Tinnitus is a common experience with up to one third of the adult population experiencing it at some time in their life. Less than 1% of the adult population have tinnitus of sufficient severity to affect their quality of life seriously (although up to 8% may seek medical advice about it). Much of the severity of tinnitus relates to the individuals' psychological response to the abnormal tinnitus signal. The prevalence of tinnitus increases in association with high frequency hearing loss. There is, unfortunately, no diagnostic test that either confirms the presence of tinnitus or its severity. Currently there is no satisfactory severity grading system. A five-point severity grading scheme is therefore proposed and the entry criteria detailed. The five severity points are: slight, mild, moderate, severe and catastrophic. Categorization as 'severe' or 'catastrophic' should be, by epidemiological definition, very rare. General guidance, theory and evidential support are contained within.

BackgroundCanal wall down and canal wall up mastoidectomy represent two surgical approaches to middle ear cleft pathology. Very few studies have examined the effects of these procedures both on the patients' well being and on the resources needed to maintain that state. In this study the authors report the outpatient attendance pattern of canal wall down mastoidectomy patientsMethodsThis is a retrospective case-note review of 101 patients who underwent a CWD mastoidectomy at Derriford Hospital, Plymouth, UK. All surgery was performed by the senior author (PCW-T) between 1985 and 1997. The main outcome measures were the frequency of outpatients' visits, clinical problems at visit and the percentage of discharged patients.ResultsThe studied patients made a total of 1341 outpatient visits between November 1985 and December 1998 with an average of 13.3 visits per patient (median of 11 visits). Almost two thirds of the group still attend for regular follow up. The greatest number of visits occurred in the first 24 months after surgery. The commonest reasons for outpatient visits were the removal of the clinical features of chronic cavity inflammation. Residual/recurrent cholesteatoma, residual perforations and structural cavity problems were infrequent.ConclusionCWD mastoidectomy carries an intrinsic morbidity resulting in a long term attendance in the outpatients.

Congenital spinal fusion, constituting the Klippel-Feil anomaly, has long been known to be associated with severe deafness in many patients. Ten such cases are described, with audiometric and tomographic assessment of the ear lesions. Of the 20 ears examined, 12 showed evidence of severe hearing loss and of these, 11 had evidence of significant inner ear dysplasia on tomography. Middle and external ear abnormalities were also demonstrated. The significance of these findings and the relation to other syndromes is discussed.

SUMMARYA case of cervical chordoma in a man of 27 years is described. The clinical features were of progressive spinal cord compression. Treatment was by transoral decompression of the second cervical vertebra and postoperative radiotherapy. The result was favourable and remained so at follow-up after 27 months. The relative merits and scope of the various approaches to the upper cervical vertebrae and the role of radiotherapy and chemotherapy are discussed. CHORDOMA is a rare tumour of the axial skeleton whose clinical features bring it within the scope of the ophthalmologist, otolaryngologist, neurosurgeon, orthopaedic surgeon and general surgeon.Since its identification in 1856 by Luschka, about 1000 cases have been reported and about 10-15 new cases are described each year. The most comprehensive recent review (Utne and Pugh, 1955) covers 505 cases from the world literature and 72 cases from the Mayo Clinic.Clinical features Statistically, chordoma accounts for 5 per cent of all benign bone tumours (Coley, 1960), less than 2 per cent of all nasopharyngeal tumours (Osborn, 1975) and less than 1 per cent of all central nervous system neoplasms (Poppen and King, 1952). The origin of a chordoma is from the notochord or its remnants (Wright, 1967).Any age group may be affected, though the peak incidence is in middle age. The sex distribution is not clear-cut, the series varying in their results. Most chordomas affect the craniocervical or the sacrococcygeal regions.The pathology and histology have been well described by Steegman (1971) and the ultrastructure by Gessaga et al. (1973). Metastasis is rare in head and neck chordoma but it may be local to lymph nodes or within the central nervous system (Holzner, 1954). Histology shows malignant features, but these correlate poorly with clinical behaviour.The diagnosis depends on radiological and histological findings. The clinical picture is one of a local space-occupying lesion arising in bone, usually without local or distant metastasis. Systemic features are undiagnostic, though lassitude and weight loss are fairly frequent. The results of haematological, serological and biochemical investigations are usually normal. There are no specific radiological changes that differentiate a chordoma from other bone tumours. Plain radiography with tomography and contrast studies are of great benefit in defining spread and involvement. Angiography is useful in that it not only excludes other pathological conditions, but also gives some indication as to the vascularity of the tumour.A biopsy may be a prelude to or accompany definitive surgery. Much has been made of the difficulty in diagnosis (Higinbotham et al., 1967), but given a good specimen, the features are unmistakable. TreatmentIncreasing optimism has been shown concerning the role of radiotherapy (Rosenqvist and Saltnan, 1959;Windeyer, 1959) as operation alone has proved inadequate. The latest reviews (Pearlman and Friedman, 1970; Pearlman et a]., 1972; Phillips and Newman, 1974) suggest that a combination of radical op...