A case of infantile fibromatosis involving the cheek of a three-year-old Sudanese girl is described. The tumour was resected through a sublabial approach.

A case of Feingold syndrome is presented with a previously undescribed association of bilateral profound hearing impairment. Computed tomography (CT) scanning revealed severe narrowing of the internal auditory meatuses at the peripheral end with non-existent auditory nerves. This pathology is significant in the future habilitation of the child, as cochlear implantation is not possible. Children with multiple congenital anomalies should have radiological investigations before hopes are raised with respect to cochlear implantation.

This study evaluated the nature and extent of problems faced by patients following discharge from hospital following laryngectomy. Semi-structured interviews investigated the practical and psychological concerns of a cohort of laryngectomy patients in a district general hospital. A number of simple practical measures can be employed to reduce early hospital re-admission following laryngectomy. Psychological morbidity is an important cause of low quality of life following laryngectomy. Formal psychological treatment could be of benefit in the management of these patients.

This case reports electrophysiological evidence for cochlear function in a child with radiological evidence of bilateral auditory nerve agenesis or severe hypoplasia. The diagnosis of auditory nerve agenesis was supported by a bilateral atresia of internal auditory canals on computed tomography (CT) scan and magnetic resonance imaging (MRI) absent auditory brainstem responses and absent behavioural responses to sound. Despite the apparent absence of an auditory nerve or spiral ganglion, electrocochleography revealed surviving cochlear function at 70–80 db HL and an abnormal electrocochleographic waveform. This case demonstrates that cochlear function may develop without afferent, or efferent innervation. It also emphasizes that cochlear function may occur in the presence of profound deafness.

Congenital cholesteatoma may originate at various sites in the temporal bone. For example, in the petrous apex, the cerebellopontine angle, the middle ear cavity, the mastoid process or the external auditory canal. The least common site being the mastoid process. We present two cases of congenital cholesteatoma of the mastoid process, each presenting with different symptoms and at different ages. Both patients underwent surgical treatment, which confirmed the diagnosis and radiological findings.

We present a case of a 63-year-old diabetic male who developed malignant otitis externa following mastoidectomy. Extensive skull base osteomyelitis caused thrombosis of the jugular bulb and subsequent paralysis of cranial nerves VII, IX, X and XII. Hewas treated aggressively with intravenous antibiotics and debridement of granulation tissue in the mastoid bowl with full recovery of the cranial nerve palsies associated with recanalization of the jugular bulb. We believe this is the first reported case of malignant otitis externa to occur following mastoidectomy with complete recovery of the cranial nerve paresis.

Nasogastric tube placement is desirable for the short-term administration of calories when oral feeding is not possible. Enteral nutrition is superior to parenteral nutrition. In some patients this method of feeding is impossible because of repeated failure of nasogastric tube placement, necessitating either general anaesthesia for enteral access, or total parenteral nutrition. We describe a new method for nasogastric tube placement aided by a fibre-optic nasendoscope which has resulted in the successful placement of over 20 nasogastric tubes in our unit and the avoidance of more invasive methods of establishing nutrition with their associated complications.

Solitary myofibroma is a recently described benign neoplasm of the skin or superficial soft tissue and it represents the adult counterpart of infantile myofibromatosis. This new clinicopathological entity is being recognized increasingly. A case of solitary myofibroma occurring in the pinna of a 50-year-old woman is presented. Such a lesion occurring in the pinna of an adult has not been reported in the literature.

Relapsing polychondritis (RP) is a relatively rare rheumatic condition of unknown aetiology. It is characterized by recurrent episodic inflammation of cartilaginous structures (nose, ear and trachea). The clinical diagnosis of polychondritis can frequently be made with confidence in the absence of histological confirmation. A 61-year-old diabetic man, with bilateral relapsing aural inflammation, left ear deafness with tinnitus and pain at the sternocostal junctions is reported. After clinical diagnosis of relapsing polychondritis steroid therapy was started. An ear cartilage biopsy was performed confirming the clinical diagnosis. Subsequently soft tissue infection occurred at the operation site. The abscess was drained and oral ciprofloxacin was given with complete resolution of the infection over 30 days. As the infection is the main cause of death in these patients, we analyse whether biopsy is absolutely necessary for the diagnosis of RP in some patients.

A patient with sudden onset pulsatile tinnitus resulting from a spontaneous carotico-cavernous fistula is presented. The case is discussed and illustrated to highlight the clinical features and natural history of a condition rarely seen by ENT surgeons.

A 17-year-old male patient was admitted because of progressive hearing loss since the age of six. His former blood and radiology investigation had revealed idiopathic hyperphosphatasia. On ENT examination bilateral thickened tympanic membranes with severe mixed-type hearing loss was diagnosed. Computerized tomography (CT) demonstrated expansion of the calvarial bones, including the temporal bones, except for the otic capsule. Middle-ear exploration revealed thickened middle-ear mucosa and a stone hard, immobile bony mass instead of the normal ossicular chain at the posterior superior part of the mesotympanum. No ossicular reconstruction could be attempted and the patient was rehabilitated with a hearing aid.

We present our experience using the Clarion® magnetless multichannel cochlear implant with a woman profoundly deafened following bilateral acoustic neuromata as a consequence of neurofibromatosis 2 (NF2). The right neuroma had been previously removed without an attempt at neural preservation. On the left, however, a posterior fossa approach had been taken with the aim of preserving hearing. Although the left cochlear nerve appeared to be undamaged at the end of the operation, no hearing thresholds could be elicited on post-operative audiometry, because of damage either to the cochlear nerve or to the blood supply to the cochlea. Round window electrical stimulation subsequently produced a perception of sound, confirming that the cochlear nerve was capable of functioning and that a cochlear implant would be effective. Because she would need regular magnetic resonance imaging (MRI) to monitor existing and future NF2 lesions, it was decided to use a magnetless Clarion® implant, which has been shown to be MRI compatible. We report our experience of using the device in this case and discuss some of the issues related to the provision of cochlear implants to patients with NF2.

Near total laryngectomy with cricohyoepiglottopexy (CHEP) allows cure of glottic carcinomas with voice preservation. The subject of this study was to evaluate CHEP in terms of tumour control and functional result in T1 and T2 glottic carcinomas.

This study reviewed retrospectively 55 consecutive cases of CHEP performed between January 1, 1981 and September 1, 1992 with the exclusion of post-radiotherapy salvage surgery. CHEP was indicated for a T1a limit to the anterior commissure and/or with dysplasia of the other vocal fold (10 cases), T1b (11 cases) and T2 (34 cases) glottic carcinomas. All our patients have a follow-up of more than five years.

The post-operative course after this surgery was generally uneventful. The median time to decannulation was 18 days, to removal of the nasogastric tube was 15 days and to discharge from hospital was 23 days. No significant difference was observed according to the preservation of one or both arytenoid cartilages. The long-term functional result can be considered to be good in three-quarters of cases, with normal oral swallowing and an easily understood voice. The remaining one quarter had a whispery voice and sometimes episodes of aspiration when swallowing liquids. In terms of oncological results, the five-year recurrence-free survival rate was 94 per cent for T1 and 84 per cent for T2. The ultimate tumour control (taking into account four cases of total laryngectomy) was 94 per cent for T1 and 93 per cent for T2.

Primary surgery by CHEP therefore appears to be a good option for early glottic carcinomas. The main problem remains that voice recovery is mediocre in one quarter of patients.

Bacterial meningitis is an important cause of acquired sensorineural deafness in childhood. Deafness following meningitis may be progressive. Previous reports have shown deterioration in hearing up to 12 years after the illness. We present two cases of sensorineural deafness following meningitis. Severe to profound sensorineural hearing losses were detected immediately after meningitis in these patients. The hearing subsequently deteriorated in both cases. Deterioration in hearing thresholds occurred 17 years after the illness in one case. In the other patient the hearing got progressively worse three years after meningitis. She subsequently required a cochlear implant.

Genetic defects of the mitochondrial DNA often cause sensorineural hearing impairment, accompaniment by disorders of organs within the body. This case report describes cochlear implantation of a 33-year-old deaf blind female with mitochondrial cytopathy. The outcome was very successful, and vastly improved quality of life for this patient. Many cases of mitochondrial cytopathy cause progressive deafness; it is, therefore, likely that other patients with this unusual disorder will present for cochlear implant assessment.

Internal carotid artery aneurysms are a rare cause of pulsatile tinnitus and conductive hearing loss but should be borne in mind when there is a suspected diagnosis of glomus jugulare or high-riding jugular bulb. Most cases are congenital. We present a case of otorrhagia which was initially thought to be a glomus jugulare, the diagnosis of internal carotid artery aneurysm was made at angiography and treated by balloon embolization.

The surgical management of Menière's disease presents a difficult and controversial problem and includes various procedures ranging from ‘placebo operations’ to vestibular nerve section. The situation is even more complex in the case where the only hearing ear is affected. We present a case of severe debilitating vertigo due to Menière's disease that was treated by chemical labyrinthectomy and cochlear implantation. This unique management strategy allowed restoration of useful hearing and relief of vertigo.

We describe a patient who developed acute angle-closure glaucoma following the application of topical intranasal cocaine. A 46-year-old woman underwent an elective antral washout under general anaesthesia and with local application of 25 per cent cocaine paste to the nasal mucosa. Twenty-four hours post-operatively the patient developed sudden painful blindness which was found to be due to acute glaucoma. Cocaine with its indirect sympathomimetic activity causes mydriasis, that can precipitate acute angle-closure glaucoma in predisposed individuals with a shallow anterior chamber. Although the incidence is rare, otolaryngologists need to be aware of this potential complication.

Branchio-oto-renal syndrome is a rare autosomal dominant condition characterized by hearing loss, branchial arch abnormalities and renal tract malformations. We present the first reported case of branchio-oto-renal syndrome associated with bilateral congenital cholesteatoma and ossicular chain abnormalities. The pathogenesis of this syndrome is described and the literature is reviewed.