Hearing outcomes in children with primary ciliary dyskinesia—a longitudinal study

https://doi.org/10.1016/j.ijporl.2005.02.013Get rights and content

Summary

Primary ciliary dyskinesia (PCD) is a congenital abnormality of ciliary structure or function. The otological manifestations of the disease include otitis media with effusion (OME). To date, the severity of hearing loss and natural progression of OME in this select group of patients has not been documented. In this retrospective observational study, we looked at the tympanograms and audiograms of all children with PCD attending the Royal Brompton Hospital multidisciplinary clinic. Our results show an improvement in both hearing thresholds and tympanograms with age (p < 0.001). Most cases resolve by the age of 12. This supports the current practice of conservative management in these patients. The problems of persistent otorrhoea and residual tympanic membrane perforation are thereby avoided with the reassurance that hearing loss will spontaneously resolve with time.

Introduction

Primary ciliary dyskinesia (PCD) is a group of congenital abnormalities of ciliary structure or function. The pathology lies either in the dynien arms or in the mobility of the cilia. The incidence of PCD is around 1 in 15,000 and it is estimated that there are 3000 cases in the UK. Additionally, there are 70 new cases born every year [1]. Clinical manifestations of PCD include neonatal respiratory distress, chronic rhinosinusitis, otitis media with effusion and subfertility. Some of these patients have situs invertus and the condition is then termed Kartagener's syndrome [2]. PCD is also rarely associated with hydrocephalus [3], congenital cardiac defects, oesophageal disease [4] and biliary atresia [5].

Otitis media with effusion (OME) is very common in children with PCD and is usually managed conservatively with regular audiological assessment, hearing aids and hearing therapy (at school and at home). This is because treatment with a ventilation tube often results in a prolonged and offensive otorrhoea [6], presumably due to abnormal mucociliary function in the eustacian tube and middle ear cleft. To date, the severity of hearing loss and the natural progression of OME in this select group of patients has not been documented. The aims of this study are to address these two questions.

Section snippets

Methods

We performed a cross-sectional retrospective observational review of all children with PCD attending the multidisciplinary clinic at the Royal Brompton Hospital. The children's symptoms and signs on otoscopy were recorded along with their pure tone audiograms and tympanograms. Ears with a ventilation tube in situ or a tympanic perforation were excluded from the study. Children too young to be assessed with an audiogram were also excluded from the study.

The children's age ranged from 3 to 15.

Results

Of a total of 92 children with PCD registered at the Royal Brompton Hospital, we analysed 71 of them. The 21 children not analysed included two, who were too young for hearing assessment with audiograms, and fourteen children who had no ENT complaints when initially assessed at the multidisciplinary clinic. Five sets of notes were unavailable despite extensive efforts to find them. From the study group of 71 children, five had a tympanic membrane perforation unilaterally and three had a

Discussion

In our study we excluded asymptomatic children with PCD (14 out of 92) as well those who had perforations or ventilation tubes (8 unilaterally out of the remaining 71). These excluded patients represent the extreme ends of the spectrum and our sample, therefore, is probably representative of the disease as a whole. Furthermore, the Royal Brompton is a national centre for PCD and a study with numbers as large as this has not been previously reported.

OME in children without an immunological,

Acknowledgement

I would like to thank Joseph Eliahoo from the statistical advisory service at Imperial College, London for his help with the statistical analysis of this paper.

References (7)

  • A. Bush et al.

    Primary ciliary dyskinesia: diagnosis and standards of care

    Eur. Respir. J.

    (1998)
  • M. Kartagener

    Zur pathogenese der bronkiectasian bei situs viscerum invertus

    Britage zur klinik and Erforshung der Tuberkulose and der lungen krankheiten

    (1933)
  • M.A. Greenstone et al.

    Hydrocephalus and primary ciliary dyskinesia

    Arch Dis. Child

    (1984)
There are more references available in the full text version of this article.

Cited by (72)

  • Primary ciliary dyskinesia

    2023, Presse Medicale
  • Primary ciliary dyskinesia in children

    2020, Journal de Pediatrie et de Puericulture
  • 71 - Primary Ciliary Dyskinesia

    2019, Kendig's Disorders of the Respiratory Tract in Children
  • Hearing loss in children with primary ciliary dyskinesia

    2018, International Journal of Pediatric Otorhinolaryngology
    Citation Excerpt :

    Management varies from conservative approaches including antibiotics and hearing aids to surgical interventions with PET. Significant hearing loss related to OME in the general pediatric population typically warrants PET, but this may not be recommended for patients with PCD due to a potentially higher rate of otorrhea after PET insertion [1,3,4,11,24]. Hadfield et al. does not recommend PET insertion in children with PCD because in addition to persistent mucoid discharge, there was no evident advantage in hearing improvement in this subset of patients [6].

View all citing articles on Scopus
1

Tel.: 44 7709443233.

2

Tel.: 44 20 8846 1020; fax: 44 20 8846 1200.

3

Tel.: 44 20 8846 1069; fax: 44 20 8846 1070.

View full text