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This paper reviews five cases of malignant otitis externa treated in Portsmouth between 1979 and 1986. These five patients constitute all the cases seen in the Portsmouth, South East Hampshire, Chichester and South West Sussex area during the period, indicating the condition to be rare. All cases showed a number of features in common: all were elderly (over 55 years); diabetic; the majority (4:1) being male. In all cases Pseudomonus aeruginosa was cultured from pus exuding from the external auditory canal. Each of the five cases was resident in the Chichester and South West Sussex part of the catchment area. Every case presented with otorrhoea and otalgia and all were treated with a combination of topical and intravenous antibiotics plus intensified diabetic control. Two cases followed ear syringing. Neurological complications seen in this series of patients included facial palsy (three cases), fifth nerve palsy, dysphagia, vocal cord palsy, ataxia and Argyll-Robertson pupil. [ABSTRACT FROM AUTHOR]

Pure-tone audiometry was carried out on members of a recently described maternal lineage with sensorineural deafness, harbouring a novel mitochondrial mutation in the gene for tRNA-ser(UCN). This revealed a characteristic pattern of symmetrical bilateral sensorineural hearing losses in each affected individual, predominantly affecting the high-frequencies, but with considerable variability between individuals. No clear correlation was observed between age and severity, but most subjects reported progressive worsening of their condition. Some members of the lineage were found to be heteroplasmic for the tRNA-ser(UCN) mutation. However, the severity of hearing loss was poorly correlated with the representation of the mutant mtDNA, indicating that other, as yet unidentified factors must be involved in the aetiology of this disorder.

We have detected a novel mitochondrial mutation in a maternal pedigree, at least 13 of whose members have sensorineural hearing loss of varying severity, but who exhibit no other pathological features. The mutation, at np 7445, converts the 3' terminal T residue of tRNA-ser(UCN) to a C, and also brings about a silent alteration to the COI stop codon. The mutation destroys an XbaI site, within which a second mutation, at np 7444, has previously been reported in association with Leber's hereditary optic neuropathy. Predominantly mutant mtDNA was found in all 13 family members surveyed, whether or not they are overtly affected by deafness, and some individuals appeared homoplasmic, within the limits of detection. The novel mutation was not found in over 600 normal controls, nor in any of 27 other maternally unrelated individuals with deafness Other mutations found in mitochondrial disorders were also absent from this pedigree.

Acute supraglottic laryngitis and epiglottitis is increasingly recognised in adults. We present a case in which the condition was the presenting feature of acute myeloid leukaemia and in which Branhamella catarrhalis was the causative organism.