OBJECTIVE:To determine an ENT undergraduate syllabus.DESIGN:Two round Delphi survey.SETTING:Email questionnaire.PARTICIPANTS:Stakeholders with a vested interest in ENT undergraduate education.MAIN OUTCOMES MEASURED:Mode and median scores for 232 learning outcomes.RESULTS:The individual learning objectives that scored most highly were related to history taking and examination, red flag symptoms, common ENT conditions including all forms of otitis, acute and chronic rhinosinusitis, thyroid disease, pharyngeal infection and airway compromise and formulation of differential diagnoses.CONCLUSIONS:Using a Delphi technique, a structured, evidence-based curriculum has been developed. This should assist those medical schools who do not currently have ENT in their curriculum but wish to reinstate it to produce a high quality teaching programme. It may also assist those medical schools who do have ENT in their curriculum to continue to develop their curriculum.
Objective: There is no consensus for the management of facial nerve schwannomas (FNS). We aim to provide a structured approach to their management based on a large series over a 19-year period. Study Design: Retrospective review. Patients/material and Methods: Overall 28 FNS patients were included in the study. Mean age at diagnosis was 46.4 years (range, 22-73). Mean duration of follow-up was 48 months (range, 3-144). Results: Facial weakness was the presenting symptom in 16 patients (57%). Most patients had multisegment involvement; the internal acoustic canal (IAC) was most commonly affected (68%). Fourteen patients were initially managed conservatively; 14 patients received primary surgical intervention. Two patients failed conservative management due to deterioration in facial nerve function and underwent surgery. No patients opted for stereotactic radiotherapy. Ten patients had primary nerve grafting (HB score outcome III/IV in 7/10 patients after mean follow-up of 34 months) and five patients had a staged VII/XII transposition (HB outcome IV in 2/4 patients and HB outcome V in the other two patients [mean follow-up of 25 months, one patient missing data]). Conclusions: The philosophy of our unit in managing FNS is to preserve facial function wherever possible. There is strong evidence from this study that such an approach is valid given the high proportion of patients who had preservation of facial function over the follow-up period and the small number of tumors that showed significant growth.
Objective: Describe the early experience of bevacizumab treatment in four patients with type II neurofibromatosis.
Objectives: To describe the outcomes from surgery for vestibular schwannomas (VS) following previous irradiation, the technical aspects of surgery, and postoperative facial function.
Objective: Magnetic resonance (MR) imaging is the primary imaging modality used to monitor patients with neurofibromatosis type 2 (NF2). Until recently, it has been contraindicated in auditory brainstem implant (ABI) recipients due to concerns over internal magnet displacement and image distortion effects. This study investigates the use of compression head bandaging and head positioning in minimizing these risks and patients' experiences of them.
Objective: We aim to determine the radiological growth characteristics in recurrent or residual sporadic vestibular schwannomas following surgical resection.
Objective: We present the early experience of bevacizumab treatment for peripheral schwannomas in three patients with either neurofibromatosis type 2 (NF2) or familial schwannomatosis.
Background: We present the results of a retrospective review of children undergoing implantation with bone-anchored hearing aids (BAHAs) at the Great Ormond Street Hospital for Children.Methods: The case notes of 71 children undergoing BAHA placement at the Great Ormond Street Hospital for Children between December 1990 and August 2002 were reviewed. Outcome measures included hearing thresholds, incidence of fixture loss, skin reaction and need for revision. Quality of life outcomes were also measured.Results: Eighty-five ears had been implanted. Fifty-four per cent of children had experienced no complications, 42 per cent had required revision surgery and 26 per cent had experienced fixture loss at some point. Young age at implantation was associated with an adverse outcome. Trauma and failure of osseointegration had been the commonest reasons for failure. A skin reaction around the abutment had occurred at some point in 37 per cent of children but had persisted for longer than six months in only 9 per cent; this had been associated with fixture loss. The use of fixture site split skin grafts had reduced problems with skin hypertrophy and hair overgrowth. Hearing thresholds when using BAHAs had been comparable to those when using bone conduction hearing aids. However, BAHAs had significant additional benefits in terms of sound quality, ease of use and overall quality of life.Conclusion: Bone-anchored hearing aids provide significant benefits over other types of hearing aid, both audiologically and in terms of quality of life. Careful selection of candidates and meticulous follow up are required in order to minimize complications.
The XIth cranial nerve or accessory nerve provides the motor supply to the sternocleidomastoid and trapezius muscles. It is frequently encountered during neck surgery, and as such is at risk of iatrogenic injury, resulting in the 'shoulder syndrome'. Historically, the nerve was sacrificed on oncological grounds during radical neck dissection. However, the basis for sacrifice is unfounded in the majority of cases, and accessory nerve sparing selective neck dissection has equal oncological efficacy. The path of the nerve in the neck is very variable, and there is not a wholly reliable landmark for its identification. However, there are a number of methods described in the literature to guide the surgeon in its identification. This paper provides a systematic review of all the methods available for identification of the accessory nerve, and comments on the reliability of each. In doing so, the detailed anatomy of the accessory nerve is also described.
The use of keels in the treatment of anterior glottic stenosis is well established. A variety of methods for keel fixation have previously been documented. We describe a simple technique of securing a Silastic keel, placed endoscopically, with a simple percutaneous suture, after laser vaporization of an anterior commissure web.
Glomus tumours of the skull base are rare, and most frequently present with symptoms of hearing loss and tinnitus. Diagnosis is often delayed due to the slow growth of the tumour. We describe the previously unreported occurrence of a glomus tumour presenting with a unilateral accessory nerve palsy.
This study prospectively follows changes in quality of life (QoL) of 40 patients undergoing surgical treatment for head and neck malignancy over a 1-year period, using the University of Washington QoL questionnaire version 4.0. The tumour types included oral, oropharyngeal, hypopharyngeal, oesophageal, laryngeal, sinonasal and thyroid carcinomas. Mean overall QoL scores were significantly worse at 3 and 6 months (P < 0.05) and returned to around preoperative scores at 12 months (P = 0.11). Oropharyngeal, laryngeal, sinonasal and thyroid subsites all maintained QoL scores at each time point. QoL scores for hypopharyngeal and oral subsites dropped markedly at 3 and 6 months but returned to around preoperative levels at 12 months. The domains most affected by treatment varied depending on tumour subsite. For the population as a whole, their appearance, activity, speech, shoulder and saliva domains were all significantly less at 12 months. Anxiety scores were significantly better at 12 months.
The tongue base and vallecula are common anatomical sites for impaction of inadvertently swallowed fish bones. Many techniques for their removal have been described but this apparently simple procedure can still pose a surgical challenge. We describe a previously unreported technique using rigid endoscopy with the 4 mm 30 degree Hopkins’ rod as a visual aid for transoral removal of the impacted bone. The technique has proved to be very well tolerated by the patient and more acceptable in terms of morbidity, time and resources than either flexible endoscopy or rigid endoscopy under general anaesthetic.
Objective: To report on the management of laryngoceles by CO2 laser-assisted endoscopic excision. Study Design: A 15-year retrospective study of 12 adult patients (7 men and 5 women) who presented with a laryngocele. Nine patients had an internal laryngocele, one patient had an external laryngocele, and two patients had combined internal-external components. Two laryngoceles were right-sided, six were left-sided, and four were bilateral. Methods: An endoscopic examination of the laryngocele was carried out for both diagnostic and therapeutic purposes. Once identified the air- or mucus-filled cyst (extending from the laryngeal ventricle into the paraglottic space and beyond the thyrohyoid membrane in some cases), the laryngocele was excised in toto, with its surrounding capsule, via endoscopic approach using the CO2 laser. Results: The main presenting symptom was dysphonia in seven patients, visible or palpable mass in the neck in three, and upper airway obstruction in the remaining two. All laryugoceles were treated with endoscopic laser excision of the internal and external components when required. The average postoperative stay in hospital was 1.8 days. Only two of the patients treated had a tracheotomy; both cases presented elsewhere with an emergency airway obstruction, which necessitated tracheotomy. In these two cases, decannulation was subsequently performed. There were no significant complications. The follow-up ranged from 6 months to 5 years. Conclusion: CO2 laser-assisted endoscopic excision of a laryngocele is a quick, precise, and safe alternative to an external approach excision (lateral thyroidotomy, laryngofissure) with fewer complications than its external counterparts, resulting in speedier rehabilitation of both the patient and his or her voice.
This report describes the development of chondroid metaplasia within a fibroepithelial polyp situated on the tongue. Although fibroepithelial polyps in this location are not unusual, chondroid metaplasia in such a lesion in this position has not, as far as the authors are aware, been described previously. The aetiology and the pathogenesis of the lesion are discussed.
Striatal nicotinic acetylcholine receptors with high affinity for nicotinic agonists are involved with the release of a number of neurotransmitters, including dopamine. Previous findings as to whether these receptors are changed in Parkinson's disease and Alzheimer's disease are inconsistent and no previous investigations have focused on these receptors in dementia with Lewy bodies and schizophrenia, which are also associated with disorders of movement. The present autoradiographic study of striatal [3H]nicotine binding in Alzheimer's and Parkinson's diseases, dementia with Lewy bodies and schizophrenia was conducted with particular reference to the potentially confounding variables of tobacco use and neuroleptic medication. [3H]Nicotine binding in both dorsal and ventral caudate and putamen was significantly reduced in Parkinson's disease (43-67%, n=13), Alzheimer's disease (29-37%, n=13) and dementia with Lewy bodies (50-61%, n=20) compared to age-matched controls (n=42). Although tobacco use in the control group was associated with increased [3H]nicotine binding (21-38%), and neuroleptic treatment in dementia with Lewy bodies and Alzheimer's disease was associated with reduced [3H]nicotine binding (up to 29%), differences between neurodegenerative disease groups and controls persisted in subgroups of Alzheimer's disease cases (26-33%, n=6, in the ventral striatum) and dementia with Lewy body cases (30-49%, n=7, in both dorsal and ventral striatum) who had received no neuroleptic medication compared to controls who had not smoked (n=10). In contrast, striatal [3H]nicotine binding in a group of elderly (56-85 years) chronically medicated individuals with schizophrenia (n=6) was elevated compared with the entire control group (48-78%, n=42) and with a subgroup that had smoked (24-49%, n=8). The changes observed in [3H]nicotine binding are likely to reflect the presence of these receptors on multiple sites within the striatum, which may be differentially modulated in the different diseases. Further study is warranted to explore which nicotinic receptor subunits and which neuronal compartments are involved in the changes in [3H]nicotine binding reported, to aid development of potential nicotinic receptor therapy.
Neurofibromatosis is a genetic condition characterized by localized over-growth of mesodermal and ectodermal tissues. Neurofibromatosis type 1 is characterized by cutaneous and skeletal abnormalities. Angiopathy is rare. We describe a new presentation of angiopathy in neurofibromatosis type 1. The patient presented with extensive bruising of the right side of her neck together with intra-oral and pharyngeal submucosal haemorrhage. Radiological investigation confirmed that an aneurysm of the right ascending pharyngeal artery had ruptured. This was successfully embolized without complication.
The human striatum, which receives dopaminergic innervation from the substantia nigra and ventral tegmental area (cell groups A8, A9 and A10), has structural and functional subdivisions both rostrocaudally and dorsoventrally. These relate to motor and non-motor origins of cortical projections and the specific areas of the substantia nigra and ventral tegmental area providing dopaminergic innervation. In the present study, we have evaluated the distribution of a number of dopaminergic parameters in the caudate, putamen and nucleus accumbens at separate coronal levels in a post mortem study in a series of elderly normal individuals aged 55–94 years, with analysis of the effect of post mortem variables. Dopamine D1 receptor density displayed a rostrocaudally declining gradient in the putamen but not in the caudate, such that at levels posterior to the anterior commisure, there was significantly lower D1 binding in the putamen compared to the caudate. The density of dopamine D2 receptors was similar in the putamen and caudate, increasing rostrocaudally. The density of dopamine uptake sites exhibited an increasing rostrocaudal gradient in the caudate, especially ventrally, but not in the putamen, where binding was more constant. The dopamine D3 receptor was concentrated in the ventral striatum, particularly the nucleus accumbens, although there was no evidence of a rostrocaudal gradient. With respect to striosome–matrix compartmentalization, there was no complete segregation, although D1 and D3 receptors were concentrated in striosomes, whereas D2 receptors and uptake sites showed higher density in the matrix. Levels of dopamine were similar in the caudate and putamen, and were significantly elevated at levels including the nucleus accumbens and the anterior commisure. Homovanillic acid and the metabolic index (homovanillic acid/dopamine ratio) were significantly higher in the putamen compared to the caudate, especially at levels from and caudal to the anterior commisure. These distributions of dopamine receptors and metabolic indicators, reflecting the different functional domains of the striatum, are relevant to the interpretation of current in vivo imaging of the dopamine transporter and receptors in neurological and psychiatric disorders. They provide information to assist in the detection of perturbations in expression, in specific diseases, at particular points on rostrocaudal, lateromedial and dorsoventral axes, a level of resolution beyond current neuroimaging capability.
Dementia with Lewy bodies (DLB) is a neuropsychiatric disease associated with extrapyramidal features which differ from those of Parkinson's disease, including reduced effectiveness of L-dopa and severe sensitivity reactions to neuroleptic drugs. Distinguishing Alzheimer's disease from DLB is clinically relevant in terms of prognosis and appropriate treatment. Dopaminergic activities have been investigated at coronal levels along the rostrocaudal striatal axis from a post-mortem series of 25 DLB, 14 Parkinson's disease and 17 Alzheimer's disease patients and 20 elderly controls. [3H]Mazindol binding to the dopamine uptake site was significantly reduced in the caudal putamen in DLB compared with controls (57%), but not as extensively as in Parkinson's disease (75%), and was unchanged in Alzheimer's disease. Among three dopamine receptors measured (D1, D2 and D3), the most striking changes were apparent in relation to D2. In DLB, [3H]raclopride binding to D2 receptors was significantly reduced in the caudal putamen (17%) compared with controls, and was significantly lower than in Parkinson's disease at all levels. D2 binding was significantly elevated at all coronal levels in Parkinson's disease compared with controls, most extensively in the rostral putamen (71%). There was no change from the normal pattern of D2 binding in Alzheimer's disease. The only significant alteration in D1 binding ([3H]SCH23390) in the groups examined was an elevation (30%) in the caudal striatum in Parkinson's disease. There were no differences in D3 binding, measured using [3H]7-OH-DPAT, in DLB compared with controls. A slight, significant decrease in D3 binding in the caudal striatum of Parkinson's disease (13%) patients and an increase in Alzheimer's disease (20%) in the dorsal striatum at the level of the nucleus accumbens were found. The concentration and distribution of dopamine were disrupted in both DLB and Parkinson's disease, although in the caudate nucleus the loss of dopamine in DLB was uniform whereas in Parkinson's disease the loss was greater caudally. In the caudal putamen, dopamine was reduced by 72% in DLB and by 90% in Parkinson's disease. The homovanillic acid : dopamine ratio, a metabolic index, indicated compensatory increased turnover in Parkinson's disease, which was absent in DLB despite the loss of substantia nigra neurons (49%), dopamine and uptake sites. These differences between DLB, Parkinson's disease and Alzheimer's disease may explain some characteristics of the extrapyramidal features of DLB and its limited response to L-dopa and severe neuroleptic sensitivity. The distinct changes in the rostrocaudal pattern of expression of dopaminergic parameters are relevant to the interpretation of the in vivo imaging and diagnosis of DLB.
Changes in the number of high-affinity nicotine binding sites have been widely reported in specific regions of the human brain during aging and in degenerative neurological diseases associated with aging, such as Alzheimer's disease. Nicotinic receptors are highly diverse and a description of the molecular subtypes affected in such conditions has not been achieved to date. To investigate the status of the alpha3 subunit-containing subtypes in such conditions, we assessed by in situ hybridisation the alpha3 mRNA density in the hippocampus, entorhinal cortex and thalamus of Alzheimer's patients and age-matched controls. No significant difference in the expression of the alpha3 mRNA, either qualitative or quantitative, was found between Alzheimer's individuals and controls in any of the analysed areas. This result suggests that the nicotine binding changes occurring in these areas in Alzheimer's patients are not correlated to a variation of the alpha3 mRNA in the same regions. Nevertheless, a negative correlation between the alpha3 mRNA density and the age was observed in the entorhinal cortex of both the Alzheimer's and the normal subjects, suggesting a potentially extensive decay of the alpha3-expressing neurons or loss of alpha3-containing receptors in intact neurons of the entorhinal cortex in the late elderly.