Primary ciliary dyskinesia (PCD) is characterized by disease of the upper and lower respiratory tract, in association with visceral mirror image arrangement in 50% of cases, due to abnormal structure and/or function of cilia. The purpose of this paper is to review the clinical features, diagnosis and management of PCD. Presentations include neonatal respiratory distress, recurrent lower respiratory tract infection, chronic rhinosinusitis and male infertility. PCD enters the differential diagnosis of bronchiectasis, atypical asthma, and unusually severe upper airway disease. Diagnosis is by a cascade of investigations, starting with the saccharin test in patients older than 10 yrs; ciliary beat frequency and pattern on light microscopy; and electron microscopy to assess ciliary morphology and orientation. It is important not to confuse primary and secondary ciliary abnormalities. Nasal nitric oxide is low in PCD, and this measurement shows promise as a screening test for PCD. Diagnosis is important, in order to prevent the development of bronchiectasis and to avoid any unnecessary otorhinolaryngological procedures. Regular follow-up is essential, and management should be multidisciplinary, with input from centres with a special interest in PCD, having access to paediatric and adult chest physicians, otolaryngologists and audiological physicians, physiotherapists, counselling services and fertility clinics. The prognosis is good, but morbidity can be considerable if PCD is incorrectly managed.

No abstract

Ménière's Society and National Institute for Health Research Imperial Biomedical Research Centre.

No abstract

Twenty-five CAT-scan-confirmed stroke patients and 25 matched controls were studied. All the stroke patients were stable 2-3 months after unilateral hemispheric stroke. Those with ear disease, other central neurological disorder, severe dysphasia and acute or chronic confusion were excluded. There were no significant differences between the groups for average pure tone hearing threshold (APTT) or ability to discriminate pre-recorded speech presented to one ear at 35 decibels (dB) above APTT. The stroke subjects had significantly impaired performance on dichotic competing sentence testing (DCST). Seventeen stroke patients but only one control subject failed DCST. Failure rate was similar for left and right stroke and for temporal and non-temporal lobe involvement. Two-thirds of patients failing DCST did so in the ear opposite the side of the cortical lesion. We conclude that (i) DCST is useful in detecting central auditory dysfunction in stroke patients; and (ii) stroke can affect central auditory perception in older patients.

Eighteen patients were seen over a period of 3 years. Twenty of their ears were diagnosed as having bullous myringitis. Pure tone audiograms and stapedial reflexes were performed within 48 h of referral. Six ears demonstrated sensorineural hearing loss, 7 ears mixed loss and 4 conductive loss. Recovery of sensorineural hearing loss complete in 8 out of 13 ears. Stapedial reflexes were elicited in 5 patients and all showed recruitment. The findings of this study confirms the fact that sensorineural hearing loss is more common in bullous myringitis than previously thought and that it is temporary in many cases. The stapedial reflex results suggest that the site of the lesion is in the cochlea.

We report a case of septicaemia and death due to occult sinusitis in an otherwise healthy adult. Septicaemia was diagnosed on clinical grounds and blood culture grew Streptococcus pneumoniae. Maxillary sinusitis was discovered incidentally on a CT scan four days after the onset of symptoms. A sinus wash-out revealed pus which on culture was positive for Streptococcus pneumoniae. The patient deteriorated gradually and died despite appropriate therapy. We conclude that sinusitis should be suspected in any case of septicaemia where the primary focus is not known and the patient does not respond quickly to treatment.