To report an unusual case of fungal mastoiditis caused by entomophthorales in an immunocompetent patient, and its management.

Case report with a review of the literature.

A 13-year-old girl presented to us with a mastoid abscess. Entomophthoromycotic infection of the mastoid was diagnosed on histopathological examination, and subsequently treated with surgical debridement and amphotericin B injection.

This is the first reported case of mastoid abscess secondary to entomophthoromycosis. Early detection and treatment contributed to this patient's good outcome.

Bilateral, spontaneous cerebrospinal fluid rhinorrhoea is extremely rare, with only one previous case report (this patient developed contralateral cerebrospinal fluid leakage four years after successful endoscopic repair). We present the first English-language report of simultaneous, bilateral, spontaneous cerebrospinal fluid rhinorrhoea.

To recommend a simple alternative endoscopic technique for simultaneous closure of bilateral, spontaneous cerebrospinal fluid rhinorrhoea.

A 47-year-old woman presented with recent onset of bilateral, spontaneous cerebrospinal fluid rhinorrhoea, a recent history suggestive of meningitis, and a past history of pneumococcal meningitis. Bony defects on both sides of the cribriform plate were closed endoscopically in the same anaesthetic session, via a uninasal, trans-septal approach, enabling both leakage sites to be sealed simultaneously.

In cases of bilateral, spontaneous cerebrospinal fluid rhinorrhoea, uninasal, trans-septal endoscopic repair is a simple and effective technique for simultaneous closure of cerebrospinal fluid leakage.

Susac syndrome comprises a triad of vestibulocochlear dysfunction, retinopathy and multifocal encephalopathy, which is characterised pathophysiologically by microangiopathy of the ear, retina and brain. Diagnosis is confirmed by magnetic resonance imaging of the brain and ophthalmological examination, which reveals branch retinal artery occlusion. Hearing loss persists in 90 per cent of patients. We present a case of successful hearing rehabilitation by cochlear implantation in a young woman with this syndrome.

A 36-year-old woman presented with neurological symptoms suggestive of encephalitis. She subsequently developed vestibulocochlear symptoms. The diagnosis was confirmed upon magnetic resonance imaging and fluorescein angiography, which showed multiple peripheral retinal arterial occlusions. Hearing loss was fluctuant but gradually progressive over nine months, to bilateral profound sensorineural hearing loss.

A left cochlear implant was placed, with a good outcome.

In this Susac syndrome patient, the outcome of cochlear implantation was encouraging, notwithstanding the possible involvement of retrocochlear pathways.

Myofibrosis of the ear is rare. Myofibromas are mesenchymal tumours which usually present in infancy but have been reported sporadically in adults.

To present a rare case of trauma as a cause of pinnal myofibrosis.

Case report. A 29-year-old soldier suffered repeated trauma from helmet wear and sports, and presented to our clinic with an exquisitely painful lump in the anti-helix of the pinna.

The lump was excised uneventfully. Pathology revealed a reactive myofibroblastic proliferation which, given the history of trauma, raised the possibility of a florid cellular repair process. The main differential diagnosis was myofibroma. Immunohistochemistry was used to exclude other possible causes.

No similar case has previously been reported. The aetiology of myofibroma is unclear, but repeated trauma may be a trigger. Histological and immunohistochemical analysis are recommended when the diagnosis is ambivalent.

To report a case of fluctuating hearing due to auditory aura, as an unusual presentation of temporal lobe epilepsy.

Case report and review of English language literature on temporal lobe epilepsy and auditory aura.

A 31-year-old man presented with intermittent symptoms of bilateral fullness in the ears associated with deafness. He was subsequently diagnosed with temporal lobe epilepsy. Further enquiry revealed a family history of epilepsy.

Auditory aura as a presentation of temporal lobe epilepsy is rarely encountered in otolaryngological practice. This case highlights the importance of obtaining detailed information on epilepsy, including any family history of epilepsy, as a routine part of history-taking in patients presenting with fluctuating hearing loss.

To report a rare case of a symptomatic malleo-incudal osteoma, and to highlight the difficulties in making the clinical diagnosis.

Case report and literature review.

Malleo-incudal osteoma is a rare cause of unilateral conductive hearing loss. Its symptoms may mimic those of other otological causes of conductive hearing loss, such as otosclerosis.

This case report highlights the challenges involved in establishing a clinical diagnosis of malleo-incudal osteoma. It also emphasises the importance of assessing the mobility of the divided ossicular chain during a planned stapedectomy.

Supracricoid laryngectomy with cricohyoidoepiglottopexy is an organ-preserving surgical technique used to treat laryngeal cancer. This procedure resects the vocal folds; however, it is unclear how the sound source and airway morphology are involved in phonation through the post-operative neoglottis.

Multidetector helical computed tomography scanning was performed on two patients who had undergone supracricoid laryngectomy with cricohyoidoepiglottopexy. The cricoid and arytenoid cartilages and the airway were visualised using three-dimensional images.

The mobility of the arytenoid cartilages was well preserved in the one patient with bilateral arytenoids, and in the other patient with only one arytenoid remaining. Two types of airway configuration were observed during phonation: one patient had a single stream airway, while the other had a combination of several streams.

In the patient with only one arytenoid remaining, the preserved arytenoid tended to be rotated excessively inward. Therefore, phonation may have also occurred in various airways followed by mucosal vibration, which may be a sound source.

Inflammatory pseudotumours are mostly seen in the lung, and occasionally in the head and neck region including the sinonasal area. Reported treatment modalities comprise corticosteroid treatment, surgical excision and radiotherapy. The latter option is required because wide surgical resection may be difficult for head and neck lesions, especially in children. However, clinicians should be aware of the risk of late-onset side effects of radiotherapy in children.

We present a two-year-old girl with a massive inflammatory pseudotumour of the maxillary sinus. Transcatheter arterial embolisation was performed, and the lesion was successfully managed without additional therapy. There was no evidence of recurrence over the next five years.

This is the first report presenting the utility of arterial embolisation for inflammatory pseudotumour.

To demonstrate the need for computed tomography imaging of the temporal bone in patients clinically suspected of otosclerosis who present with atypical symptoms or audiological findings.

We present two patients with bilateral conductive hearing loss and suspected otosclerosis in whom third mobile window lesions were revealed. The first patient had bilateral large vestibular aqueducts and bilateral fenestral otosclerotic foci. Computed tomography imaging of the second case revealed bilateral superior semicircular canal dehiscence and bilateral cochlear clefts, mimicking an otosclerotic focus in the fissula ante fenestram.

Differentiating third mobile window lesions from otosclerosis as the cause of a conductive hearing loss is essential before considering stapes surgery, as such treatment would be unnecessary and potentially harmful.

We report a very rare complication of nasal surgery: dislocation of the inferior turbinates into the nasopharynx, presenting as sleep apnoea and persistent nasal obstruction.

A 56-year-old woman presented with a history of obstructive sleep apnoea for one year and nasal obstruction for seven years. She had undergone nasal surgery one year previously. Non-contrast computed tomography of the nose and paranasal sinuses showed a mass near the posterior choana on both sides, confirmed by nasal endoscopy. The displaced turbinates were removed, after resecting their attachment at the posterior choanae, and the patient's symptoms were relieved.

This is a very rare complication of nasal surgery; to the best of our knowledge, we report only the second published case in the English language literature. We suggest that such cases be closely followed up post-operatively.

To assess the quality of life of UK children with sleep-disordered breathing undergoing adenotonsillectomy, by using the Obstructive Sleep Apnoea 18 questionnaire and determining score changes and effect sizes.

Prospective, longitudinal study.

The otolaryngology department of a university teaching hospital in Northern England.

Twenty-eight children for whom adenotonsillectomy was planned as treatment for sleep-disordered breathing, and who had either a clinical history consistent with obstructive sleep apnoea or a polysomnographic diagnosis.

The Obstructive Sleep Apnoea 18 questionnaire, a previously validated, disease-specific quality of life assessment tool; changes in questionnaire scores and effect sizes were assessed.

The Obstructive Sleep Apnoea 18 questionnaire was administered to each child's parent pre-operatively, then again at the follow-up appointment. Questionnaire scores ranged from 1 to 7. Score changes were analysed using the paired t-test; effect sizes were calculated using 95 per cent confidence intervals.

Complete data were obtained for 22 children (mean age, 61 months). Ten had undergone pre-operative polysomnography. Twenty-one children underwent adenotonsillectomy (one underwent tonsillectomy). Median follow up was eight weeks (interquartile range, six to 11 weeks). Following surgery, the overall mean score improvement was 2.6 (p < 0.0001) and the mean effect size 2.4 (95 per cent confidence interval 1.9 to 2.8). There were significant improvements in each of the individual questionnaire domains, i.e. sleep disturbance (mean score change 3.9, p < 0.0001), physical suffering (2.2, p < 0.0001), emotional distress (2.0, p = 0.0001), daytime problems (1.8, p = 0.0001) and caregiver concerns (2.6, p < 0.0001).

In these children with sleep-disordered breathing treated by adenotonsillectomy, Obstructive Sleep Apnoea 18 questionnaire results indicated significantly improved mean score changes and effect sizes across all questionnaire domains, comparing pre- and post-operative data.

We present a patient with mitochondrial hearing loss and a novel mitochondrial DNA transition, who underwent successful cochlear implantation.

An 11-year-old girl showed epilepsy and progressive hearing loss. Despite the use of hearing aids, she gradually lost her remaining hearing ability. Laboratory data revealed elevated lactate levels, indicating mitochondrial dysfunction. Magnetic resonance imaging showed diffuse, mild brain atrophy. Cochlear implantation was performed, and the patient's hearing ability was markedly improved. Whole mitochondrial DNA genome analysis revealed a novel heteroplasmic mitochondrial 625G>A transition in the transfer RNA gene for phenylalanine. This transition was not detected in blood DNA from the patient's mother and healthy controls. Mitochondrial respiratory chain activities in muscle were predominantly decreased in complex III.

This case indicates that cochlear implantation can be a valuable therapeutic option for patients with mitochondrial syndromic hearing loss.

To report outcomes for the first known cochlear implantation procedures in two patients with Brown–Vialetto–Van-Laere syndrome.

Two adult patients (a brother and sister) with post-lingual sensorineural deafness associated with Brown–Vialetto–Van-Laere syndrome. The female patient presented with a milder form of the syndrome.

Cochlear implantation.

Post-implantation speech discrimination scores.

Auditory evoked potential testing suggested pathological changes in both patients' cochleae, auditory nerves, brainstem and (probably) central auditory pathways. In the male patient, despite implantation of the better ear, the Bamford–Kowal–Bench sentence score was zero at 21 months post-implantation. In the female patient, Bamford–Kowal–Bench sentence scores at six months post-implantation were 25 per cent in quiet and 3 per cent in noise.

These poor clinical outcomes appear to be related to retrocochlear and probable central auditory pathway degeneration.

We report a rare case of tuberculosis presenting with bilateral vocal fold palsy.

Case report and discussion of ENT manifestations of tuberculosis.

A 39-year-old man presented with stridor and bilateral vocal fold palsies, and underwent an emergency tracheostomy. Intra-operatively, a mass was identified overlying the thyroid cartilage. Fine needle aspiration cytology of this mass showed well formed granulomas but negative Ziehl–Nielsen staining. Computed tomography showed the mass to be surrounding the thyroid cartilage, causing airway occlusion; pulmonary infiltrates were also seen. The patient was commenced on tuberculosis treatment. Subsequent sputum samples from the tracheostomy confirmed the diagnosis. The patient responded very well to treatment, and was successfully decannulated after one week.

The incidence of tuberculosis in the UK is increasing, particularly in urban areas. The otolaryngologist may encounter a wide variety of presentations. Diagnosis requires a high index of clinical suspicion.

We present the first reported case of sebaceous adenoma of the palate, to our knowledge.

Case report and review of the English language literature, with a focus on the pathological spectrum of sebaceous glands.

A woman presented with a growth on the hard palate, which was excised and examined. Histopathological analysis showed features of sebaceous adenoma. Further evaluation with Sudan Black B special stains and Ki 67 immunohistochemistry was performed.

Sebaceous adenomas in the oral cavity are very rare, with only 10 cases previously reported in the English language literature. Sebaceous adenoma of the palate has not previously been reported, to our knowledge. We discuss the role of special stains and immunohistochemistry in the diagnosis of this tumour.

There have been few previous reports of intraosseous schwannomas within the mandible with extension into the cranium. We report two such cases and discuss the relevant clinical features, radiological manifestations and treatment protocols.

Two case reports of trigeminal schwannoma of the mandible with intracranial extension, including analysis of clinical, radiological and pathological aspects.

Panoramic radiographs showed both tumours as multilocular radiolucencies. Solid and cystic components were seen on computed tomography and magnetic resonance imaging. The two tumours extended into the cranium through the pterygomandibular space and an obviously expanded foramen ovale.

Trigeminal schwannoma of the mandible can develop to involve intracranial extension. Radiological identification of an expanded foramen ovale may facilitate pre-operative identification.

We report a unique case of a mucocele of a pneumatised uncinate process.

Clinical, radiological and intra-operative findings are presented.

A 43-year-old woman was referred to our ENT department suffering from persistent symptoms of left-sided sinusitis. Although an initial computed tomography scan suggested a lacrimal duct mass, dacrocystography showed free flow through the nasolacrimal duct. The patient underwent surgery, revealing a mucocele within a pneumatised uncinate process.

This patient's clinical, radiological and intra-operative findings illustrate how variations in sinus anatomy can pose a diagnostic challenge.

Epithelial-myoepithelial carcinoma is an uncommon, low-grade carcinoma that generally occurs in the salivary glands. A few cases of epithelial-myoepithelial carcinoma arising in the nasal cavity have been reported. We describe a unique case of aggressive epithelial-myoepithelial carcinoma in the nasal cavity.

A 36-year-old woman presented with a mass in her left nasal cavity. Histopathological evaluation revealed it to be an epithelial-myoepithelial carcinoma with overt nuclear atypia, frequent mitoses and necrosis. The tumour recurred in the contralateral nasal cavity 15 months following primary excision. Medial maxillectomy and radiation therapy were performed. Seven-month follow up revealed extensive bone metastases.

We report a rare case of aggressive epithelial-myoepithelial carcinoma in the nasal cavity, with high-grade histology.

We report an extremely rare case of extramedullary plasmacytoma.

Case report and review of the English-literature concerning extramedullary plasmacytoma and multiple myeloma.

We present an unusual case of multiple extramedullary plasmacytomas, which, over a protracted course of 30 years, presented on different occasions at three separate sites in the head and neck. The patient was managed surgically on all occasions, and was disease-free at the time of writing.

Following review of the literature, we believe this to be the only case with this extremely unusual presentation. This case is noteworthy, not only because of the rarity of extramedullary plasmacytoma, but also because it highlights a number of important clinical issues. The diagnosis and management of extramedullary plasmacytoma require close cooperation between multiple disciplines.