No evidence of cholesteatoma in untreated otitis media with effusion in children with primary ciliary dyskinesia

Ghedia, Reshma; Ahmed, Jahangir; Navaratnam, Annakan; Harcourt, Jonny

doi:10.1016/j.ijporl.2017.12.015

Cited by 11 publications

(13 citation statements)

References 26 publications

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“…Retrospective medical record review studies of children provide the most current knowledge about PCD-associated ear problems; however, test result and symptom records are not standardized . In these studies, acute ear problems appear to improve with age—probably because of eustachian tube anatomical changes and its changing angle with respect to the base of the skull.…”

Section: Introductionmentioning

confidence: 99%

Characteristics of Otologic Disease Among Patients With Primary Ciliary Dyskinesia

Goutaki

Lam

Alexandru

et al. 2023

JAMA Otolaryngol Head Neck Surg

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ImportanceOtologic disease is common among people with primary ciliary dyskinesia (PCD), yet little is known about its spectrum and severity.ObjectiveTo characterize otologic disease among participants with PCD using data from the Ear-Nose-Throat Prospective International Cohort.Design, Setting, and ParticipantsThis cross-sectional analysis of baseline cohort data from February 2020 through July 2022 included participants from 12 specialized centers in 10 countries. Children and adults with PCD diagnoses; routine ear, nose, and throat examinations; and completed symptom questionnaires at the same visit or within 2 weeks were prospectively included.ExposuresPotential risk factors associated with increased risk of ear disease.Main Outcomes and MeasuresThe prevalence and characteristics of patient-reported otologic symptoms and findings from otologic examinations, including potential factors associated with increased risk of ear inflammation and hearing impairment.ResultsA total of 397 individuals were eligible to participate in this study (median [range] age, 15.2 [0.2-72.4] years; 186 (47%) female). Of the included participants, 204 (51%) reported ear pain, 110 (28%) reported ear discharge, and 183 (46%) reported hearing problems. Adults reported ear pain and hearing problems more frequently when compared with children. Otitis media with effusion—usually bilateral—was the most common otoscopic finding among 121 of 384 (32%) participants. Retracted tympanic membrane and tympanic sclerosis were more commonly seen among adults. Tympanometry was performed for 216 participants and showed pathologic type B results for 114 (53%). Audiometry was performed for 273 participants and showed hearing impairment in at least 1 ear, most commonly mild. Season of visit was the strongest risk factor for problems associated with ear inflammation (autumn vs spring: odds ratio, 2.40; 95% CI, 1.51-3.81) and age 30 years and older for hearing impairment (41-50 years vs ≤10 years: odds ratio, 3.33; 95% CI, 1.12-9.91).Conclusion and RelevanceIn this cross-sectional study, many people with PCD experienced ear problems, yet frequency varied, highlighting disease expression differences and possible clinical phenotypes. Understanding differences in otologic disease expression and progression during lifetime may inform clinical decisions about follow-up and medical care. Multidisciplinary PCD management should be recommended, including regular otologic assessments for all ages, even without specific complaints.

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Section: Introductionmentioning

confidence: 99%

Characteristics of Otologic Disease Among Patients With Primary Ciliary Dyskinesia

Goutaki

Lam

Alexandru

et al. 2023

JAMA Otolaryngol Head Neck Surg

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“…We found that OME caused hearing impairment in 39% of the et al, 2012). However, other studies do not report a clear effect of tube placement on hearing loss in PCD patients (Ghedia, Ahmed, Navaratnam, & Harcourt, 2018;Kreicher, Schopper, Naik, Hatch, & Meyer, 2018). Moreover, chronic problematic otorrhoea (up to 50%) following insertion of ventilation tubes in PCD patients has been reported, adversely affecting both hearing and quality of life (Andersen, Alanin, von Buchwald, & Nielsen, 2016;Campbell, Birman, & Morgan, 2009;El-Sayed, Al-Sarhani, & Al-Essa, 1997;Wolter et al, 2012).…”

Section: Discussionmentioning

confidence: 98%

Primary ciliary dyskinesia in Volendam: Diagnostic and phenotypic features in patients with a CCDC114 mutation

Israëls

Gogh

et al. 2022

American J of Med Genetics Pt C

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Primary ciliary dyskinesia (PCD) is a heterogeneous disease, with impaired mucociliary clearance causing respiratory tract infections. A founding CCDC114 mutation has led to a relatively homogeneous and large Dutch PCD population in Volendam. Our aim was to describe their phenotype. Therefore, all Volendam PCD patients seen at the Amsterdam UMC were included in this study. Data were collected on lung function, microbiology, radiology, and ear-nose-throat (ENT) symptoms. A mixed effects model estimated lung function decline in %point per year (95% confidence interval [CI]). Thirty-three (60%) out of approximately 56 Volendam PCD patients were treated at our center and included in this study. Only 30% of patients had situs inversus. FEV 1 declined in children (À1.43%/year, CI: À1.80/À1.05), but not in adults (0.01%/year, CI: À0.36/0.38). Pseudomonas aeruginosa was cultured in 21% of children and 60% of adults, respectively. Patients who have been infected at some point with P. aeruginosa had a steeper decline in FEV 1 as compared to patients that have never been infected. Neonatal symptoms (79%) and ENT problems (94%) were common; fertility issues however, were not (11%) common. Compared to other PCD cohorts, the Volendam/CCDC114 patients have a moderately severe phenotype with lung function decline predominantly occurring in childhood.

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“…36 As the problem is that of mucociliary dysfunction, the Eustachian tube functions normally and so retraction and cholesteatoma are no more common than expected. 39 After two years of age, the ethmoid sinuses are adequately developed for polyposis: a feature of children with cystic fibrosis. 40,41 We observed none in the 26 patients without beating cilia.…”

Section: Discussionmentioning

confidence: 99%

“…36 As the problem is that of mucociliary dysfunction, the Eustachian tube functions normally and so retraction and cholesteatoma are no more common than expected. 39…”

Section: Discussionmentioning

confidence: 99%

In vitromeasurement of ciliary beat frequency in 92 children with recurrent respiratory tract problems

Drake-Lee

Green²,

McDermott

2022

J. Laryngol. Otol.

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Background Chronic or recurrent mucoid respiratory tract symptoms may be difficult to diagnose. Method Ninety-two children with chronic respiratory symptoms were divided into 4 groups: 18 children with refractory asthma, 10 with bronchiectasis without dextrocardia, 18 with dextrocardia and 46 with recurrent respiratory tract infections. Except for five neonates, cytology samples were taken under general anaesthesia. Ciliary beat frequency was measured photometrically and analysed by in-house computer software. Results Nasal polyps were found in one child with normal ciliary beat frequency. Twenty-six children had no beating cilia (male to female ratio, 15:11). The effect of increasing temperature on the ciliary beat frequency of the remaining 66 patients was evaluated (42 patients, more than 30°C, median, 8.3 Hz; 24 patients, 30–37°C, median, 11.8 Hz; p = 0.0003). Conclusion The measurement of ciliary beat frequency is part of the diagnostic work up of patients with persistent or recurrent respiratory tract infections.

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No evidence of cholesteatoma in untreated otitis media with effusion in children with primary ciliary dyskinesia

Cited by 11 publications

References 26 publications

Characteristics of Otologic Disease Among Patients With Primary Ciliary Dyskinesia

Characteristics of Otologic Disease Among Patients With Primary Ciliary Dyskinesia

Primary ciliary dyskinesia in Volendam: Diagnostic and phenotypic features in patients with a CCDC114 mutation

In vitromeasurement of ciliary beat frequency in 92 children with recurrent respiratory tract problems

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