CADASIL (cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy) is an autosomal dominant angiopathy characterized by recurrent cerebrovascular events, migraine and dementia. We describe a case of sensorineural hearing loss as the presenting feature of this condition. We have found no previous reports in the world literature of CADASIL presenting with a sudden sensorineural hearing loss. The significance of questioning a patient with regard to family history is exemplified in this case.

Stridor causing respiratory failure is an ENT and anaesthetic emergency requiring prompt management to secure a clear airway. We describe a case of subacute partial upper airway obstruction due to a large laryngeal carcinoma in an 81-year-old male resulting in respiratory failure. The patient became apnoeic after gaseous induction of general anaesthesia, and after two failed intubation attempts an emergency transtracheal airway catheter was placed by the surgeon under direct vision below the cricothyroid membrane, as this had tumour involvement. The patient was subsequently manually jet-ventilated with ease until a formal tracheostomy was made. Where difficulties with tracheal anatomy are encountered due to the presence of pathology, the insertion of a temporary airway catheter for jet ventilation by the surgeon can buy valuable time and be life-saving.

We report the cases of two laryngectomy patients who were diagnosed with de novo nasal polyposis, more than 15 years after their laryngectomies. This phenomenon has not previously been reported in the literature. We discuss the changes in nasal physiology of the laryngectomy patient, with regards to the aetiology of polyp formation, and highlight the importance of examining the nose in such patients.

We report a case of laser arytenoidectomy for bilateral abductor palsy of the vocal fold in a patient with Parkinson's disease. Parkinson's disease is known to be a rare cause for bilateral vocal fold palsy and this is the second case reported in the English literature. Majority of the reports to date are in the non-English literature. Tracheostomy has been the classic treatment option offered to these patients. To date no report has been found in the literature about a laser arytenoidectomy being performed in a patient suffering from bilateral vocal fold palsy in Parkinson's disease. We have suggested this option as it improves the quality of life of the individual and avoids the difficulty faced by a Parkinson's patient in managing tracheostomy care due to bradykinesia, rigidity and tremor.

Chordomas are rare neoplasms originating along the neuraxis. Although they do not usually show cytological atypia, metastases have been reported in 30 per cent of cases. Survival rates in cases of skull base locations are low, and local recurrence is common after local excision. Radiation therapy is used in post-operative treatment and proton radiation therapy as the primary treatment. In the present paper we present the case of a 50-year-old Caucasian man affected by chordoma of the clivus, with liver and chest metastases, relapsed after several surgical local excisions, to discuss improvements in therapeutic and imaging techniques. Indium111 (In111) pentetreotide single photon emission computed tomography (SPECT) was employed to assess the presence of somatostatin receptors and to treat the tumour with radiolabelled Y90-DOTA-lanreotide. Imaging, performed 2 months afterwards, showedstable disease in the lungs but a local progression in the metastases, in comparison with pre-treatment uptake. These data suggest the usefulness of radiolabelled somatostatin analogues in the diagnosis and therapy of chordomas.

Choanal polyps almost always appear as solitary growths and most commonly arise from the maxillary sinus. Sphenochoanal and ethmoidochoanal polyps are extremely rare. Co-existence of more than one choanal polyp is even more infrequent. We present a patient with an antrochoanal and an accompanying sphenochoanal polyp for the first time in the literature. We discuss the clinical presentation, pathogenesis and surgical management of this rare clinical entity.

We present the case of Munchausen’s syndrome by proxy (MSBP) in which the mother spat and placed blood in her child’s ear, and videoed the result, in order to gain the attentions of the medical profession. This is the first case report of this kind and may represent a disturbing trend in the use of digital photography and video to support factitious illnesses.

Parathyroid carcinoma is very rare and when diagnosed can be difficult to treat as more than 50 per cent have persistent or recurrent disease. Patients generally present with profound symptoms of hypercalcaemia and a palpable neck mass. We describe a case of parathyroid carcinoma in primary hyperparathyroidism and a case in tertiary hyperparathyroidism in a renal dialysis patient. Most studies in the literature are retrospective but recent trends in presentation, treatment options and complications have been reviewed and are summarized in this paper.

Cholesterol granulomas of the middle ear tend to be benign in nature, frequently seen by otologists during cholesteatoma or chronic otitis media surgery and easily dealt with by evacuation. In contrast, cholesterol granulomas of the petrous apex appear to have a more aggressive nature and present significant pathological threats. We present a case of a large destructive cholesterol granuloma of the middle ear eroding the middle and posterior cranial fossa bone and dura to exert a mass effect upon the temporal lobe. We emphasize the destructive potential of this extremely rare middle-ear cholesterol granuloma, and draw attention to the distinction between this variant and the commonplace and benign form of the lesion more usually found at this site. We also present the current theories of the aetiology of cholesterol granuloma formation with possible explanations for the rare aggressive behaviour.

Haemorrhage, throat pain and otalgia are common complications following tonsillectomy. Haemorrhage is rarely life-threatening but in this paper we describe a fulminant secondary haemorrhage due to an aberrant external carotid artery in an eight-year-old boy. Acute surgical intervention with ligation of the external carotid artery was needed to control the bleeding.

Arteriovenous fistula of the superficial temporal artery is a rare condition most commonly caused by trauma. Traditional surgical treatment has been superseded by endovascular embolization. We present the case of a 40year-old man with a traumatic arteriovenous fistula of the superficial temporal artery who was treated by endovascular embolization. The advantages of this approach are discussed, along with a brief history of the condition

Subjective tinnitus (heard only by the patient) is a common otological complaint. Objective tinnitus (heard by the examiner as well as the patient) is extremely rare. There are only a few cases of objective tinnitus, secondary to middle-ear myoclonus, described in the literature.

We present the case of a child with bilateral, congenital, objective tinnitus, secondary to middle-ear myoclonus, with otherwise normal hearing thresholds (250Hz-8kHz), and with no evidence of intra-cerebral or systemic disorders. No similar case has been reported in the world literature.

Acute tonsillitis is a common condition and usually runs a benign course. However life-threatening complications do still occur, even in this postantibiotic era. Infection can spread downwards into the mediastinum through the anatomic cervical spaces, causing widespread cellulitis, necrosis, abscess formation and sepsis. We present a case of descending mediastinitis in an 18-year-old woman, arising from her first episode of tonsillitis and treated successfully by surgical drainage. We believe that an awareness of this complication, early diagnosis using computed tomography scanning, and prompt, adequate surgical drainage will reduce morbidity and mortality.

Tubular apocrine adenoma (TAA) is a very rare sweat gland tumour. Comprehensive review of the literature reveals that TAA in the external auditory canal (EAC) has not yet been reported. We report a case of TAA in the EAC, together with characteristic histopathological findings.

Achalasia is a motility disorder of the oesophagus that typically presents with dysphagia, regurgitation and chest pain. A rare presenting symptom is stridor. A case of previously treated achalasia re-presenting with stridor is described and associated imaging presented.

The authors describe a patient who presented with a left sublingual swelling. Ultrasonic and sialographic investigation showed no salivary tissue in the expected left submandibular region, but a large salivary gland in the left floor of the mouth deep to the mylohyoid muscle. This is likely to represent an ectopically positioned submandibular gland.

Canalicular adenomas are a rare form of benign tumour that occur in salivary glands, occurring mainly in the upper lip and minor salivary glands of the buccal mucosa. The authors report the fifth case and the first in the otorhinolaryngological literature of a canalicular adenoma of the parotid gland. Its specific histological features are difficult to detect on fine needle aspiration and its multifocal nature can lead to recurrence and this must be considered in the clinical management.

Congenital absence of the parotid gland is extremely infrequent. We presenthere a case of unilateral parotid gland agenesis with pleomorphic adenoma of the contralateral parotid gland. Even though pleomorphic adenoma is the most common tumour of the parotid gland, to our knowledge this is the first case of these two conditions being seen together.

We present the case of a previously healthy 59-year-old man who was under treatment for scleritis and episcleritis when he developed a parotid-gland swelling and pus-producing sinus. On surgical exploration, the features were those of a parotid abscess, but the lesion not only failed to heal post-operatively but increased in size very significantly. There was also severe necrotizing keratitis of the eyes. Due to clinical suspicion and a positive antineutrophil cytoplasmic antibodies test, Wegener’s granulomatosis was diagnosed and the patient successfully treated with cyclophosphamide and steroids. Previously, a number of cases of Wegener’s granulomatosis causing salivary-gland swelling have been reported in the literature; this is the first case in which the disease has masqueraded as a parotid abscess.