Otitis media prone children with cystic fibrosis: A new normal

McCoy, Jennifer L.; Kaffenberger, Thomas M.; Yang, Tiffany S.; Dohar, Joseph E.

doi:10.1016/j.amjoto.2021.103137

Cited by 1 publication

(2 citation statements)

References 36 publications

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“…Several studies have identified a higher prevalence of hearing loss in CF patients; this hearing loss is due to not only ototoxic effects that cause sensorineural hearing loss, but also due to conductive conditions that may be linked with otitis media and ET dysfunction 17,18,[42][43][44] . Accumulating evidence supports that individuals with CF have a higher prevalence of developing otologic disorders, including otitis media 16,19,45,46 . Health of the middle ear is very likely related to CHL in patients with CF, yet the middle ear has not been extensively studied using physiologically relevant in-vitro models.…”

Section: Discussionmentioning

confidence: 99%

“…However, most studies were not specifically designed to test for middle ear disorders, such as middle ear infection or CHL. Several recent studies conducted with traditional audiometric and otologic techniques found that the CF group had a significantly higher occurrence rate of OME and CHL compared to the control group 18,19 . In CHL due to OME, sound energy conduction may be attenuated by as much as 30-40 dB 20 .…”

Section: Introductionmentioning

confidence: 92%

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A potential acoustic role for CFTR ion channel in conductive hearing loss

Liyanage,

Mun,

Carraro

et al. 2023

Preprint

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Loss-of-function mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene cause cystic fibrosis (CF). The middle ear and eustachian tube could be adversely affected in CF. In this study, we provide evidence of the role of CFTR function in conductive hearing. We developed an in-situ model to determine CFTR dependent fluid secretion in the middle ear using native mouse auditory capsule. A unique middle ear-on-a-chip was developed to address the functional and molecular basis of conductive hearing impairment. Using single-cell transcriptomics, middle ear cell composition and the associated transcriptomic signature were compared between CF and WT groups. A specialized subset of epithelial cells expressed CFTR with an overlapping signature with secretory epithelial cells. Genes related to ciliogenesis, hearing and ossification were significantly altered in CF mice middle ear. Our data suggest that CF middle ear may be at higher risk for conductive hearing loss.

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