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Evaluation of otorhinolaryngological manifestations in patients with primary ciliary dyskinesia
  • +5
  • Onder Gunaydın,
  • Ergin Eroğlu,
  • Burçay Tellioğlu,
  • Nagehan Emiralioğlu,
  • ugur ozcelik,
  • Ebru Yalcin,
  • Deniz Dogru,
  • Nural Kiper
Onder Gunaydın
Hacettepe Universitesi Hastaneleri

Corresponding Author:[email protected]

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Ergin Eroğlu
Hacettepe Universitesi Hastaneleri
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Burçay Tellioğlu
Hacettepe Universitesi Hastaneleri
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Nagehan Emiralioğlu
Hacettepe Universitesi Hastaneleri
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ugur ozcelik
Hacettepe Universitesi Hastaneleri
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Ebru Yalcin
Hacettepe Universitesi Hastaneleri
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Deniz Dogru
Hacettepe Universitesi Hastaneleri
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Nural Kiper
Hacettepe Universitesi Hastaneleri
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Abstract

Background: Primary ciliary dyskinesia (PCD) is a genetic disease characterized by recurrent respiratory tract infections. Although the pulmonary manifestations of the disease are well defined, data on otorhinolaryngological complications are insufficient. This study aimed to reveal the prevalence, clinical course and related factors of ear-nose-throat (ENT) manifestations in PCD patients. Methods: PCD patients followed in the ENT department of our center for the last 21 years were included in this retrospective observational study. The frequency of sinonasal and otological diseases, the relationships between clinical and demographic data, and possible risk factors for otorhinolaryngological findings were investigated. Results: Of the 121 patients recruited, 53% were male and the median age at diagnosis was 7 (1 month - 20 years) years. The most common ENT manifestation was otitis media with effusion (OME) (66.1%), followed by acute otitis media (43.8%), acute rhinosinusitis (ARS) (28.9%), chronic rhinosinusitis (CRS) (27.3%), and chronic otitis media (10.7%). The mean age of patients with ARS (18 vs. 16 years, p=0.045) and CRS (19 vs. 16 years, p=0.028) was higher than those without. The annual number of ARS was positively correlated with age (r= 0.170, p=0.06). A total of 45 patients had audiograms and the most common finding was conductive hearing loss (53.3%). OME significantly increased this risk of tympanic membrane injury (OR: 8.6, 95% CI: 3.6-20.3, p<0.001). Although not statistically significant, hearing loss was more common in patients who had OME compared to those who did not (76.5% vs. 45.5%, OR: 3.9, 95% CI: 0.94-16.2, p=0.062). Conclusions: Otorhinolaryngologic diseases are common in PCD patients and the awareness of ENT physicians should be expanded. Our study showed that with increasing age, patients may present with ARS and CRS more. Presence of OME is the most important risk factor for tympanic membrane damage and the most common type of hearing loss is conductive failure.