Laryngeal ulceration in Behçet's disease: the role of centres of excellence in the UK.

INTRODUCTION: Behçet's disease (BD) is a rare complex multisystem inflammatory condition characterized by the triad of symptoms: aphthous ulcers of the mouth and genitalia and uveitis. CASE REPORT: We present the case of a 31-year-old Caucasian male who had a prolonged journey from first presentation until diagnosis of BD. For 11 years, he presented symptoms affecting the oral cavity and oropharynx, with worsening odynophagia and dysphagia and ultimately, development of stridor. Flexible laryngoscopy showed significant laryngopharyngeal ulceration and scarring. Treatment was with colchicine, corticosteroids and azathioprine and supervised by one of three newly established BD Centres of Excellence in the UK. DISCUSSION: Although uncommon, ENT manifestations in patients with BD should be taken into consideration to allow for early recognition and treatment of what can become a life-threatening condition. In such situations, early referral to a BD Centre of Excellence is essential to provide confirmation of diagnosis and supervision of treatment.

UK and Ireland experience of bone anchored hearing aids (BAHA) in individuals with Down syndrome.

OBJECTIVE: To evaluate the indications, surgical techniques and post-operative problems seen in children with Down syndrome fitted with bone anchored hearing aids (BAHA). METHODS: A postal survey of all registered United Kingdom and Ireland BAHA centres. RESULTS: There was a 98% response rate to the survey. Eighty-one centres in the United Kingdom and Ireland undertake BAHA surgery. Eighteen centres were identified to have undertaken BAHA surgery on individuals with Down syndrome. A total of 43 individuals were implanted. Twenty-four cases were under the age of 16. Soft tissue complications were encountered in 21 patients (49%). Osseointegration failures were encountered in four cases (9%). There was a high level of satisfaction with the BAHA system amongst patients, parents and carers. CONCLUSIONS: BAHA is a valuable method of hearing amplification in children with Down syndrome. It should be considered not as a primary method of amplification, but in the overall management of individuals with Down syndrome after conventional hearing aids and/or ventilation tubes have been considered or already failed. The survey shows a high patient and carer satisfaction with the system, despite short term early soft tissue complications.