Learning Objectives:

Introduction: Primary Ciliary Dyskinesia (PCD) describes a group of inherited disorders that result in abnormal ciliary motion leading to mucous stasis. Clinical features almost always include otitis media with effusion (OME). PCD patients provided us with a cohort of patients with OME that is not treated with ventilatory tube insertion as these have been shown to result in chronic ottorhoea, early extrusion and persistent perforation without significant improvement to hearing in the long term. The most popular theory of cholesteatoma formation is invagination of the tympanic membrane due to a negative middle ear pressure, as found in OME. We used this cohort to investigate whether children with PCD and OME were predisposed to Cholesteatoma formation.

Methods: We performed a retrospective observational review of all the children attending a multi-disciplinary PCD clinic at a national quaternary referral centre. With thorough review of the documentation from their clinic appointments and audiology assessments we collected data regarding the management of the OME.

Results: We found that out of 144 patients on their database, almost all of them had a diagnosis of otitis media with effusion at some point. Of these, the only children who had insertion of a ventilatory tube were those who had the procedure before the diagnosis of PCD was made. The majority of children had none or mild hearing loss and therefore did not require any intervention. Those with moderate to severe hearing loss were referred for fitting of hearing aids. None of the children with OME were diagnosed with a Cholesteatoma in the time they had been followed up in the PCD clinic.

Conclusions: Cholesteatoma has not been found to occur in children with PCD despite having OME which is conservatively managed. This contradicts the traditional invagination theory of cholesteatoma formation and in these patients, the mucosal stasis may in fact provide a protective factor against cholesteatoma formation.