Preview

M.Ya. Studenikin Russian Pediatric Journal

Advanced search

Hearing disorders in children with muco-obstructive lung diseases

https://doi.org/10.46563/2026-1-1-45-52

Abstract

Introduction. Cystic fibrosis (CF) primary ciliary dyskinesia are hereditary diseases characterized by impaired mucociliary clearance and chronic respiratory tract involvement. 
Aim. To present current literature data on the pathogenesis of hearing loss in muco-obstructive lung diseases and detect risks of aminoglycoside ototoxicity under the modulating therapy for CF in children. There was performed an analysis of scientific literature data from international databases (Scopus, Web of Science, PubMed) and Russian scientific libraries (eLIBRARY.RU, CyberLeninka). Differences in the nature of hearing loss in СF and primary ciliary dyskinesia were established. For the primary ciliary dyskinesia there is characteristic hearing loss, whereas in CF the risk of sensorineural hearing loss persists due to the ototoxicity of aminoglycosides caused by targeted therapy. Personalized treatment and prevention of these forms of pathology in children help reduce risks, necessitating the development of specialized monitoring algorithms. The need for a differentiated approach to monitoring auditory function in patients with muco-obstructive lung diseases has been established.
Contributions: Sokolova V.N. — concept and design of the study; Sokolova V.N., Malyavina U.S., Simonova O.I. — material collection and processing; Sokolova V.N., Malyavina U.S. — writing the text; Simonova O.I., Kondratchikov D.S. — editing the text. All authors approved the final version of the manuscript and are responsible for the integrity of all parts of the article.
Acknowledgments. The study had no sponsorship.
Conflict of interest. The authors declare no conflict of interest.

Received: January 23, 2026

Accepted: February 10, 2026

Published: February 27, 2026

About the Authors

Vera N. Sokolova
National Medical Research Center for Children’s Health
Russian Federation

Vera N. Sokolova, MD, PhD, senior researcher, Laboratory of scientific foundations of otorhinolaryngology

e-mail: sokolova-doki@mail.ru



Ulyana S. Malyavina
National Medical Research Center for Children’s Health
Russian Federation


Olga I. Simonova
National Medical Research Center for Children’s Health; I.M. Sechenov First Moscow State Medical University (Sechenov University); Morozov Children’s Municipal Clinical Hospital
Russian Federation


Dmitriy S. Kondratchikov
National Medical Research Center for Children’s Health
Russian Federation


References

1. Lucas J.S., Carroll M. Primary ciliary dyskinesia and cystic fibrosis: different diseases require different treatment. Chest. 2014; 145(4): 674–6. https://doi.org/10.1378/chest.13-2590

2. Cockx M., Gouwy M., Van Damme J., Struyf S. Chemoattractants and cytokines in primary ciliary dyskinesia and cystic fibrosis: key players in chronic respiratory diseases. Cell. Mol. Immunol. 2018; 15(4): 312–23. https://doi.org/10.1038/cmi.2017.118

3. Pereira R., Barbosa T., Cardoso A.L., Sá R., Sousa M. Cystic fibrosis and primary ciliary dyskinesia: Similarities and differences. Respir. Med. 2023; 209: 107169. https://doi.org/10.1016/j.rmed.2023.107169

4. Kinghorn B., Rosenfeld M., Sullivan E., Onchiri F.M., Brown M.D., Szczesniak R., et al. Comparison of longitudinal outcomes in children with primary ciliary dyskinesia and cystic fibrosis. Ann. Am. Thorac. Soc. 2024; 21(12): 1723–32. https://doi.org/10.1513/AnnalsATS.202311-1008OC

5. Kartal Y., Bozdemir Özel C., Çakmak A., Sonbahar Ulu H., İnal İnce D., Ademhan Tural D., et al. The relationship between lung function, exercise capacity, oxidant and antioxidant response in primary ciliary dyskinesia and cystic fibrosis. Turk. J. Pediatr. 2024; 66(3): 309–22. https://doi.org/10.24953/turkjpediatr.2024.4581

6. Whitsett J.A. Airway epithelial differentiation and mucociliary clearance. Ann. Am. Thorac. Soc. 2018; 15(Suppl. 3): 143–8. https://doi.org/10.1513/AnnalsATS.201802-128AW

7. Hill D.B., Button B., Rubinstein M., Boucher R.C. Physiology and pathophysiology of human airway mucus. Physiol. Rev. 2022; 102(4): 1757–836. https://doi.org/10.1152/physrev.00004.2021

8. Zakharova G.P., Yanov Yu.K., Shabalin V.V. The Mucociliary System of the Upper Respiratory Tract [Mukotsiliarnaya sistema verkhnikh dykhatel’nykh putei]. St. Petersburg: Dialog; 2010. https://elibrary.ru/vtqwdn (in Russian)

9. Kapranov N.I., Kashirskaya N.Yu. Cystic fibrosis. In: Rozinova N.N., Mizernitsky Yu.L., eds. Chronic Lung Diseases in Children. Chapter 6 [Khronicheskie zabolevaniya legkikh u detey. Glava 6]. Moscow: Praktika; 2011: 94–107. (in Russian)

10. Kinghorn B., McNamara S., Genatossio A., Sullivan E., Siegel M., Bauer I., et al. Comparison of multiple breath washout and spirometry in children with primary ciliary dyskinesia and cystic fibrosis and healthy controls. Ann. Am. Thorac. Soc. 2020; 17(9): 1085–93. https://doi.org/10.1513/AnnalsATS.201905-375OC

11. Lobo J., Zariwala M.A., Noone PG. Primary ciliary dyskinesia. Semin. Respir. Crit. Care Med. 2015; 36(2): 169–79. https://doi.org/10.1055/s-0035-1546748

12. Kondratieva E.I., Avdeev S.N., Kiyan T.A., Mizernitsky Yu.L. Classification of primary ciliary dyskinesia. Pul’monologiya. 2023; 33(6): 731–8. https://doi.org/10.18093/0869-0189-2023-33-6-731-738 (in Russian)

13. Takeuchi K., Abo M., Date H., Gotoh S., Kamijo A., Kaneko T., et al. Practical guide for the diagnosis and management of primary ciliary dyskinesia. Auris Nasus Larynx. 2024; 51(3): 553–68. https://doi.org/10.1016/j.anl.2024.02.001

14. Hackenberg S., Renson A., Röseler S.M., Baumann I., Topcuoglu M.Y., Hebestreit H. Pädiatrische rhinologie. Laryngorhinootologie. 2024; 103(S 01): 188–213. https://doi.org/10.1055/a-2178-2957

15. Palchun V.T., Gurov A.V., Mikhaleva L.M., Gordienko M.V. Modern approaches to assessing the significance of chronic inflammation in the mucous membrane of the middle ear. Vestnik otorinolaringologii. 2018; 83(1): 75–80. https://doi.org/10.17116/otorino201883175-80 (in Russian)

16. Selimoglu E. Aminoglycoside-induced ototoxicity. Curr. Pharm. Des. 2007; 13(1): 119–26. https://doi.org/10.2174/138161207779313731

17. Lanvers-Kaminsky C., Ciarimboli G. Pharmacogenetics of drug-induced ototoxicity caused by aminoglycosides and cisplatin. Pharmacogenomics. 2017; 18(18): 1683–95. https://doi.org/10.2217/pgs-2017-0125

18. Steyger P.S. Mechanisms of ototoxicity and otoprotection. Otolaryngol. Clin. North Am. 2021; 54(6): 1101–15. https://doi.org/10.1016/j.otc.2021.08.007

19. Gaafar D., Baxter N., Cranswick N., Christodoulou J., Gwee A. Pharmacogenetics of aminoglycoside-related ototoxicity: a systematic review. J. Antimicrob. Chemother. 2024; 79(7): 1508–28. https://doi.org/10.1093/jac/dkae106

20. Akbari N., Lamooki F.M., Amin M.R., Disnad S.E., Yousefinejad V., Goharnia N. An update on ototoxicity: from a genetic perspective. J. Toxicol. Sci. 2025; 50(6): 245–61. https://doi.org/10.2131/jts.50.245

21. Saibene A., Urbanelli A., Coudré C., Nitro L., Vroegop A., Lejeune B., et al. Otolaryngological manifestations of cystic fibrosis in children: A systematic review. Int. J. Pediatr. Otorhinolaryngol. 2025; 189: 112238. https://doi.org/10.1016/j.ijporl.2025.112238

22. Yilmaz Yegit C., Ergenekon P., Yanaz M., Ozturk Akar N., Toktas Yavuz F., Molla Kafi H., et al. The association between aminoglycoside exposure and ototoxicity in children with cystic fibrosis. Respiration. 2025; 104(1): 48–57. https://doi.org/10.1159/000541447

23. Jørgensen F., Petruson B., Hansson H.A. Extensive variations in nasal mucosa in infants with and without recurrent acute otitis media. A scanning electron-microscopic study. Arch. Otolaryngol. Head Neck Surg. 1989; 115(5): 571–80. https://doi.org/10.1001/archotol.1989.01860290029010

24. Liu X., Cong N., Cheng X., Ma R., Wang J., Huang Y.B., et al. The role of the notch signal pathway in mucosal cell metaplasia in mouse acute otitis media. Sci. Rep. 2017; 7(1): 4588. https://doi.org/10.1038/s41598-017-04639-z

25. Mainz J.G., Koitschev A. Pathogenesis and management of nasal polyposis in cystic fibrosis. Curr. Allergy Asthma Rep. 2009; 9(3): 223–30.

26. Wijers C.D., Chmiel J.F., Gaston B.M. Bacterial infections in patients with primary ciliary dyskinesia: Comparison with cystic fibrosis. Chron. Respir. Dis. 2017; 14(4): 392–406. https://doi.org/10.1177/1479972317694621

27. Polyakov D.P., Daikhes N.A., Bazanova M.V., Melyanovskaya Yu.L. Assessment of the risk of hearing pathology in children with cystic fibrosis. Vestnik otorinolaringologii. 2024; 89(3): 29–35. https://doi.org/10.17116/otorino20248903129 (in Russian)

28. Ishanova Yu.S., Zonenko O.G., Dyakonova I.N., Shumilov P.V., Rakhmanova I.V. Objective control of hearing ability of children with cystic fibrosis who have not complained of hearing loss. Vestnik vosstanovitelnoy meditsiny. 2018; (3): 97–100. https://elibrary.ru/utxlgl (in Russian)

29. Ghedia R., Ahmed J., Navaratnam A. Hearing loss in children with primary ciliary dyskinesia. Int. J. Pediatric Otorinolaryngology. 2018; 104: 161–5. https://doi.org/10.1016/j.ijporl.2017.11.005

30. Andersen T.N., Alanin M.C., von Buchwald C., Nielsen L.H. A longitudinal evaluation of hearing and ventilation tube insertion in patients with primary ciliary dyskinesia. Int. J. Pediatr. Otorhinolaryngol. 2016; 89: 164–8. https://doi.org/10.1016/j.ijporl.2016.08.011

31. Wolter N.E., Dell S.D., James A.L., Campisi P. Middle Ear Ventilation in Children with Primary Ciliary Dyskinesia. Int. J. Pediatr. Otorhinolaryngol. 2012; 76(11): 1565–8. https://doi.org/10.1016/j.ijporl.2012.07.011

32. Ghedia R., Ahmed J., Navaratnam A., Harcourt J. No evidence of cholesteatoma in untreated otitis media with effusion in children with primary ciliary dyskinesia. Int. J. Pediatr. Otorhinolaryngol. 2018; 105: 176–80. https://doi.org/10.1016/j.ijporl.2017.12.015

33. Rivetti S., Romano A., Mastrangelo S., Attinà G., Maurizi P., Ruggiero A. Aminoglycosides-related ototoxicity: mechanisms, risk factors, and prevention in pediatric patients. Pharmaceuticals (Basel). 2023; 16(10): 1353. https://doi.org/10.3390/ph16101353

34. Shubnikova E.V., Velts N.Yu. Ototoxicity of aminoglycosides: modern concepts. Antibiotiki i khimioterapiya. 2022; 67(11-12): 79–90. https://doi.org/10.37489/0235-2990-2022-67-11-12-79-90 https://elibrary.ru/fvhsrq (in Russian)

35. Bravo O., Ballana E., Estivill X. Cochlear alterations in deaf and unaffected subjects carrying the deafness-associated A1555G mutation in the mitochondrial 12S rRNA gene. Biochem. Biophys. Res. Commun. 2006; 344(2): 511–6. https://doi.org/10.1016/j.bbrc.2006.03.143

36. Ouyang L., Ma L., Feng Y. Protective effects of MET channels on aminoglycosidesand cisplatin-induced ototoxicity. Int. J. Med. Sci. 2025; 22(3): 732–44. https://doi.org/10.7150/ijms.103270

37. Gaffney P.J., Shetty K.R., Yuksel S., Kaul V.F. Antioxidant therapies in the treatment of aminoglycoside-induced ototoxicity: a meta-analysis. Laryngoscope. 2025; 135(4): 1278–86. https://doi.org/10.1002/lary.31902

38. Ege T., Tao L., North B.J. The role of molecular and cellular aging pathways on age-related hearing loss. Int. J. Mol. Sci. 2024; 25(17): 9705. https://doi.org/10.3390/ijms25179705

39. Abreu-Silva R.S., Rincon D., Horimoto A.R., Sguillar A.P., Ricardo L.A., Kimura L., et al. The search of a genetic basis for noise-induced hearing loss (NIHL). Ann. Hum. Biol. 2011; 38(2): 210–8. https://doi.org/10.3109/03014460.2010.513774

40. Huth M.E., Ricci A.J., Cheng E.C. Aminoglycoside antibiotics and the cystic fibrosis transmembrane conductance regulator (CFTR): implications for ototoxicity. J. Biol. Chem. 2011; 286(5): 3699–707. https://doi.org/10.1074/jbc.M110.183673

41. Al-Malky G., Dawson D.D., Sirimanna S.J. Aminoglycosideinduced cochlear toxicity in cystic fibrosis: the role of CFTR mutations and the mitochondrial 12S rRNA gene. J. Cyst. Fibros. 2015; 14(4): 448–54. https://doi.org/10.1016/j.jcf.2014.10.003

42. Thomas J.P., Siracusa C., Kessel A. CFTR modulators and ototoxicity: a pharmacovigilance study. J. Cyst. Fibros. 2019; 18(6): 868–71. https://doi.org/10.1016/j.jcf.2019.05.008

43. Nussstein H., Urbantat R.M., Fentker K., Loewe A., Duerr J., Haji M., et al. Changes in sputum viscoelastic properties and airway inflammation in primary ciliary dyskinesia are comparable to cystic fibrosis on Elexacaftor/Tezacaftor/ Ivacaftor therapy. Eur. Respir. J. 2025; 67(2): 2500616. https://doi.org/10.1183/13993003.00616-2025

44. Cederroth C.R., Dyhrfjeld-Johnsen J., Canlon B. Pharmacological approaches to hearing loss. Pharmacol. Rev. 2024; 76(6): 1063–88. https://doi.org/10.1124/pharmrev.124.001195

45. Giersch A.B.S., Morton C.C. Newborn screening for deafness/ hard of hearing in the genomic era. Clin. Chem. 2025; 71(1): 54–60. https://doi.org/10.1093/clinchem/hvae193

46. Kros C.J., Steyger P.S. Aminoglycosideand cisplatin-induced ototoxicity: mechanisms and otoprotective strategies. Cold Spring Harb. Perspect. Med. 2019; 9(11): a033548. https://doi.org/10.1101/cshperspect.a033548

47. Fogliano C., Motta C.M., Avallone B. Salicylate attenuates gentamicin-induced ototoxicity and facilitates the recovery in the basilar papilla of the lizard Podarcis siculus. Neurotoxicology. 2022; 93: 301–10. https://doi.org/10.1016/j.neuro.2022.10.013

48. McGovern M.M., Cox B.C. Hearing restoration through hair cell regeneration: A review of recent advancements and current limitations. Hear Res. 2025; 461: 109256. https://doi.org/10.1016/j.heares.2025.109256


Review

For citations:


Sokolova V.N., Malyavina U.S., Simonova O.I., Kondratchikov D.S. Hearing disorders in children with muco-obstructive lung diseases. M.Ya. Studenikin Russian Pediatric Journal. 2026;1(1):45-52. (In Russ.) https://doi.org/10.46563/2026-1-1-45-52

Views: 178

JATS XML


Creative Commons License
This work is licensed under a Creative Commons Attribution 4.0 License.


ISSN 3033-9006 (Print)
ISSN 3033-9014 (Online)