We report a rare case of small cell neuroendocrine carcinoma of the nasal cavity and paranasal sinuses with intracranial extension, and discuss the management of this rare tumour.

Small cell neuroendocrine carcinoma involving the nasal cavity and paranasal sinuses with intracranial extension may be treated successfully with surgery alone, without development of local recurrence.

Small cell neuroendocrine carcinoma is a locally aggressive tumour with a high rate of recurrence. Early and aggressive surgical excision with or without radiotherapy or chemotherapy can improve a patient's outcome and prognosis. Regular follow up is needed to detect any local or distant recurrence.

We report an extremely rare case of a giant invasive prolactinoma presenting as a nasopharyngeal tumour.

Case report and literature review regarding giant prolactinoma.

Giant prolactinoma is a rare form of prolactinoma which accounts for 0.5–4.4 per cent of all pituitary tumours. It is more common in men and generally presents with symptoms of endocrine disturbance or neurological deficit.

To our knowledge, this is the first report of giant prolactinoma presenting with symptoms of sinusitis and nasopharyngeal tumour.

We report the case of a rare angiosarcoma, retiform haemangioendothelioma, in an 18-year-old young man, which presented as a recurrent ulcerating lesion of the left pinna.

Case report and literature review of retiform haemangioendothelioma. This is a low grade angiosarcoma with a high local recurrence rate and low metastasis rate, and was first described in 1994 by Calonje et al.

This patient represents only the third report of lymph node metastasis in a case of retiform haemangioendothelioma. To date, 31 cases of the tumour have been reported. Histological diagnosis of this group of vascular neoplasms can be challenging, as their histopathological appearance is intermediate between haemangioma and angiosarcoma.

Surgical excision remains the primary treatment modality, with adjuvant radiotherapy recommended in patients with large tumour size, local recurrence and lymph node metastasis, as seen in this case.

Non-arteritic ischaemic optic neuritis is a known post-operative complication of ophthalmological and maxillofacial surgery, but has not been widely described as a potential consequence of head and neck surgery.

To highlight non-arteritic ischaemic optic neuritis as a potential risk in patients undergoing head and neck surgery.

Case report of a 60-year-old man undergoing total laryngectomy and bilateral neck dissection for laryngeal squamous cell carcinoma.

On day 14 post-operatively, the patient suffered substantial oral bleeding secondary to an internal jugulo-neopharyngeal fistula. Following emergency haemostatic measures, the patient was immediately aware of visual disturbances. The ophthalmologists concluded that these were due to non-arteritic ischaemic optic neuritis, caused by acute hypoxaemia secondary to substantial blood loss.

The prognosis of non-arteritic ischaemic optic neuritis is poor. Thus, it is crucial that otolaryngologists are aware of this complication of head and neck surgery, as immediate diagnosis and treatment can help prevent worsening visual loss.

Vestibular schwannomas are the hallmark of neurofibromatosis type two. They are difficult to treat, due to their bilateral presentation and the quest for hearing preservation. Our report describes a new treatment approach in this clinical scenario.

We report two cases which confirm that bevacizumab, a monoclonal antibody targeting vascular endothelial growth factor, causes regression of vestibular schwannomas in patients with a previous history of gamma knife radiosurgery or failed treatment with another form of vascular endothelial growth factor targeted therapy.

In 2009, Plotkin et al. reported the volumetric response of vestibular schwannomas to bevacizumab treatment, both in untreated patients and in patients previously treated with erlotinib, an epidermal growth factor receptor inhibitor. The presented cases support the use of bevacizumab to treat vestibular schwannomas. Given the extremely slow growth of these tumours, we note the rapidity of volume reduction following bevacizumab therapy.

Description of a female patient with diagnosed Kjer's disease and sensorineural hearing loss, who specifically complained of a progressive inability to understand speech in noisy situations.

Case report.

A 30-year-old, Caucasian woman with Kjer's disease.

Audiological assessment showed low-frequency sensorineural hearing loss and a disproportionate deterioration in speech discrimination. This inconsistency gave rise to suspicion of possible aggravation. Follow-up testing showed that brainstem responses were absent, while clear otoacoustic emissions and cochlear microphonics were present. Hearing aids were fitted but no improvement was shown.

This case shows a combination of auditory neuropathy and Kjer's optic neuropathy. It also illustrates that the combination of unexplained hearing loss and apparently inconsistent audiometric outcomes may be associated with auditory neuropathy. Such unexpected hearing evaluation outcomes may be due to other neurological conditions, such as Kjer's disease.

Intranasal teeth are uncommon. Causes include trauma, infection, anatomical malformations and genetic factors. They present mainly in children, and many are asymptomatic.

This report describes the finding of a tooth that had been displaced into the nasal cavity in a six-year-old girl. The history, clinical examination, findings and operative treatment are described.

The child presented with nasal symptoms. Examination revealed a tooth in the right nasal cavity, confirmed by a lateral cephalogram radiograph. It was extracted under general anaesthesia. At follow up, the child was asymptomatic.

This is an unusual case of a child presenting with an intranasal tooth and nasal symptoms following trauma a number of years earlier. The child underwent extraction of the tooth, and recovered well without any complications.

We report an extremely rare case of the oropharyngeal form of tularaemia, causing a parapharyngeal abscess.

A 48-year-old woman presented with fever, sore throat, breathing difficulty and a right-sided neck swelling. This mass had previously been treated with penicillin without response, and had already been surgically drained once in another hospital. On physical examination, the tonsils were exudative and hypertrophic and the pharynx was hyperaemic. A fluctuant, 4 × 4 cm mass was seen on endoscopic examination, originating from the left parapharyngeal area and protruding towards the pyriform sinus, and partly obstructing the airway. Microagglutination test antibody titres for Francisella tularensis were positive (1/1280). The patient healed completely after definitive drainage of the abscess and antimicrobial therapy for 14 days (streptomycin, 2 × 1 g intramuscularly).

Tularaemia should be considered in the differential diagnosis of patients presenting with tonsillopharyngitis, cervical lymphadenitis and parapharyngeal abscess who do not respond to treatment with penicillin, even if they do not live in an endemic region.

Abnormalities of the third branchial arch are less common than those of the second arch and usually present with left thyroid lobe inflammation. This paper describes 15 cases of pyriform sinus fistulae of third branchial arch origin usually presenting as recurrent thyroid abscess on the left side.

A retrospective review of 15 cases of third arch fistulae managed 2000 and 2008, diagnosed based on histopathology and radiological evidence of a fistulous tract, and treated with fistulectomy with left hemithyroidectomy.

All patients (six boys and nine girls, aged three to 15 years) presented with recurrent low neck inflammation. Pre-operative ultrasound, computed tomography fistulography and barium swallow demonstrated a third arch fistulous tract, left-sided in all cases. The fistula was detected intra-operatively and pathologically in all cases. Surgery (successful in all cases) emphasised complete recurrent laryngeal nerve and ipsilateral pyriform sinus exposure, to facilitate tract excision, with left hemithyroidectomy. There was no recurrence over three to five years' follow up.

Paediatric recurrent low neck inflammatory episodes, due to thyroidal abscess, especially left-sided, should raise suspicion of pyriform sinus fistulae.

We report a case of traumatic cerebrospinal fluid rhinorrhoea following nasal packing with a Rapid Rhino inflatable balloon pack, as treatment for epistaxis.

Case report and review of the literature regarding this complication.

A thorough literature search was performed using PubMed. To our best knowledge, there have been no previous reports of cerebrospinal fluid rhinorrhoea following nasal packing. In our case, cerebrospinal fluid leakage occurred due to fracture of the middle turbinate at its superior skull base insertion. The cerebrospinal fluid leak resolved spontaneously without the need for surgical intervention.

This case report highlights not only the need for an appreciation of sinonasal anatomy and the potential risks associated with nasal packing, but also the need for adequate training in the use of commercially available nasal packs by medical practitioners attending patients with epistaxis.

We report a modified surgical technique for transplanting an ectopic, lingual thyroid to the submandibular space, in order to maintain thyroid function while relieving obstructive symptoms.

A 52-year-old woman complained of progressive dysphagia and dyspnoea. Ectopic lingual thyroid tissue was diagnosed. The ectopic thyroid gland was transplanted into the submandibular region via a lateral pharyngeal approach. A random muscle pedicle was prepared to provide a vascular supply to the transposed gland.

Twelve-month follow up confirmed the survival of the transplanted thyroid gland, with preserved thyroid function.

Surgical transplantation of a lingual thyroid to the submandibular region offers an alternative treatment method for this anomaly, which avoids the need for resection and lifelong thyroxine replacement.

To report a case of Wegener's granulomatosis mimicking skull base osteomyelitis.

A case of Wegener's granulomatosis is reported in a diabetic woman presenting with facial palsy and hearing loss. The clinical course of the disease was very similar to that of skull base osteomyelitis, especially since the patient was diabetic and the ear swab was positive for pseudomonas. The definitive diagnosis was made based on clinical presentation, imaging and serum antineutrophil cytoplasmic antibody testing. The patient was started on immunosuppressants, and her hearing loss and facial palsy recovered.

Wegener's granulomatosis is a systemic disease with various manifestations. A high index of clinical suspicion is required to reach the correct diagnosis. The clinician should be alerted to the possibility of Wegener's granulomatosis in the presence of: systemic upset out of proportion to the apparent intensity of the local lesion; middle-ear disease failing to respond to conventional treatment; and a consistently raised erythrocyte sedimentation rate.

To demonstrate the usefulness of pre-operative selective embolisation of the thyroid arteries in an unusual case with a large, vascular thyroid tumour.

A 29-year-old man presented with a large papillary carcinoma of the thyroid (weighing approximately 300 g on palpation) with extension to the mediastinum and compression of the trachea. A computed tomography scan of the neck and thorax revealed a large tumour the solid part of which was enhanced after contrast medium application, indicating a substantial vascular supply. Pre-operative selective embolisation of both superior thyroid arteries and one inferior thyroid artery, using gelatin sponge particles, was performed 4 days before surgery, under conventional angiography. After selective embolisation of these thyroid arteries, the patient experienced mild anterior neck pain and mild fever. This procedure allowed a significant reduction in blood perfusion to the tumour, which facilitated its surgical removal without blood transfusion.

Pre-operative selective embolisation of both superior thyroid arteries and one inferior thyroid artery may be an effective, minimally invasive procedure for patients with a large, vascular thyroid tumour.

We report the use of triamcinolone injections to correct severe nasal deformity due to sarcoidosis, as an alternative to formal surgical rhinoplasty.

A 30-year-old woman with a long-standing history of sarcoidosis presented to a tertiary referral rhinology clinic complaining of breathing difficulty and nasal deformity. Flexible nasoendoscopy revealed red nasal plaques typical of nasal sarcoidosis, together with significant widening of the nasal bridge. Triamcinolone, a long-acting corticosteroid, was injected both intralesionally and subcutaneously over the nasal dorsum, at zero, three and eight months, resulting in long-lasting improvement of the nasal shape.

Sarcoidosis is a non-caseating, granulomatous, epithelioid inflammation. Otorhinolaryngological manifestations occur in approximately 10 per cent of patients; however, there is little published experience of nasal reconstruction in such patients. We describe a quick, simple and relatively cost-effective technique, with little or no co-morbidity, with which to improve the aesthetic and symptomatic outcomes of nasal sarcoidosis.

We report an unusual case of a fungus ball in the concha bullosa, without involvement of the paranasal sinuses.

A 29-year-old woman presented complaining of nasal obstruction and postnasal discharge. Paranasal computed tomography demonstrated that the concha bullosa in the right middle turbinate was filled with a high-density material. The patient underwent endoscopic resection of the concha bullosa, during which a dark brown, cheese-like material was found. Histopathological examination of the excised specimen revealed a fungus ball.

Fungus balls are non-invasive accumulations of dense fungal concretions that generally affect healthy individuals. In the sinonasal region, they occur most frequently in the maxillary sinus. Although fungus ball formation in the concha bullosa is unusual, it should be considered in the differential diagnoses of middle turbinate and sinonasal pathology.

To demonstrate the need for computed tomography imaging of the temporal bone before considering revision stapes surgery in patients with recurrent or residual conductive hearing loss.

We report the case of a high-riding jugular bulb with an associated jugular bulb diverticulum, which was dehiscent towards the vestibular aqueduct, in a patient with confirmed otosclerosis who did not experience hearing improvement after stapedotomy.

This case demonstrates the usefulness of temporal bone computed tomography in the evaluation of patients with otosclerosis in whom stapedotomy has not improved hearing. In such patients, revision surgery to address residual hearing loss would eventually prove unnecessary and avoidable.

Rhinoscleroma and Rosai–Dorfman disease have been reported to coexist in the same patient at different sites. Rosai–Dorfman disease may have an aetiological relationship with rhinoscleroma, although this has not yet been proved.

A case of a 42-year-old woman with recurrent nasal masses is presented, with histopathological proof of both conditions coexisting in the same nasal lesion.

The aetiopathology, clinical features and treatment of both diseases are discussed and a literature survey is reported. Histologically, the presence of Mikulicz cells with entrapped, rod-like, Gram-negative bacilli and Russell bodies suggests rhinoscleroma. Emperipolesis and S-100-positive histiocytes confirm the diagnosis of Rosai-Dorfman disease. The presence of both in the same slides from affected tissues has never been demonstrated before. In the light of this evidence, the author believes that rhinoscleroma must be considered in the aetiology of Rosai–Dorfman disease.

This case report aims to raise awareness amongst clinicians of ear presentation of cutaneous borrelia.

We report a recent case of borrelia lymphocytoma cutis benigna in a child presenting with unilateral earlobe swelling, who was otherwise well. A review of the English language literature, including management of the disease, is also presented.

This case highlights the fact that borrelia lymphocytoma should be included in the differential diagnosis of a persistent, unilateral, inflamed, swollen earlobe in an otherwise healthy child.

Dermoids presenting in early life with a sinus tract along the midline of the nasal dorsum are well described. We present three very unusual variants of this condition.

Case one was a two-year-old child with a pit in the philtrum of the lip. The tract pierced the nasal spine and extended towards the crista galli. Case two was a 15-month-old child with two separate sinus openings on the dorsum, one below and one above a dermoid cyst. Case three was a 17-year-old adolescent with neurological disability. Scanning showed a dermoid cyst deep in the anterior skull base, but the sinus tract had been obliterated by bony overgrowth due to long term use of antiepileptic medication.

Cases one and two were successfully managed by complete excision via an external rhinoplasty approach. Case three, in contrast, was not operated upon due to bony obliteration of the sinus tract.

Surgeons dealing with nasal dermoids must be able to adapt to the variable anatomical and clinical features of these lesions.

Wegener's granulomatosis is a rare but well recognised autoimmune necrotising vasculitis. Presentation of disease in the head and neck is common and mostly consists of nasal crusting, blockage and bloody discharge. Neurological presentation is very uncommon.

We report a patient who presented to the medical emergency services with signs and symptoms of meningitis, but who was eventually diagnosed with Wegener's granulomatosis. A literature search on this topic was carried out using Medline and Embase (1996 to 2011), searching for ‘Wegener's granulomatosis’ and ‘meningitis’.

After thorough neurological and medical investigation, a combination of brain computed tomography, lumbar puncture, nasal biopsy and laboratory results refuted the diagnosis of meningitis and confirmed the diagnosis of Wegener's granulomatosis.

To the best of our knowledge, this is the first English-language case report of a patient with Wegener's granulomatosis presenting with symptoms of meningitis unconfirmed on computed tomography and lumbar puncture.