Review article
Management of otitis media with effusion in children with primary ciliary dyskinesia: A literature review

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Abstract

Objective

Primary ciliary dyskinesia is an autosomal recessively inherited group of disorders of ciliary ultrastructure. Otolaryngologists are frequently involved in the management of some of the most common symptoms of primary ciliary dyskinesia including chronic rhinitis, sinusitis and otitis media with effusion. A dilemma for otorhinolaryngologists is whether ventilation tubes are of benefit in children with primary ciliary dyskinesia and otitis media with effusion and what effective alternatives exist. This paper aims to address this issue via a literature review and case presentation.

Methods

An extensive review of the literature was undertaken and a discussion of the advantages and disadvantages of ventilation tubes in the management of otitis media with effusion in these children is presented and compared with that of the general population. We present a case of a 9 months old boy with Kartagener's Syndrome and chronic bilateral otitis media with effusion to illustrate our findings.

Results

Eight papers were identified, all with small study numbers. The main outcome measures were hearing, otorrhoea and tympanic membrane structural changes. The natural history of otitis media with effusion and hearing loss in primary ciliary dyskinesia appears to be fluctuant into adulthood. Therefore, otitis media with effusion in primary ciliary dyskinesia does not resolve by the age of 9 years, regardless of treatment, as previously assumed. Ventilation tube insertion improves hearing in primary ciliary dyskinesia, but may lead to a higher rate of otorrhoea when compared to the general population. Tympanic membrane changes were clinically insignificant.

Our patient eventually underwent successful insertion of bilateral ventilation tubes with a marked improvement in hearing and language with minimal otorrhoea.

Conclusion/Discussion

The highest level of evidence found for the management of otitis media with effusion in children with primary ciliary dyskinesia was level IV. Currently, the evidence is inconclusive and conflicting. Whilst our results are promising, clearly higher quality research on a larger number of patients is required to definitively evaluate the management options for otitis media with effusion in these children.

Introduction

Primary ciliary dyskinesia (PCD), formerly known as immotile cilia syndrome, is a rare cause of chronic airway disease in white children [1]. PCD is an autosomal recessive, heterogenous group of disorders of ciliary ultrastructure with variable penetrance [2], [3], [4]. PCD is characterized by cilia that beat more slowly than normal, or with abnormal beat patterns, that result in impaired mucociliary clearance (MCC). PCD affects both genders equally and has an incidence varying from 1 in 15,000 to 1 in 40,000 births [1], [3], [5]. Kartagener's syndrome (KS) is a subgroup of these children with a triad of sinusitis, bronchiectasis and situs inversus and affects approximately 50% of PCD patients [6].

Otolaryngologists are frequently involved in the management of many of the common symptoms of PCD and KS including chronic rhinitis, sinusitis and otitis media with effusion (OME). OME is defined as the presence of middle ear fluid without symptoms or signs of acute inflammation of the middle ear [7] and [8]. Otitis media with effusion (OME) is the most common cause of acquired conductive hearing loss in childhood [9] with a 10–30% prevalence in the 1–3 year age group [7]. The treatment of chronic OME in children aims to improve hearing and to prevent the possible sequelae of long-term hearing loss such as impairments to speech and language development. Treatment also aims to prevent other potential sequelae of OME such as tympanic membrane atelectasis and retraction pockets, ossicular chain erosion or necrosis and cholesteatoma.

The dilemma for Otolaryngologists is whether ventilation tubes (VT) are of benefit in these children or whether, by placement of VT, a dry ear with OME and hearing loss is converted to a chronically discharging ear with OME and hearing loss. This paper presents a review of the current literature on the natural history of OME in PCD and also of the treatment of OME with VT in children with PCD. We look at the factors of hearing loss, post-operative otorrhoea and tympanic membrane changes. We report our experience and follow-up with one such patient.

Section snippets

Method

A review of the literature was undertaken to examine the natural history and treatment of OME in PCD. The literature review encompassed a search of the following databases: Premedline and Medline (1950–2008), Embase, Cochrane Central Register of Controlled Trials, Cochrane Database of Systematic Reviews, Database of Abstracts of Reviews of Effects, ACP Journal Club and Google Scholar. Also, a hand search of references of the relevant literature was undertaken and some authors were contacted for

Case report

K.M. was referred to our clinic at 9 months of age for an opinion regarding the management of his bilateral chronic OME. K.M. has dextrocardia, complete situs inversus, bronchiectasis and chronic sinusitis. Diagnosis of KS at birth was delayed due to a mislabeled chest X-ray (CXR) (Fig. 1). The CXR was performed due to respiratory distress after an uneventful pregnancy and delivery. A repeat CXR at six weeks of age, due to continued respiratory distress and feeding difficulties, revealed

Results

Eight papers were identified. Two studies specifically assessed the natural history of 87 patients with OME and PCD [10], [11]. Six papers reviewed the treatment of 81 patients with PCD and OME [6], [12], [13], [14], [15], [16]. All but 2 of the 8 papers identified [10], [16] were published 10 years ago or more. The 8 papers reviewed comprise: 5 retrospective observational studies/case series [6], [10], [12], [13], [15], one cross-sectional study [11], one case report [16] and one letter in

Discussion

KS is named after a paediatrician who described the triad of bronchiectasis, situs inversus and sinusitis in 4 patients in 1933 [30]. The aetiology and pathogenesis of KS was determined in 1975 when Pedersen and Rebbe [31], and then Afzelius et al. [32] in 1976, described the association of abnormal ultrastructure of spermatozoa with sinusitis, bronchiectasis and situs inversus in infertile men. More recently, Nonaka et al. [33], [34] suggested the role of the motile monocilia of the embryonic

Conclusion

Children in the general population with OME have a conductive hearing loss, which is usually temporary, variable in severity and duration, and may be asymmetric [47]. Normal hearing is usually achieved with conservative management by the age of 8 years [68], [69]. Hearing loss in children with OME and PCD is usually persistent throughout childhood, continues well into adulthood [10], [11], [15], [17], is variable in severity [10], [11] and is bilateral. By providing VT for children with PCD,

Conflict of interest statement

This study was not funded and no author had any conflict of interest regarding this article.

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