We reviewed the clinicopathological characteristics of 13 cases of calcifying epithelial odontogenic tumour (CEOT) (Pindborg tumour) diagnosed in the Division on Stomatology, Institute for Medical Research, Kuala Lumpur, over a 29-year period. There were eight female and five male patients. These consisted of eight (61.5 per cent) Malays, three (23.1 per cent) Chinese, one (7.7 per cent) Indian and one (7.7 per cent) Melanau. Their ages at presentation ranged from 19–61 years (mean age, 31.8 years). There were 12 central and one peripheral CEOT. Of these, 76.9 per cent of cases were located in the maxilla, the remaining in the mandible. The commonest clinical diagnosis was a dentigerous cyst (66.7 per cent). Enucleation was the main mode of treatment. Histologically, sheets and strands of polyhedral epithelial cells containing eosinophilic, homogeneous globules with Liesegang rings were observed. One case also showed extensive calcification and clear cell differentiation. Immunohistochemistry revealed a variable keratin staining of the CEOT epithelium, confirming its heterogeneity.

A three-generation family with Saethre-Chotzen syndrome and an isolated case are presented. The proband presented with conductive hearing loss. His mother and grandmother showed minor features of the syndrome including conductive hearing loss.

Symptoms of the craniosynostosis syndromes can include stapes ankylosis, a fixed ossicular chain in a too small epitympanum, and small or even absent mastoids. The proband was treated with a boneanchored hearing aid (BAHA) instead of reconstructive middle ear surgery. Current literature on the results of ear surgery is reviewed. In general, reconstructive middle ear surgery should only be considered if congenital anomalies of the middle ear are the only presenting symptom. In cases with additional anomalies such as atresia of the ear canal or damage due to chronic ear infections, the outcome of reconstructive surgery to correct the anomalous ossicular chain is unsatisfactory. In such cases the BAHA is probably the best solution.

Whilst the oral contraceptive pill (OC) has been implicated on a number oif occasions as a cause of sensorineural hearing loss, there are no published reports linking hormone replacement therapy (HRT) to otological symptoms. A case of sensorineural loss with tinnitus following commencement of HRT is described, followed by a discussion outlining the fundamental differences between the OC and HRT, thus expaining why a vascular aetiology is unlikely. It is hypothesized that otological symptoms in such cases may be due to the effect of oestrogens on electrolyte balance disturbing inner ear function and also a direct effect on the auditory pathways mediated in part by alterations in neurotransmitter receptor concentrations.

The ‘Darling’ elbow rest provides a compact, easily manoeuvrable platform for the surgeon's elbow during endoscopic sinus surgery, stabilizing the endoscope and increasing operative safety.

A case is described of a primary neuroblastoma of the facial nerve in a 13-year-old boy presenting with a recurrent facial paralysis. This tumour was excised preserving the nerve and followed with post-operative radiotherapy. The pathology of the tumour is described and facial nerve tumours briefly discussed as a cause of facial palsy. There have been no other cases of a primary neuroblastoma of the facial nerve arising at this site reported in the last 20 years.

Antral puncture and lavage through the inferior meatus is a minor but common otolaryngological procedure, usually performed under local anaesthesia. We describe a new method of introducing local anaesthetic into the inferior meatus, via the use of a soft intravenous cannula connected to a syringe containing 10 per cent cocaine paste. We have called this new technique inferior meatal cannula anaesthesia (IMCA).

Non-tuberculous mycobacterial (NTM) infections usually present as an enlarged lymph node in the neck of a non-immunocompromised child. The differential diagnosis includes bacterial adenitis, malignant disease and tuberculosis. The definitive diagnosis relies upon isolating the organisms in culture. The treatment is complete surgical excision with, or without, anti-tuberculous chemotherapy

Ten cases of NTM infections are presented with a discussion of the aetiology and treatment of this condition.

We report a rare case of relapsing polychondritis with an initial symptom of inner ear involvement. This 53-year-old Japanese man experienced a hearing difficulty, tinnitus in both ears, and dizziness of sudden onset, but lacked auricular chondritis at that time, which is the most frequent finding in relapsing polychondritis. Thus it was difficult to reach a correct diagnosis. Steroid therapy, with oral prednisolone 15 mg daily, was effective. Almost two months after we began the steroid therapy, the patient complained of losing interest in his work and reported a hallucination vision on the TV screen, so the dose of prednisolone was decreased to 10 mg. The hallucinations then disappeared, but the serum level of C-reactive protein increased highly. To reduce the dose of prednisolone, we tried low-dose oral methotrexate. However, we had to discontinue it when the patient experienced severe vomiting and diarrhoea. As adjuvant therapy, we then administered Sho-saiko-to, Chinese herbal medicines with few side effects. Symptoms and laboratory abnormalities then improved markedly.

Multiple sclerosis is known to affect the myelin of the auditory pathway resulting in acute hearing loss. Two cases of sudden deafness due to multiple sclerosis have been evaluated by conventional audiometry, brainstem auditory evoked response audiometry and transtympanic electrocochleography. The abnormalities of the compound action potential in both patients (enhanced latency, abnormal adaptation using fast stimulus rate) and the normal receptor potentials (cochlear microphonic, summating potential), as well as the absence of brainstem responses suggest a disturbance of synchronization at the level of the first auditory neurone. The electrocochleography provides valuable information for the topodiagnosis of this and other neural hearing losses, especially in the absence of reliable brainstem responses.

Collapse of the external auditory meatus during audiometry can lead to spuriously increased hearing thresholds being obtained, particularly at high frequencies, and may simulate conditions such as noise-induced hearing loss, presbyacusis and retrocochlear pathology. Consequently, inappropriate investigations and management may be undertaken. Two patients with elevated thresholds secondary to ear canal collapse are described. The implications of initially failing to identify the true nature of their ‘hearing losses’ are highlighted and strategies to avoid such pitfalls are discussed.

This case describes an apparent improvement in hearing sensitivity in a young girl over a period of 12 months, after she was fitted with binaural hearing-aids. Discrepancies between objective and subjective test results are highlighted. Even if the underlying reason for the conflicting test results was due to poor listening skills or test error, this child behaved like a hearing-impaired child and her performance improved after hearing-aid use. The case also illustrates the application of earphone testing from six months of age. The importance of closely monitoring all children who are fitted with hearing-aids is highlighted.

Superficial siderosis is a rare central nervous system disorder characterized by deafness, ataxia, and pyramidal signs. The hearing loss is believed to be predominantly neural and is usuallly progressive and bilateral. Careful assessment is therefore necessary to determine the best approach to hearing rehabilitation. A case is presented of usperficial siderosis in a young woman who has benegited significantly from cochlear implantation using the Nucleus dvice.

A case of nasal septal perforation secondary to cryoglobulinaemia is reported. In this instance the cryoglobulinaemia was due to Waldenstrom's macroglobulinaemia (lymphoplasmocytic lymphoma with IgM paraprotein) in which the paraprotein was a potent cryoglobulin.

Cochlear implantation is now a routine clinical procedure for deaf patients in many countries (Gibson, 1987). It replaces the function of damaged cochlear hair cells and, therefore, detects sound and by electrical stimulation produces an appropriate signal in the remaining cochlear nerve fibres (House and Berliner, 1991). Wegener's granulomatosis is an uncommon auto-immune disease. It has a peak incidence at the fifth decade with slight male predominance. Nasal problems are the predominant presentation with otological manifestations presenting rarely. We report a case of Wegener's granulomatosis presenting with total hearing loss and after right cochlear implantation a free field threshold of 40 dB and 20 per cent Bamford-Kowal-Bench (BKB) speech test.

We conclude that Wegener's granulomatosis is not an absolute contraindication for cochlear implantation.

Posterior rhinoscopy remains a valuable yet often difficult examination by conventional means. Fibreoptics, although providing an excellent alternative, may not always be available. We have combined a St. Clair Thomson's mirror with a Lack's metallic tongue depressor for ease of examination and patient cooperation. It is possible to examine the nasopharynx with 6 mm, 8 mm, 10 mm and 12 mm mirrors. The instrument may be boiled, is sturdy, economical and extremely useful in paediatric practice.

Wegener's granulomatosis is a multi-system disease characterized by granuloma formation and a necrotizing vasculitis. It classically presents with involvement of the upper and lower respiratory and renal systems. However locoregional disease is common and may include otological manifestations. Facial nerve palsy has been reported during the course of the disease process but it is extremely rare for it to be the presenting feature. Previously reported cases have involved a protracted diagnostic process including exploratory tympanotomy, mastoidectomy and facial nerve decompression. We report a case of Wegener's granulomatosis which presented with a facial nerve palsy. An early diagnosis was achieved by measurement of the erythrocyte sedimentation rate (ESR), followed by serological assay of cytoplasmic anti-neutrophil cytoplasmic antibody (cANCA). Histological examination of nasal mucosal biopsies confirmed granuloma formation consistent with a diagnosis of Wegener's granulomatosis. This allowed early treatment with immunosuppressive therapy and avoided unnecessary and potentially hazardous middle ear surgery.

Three cases of patients with acute middle ear infections complicated by a sixth nerve palsy are described. The possible causes of sixth nerve palsy in such cases are discussed. We propose that where there is a sixth nerve palsy without petrositis and without raised intracranial pressure, it can arise as a result of spreading phlebitis along the inferior petrosal sinus from the lateral sinus.

Pleomorphic adenoma arising in the external auditory canal is rare. We report the case of a 38-year-old man. To better grasp the clinical features and natural history of this uncommon tumour, we also reviewed the world wide literature and found 24 similar cases, which we analysed together with our own.

The acquired immune deficiency syndrome (AIDS) epidemic and other potentially fatal blood-borne infectious diseases have produced a heightened awareness for preventing accidental inoculation of surgeons and operating theatre staff. It is incumbent upon the head and neck surgeon to use safe and effective surgical technique in these patients to prevent such an event. We are seeing an increasing number of patients with AIDS and hepatitis undergoing tracheotomy. We present a sharps-free technique for tracheotomy which is safe for the hospital staff and the patient.

With the recent advances in the management of vestibular schwannomas, it is possible not only to save the facial nerve function but also preserve hearing in a small percentage of cases. Difficulties arise while managing patients with vestibular schwannoma in their only hearing ear. In this article we summarize our experience in managing seven of these patients. We recommend a watch and wait policy with a regular follow-up with audiometric testing and gadolinium-enhanced magnetic resonance imaging (MRI). Gamma knife radiosurgery is advised in cases with deterioration of hearing or increase in tumour size. Surgery is usually avoided unless there are brainstem compression symptoms.