National survey of hearing assessment in patients with cystic fibrosis in the UK

Abstract

Background: Hearing loss is a major health and social problem that affects everyday life.Patients with Cystic Fibrosis (CF) are at high risk of hearing loss as a result of receiving multiple courses of aminoglycoside antibiotics.In addition some patients also carry the mitochondrial mutation m.1555A>G which leads to severe and rapid onset hearing loss when exposed to aminoglycosides.Identifying patients at risk of hearing loss is essential to early recognize, prevent and treat. Aim: To evaluate the current practice of hearing assessment in patients with CF among the UK CF centers. Method: A six questions online anonymous survey was distributed to all UK pediatric and adult CF centers. Results: 21/49(43%)CF centre responded.Only 5/21(23.8%) do not routinely assess if patients with CF have any hearing concern.At annual review of patients with CF; 12/21(57%) routinely check if any hearing concerns and 10/21(47.6%) perform routine hearing test.18/21(85.7%) do not use aminoglycosides for patients with hearing loss. 17/21(80.9%) have alternative antibiotics to aminoglycoside listed at local hospital CF guideline. 3/21(14.2%) routinely refer patients with CF for genetic analysis of aminoglycoside ototoxicity mutation m.1555A>G before starting aminoglycoside. 8/21(38%) refer CF patients with sensorineural hearing loss for genetic analysis of aminoglycoside ototoxicity mutation m.1555A>G. Conclusion: This survey indicates a wide variation in current practice among the UK CF centers considering hearing assessment, screening and testing for genetic risk factor in patients with CF including the ones who receive aminoglycosides.A national guideline of hearing assessment among this group of patients is required.