ESPE Abstracts

Objectives: Hearing loss and ENT problems are frequently seen in children with Achondroplasia. Current international consensus guidance recommends audiological assessment before the age of one year and thereafter in childhood in presence of speech delay, hearing difficulties or features of middle ear effusion. In January 2019, we began a programme of annual hearing screening for children with Achondroplasia residing in Glasgow who attend the Complex Bone Clinic (CBC) at Royal Hospital for Children Glasgow (RHCG). Our aim is to assess whether this screening programme is an effective use of resources, with a worthwhile rate of detecting new otological problems.

Methods: Data was collected on age, ear and hearing symptoms, audiometric test results and subsequent outcomes for the first four calendar years of the screening programme. The Primary outcome was the pick-up rate of new hearing screening problems at each screening visit.

Results: 10 Children with a diagnosis of achondroplasia were identified who lived within Greater Glasgow and Clyde. Seven Children (Five Females) participated in the in the annual hearing screening programme at some point with a Median Age of 10.8 years (Range 6.1 to 18.4 years). The pick-up rate of new problems was as follows: Of the seven children who participated, six had an episode of hearing loss documented at some point, with prevalence in individual years ranging from 25-80%. One child had normal hearing throughout. One Child had pre-existing conductive hearing loss which remains managed with hearing aids. Three had a new episode of hearing loss detected by screening and were referred to ENT (Ear, Nose, and Throat) surgeons. An additional child was also referred to ENT for treatment. One Child had hearing loss which resolved following treatment then recurred.

Conclusions: Results at this stage suggest there is a worthwhile pick-up rate of new otological problems and continued detection beyond inaugural year highlights annual screening is likely beneficial for this cohort, although larger case numbers are required.