweating of the external auditory canal is extremely rare. A clinical case, that is only the second in the English literature, is presented. The potential pathogenesis and its treatment options are discussed.

Idiopathic diffuse erosion of the skull base presenting as cerebrospinal fluid rhinorrhoea.

We present an unusual case of generalized erosion of the skull base. We have not found a similar case reported in the world literature. The presenting symptom was spontaneous cerebrospinal fluid (CSF) rhinorrhoea which arose from a bony defect associated with herniation of the right temporal lobe into the sphenoid sinus. We discuss the management of such a case including imaging of the skull base and the endoscopic repair of the bony defect.

Chondroma of the external auditory canal.

Chondroma, a benign tumour which is common at many sites in the body, has not been reported in the English literature in the bony external auditory canal. The first such case at this anatomical site is described and the pathogenesis is considered.

Surgical aspects of cochlear implantation in young children: a review of 115 cases.

A review of the surgical complications of the first 115 patients who received a cochlear implant in this program demonstrate a negligible complication rate in the perioperative period and a low complication rate in the long term. The surgical technique is described in detail and the reasons for this low complication rate are analysed.

Aberrant common carotid artery encountered during free jejunal graft repair of oesophageal stricture.

The origin and course of the carotid arterial system in the superior mediastinum and neck are remarkably constant. Although variations are rare, they can have important implications in certain clinical problems. In this report, a patient underwent free jejunal graft repair of a cervical oesophageal stricture caused by radiotherapy for a post-cricoid carcinoma. The common carotid artery on the side of the surgical approach was found to cross the midline to the other side of the neck, and created difficulties both with the procedure and future management. This has not been previously reported.

Compositional differences between bilateral middle ear effusions in otitis media with effusion: evidence for a different etiology?

The aim of this study was to clarify the site of primary pathology in otitis media with effusion. Effusions were collected from 64 children with bilateral effusions at the time of myringotomy. The rheological properties and biochemical compositions of effusions were measured for 23 pairs of effusions, and the levels of the inflammatory mediators TNF alpha, IL-1beta, and IL-8 were measured in 41 pairs using specific enzyme-linked immunosorbent assays (ELISAs). Measurements from paired ears were compared using analysis of variance (ANOVA) tests and significant differences were found for reduced specific viscosity, mucin content, protein content, and levels of IL-8. The results demonstrate that the two ears have different immunological processes or rates of processes which might explain the significantly different rheological properties of effusions. This suggests that each ear undergoes pathological changes independently and has implications for using the opposite ear as a control in clinical trials.

Susac's syndrome: a rare cause of fluctuating sensorineural hearing loss.

Susac's syndrome is extremely rare and is characterized by the clinical triad of encephalopathy, retinal artery occlusion and deafness. A case of this rare syndrome is presented and for the first time the fluctuating nature of the hearing loss is demonstrated. The site and nature of the hearing loss has been investigated. The value of neural hearing loss as a method of monitoring the clinical severity is discussed.

Middle ear infection and sixth nerve palsy.

Three cases of patients with acute middle ear infections complicated by a sixth nerve palsy are described. The possible causes of sixth nerve palsy in such cases are discussed. We propose that where there is a sixth nerve palsy without petrositis and without raised intracranial pressure, it can arise as a result of spreading phlebitis along the inferior petrosal sinus from the lateral sinus.