Otolaryngological manifestations of cystic fibrosis in children: A systematic review

Purpose: Cystic fibrosis (CF) is the most common autosomal recessive disorder in the Caucasian population. Otolaryngological manifestations pose a significant impact on the quality of life of children with CF. The primary aim of this review is to provide a state of the art update of current literature on the otolaryngological manifestations of CF in children. Methods: We systematically reviewed the PubMed, Cochrane Library, Embase and Web of Science databases, for prospective studies including pediatric patients with cystic fibrosis, reporting on otolaryngological manifestations. After assessment of the risk of bias and quality of the included studies, data were extracted. Results: The search retrieved 6745 unique items after duplicate removal. After three selection rounds and quality assessment, 38 articles were ultimately retained for data extraction. The total number of participants in the studies was 1981. Most studies were prospective cohort studies (n = 28). The articles were divided into six groups (ear/speech disorders - aminoglycoside ototoxicity (n = 10); otolaryngology-related quality of life (n = 5); nasal and paranasal sinuses (n = 11); sleep disorders (n = 3); paranasal sinuses imaging (n = 5); other (n = 4)). Conclusion: The most common otolaryngological manifestation of children with CF is chronic rhinosinusitis, but CF can have other otolaryngological-related pathologies such as hearing loss, middle ear problems, sleep apnea syndrome, and decreased smell and/or taste functions. We found a considerable gap in the literature if we would draw evidence-based conclusions on diagnosis and management of otolaryngological manifestations in children with CF.