Wegener's granulomatosis is generally a chronic, indolent, inflammatory condition, treated with cytotoxics (cyclophosphamide) and corticosteroids.

This paper reports an unusual case of acute fulminant Wegener's granulomatosis that failed to respond to conventional treatment, but showed a dramatic response to rituximab, which is a relatively new form of treatment for resistant cases.

As well as describing the case (with photographic illustrations), the current paper provides a review of the literature, focusing on acute Wegener's granulomatosis and frequency of resistance to ‘conventional’ forms of treatment. There is also an evaluation of the evidence for the effectiveness of rituximab in resistant Wegener's granulomatosis.

The patient responded remarkably well to rituximab and had no disease recurrence at 24 months' follow up.

Clinicians should be aware of the acute fulminant form of Wegener's granulomatosis, as a delay in diagnosis and treatment may have fatal consequences. The paper also highlights the dramatic response to rituximab experienced by the patient.

To review the safety of thyroidectomy combined with cervical neck dissection without drainage, in patients with papillary thyroid carcinoma.

Two groups were defined depending on whether cervical neck dissection was or was not performed (groups one and two, respectively).

Group one included 153 patients with central neck dissection and 52 patients with central and lateral neck dissection. Group two included 121 patients. Post-operative drainage was not used in either group. Overall, 17 patients (5 per cent) developed post-operative haematoma and/or seroma: 12 patients (6 per cent) in group one and 5 patients (4 per cent) in group two. There were no major bleeding episodes; only minor bleeding or seroma was encountered, not requiring surgical intervention. Overall, 91 per cent of patients had a post-operative stay of 1 day. The number of peri-operative local complications and length of stay did not differ significantly between the two groups.

Thyroidectomy plus cervical neck dissection without drainage is safe and effective in the treatment of papillary thyroid carcinoma.

Bilateral sensorineural hearing loss associated with recurrent urticarial skin lesions may be signs of underlying Muckle–Wells syndrome. Previous reports have described the hearing loss to be progressive in nature.

To our knowledge, this paper presents the first published case of sudden onset, bilateral sensorineural hearing loss associated with urticarial vasculitis due to underlying Muckle–Wells syndrome.

The patient underwent a cochlear implantation with a modest outcome.

Cochlear implantation may help to rehabilitate sudden hearing loss associated with this condition, but early diagnosis may allow treatment with interleukin-1β inhibitors such as anakinra.

Myopericytoma is a relatively recently described skin and soft tissue tumour that demonstrates perivascular myoid cell or pericytic differentiation. Whilst the range of anatomical locations has expanded to include visceral locations, head and neck myopericytomas are rarely documented. There have been no previous reports of aural myopericytoma.

This paper reports the clinicopathological features of a biopsy-proven, slow-growing, 20 × 20 mm, polypoid myopericytoma that involved the external auditory canal and tragus in an 18-year-old woman. Excision was curative.

Heightened clinicopathological awareness of the expanding anatomical distribution of myopericytoma is critical to its diagnosis when it presents in unusual and novel locations. Myopericytoma should be added to the range of external auditory canal neoplasms, especially those characterised by an admixture of spindle cells and a prominence of blood vessels, including those with a haemangiopericytomatous pattern.

The tensor tympani muscle is the largest muscle within the middle ear. Its voluntary contraction is a very unusual event. Only a few papers have documented its audiometric effects.

To report an unusual case of voluntary tensor tympani muscle contraction and describe its audiometric effects.

A 27-year-old man, who presented complaining of voluntarily evoked bilateral tinnitus, was found to be able to voluntarily contract the tensor tympani muscle in both ears simultaneously. Audiograms were performed under conditions of rest and maximal contraction of the tensor tympani muscle. The most remarkable effects were conductive hearing loss at lower frequencies and an increase in middle-ear impedance.

The importance of the tensor tympani muscle in middle-ear physiology remains unclear. It has been related to the attenuation of sounds produced during the mastication process. Voluntary control over the tensor tympani muscle is an extremely rare event. However, an understanding of the potential audiometric effects of its contraction could aid the diagnosis of hearing disorders.

This paper reports a case of Dirofilaria repens infection in a patient who presented with an anterolateral neck swelling. Dirofilaria repens infection of the neck region is rare even in countries where dirofilarial infestation is endemic. The diagnosis is made by identifying the worm in surgical or pathological specimens.

A 47-year-old man presented with an 8-week history of non-tender, right-sided, lower anterolateral neck swelling and weight loss. An ultrasound scan showed a cystic lesion containing a living worm. The cyst was excised and the patient showed full recovery at follow up.

To our knowledge, there has been no previous report of an anterolateral neck swelling secondary to Dirofilaria repens infection in Europe. Our case is unusual because of the rarity of Dirofilaria repens presenting as a neck swelling.

To study the outcome of endonasal endoscopic surgery for adenoid cystic carcinoma of the sinonasal tract over a five-year follow-up period.

Retrospective analysis.

Four consecutive patients with adenoid cystic carcinoma of the sinonasal tract, who had undergone endonasal endoscopic surgery, were reviewed regarding age at diagnosis, sex, primary site, tumour-node-metastasis staging, treatment modalities, histopathological findings, duration of follow up, distant metastases and treatment outcome.

All patients were diagnosed at an advanced stage and had post-operative adjuvant radiotherapy. Three patients underwent endoscopic endonasal resection and one endoscopic assisted craniofacial resection. The most common primary site was the ethmoid sinus (three patients). Three patients had no evidence of recurrence. One patient who had undergone partial clearance via endoscopic endonasal resection developed cervical node metastases a year after treatment; this patient also developed distant metastases.

Adenoid cystic carcinoma is difficult to treat. Sinonasal tract tumours can be resected via endoscopic endonasal resection or endoscopic assisted craniofacial resection, but prolonged follow up is advisable. Radiotherapy is an important adjuvant treatment.

A spontaneous cerebrospinal fluid leak can sometimes only become apparent following grommet insertion and usually represents dehiscence of the tegmen tympani, which is an uncommon condition.

This report aimed to reaffirm the importance of recognising this unusual presentation and outline management options.

A 63-year-old man with conductive hearing loss and type B (flat) tympanometry underwent grommet insertion into his left ear, which resulted in cerebrospinal fluid otorrhoea. A defect of the tegmen tympani was found. This was successfully repaired via a transmastoid approach using a multi-layered grafting technique.

Dehiscence of the tegmen tympani is uncommon and may only come to light following grommet insertion, which may be problematic for the uninformed otolaryngologist. Education is important to ensure early recognition and appropriate management.

We report a rare case of concurrent myeloid sarcoma and acute fulminant invasive fungal sinusitis in a patient with relapsed acute myeloid leukaemia.

A 73-year-old man was diagnosed with acute myeloid leukaemia and developed relapse one year later. After two courses of azacytidine, he began suffering from a dull pain in the left temporal and orbital regions. Sinus computed tomography showed a localised lesion in the left ethmoid sinus, which rapidly progressed to an extensive intracranial mass within one month. Surgical debridement was performed, and histopathological analysis revealed the coexistence of myeloid sarcoma and acute fulminant invasive fungal sinusitis. The patient responded well to prompt surgical debridement, antifungal medication and radiotherapy.

Coexistence of sinonasal myeloid sarcoma and acute fulminant invasive fungal sinusitis poses an urgent diagnostic and management challenge to clinicians. Timely recognition of this rare comorbid condition is warranted as application of appropriate treatment can save lives.

We describe a rare case of sudden onset of unilateral sensorineural hearing loss occurring as the first symptom of moyamoya disease, which is characterised by progressive stenosis of the intracranial internal carotid arteries and their proximal anterior cerebral arteries and middle cerebral arteries.

Case report and review of the world literature regarding moyamoya disease with hearing loss.

The reported patient had moyamoya disease that initially presented as sudden, unilateral sensorineural hearing loss. Magnetic resonance imaging showed occlusion of the anterior cerebral, middle cerebral and distal internal carotid arteries bilaterally. The possible mechanism of this patient's sudden sensorineural hearing loss may have been vascular occlusion resulting from thrombotic narrowing or blockage by plaque.

The described patient represents the first reported case of sudden onset, unilateral sensorineural hearing loss occurring as the first symptom of moyamoya disease. The possibility of a vascular lesion such as moyamoya disease should be considered in patients with sudden sensorineural hearing loss, especially children, young adults and Asian patients. Due to this disease's poor outcome, early diagnosis and treatment are important to prevent stroke.

Rhinosporidiosis primarily affects the mucous membranes of the nose and nasopharynx. The disseminated form of this chronic fungal disease is extremely rare.

The authors present a case of disseminated rhinosporidiosis in an immunocompetent patient with involvement of the skin, subcutaneous tissue, muscle, bone, penis and urethra, and with a long-standing primary lesion in the nose.

A late or atypical presentation of rhinosporidiosis may cause diagnostic dilemma. Fine needle aspiration cytology of the tumoural lesions may establish the diagnosis. Histopathology is confirmatory. The subcutaneous masses may be solid or cystic. Ulceroproliferative lesions need to be differentiated from malignancies.

This is the first reported case of truly disseminated rhinosporidiosis with simultaneous involvement of multiple anatomically unrelated sites in a single patient. This is also the first reported case of cystic rhinosporidiosis. The possibility and sequelae of spontaneous regression of rhinosporidiosis are also discussed.

We report the rare case of a 55-year-old woman with an asymptomatic left sphenoid sinus pseudomeningocele mimicking a mucocele.

A 55-year-old woman was found to have an incidental mass in the left sphenoid sinus on computed tomography. Magnetic resonance imaging confirmed the presence of a mucocele. A left endoscopic sphenoidotomy was performed to drain the mucocele. Despite an anatomical puncture through a stenosed sphenoid ostium, alarmingly, the opening leaked cerebrospinal fluid. A dehiscent lateral wall was identified with a dural opening communicating with the sphenoid sinus. This was immediately repaired with a free nasal septal mucosal graft. Six months post-operatively, the patient remained free from cerebrospinal fluid leakage.

Pseudomeningoceles are extremely rare in the absence of trauma or iatrogenic injury. Surgeons should be alert to their presence as they can mimic a unilateral mucocele or nasal polyp. Endoscopic management lends itself to a single-stage repair.

The key to avoiding damage to the horizontal facial nerve in middle-ear surgery is to formally identify the nerve in the early stages of the procedure.

In the non-infected ear this can be achieved relatively easily by identifying the oval window niche. However, in the infected ear with cholesteatoma, the safest landmark to use is the processus cochleariformis, which can be identified by three different methods.

In an infected ear that is full of granulation tissue and/or cholesteatoma, the horizontal facial nerve can be reliably identified by locating the processus cochleariformis using the three methods described. This avoids damage to the nerve and important structures around it.

The surgical trainee has to acquire surgical skills in an era of reduced training hours and greater demands for efficient use of operating theatre time. Many surgical specialties are utilising model and simulation-based training to provide safe, low-pressure training opportunities for today's trainee.

This paper describes a simple, relatively inexpensive tonsillectomy model that enables the practice of tonsil removal and ligation of bleeding vessels. The model is beneficial for the patient, trainee and trainer.

The pseudo mouth and active bleeding components of this model provide the trainee with a relatively inexpensive, realistic model with which to gain confidence and competence in the skill of ligating tonsillar blood vessels with a tonsil tie.

Septoplasty is one of the most common otolaryngological operations. It is often dismissed as a simple procedure, despite the wide range of potential complications. We describe the first reported case of unilateral hemiplegia as a complication of septoplasty.

A 51-year-old man presented with right hemiplegia following a septoplasty and turbinoplasty procedure carried out elsewhere. Cranial imaging showed a breakthrough fracture of the left sphenoid sinus anterior wall and clivus, with a haemorrhagic area in the left paramedian pons, which was responsible for the patient's right hemiplegia. Despite neurological and physiotherapeutic rehabilitation, the patient gained only partial recovery from his right hemiplegia.

Good intra-operative visualisation and appropriate surgical technique are essential to prevent complications and achieve a functional nasal airway. The importance of the presented case to the pre-operative informed consent process is underlined.

To establish the diagnostic adequacy of ultrasound-guided fine needle aspiration cytology samples at the East Berkshire neck lump clinic, and to perform a cost-benefit analysis related to the hypothetical addition of an on-site cytology technician (required to review fine needle aspiration specimen adequacy).

The adequacy of all ultrasound-guided fine needle aspiration procedures was reviewed from 1 January to 30 June 2011. These results were used in the cost-benefit analysis related to on-site cytology assessment.

Of the 307 ultrasound-guided fine needle aspiration cytology procedures performed over 6 months, 67 (22 per cent) were reported to be non-diagnostic. Operator experience was found to correlate significantly with diagnostic adequacy (p < 0.001). Only 5 per cent of all fine needle aspirations were initially non-diagnostic but diagnostic on repeat sampling. This suggests that the financial and time costs of on-site fine needle aspirate adequacy assessment would outweigh any benefit.

In this series, the experience of individuals performing fine needle aspirations was the most important factor related to adequacy.

To introduce pseudoaneurysm of the sphenopalatine artery as the possible aetiology of acute massive epistaxis in patients with a history of orthognathic surgery accompanied by Le Fort I osteotomy.

Case report and literature review.

This paper reports a case of acute life-threatening epistaxis following Le Fort I osteotomy. Computed tomography and angiography showed a pseudoaneurysm of the sphenopalatine artery, which was successfully treated by endovascular embolisation.

Although a pseudoaneurysm of the sphenopalatine artery following Le Fort I osteotomy is extremely rare, it should be considered as the possible aetiology of acute massive epistaxis in patients with a history of orthognathic surgery accompanied by Le Fort I osteotomy.

To present the first case of middle-ear actinomycosis in the UK in the last 60 years. The diagnosis and management of actinomycosis of the middle ear is also presented, as well as a review of the recent literature.

This paper reports the case of a nine-year-old girl who presented with recurrent otorrhoea caused by actinomycosis. Mastoid exploration with clearance of the infected tissue, in conjunction with six months amoxicillin, resulted in long-term disease remission. Histology revealed Gram-positive, silver avid (on Grocott staining) and diastase-resistant periodic acid-Schiff negative staining of colonies; this profile was in keeping with the characteristic ‘sulphur granules’ of actinomycosis.

Actinomycosis of the middle ear and mastoid is rare, with less than 45 cases presented in the worldwide literature. This case confirms that the disease should be treated with full surgical clearance and long-term antibiotics.

Concha bullosa is the pneumatisation of intranasal conchae (usually the middle turbinate, and rarely the inferior or superior turbinate); however, the term is generally used to describe aeration of the middle concha. Superior concha bullosa is a rare finding, and only a few cases of inferior concha bullosa have been reported in the medical literature. When symptomatic, concha bullosa may cause various problems including nasal congestion, headache, postnasal drip, anosmia and, sometimes, epiphora.

Computed tomography, following history-taking and physical examination, is a valuable tool in diagnosing turbinate pneumatisation. This article presents a very rare case with bilateral triple conchae pneumatisations.

The symptomatology, diagnosis and treatment options for cases of multiple concha bullosa are discussed. The surgical interventions performed in the presented case are briefly described.

The presented patient had pneumatisation of all six turbinates. In such cases, we propose that this condition be termed ‘conchae bullosis’ rather than ‘conchae bullosa’, in a similar fashion to the use of nasal polyposis as the plural form of nasal polyp.