Tympanic Membrane Retractions in patients with Williams Syndrome: A Controlled Study

Introduction  The role of elastin in tympanic retractions and chronic otitis media is not well established. Williams Syndrome (WS), a pathology related to a mutation in the elastin gene, could generate tympanic retractions. Objective  To compare the prevalence of tympanic retractions among patients with WS and controls. Methods  WS patients ( n  = 43 ears) and controls ( n  = 130 ears) were evaluated by digital otoscopic examination and the degree of tympanic membrane retraction was classified by 2 blinded experienced otolaryngologists. Results  The agreement rate between the evaluators was 71.1% for pars tensa and 65% for pars flaccida retraction ( p  < 0.001). The pars tensa and pars flaccida retractions are present in patients with WS after an adjusted residue of respectively - 2.8 and - 2.6 ( p  = 0.011 and p  = 0.022) compared with controls. Conclusions  Tympanic membrane retractions are not more common in the WS group when compared with controls.