Clinics in diagnostic imaging (111): X-linked congenital mixed deafness syndrome.

Two siblings, boys aged five and six years old, presented with mixed hearing loss. Computed tomography of the temporal bones showed bulbous dilatation of the internal auditory canals and incomplete separation with the basal turn of the cochlear, consistent with the diagnosis of X-linked congenital progressive mixed deafness syndrome. The diagnosis and management of this rare condition is discussed.

Endolymphatic sac tumour: a rare cause of recurrent vertigo.

Endolymphatic sac tumour occurring in a 32-year-old man presenting with Meniere's like symptoms of recurrent vertigo, hearing loss and tinnitus is described. Magnetic resonance imaging and computed tomography showed a vascular bone tumour centred over the retrolabyrinthine aspect of the temporal bone where the endolymphatic sac was located. Surgical excision via a translabyrinthine approach was performed. Endolymphatic sac tumours are rare papillary adenocarcinomas that arise from the endolympatic sac. It can be mistaken both on radiology and histology for other tumours such as paragangliomas, renal or papillary thyroid carcinoma metastases. Surgical excision is the treatment of choice but sacrifice of the auditory and facial nerve may be needed in advanced cases to achieve tumour clearance.

Vestibular rehabilitation therapy for the dizzy patient.

A customised vestibular rehabilitation therapy (VRT) programme is an important treatment modality in patients with vestibular dysfunction resulting in motion-provoked vertigo, oscillopsia (gaze instability), disequilibrium and gait disturbances. We discuss in this paper the patient selection criteria for VRT, rehabilitation strategies for unilateral and bilateral vestibular deficits, and some of the compelling evidence to support the use of VRT in treating such patients.

Why generic and disease-specific quality-of-life instruments should be used together for the evaluation of patients with persistent allergic rhinitis.

BACKGROUND: The importance of assessing health-related quality of life (HRQL) of patients with allergic rhinitis (AR) has been well established, but the specific roles of rhinitis-specific or general health instruments have not been delineated. OBJECTIVE: We analysed the psychometric properties of a disease-specific instrument, the Rhinoconjunctivitis Quality-of-Life Questionnaire (RQLQ) and the general health instrument, the Medical Outcome Short-Form 36 (SF-36) as they are employed in combination in patients with persistent AR in clinical practice. METHOD: We analysed the data collected from a prospective study of 43 newly diagnosed patients with persistent AR and 44 controls. We interviewed the patients four times, at baseline, weeks 4, 8 and 10. RESULTS: The RQLQ and SF-36 have good discriminative property, internal consistency, and test-retest reliability. The RQLQ is superior to the SF-36 as an evaluative instrument because more of its domains respond to change, the magnitude of change was greater, and the response was faster. The SF-36 is more susceptible to floor and ceiling effects. Both instruments are unsuitable for mildly symptomatic patients based on Rasch model analysis. Each questionnaire assesses a distinct and significant portion of the total HRQL of persistent AR. CONCLUSION: The SF-36 and RQLQ are good for discriminating rhinitis patients from controls, but the former is poor for detecting changes in QOL. Both are inappropriate for mildly symptomatic patients. Each instrument measures non-overlapping halves of the measurable HRQL. For an assessment of the HRQL in persistent AR that is complete and responsive both instruments should be employed together.