Sensorineural hearing loss (SNHL) has been a well-documented complication of sickle cell disease in the literature from West Africa, West Indies, United States of America and the Middle East. We present a study of 52 patients with homozygous sickle cell disease and 36 control patients with haemoglobin genotype AA, matched for age and sex. Seven patients with sickle cell disease (13.5 per cent) were found to have sensorineural hearing loss i.e.>20 dB at two or more frequencies, while all the patients in the control group had normal hearing (p<0.05).Our study shows the incidence of SNHL in the UK to be similar to that reported in the US A and much lower than that found in malaria endemic areas of the tropics.We highlight the factors which we consider responsible for these differences and suggest that the crucial period in the development of SNHL in sickle cell disease may be intra-uterine or during the first few years of life. All sickle cell patients should be encouraged to have regular hearing assessment.
Forty-five patients with homozygous sickle cell disease who had tonsillectomy for recurrent tonsillitis, when compared with 45 matched controls with haemoglobin genotype AA, showed significant differences in the clinical manifestations and complications of recurrent tonsillitis between the two groups. Although throat swabs in the sickle cell group were mostly negative because they were on prophylactic penicillin, all tonsils harboured Streptococcus pneumoniae when cultured. This study suggests the tonsils to be the more specific source of pneumococcal infection that causes systemic complications which increase morbidity and mortality in sickle cell disease. Although the sickle cell patients may be less clinically symptomatic with tonsillitis, the incidence of serious complications caused by pneumococcal infections, now shown to arise from the tonsils, is significant. Adenotonsillar hypertrophy is linked with an increased risk of a sleep apnoea which causes serious neurological complications such as cerebral infarction and stroke. Tonsillectomy has greatly reduced the incidence of complications from pneumococcal infections in the sickle cell group and should therefore be recommended for sickle cell patients taking prophylactic penicillin and still developing pneumococcal infections.
Metastatic carcinoma of the ethmoids is very rare. Of the few cases reported in the literature, the majority are renal in origin. Although metastases from the gastrointestinal tract have been reported. there are no known cases of metastases from the stomach.We present a rare case of metastatic adenocarcinoma of the ethmoids in a patient previously treated for adenocarcinoma of the stomach and describe the possible mechanisms of spread. The patient had a craniofacial resection of the tumour but unfortunately died four weeks post-operatively from acute liver failure probably caused by a liver metastasis.
A cause of nasal obstruction in sickle cell anaemia not previously described in the literature is presented. The pathophysiology is the same as in priapism because of the similarities in the vascular arrangement of the turbinate and the penis. Various treatment options are considered but the best results were achieved by surgical reduction of the inferior turbinates.
An unusual case of squamous cell carcinoma of the middle ear and mastoid in which syncope was a major presenting feature is reported. No such case has been reported in the literature. A possible explanation is offered.
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