Schwannoma arising from the tympanic membrane is a rare neoplasm. This report describes an external ear canal mass obscuring the tympanic membrane. A transcanal approach identified a tumour adhered to the tympanic membrane. The tumour was excised without myringoplasty. Pathology confirmed the diagnosis of schwannoma. Clinical examination revealed no evidence of recurrence during a follow-up period of one year. The possible origins of schwannoma of the tympanic membrane and lesion management are also discussed.

Glomus tympanicum tumour is one of the commonest neoplasms of the middle ear. It is more common in females. The most common presenting symptom is pulsatile tinnitus. A retrotympanic mass is found in the middle ear in all the cases. Computed tomography (CT) scan is the investigation of choice, however in difficult cases magnetic resonance imaging (MRI) can be helpful. Surgery is regarded as the gold standard of treatment in spite of the fact that radiation therapy has also been advocated in the literature. In this series 17 cases of glomus tympanicum treated at Gruppo Otologico Piacenza – Rome (Italy) were analysed retrospectively. A simple and safe technique has been described. All the cases were female and treated by surgery. The tumour was removed completely in all the cases and the ossicular chain kept intact. Recurrence was encountered in only one case after nine years. Surgical removal of the tumour is recommended as the treatment of choice with the following advantages: complete removal, a low complication rate, a low recurrence rate, acceptable hearing level and minimum morbidity.

We report a case of mucoepidermoid carcinoma (MEC) originating from the eustachian tube and middle ear. A 31-year-old male who presented with otorrhoea and methicillin-resistant Staphylococcus aureus (MRSA) in the right ear was admitted to hospital due to cerebral infarction and deep vein thrombosis. After recovery, biopsies from a granulomatous mass found in the middle ear during operation for chro ic otitis media revealed intermediate-grade MEC and a nasopharyngeal mass identified after surgery also revealed the same result. He received combined radiation therapy and chemotherapy and no residual or recurrent tumour was detected after two years of follow-up.

We present a case of Hodgkin's disease of the mastoid associated with upper cervical lymph node involvement without disseminated disease. This patient was treated with chemotherapy alone and lesions responded rapidly. At present the patient is well, with no evidence of recurrence or extension of this disease. Hodgkin's disease of the middle ear and mastoid is an extremely rare disease. After a wide search in the literature no cases of Hodgkin's disease arising in the middle ear and mastoid have been found.

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.

Solitary schwannomas are relatively rare. There has been only one previously reported case, in the English literature, of a schwannoma arising in the external auditory canal. We present a case which was managed by local excision and was unassociated with cochlear or retrocochlear pathology.

We present a case of juvenile xanthogranuloma of the external ear canal in a 51-year-old woman and a review of the literature.

The clinical and pathological features of glandular tumours of the external auditory meatus are presented. Their heterogenous clinical and pathological features make the collective term ‘ceruminoma’ ambiguous andmisleading. The spectrum of pathological behaviour and histological features demonstrated by these interesting tumours necessitate a broader classification system. In our hospital 32 patients presented with tumours of the external auditory meatus over a 30-year period, of which seven were glandular in origin. A review of the histology of these glandular tumours enabled us to reclassify them as adenoma, cylindroma, adenoid cystic carcinoma or ceruminous adenocarcinoma. Together with the less common mucoepidermoid carcinoma and pleomorphic adenoma this subdivision forms a basis for a more meaningful classification system with prognostic and therapeutic implications specific to each tumour type. The term ‘ceruminoma’ should no longer be used unqualified.

Abstract: Primary extramedullary plasmacytoma (PEP) is an uncommon neoplasm of plasma cell origin which afflicts the head and neck mainly. In this study we report a rare case of a 34-year-old man who presented with left ear tinnitus, hearing loss, blocked feeling and headache. Exploratory tympanotomy revealed a mass extending into the attic and the mastoid antrum. Following canal wall-up mastoidectomy, the tumour was carefully removed. Histological examination (including immunoperoxidase staining) and thorough clinical, laboratory and radiological evaluation revealed an exclusively cytoplasmic monoclonal IgG immunoglobulin PEP. The combination of surgery (including a second-look procedure) and radiotherapy used in this case may be an over-treatment. However, the patient is still disease-free seven years after his first admission to hospital.

This paper offers an account of the contemporary surgical approach to advanced tumours of the external ear based on a series of 11 patients. There were eight squamous, two basal cell carcinomas and one mucoepidermoid tumour. The traditional method of excision was slightly modified by performing microsurgical dissection of the lateral part of the temporal bone rather than chisel osteotomies, and then including it en bloc with the involved soft tissues. The defect was then closed using a scalp or myocutaneous flap and this combination of otological and reconstructive expertise has proved satisfactory. Four patients are alive with no evidence of disease a mean of 4.2 (range 1.0–7.0) years from surgery: two patients who remained free of disease have subsequently died of unrelated conditions 12 and 24 months post-operatively, and in three cases death from recurrent disease occurred a mean of 1.4 (range 0.9–2.1) years after our surgery. There were two postoperative deaths. Based on the actuarial survival of 36 per cent and a successful disease clearance rate of 54 per cent, our conclusion is that the outlook of this condition has not dramatically improved since the original descriptions of the management of this problem first appeared, although intervention remains justifiable because of the potential curability and relief of symptoms.

Pilomatrixoma is a benign skin tumour arising from the outer hair root matrix. It is an uncommon tumour which favours the hair bearing area of the head and neck. The preauricular area is a well documented site, but to our knowledge, there are no reports of a pilomatrixoma arising in the external auditory meatus. We present such a case and discuss the historical background along with its pathological and clinical features.

Ceruminous glands should no longer be regarded as purely apocrine glands, but as apoeccrine glands with both apocrine and eccrine modes of secretion. We present two cases of pleomorphic adenoma of ceruminous glands, among the rarest of such tumours. The use of such terms as ‘ceruminoma’ and ‘hidradenoma’ should finally be abandoned, and ‘ceruminous gland tumour’ used instead as a generic term. Classification should be based on Wetli's prototype (adenoma, pleomorphic adenoma, adenoid cystic carcinoma and adenocarcinoma), with the addition of benign eccrine cylindroma and syringocystadenoma papilliferum; the inclusion of mucoepidermoid carcinoma should await full validation. Wide local excision is necessary for all tumours, with only follow-up for histologically benign neoplasms. Malignant tumours need early aggressive surgery and radiotherapy. If marginal invasion cannot be assessed histologically, then adenoma and adenocarcinoma cannot be distinguished and we suggest that the tumour be reported as ‘of uncertain malignant potential’. Long-term studies are needed to confirm or refute the view that all ceruminous gland tumours are potentially malignant.

We present a rare case of a transitional papilloma of the middle ear, occurring several years after excision of a similar lesion from the nose. There was no continuity between the two lesions suggesting that these tumours can be multicentric in origin.

A salivary gland choristoma is an extremely uncommon tumour in the middle ear space. It appears to be a developmental abnormality and may be associated with abnormalities of adjacent structures.

It usually presents with unilateral conductive deafness which may be long–standing and the tumour often pursues a benign, slow growing course. It is usually possible to excise it, but problems may arise as there may be variable associated anatomical abnormalities of the middle ear.

We present the nineteenth recorded case, review the literature and discuss the management of this condition.