Olfaction and Gustation in Children With Primary Ciliary Dyskinesia.

Abstract

Primary ciliary dyskinesia (PCD) is a rare autosomal recessive disorder whereby abnormal cilia cause a wide array of respiratory tract manifestations including chronic rhinosinusitis. The purpose of this study was to determine whether olfaction and gustation are impaired in children with PCD. Cross-sectional study. Tertiary pediatric academic hospital. Children with confirmed PCD based on having at least 1 of 3 approved diagnostic criteria as per The American Thoracic Society guidelines were recruited from The PCD Clinic in our tertiary care pediatric hospital. Odor identification ability was tested using the Universal Sniff (U-Sniff) test and taste threshold was measured using an electrogustometer. The main outcome of this study is to determine the incidence of olfactory dysfunction in children with PCD and investigate if there is an associated gustatory dysfunction. Twenty-five children participated (14 male, 11 female), The median age was 10.8 years (range: 4.1-17.9 years). Only 4/25 (16%) complained of olfactory dysfunction prior to testing. None of the patients complained of dysgeusia. However, 48% (12/25) scored less than 7 on the U-Sniff, signifying hyposmia or anosmia. In contrast, scores obtained by electrogustometry were in the normal range. There was no correlation between performance on the U-Sniff and electrogustometry testing. Olfactory impairment in children with PCD is common but underrecognized by patients. This is not associated with abnormal gustation. Among other, this places children with PCD at an increased risk with respect to smelling a fire or detecting spoiled or poisonous food.