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Cilia and Ear.

Gioia Piatti1, Maria Margherita De Santi2, Sara Torretta3

  • 11 Department of Pathophysiology and Transplantation, University of Milan, Fondazione IRCCS Ca' Granda, Ospedale Maggiore Policlinico, Division of Bronchopneumology, Milan, Italy.

The Annals of Otology, Rhinology, and Laryngology
|March 15, 2017
PubMed
Summary

Primary ciliary dyskinesia (PCD) frequently causes chronic middle ear effusion in childhood. Adults with PCD require ongoing otological monitoring due to potential upper airway complications.

Keywords:
adultsaudiologyearotitis mediaotological diseasesprimary ciliary dyskinesia

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Area of Science:

  • Otolaryngology
  • Genetics
  • Audiology

Background:

  • Primary ciliary dyskinesia (PCD) is a rare genetic disorder affecting motile cilia.
  • Otological manifestations are common in PCD, but their long-term prevalence in adulthood is less understood.

Purpose of the Study:

  • To determine the prevalence of otological complications in adults diagnosed with primary ciliary dyskinesia.
  • To assess the audiological and vestibular status of adult PCD patients.

Main Methods:

  • A cohort of 23 adult PCD patients underwent detailed medical history focusing on ear, nose, and throat (ENT) symptoms and surgical history.
  • Objective ENT examination, audiometry, tympanometry, otoacoustic emission testing, and vestibular function evaluation were performed.

Main Results:

  • 52% of patients reported chronic middle ear effusion in childhood; no patients had undergone ear surgery.
  • 11 patients had conductive hearing impairment, and 1 had unrelated sensorineural hearing loss; 8 had present distortion product otoacoustic emissions (DPOAE).
  • No vestibular alterations were detected; normal hearing was observed in 11 patients.

Conclusions:

  • Chronic middle ear effusion is highly prevalent in childhood PCD.
  • Adults with PCD warrant careful otological monitoring, especially considering frequent upper airway issues like chronic rhinosinusitis and nasal polyposis.