Introduction
Primary ciliary dyskinesia (PCD) is a heterogeneous inherited ciliopathy
characterized by impaired airway mucosal clearance. The disease mainly
presents with recurrent upper and lower respiratory tract infections
(LRTI) and their complications.1,2 Situs inversus or
infertility may accompany the clinical picture. Due to the low incidence
and undiagnosed mild cases, the accurate prevalence is unclear, but is
estimated from 1:40,000 to 1:7,550.3 Although there is
no gold standard diagnostic test, nasal nitric oxide (nNO), high-speed
video microscopy analysis (HSVA), transmission electron microscopy (TEM)
and genetic tests are used for diagnosis.4,5 The
European Respiratory Society task force recommends exploring PCD in
patients with persistent wet cough, situs or congenital heart anomalies,
persistent rhinitis, chronic otitis, or neonatal respiratory
morbidities.6
It is well known that otorhinolaryngological manifestations such as
chronic or recurrent rhinosinusitis, middle ear infections, otitis media
with effusion are quite common in PCD patients.7–9Accordingly, permanent hearing loss, vestibular and balance impairments
have also been shown.10,11 However, although pulmonary
diseases are well defined in these patients, the data in the field of
otolaryngology are not satisfactory. Pieces of knowledge are
inconsistent because ear-nose-throat (ENT) studies are mostly
single-center, retrospective, non-standardized, and have a small number
of patients.12 Recently, the study protocol of the
first international, prospective ENT cohort in PCD patients was
published and the study is ongoing now.13 The aim of
our study was to determine the prevalence of sinonasal and otological
disorders in the PCD cohort of 121 patients and to reveal the ENT
findings with associated clinical and demographic factors.