SUMMARY The invariable presence of otitis media with effusion in children with cleft palate aged from 2 to 20 months is confirmed in a prospective trial, and the diagnosis was made in each case by myringotomy. Treatment planning should take account of this, and long term ventilation of at least one ear seems to be mandatory in order to correct bilateral congenital deafness. Close cooperation between otologist and plastic surgeon is essential for diagnosis and treatment of otitis media in these patients.The association between hearing loss and a cleft palate is well established. 1-4 The hearing loss observed in adults with cleft palate is usually conductive and related to scarring, adhesions, and perforations of the tympanic membrane. It is suggested that these symptoms are late sequelae of -untreated or unresolved middle ear effusions.5 6 Much recent research centres on otological problems in children and infants with cleft palate.
This paper presents the results of a national comparative audit of mastoid surgery, retrospectively analysing 611 operations by 55 consultants. Confidentiality for both patient and surgeon was assured. For cholesteatoma 80% of mastoidectomies performed were open cavity procedures and for other middle ear disease 58% used an intact canal wall technique. Thirty-two surgeons used only an open cavity mastoidectomy for cholesteatoma during the period of the audit. There was a statistically significantly greater number of wet ears with open cavities than with closed cavities, but the frequency of the operator did not correlate with the dry ear rate. Ten serious complications (facial palsy and dead ear) were recorded. All occurred in open cavity mastoidectomies. The post-operative hearing status was unknown in 40% of patients.
Despite the setbacks successes have been achieved. Of the 18 SCID patients treated for their life threatening condition, 17 are still alive and have had a healthy, functioning immune system for up to five years and a good quality of life. In addition to monogenic conditions, gene therapy also represents a viable treatment for multigenic disorders, such as cancer, either as a standalone treatment or in combination with chemotherapy or radiotherapy. A huge amount of work is yet to be done, but with efficient monitoring of trials and continuous improvements of viral vectors, gene therapy may still represent an important addition to the treatment armamentarium for a range of diseases.
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