The multichannel auditory brainstem implant (ABI) provides the potential for hearing restoration in patients with neurofibromatosis type 2 (NF2). Programmes for auditory brainstem implantation have been established in two Australian centres. Eight patients have been implanted under the protocol of an international multi-centre clinical trial. Three patients had ABI insertion at the time of first side tumour removal, four at second side tumour removal and one after previous bilateral surgery where there was some residual tumour. The translabyrinthine approach was used in all cases. Successful positioning of the electrode array was achieved in seven of eight patients, all of whom achieved auditory perception with electrical stimulation. Intra-operative electrically evoked auditory brainstem response testing was successful in four patients and was useful in confirming correct electrode position. In six cases postoperative psychophysical and auditory perception testing demonstrated that useful auditory sensations were achieved. Five of these patients regularly used the implant. In one patient electrode placement was unsuccessful and only non-auditory sensations occurred on stimulation. In the remaining patients nonauditory sensations were minimal and avoidable by selective electrode programming. Auditory brainstem implantation should be considered in patients with NF2. The greatest benefit is seen in patients without debilitating disease who have non-aidable hearing in the contralateral ear.

A pulsating ear drum is a rare otological finding and usually indicates a vascular pathology. We report the case of a woman who presented with pulsatile tinnitus following an upper respiratory tract infection. She was found to have bilateral pulsating tympanic membranes on otological examination, in association with bilateral dehiscence of the tegmen tympani and a spontaneous encephalocele on the right side and a meningocele on the left side, demonstrated on computerized tomography (CT) scan of her temporal bones.

The incidence of an aberrant internal carotid artery in the middle ear is approximately one per cent and most patients are asymptomatic. We present two patients with an aberrant internal carotid artery who presented with pulsatile tinnitus and an intra-tympanic mass. Here we discuss the clinical presentation, relevant radiographic investigations and further management of these patients.

Histiocytic necrotizing lymphadenitis, or Kikuchi’s disease, is a self-limiting cervical lymphadenitis of unknown origin. Since no specific treatment has been reported for Kikuchi’s disease to date, once a diagnosis of Kikuchi’s disease has been established, the role of the physician has been limited only to treating the symptoms. Sometimes, however, the clinical manifestations of the disease can be very distressing for the patients. Thus, a more aggressive form of treatment may be required for the patients who suffer from severe and persisting symptoms and recurrence. We present three cases of Kikuchi’s disease that benefited significantly from systemic administration of prednisone.

Primary chordoma of the paranasal sinuses are extremely rare tumours, with only a small number of cases verified and published in the literature. It appears that only five of these cases involved the ethmoid sinuses either as a primary or by local invasion, and of these documented cases only one other has been found to solely involve the ethmoid sinus. We present a case of primary ethmoid sinus chordoma treated by wide local surgical excision and present a review of the literature with regard to prevalence and treatment rationale.

Before contemplating surgery for cerebrospinal fluid (CSF) rhinorrhoea it is vital that the correct diagnosis is established. This can be done using immunofixation of beta-2-transferrin, that is nearly always positive in cases of CSF rhinorrhoea. Fluorescein lumbar puncture is useful in establishing the exact site of a leak and also in confirming the absence of a leak where the clinical suspicion is high but the beta-2-transferrin is negative. High resolution computed tomography (CT) scanning is a useful radiological investigation for identifying a bony defect. We present nine patients who presented with clear rhinorrhoea that was clinically highly suggestive of a CSF leak. Three of these patients had undergone previous craniotomies for presumed CSF rhinorrhoea. The diagnosis of CSF rhinorrhoea was excluded in all patients using beta-2-transferrin with or without fluorescein lumbar puncture. The authors believe that measurement of beta-2-transferrin should be mandatory before surgery for CSF rhinorrhoea.

Osteosarcomatosis (also known as multifocal osteosarcoma or multiple sclerotic osteosarcoma) is a rare condition, characterized by multiple skeletal lesions at initial presentation. We describe a case of osteosarcomatosis involving craniofacial bones presenting with cranial nerve palsies. In addition, a brief review of the literature is given, with a discussion of this highly aggressive form of osteosarcoma.

Plasma cell tumours of the mastoid are rare malignancies of haematological origin. Two patients are described with a plasma cell tumour in the mastoid bone. In one patient it concerned a solitary plasmacytoma of the bone and in the other a focus in the mastoid of a recently diagnosed multiple myeloma. Symptoms in these patients were non-specific. Computed tomography (CT) and magnetic resonance image (MRI) scanning showed a nonspecific space-occupying lesion in the mastoid. The diagnosis was made on immunohistological examination, that showed diffuse sheets of monoclonal plasma cells in a clear matrix. When a plasmacytoma lesion is found multiple myeloma should always be excluded. Treatment in case of solitary plasmacytoma of the mastoid bone consists of radiotherapy. In case of localization in the mastoid of multiple myeloma the treatment consists of palliative chemotherapy.

The role of routine coagulation studies in the management of patients suffering from epistaxis is unclear. In an attempt to address this issue the case notes of all emergency admissions for epistaxis to a large Scottish teaching hospital were retrospectively reviewed over a one-year period. One hundred and forty patients (63 male, 77 female) were admitted between January and December 1998. The patients who had coagulation studies were identified and their results analysed. A total of 121 patients (86.4 per cent) had coagulation studies performed. Of these, 10 (8.3 per cent) had abnormal results and all were taking warfarin or a combination of warfarin and aspirin. No other coagulation abnormalities were identified. This study supports the view that there does not appear to be a role for routine coagulation studies in patients admitted with epistaxis. The investigation for potential haemostatic disorders should be performed when clinically indicated and, if necessary, in consultation with the haematology service.

This paper reports a case of spontaneous indirect carotid cavernous fistula that presented with pulsatile tinnitus, left-sided temporal headache and left-sided ptosis. The pulsatile tinnitus, its aetiology and investigation are discussed. The importance of pulsatile tinnitus is highlighted, with a discussion of carotidcavernous fistulas. This case illustrates that clinically silent cavernous sinus thrombosis can give rise to spontaneous indirect carotid cavernous fistula. Magnetic resonance imaging angiography was used in diagnosis. Treatment ranges from observation, as in our case, to transvenous endovascular techniques.

Amyloidosis of the upper aerodigestive tract is relatively rare. A case of localized amyloidosis involving all components of Waldeyer’s ring with added laryngeal involvement is described. This has not been previously reported. A literature review of this conditions is presented.

We report what we believe to be only the 10th case of palatal necrosis secondary to cocaine abuse in a 33-yearold female patient. Extensive necrosis also involved the cartilaginous and bony septum and paranasal sinuses. Following exclusion of other mid-line destructive diseases her treatment involved saline douches and cessation of cocaine. She remains under review within the department with no evidence of progressive disease. We present a review of the other nine cases of palatal necrosis reported in the world literature and demonstrate a greater incidence in female users. The various presenting conditions of cocaine abuse encountered within the head and neck region by the otorhinolaryngologist are then discussed.

Fine needle aspiration cytology (FNAC) is commonly used in the diagnostic work-up of head and neck masses. Complications are extremely uncommon. We describe a case of monocular blindness following FNAC of a neck mass. To our knowledge this is the first case described in the literature.

Since 1994, a US Food and Drug Administration clinical trial evaluated the multichannel auditory brainstem implant (ABI) on 92 subjects with neurofibromatosis type 2 (NF2). The trial has shown that 85 per cent of patients receive auditory sensations. A small number of patients demonstrate a clinically significant degree of open-set sentence recognition in the sound-alone condition; however, when the ABI is combined with lip-reading cues, 93 per cent of patients demonstrate improved sentence understanding at three to six months. In addition, the majority of recipients report daily use of their devices, and satisfaction with the decision to receive the ABI.

We report a rare case of isolated extramedullary plasmacytoma (EMP) of the right true vocal fold in a 38-year-old male with a one-year history of hoarseness. Immunohistochemical staining of plasma cells in the tumour, showed over 90 per cent of them to be positive for kappa light chains. After two attempts at local surgical excision and recurrence within 10 months, the tumour was irradiated.

Only seven reported cases of isolated EMP of the true vocal fold are reported in the literature. The therapeutic options are discussed.

Although radiation damage to the inner ear can be induced experimentally in animals, its incidence in humans as a complication of therapy for nasopharyngeal carcinoma (NPC) remains a matter of conjecture due to inadequate histological validation. A case of advanced NPC with a mixed conductive and neurosensory hearing loss is presented that at necropsy showed tumour invasion of the VIIIth cranial nerve in the internal auditory meatus with associated infection. The architecture of the organ of Corti was well-preserved despite heavy doses of radiation but degeneration, the cause of which could not be substantiated, was present in the auditory nerve pathway. A diagnosis of radiation damage to the end-organ of hearing in the absence of supporting histological evidence should be made with caution.

A 63-year-old woman had disabling positional vertigo for four months. She showed intense direction-changing apogeotropic nystagmus. Conservative treatment failed to resolve her vertigo. From the findings of the nystagmus, cupulolithiasis of the right lateral canal was suspected. Plugging of this canal successfully eliminated the nystagmus and positional vertigo. Positional vertigo can sometimes be disabling and unresponsive to conservative therapy. Careful analysis of the nystagmus may allow selection of the most appropriate treatment.

Porencephalic cyst expanding into the ear is a very rare complication of temporal bone fracture. We report a case of a 20 – year – old male who developed a traumatic porencephalic cyst of the temporal lobe expanding into the ear through a tegmen fracture in association with a cholesteatoma. The clinical presentation was otitic meningitis. This occurrence was not encountered in any of the cases reviewed in the literature. The diagnosis, pathogenesis, treatment and prognosis of this condition are reviewed.

The 18q-syndrome is associated with hearing impairment in 50-80 per cent of cases. The hearing loss may be sensorineural or conductive. A high proportion of cases are associated with narrow or stenosed external auditory canals. This may be a useful clinical pointer to the syndrome. Two cases with impaired hearing are presented in this paper including one case with complex external ear and middle ear malformations. The clinical and audiological features in each case are described.

Several lipomas of the nasal cavity and paranasal sinus have previously been reported to date. We present a 21-year-old case with nasal lipoma situated on the left posterior of part of the nasal septum, operated on endoscopically. The present case is, to our knowledge, the first report on nasal lipoma in the adult.