Keloid scarring is a benign hyperproliferation of fibrous tissue occurring at a wound healing site. Keloid formation related to the ear is generally the result of ear-piercing, mainly causing cosmetic disfigurement. We present an unusual case of keloid formation at a previous meatoplasty incision scar in a 10-year-old Caucasian with a modified radical mastoid cavity. This lesion prevented the cavity from self-cleaning and obstructed microscopic evaluation of the cavity. Treatment was successfully performed by surgical excision, with closure of the defect using supra-keloid skin flaps, followed by serial steroid injection therapy.

Lemierre's syndrome is characterized by acute oropharyngeal infection complicated by internal jugular venous thrombosis secondary to septic thrombophlebitis, and metastatic abscesses. We report a case of Lemierre's syndrome in an 18-year-old Caucasian woman presenting with a peritonsillar abscess and ipsilateral VIth cranial nerve palsy.

Benign mucous membrane pemphigoid is a rare autoimmune disorder affecting the upper aero-digestive tract and conjunctivae. This is a case presentation of benign mucous membrane pemphigoid affecting the oral mucosa, pharynx, oesophagus and larynx, leading to cicatricial lesions in the pharynx and larynx, causing dysphagia, hoarseness and stridor. The alternative forms of management for laryngeal scarring due to this disease are explained. The patient was later diagnosed with advanced renal cell carcinoma, raising the possibility of cicatricial pemphigoid manifesting as a paraneoplastic syndrome of underlying renal cell carcinoma.

Keratosis obturans is characterized by the accumulation of desquamated keratinous material in the bony portion of the external auditory canal. Classically, it is reported to present with severe otalgia, conductive hearing loss and global widening of the external auditory canal. Extensive erosion of the bony meatus, with exposure of the facial nerve, has been previously reported, but no case of facial nerve palsy has as yet been published. We report the first published case, to our knowledge, of a unilateral facial nerve palsy secondary to neglected keratosis obturans.

A patient presented with unilateral, right-sided hearing loss and tinnitus and underwent gadolinium-enhanced magnetic resonance imaging (MRI). A pure tone audiogram showed a right-sided sensorineural hearing loss. The MRI scan was initially negative but when repeated seven years later, following a further deterioration of symptoms, it showed a 2 mm, right-sided acoustic neuroma.

This case has great potential significance for the diagnosis of acoustic neuroma, and it may raise medico-legal issues regarding the exclusion of this diagnosis. The case illustrates that a single negative scan may not be adequate if pure tone audiograms show deterioration in hearing loss.

The sarcomatoid variant is a rare subtype of salivary duct carcinoma that comprises epithelial and sarcomatoid components. The authors present the first reported case of sarcomatoid salivary duct carcinoma occurring in the larynx, which was successfully treated by partial laryngectomy with ipsilateral neck dissection, with no evidence of recurrence after five years.

Microscopic analysis revealed a composite epithelial and sarcomatoid tumour. The epithelial component was characterized by papillary proliferation of glandular and ductal structures lined with multilayered epithelial tumour cells. The sarcomatoid component was characterized by pleomorphic spindle cells with cytologic atypia, accompanied by small lymphoid cells, extensive proliferation of blood vessels and focal myxoid changes.

To the best of our knowledge, only one case of sarcomatoid salivary duct carcinoma outside a major salivary gland has been reported previously in the literature, and this is the first report of sarcomatoid salivary duct carcinoma arising from the larynx.

With the exception of moderately differentiated neuroendocrine carcinoma of the larynx, neuroendocrine carcinomas are very rare head and neck malignancies. We report a case of a small cell neuroendocrine carcinoma in the petrous apex of the temporal bone. This unusual site for this tumour has not yet been reported in the literature. We confirmed the location and extent of the primary tumour by positron-emission tomography scan. The final histopathological diagnosis was of small cell carcinoma, and this was confirmed by immunohistochemistry. We treated this patient with surgery followed by radiotherapy and chemotherapy. After the treatment was completed there was subtotal remission of the tumour, with no distant metastases.

Classical Ménière's disease is rarely found in children and literature regarding it is scarce. In general, the frequency of Ménière's disease in children is only 0.4–7.0 per cent of that in adults. The progression pattern of Ménière's disease in children is not known well. Here, we report three cases of Ménière's disease in children less than 15 years old, treated over nine years. The three cases comprise 14- and 13-year-old boys and a nine-year-old girl. Two of the three patients initially complained only of recurrent bouts of vertigo, without any tinnitus, ear fullness or hearing impairment. In all three cases, the early pure tone audiograms showed only high tone frequency loss, regardless of subjective hearing loss, and the decrease in the hearing threshold was observed one to eight years after the dizziness attacks began. The hearing threshold was usually decreased to a level of mild or moderate hearing impairment. After diuretic treatment, vertigo was generally well controlled, and some cases showed improvement in hearing. Of the total number of patients with Ménière's disease who visited our department over nine years, 2.6 per cent (3/114) were children, and the overall incidence of Ménière's disease in children with vertigo was 2.0 per cent (3/147). In conclusion, Ménière's disease in children rarely develops and may have characteristics of high tone loss in initial audiograms.

Teratomas and dermoids or hairy polyps of the head and neck are rare congenital tumours, often confused and reported to appear as single masses, that may cause upper aerodigestive tract impairment. Their histological classification, origin and embryological basis of development have been a topic of debate in the past and recent literature.

We report a case of an infant presenting with a multifocal bigerminal teratoma in the nasopharynx and the neck, causing a deep neck infection, and discuss the unique clinicopathological aspects of this case.

Inverted papilloma is a rare, benign tumour representing only 0.5 to 4 per cent of all sinonasal neoplasms; its involvement of the middle ear is extremely rare. We present a case of multicentric inverted papilloma in the sinonasal region and middle ear in a 54-year-old man. The patient later developed neck metastasis secondary to malignant transformation of the inverted papilloma in the middle ear.

Epithelioid haemangioendothelioma (EHE) is an uncommon vascular neoplasm which occurs rarely in the head and neck and has an unpredictable clinical behaviour. It is characterized by round or spindle-shaped endothelial cells with cytoplasmic vacuolation. Most often, EHE arises from the soft tissues of the upper and lower extremities, and it has borderline malignant potential. We describe the first reported case of EHE in the parapharyngeal space, which was treated successfully via a transcervical–transparotid approach (following angiography with embolization), without massive intra-operative bleeding or facial nerve damage.

We describe a case in which reconstruction of the tendon of the tensor tympani muscle was necessary for the successful restoration of sound conduction.

The right ear of a nine-year-old boy was treated for cholesteatoma with staged surgery. During the first operation, the tendon was cut to ensure good visibility in the tympanic cavity. Post-operatively, maintenance of aeration of the middle ear required ventilation tubes at first and Valsalva manoeuvres later on. The position of the reconstructed tympanic membrane varied a great deal, moving between the medial wall of the tympanic cavity and extreme bulging. This made exact measurement of a columella for ossicular reconstruction impossible.

The preserved handle of the malleus was bound to the cochleariform process with ionomer cement, using a piece of surgical suture material as a substitute for the tendon. This arrangement prevented the tympanic membrane from undergoing excessive lateral movement after inflation and the ossicular chain was replaced with a successful ossiculoplasty with an autogenous bone ‘drum to footplate’ columella. The pre-operative 55.0 dB air–bone gap decreased immediately to 3.3 dB, widening after three years to 15.0 dB.

Aims: To demonstrate the efficacy of bilateral medialization thyroplasty as a treatment for severe, chronic aspiration. To review the literature on surgical options available in the treatment of severe aspiration.

Materials and methods: Three cases that underwent bilateral medialization thyroplasty are described. The technique used was the standard medialization thyroplasty described by Isshiki as a unilateral procedure. The assessment and rehabilitation of these patients is discussed.

Results: All patients stopped aspirating following surgery. One patient returned to a normal diet and one patient returned to a solid diet. All patients required a permanent tracheostomy.

Conclusion: Bilateral medialization thyroplasty offers an effective surgical option in the treatment of severe, chronic aspiration. It maintains good voice, with a possible return to oral diet. The operation is easily reversible if the patient's condition alters.

We report a rare case of myxofibrosarcoma arising in the neck. A 55-year-old man presented with a two-year history of left-sided, painless, submandibular swelling. Computed tomography and magnetic resonance imaging (MRI) revealed an 80 × 35 mm, well defined, lobulated, submandibular tumour extending to the parapharyngeal space. The tumour showed uniformly low intensity and marked hyperintensity in T1- and T2-weighted MRI scans, respectively, and was scarcely enhanced by gadolinium. A tentative diagnosis of lymphangioma or plunging ranula was made, and the patient underwent local injection of OK-432, which proved to be ineffective. Resection of the tumour was then performed via a transcervical approach. The tumour was histopathologically and immunohistochemically diagnosed as a low-grade myxofibrosarcoma. The patient's post-operative clinical course was uneventful, and the patient was free of disease 27 months after surgery. The pathology, clinical characteristics and treatment of myxofibrosarcoma are bibliographically reviewed.

Extracranial spread of meningiomas to involve the middle ear is very rare. We present the case of a 43-year-old woman with a known cerebellopontine angle meningioma who subsequently presented with left-sided otalgia and a middle-ear mass extruding through the tympanic membrane due to local invasion of the meningioma. The tumour was excised surgically. A discussion of the relevant literature is also presented.

Craniocervical necrotizing fasciitis (CCNF) has a potentially high morbidity and mortality. Late presentation, shock and disseminated intravascular coagulation are associated with a particularly poor prognosis. Early recognition and aggressive treatment is advised. A case report is presented of a late presentation of necrotizing fasciitis of the face, neck and anterior thoracic wall. Despite adverse prognostic indicators, surgical debridement was performed. Intensive medical treatment included continuous renal replacement therapy and intravenous immunoglobulin. Survival in this case illustrates that the window of opportunity for surgically treating CCNF extends to the advanced stages of the disease, albeit with increased morbidity.

Rehabilitation of voice and speech after total laryngectomy has become established practice in recent years. A voice prosthesis is placed within a surgically produced fistula between the trachea and upper oesophagus and acts as a one way valve, allowing passage of pulmonary air from the trachea into the oesophagus and preventing aspiration of food and fluid from the oesophagus into the trachea. Persistent leakage through or around these prostheses is a recognized complication, the aetiology of which can vary widely, from mechanical issues with the prostheses themselves to anatomical and physiological issues associated with the reconstructed pharynx.

We report a new technique of using Dysport® in the management of intractable central leakage due to premature and forceful closure of the upper oesophageal sphincter during swallowing. This resulted in the pooling of fluids around the posterior flange of the prosthesis. This, along with the increased pressure from the muscle contraction, led to central leakage, as identified on videofluoroscopy. An injection of Dysport paralysed the upper oesophageal sphincter, preventing pooling of fluids around the prosthesis and the forcing open of the valve. The effect was to eliminate the leakage, and the patient did not require further injections over the following 22 months.

Interdigitating dendritic cell sarcomas (IDCSs) are extremely uncommon tumours that arise predominantly in lymphoid tissue. We report a case of an IDCS arising in the parotid gland of a 73-year-old man. Clinically, a primary salivary gland tumour was suspected but fine needle aspiration cytology suggested a soft tissue tumour. A diagnosis of IDCS was made on histopathological examination of the resection specimen, with subsequent confirmation by electron microscopy. Given the extreme rarity of this tumour at this site, it is unlikely to be a common diagnostic problem, but the importance of multiple diagnostic modalities is emphasized. The findings of cytology, histology, immunohistochemistry and electron microscopy have not previously been described together in a single case report of this tumour.