Diagnosis and Rehabilitation Approaches in Children with Hearing Loss and Deafness in Russian Federation
https://doi.org/10.15690/pf.v22i4.2939
Abstract
Impaired hearing in children such as hearing loss and deafness is crucial medical and social topic. Auditory function deterioration in a child can lead to mental and speech development delays. Preventive measures and timely diagnosis of defective hearing are extremely important to avoid any difficulties with social adaptation. This article covers key methods for diagnosis deafness and hearing loss in children, considers treatment of children with acute and chronic sensorineural hearing loss, and discusses issues of deafness surgical treatment via cochlear implantation in children. Cochlear implantation proved to be the most effective method for rehabilitation and social adaptation of children with significant hearing loss or deafness. Children with grade IV hearing loss and deafness can fully develop and integrate into society after successful cochlear implantation surgery and high-quality postoperative rehabilitation. This article contains basic data that pediatricians and otorhinolaryngologists should know when working with children with severe sensorineural hearing loss. Cochlear implantation is the advanced and effective technique helping children suffering from grade IV sensorineural hearing loss and deafness.
About the Authors
Valentin I. Popadyuk
RUDN University
Russian Federation
Disclosure of interest:
Russian Federation
MD, PhD, Professor
Moscow
Disclosure of interest:
Not specified.
Evgenii S. Savelev
RUDN University ; The National Medical Research Center of Otorhinolaryngology
Russian Federation
Disclosure of interest:
Russian Federation
MD
6, Miklukho-Maklaya Str., Moscow, 117198
Disclosure of interest:
Not specified.
Elena E. Saveleva
Bashkir State Medical University
Russian Federation
Disclosure of interest:
Russian Federation
MD, PhD, Associate Professor
Ufa
Disclosure of interest:
Not specified.
References
1. Tavarkiladze G.A. Hearing loss and deafness — global problem of the modern health care. Almanac of the Institute of Special Education. 2021;(45):1–8. (In Russ).
2. Deafness and hearing loss. 26 February 2025. In: World Health Organization: Official website. (In Russ). Доступно по: https://www.who.int/ru/news-room/fact-sheets/detail/deafness-and-hearing-loss. Ссылка активна на 31 июля 2025.
3. Harrison Bush AL, Lister JJ, Lin FR, et al. Peripheral hearing and cognition: evidence from the Staying Keen in Later Life (SKILL) study. Ear Hear. 2015;36(4):395–407. doi: https://doi.org/10.1097/AUD.0000000000000142
4. O’Donoghue GM, Pisoni D. Auditory and linguistic outcomes in pediatric cochlear implantation. In: Cochlear implants. Waltzman SB, Roland JT, Jr., eds. 3rd edn. New York: Thieme; 2014. pp.158–165.
5. Brody JE. Hearing loss costs far more than the ability to hear. September 28, 2015. In: The New York Times: Website. Available online: https://archive.nytimes.com/well.blogs.nytimes.com/2015/09/28/hearing-loss-costs-far-more-than-ability-tohear/?_r=0. Accessed on August 1, 2025.
6. Pashkov AV, Namazova-Baranova LS, Vishneva EA, et al. Hearing Loss Effect on the Educational Process in Children and Adolescents. Voprosy sovremennoi pediatrii — Current Pediatrics. 2020;19(4):272–278. (In Russ). doi: https://doi.org/10.15690/vsp.v19i4.2134
7. Daikhes NA, Machalov AS, Balakina AV, et al. Audiological features of the management of patients who underwent surgical interventions on the structures of the middle ear during the use of the cochlear implantation system. Rossiiskaya otorinolaringologiya = Russian Otorhinolaryngology. 2022;21(4):103–112. (In Russ). doi: https://doi.org/10.18692/1810-4800-2022-4-103-112
8. Koroleva IV, Kuzovkov VE. Cochlear Implantation — a High-Tech Method of Hearing Restoration in Deaf Children. Consilium Medicum. Pediatrics (Suppl.). 2015;(2):46–50. (In Russ).
9. Altman YaA. Rukovodstvo po audiologii. Moscow: DMK-Press; 2003. 359 p. (In Russ).
10. ЧChibisova SS, Markova TG, Alekseeva NN, et al. Epidemiology of hearing loss in children of the first year of life. Vestnik otorinolaringologii = Russian Bulletin of Otorhinolaryngology. 2018;83(4):37–42. (In Russ). doi: https://doi.org/10.17116/otorino201883437
11. Boboshko MYu, Savenko IV, Garbaruk ES, et al. Prakticheskaya surdologiya. St. Petersburg: Dialog; 2021. 420 p. (In Russ).
12. Tavartkiladze GA, Zagoryanskaya ME, Rumyantseva MG. Metodiki epidemiologicheskogo issledovaniya narushenii slukha: Methodological recommendations. Moscow; 2006. 27 p. (In Russ).
13. Fortnum HM, Summerfield AQ, Marshall DH, et al. Prevalence of permanent childhood hearing impairment in the United Kingdom and implications for universal neonatal hearing screening: questionnaire based ascertainment study. BMJ. 2001;323(7312):536–540. doi: https://doi.org/10.1136/bmj.323.7312.536
14. Neirosensornaya tugoukhost’ u detei: Clinical guidelines. Union of Pediatricians of Russia; National Medical Association of Otolaryngologists. Ministry of Health of Russia; 2024. 65 p. (In Russ).
15. Watkin P, Baldwin M. The longitudinal follow up of a universal neonatal hearing screen: the implications for confirming deafness in childhood. Int J Audiol. 2012;51(7):519–528. doi: https://doi.org/10.3109/14992027.2012.673237
16. Deev IA, Kobyakova OS, Starodubov VI, et al. Obshchaya zabolevaemost’ detskogo naseleniya Rossii (0–14 let) v 2023 godu: Statistical materials. Moscow: Central Research Institute of Public Health of the Russian Ministry of Health; 2023. 156 p. (In Russ).
17. Markova TG. Nasledstvennye narusheniya slukha. In: Otorinolaringologiya: National guidelines. Palchun VT, ed. 2nd edn. Moscow: GEOTAR-Media; 2016. pp. 723–732. (In Russ).
18. Tufatulin GSh., Koroleva IV. Organizatsiya surdologicheskoi pomoshchi detyam. St. Petersburg: Publishing house of NWSMU named after I.I. Mechnikov; 2021. 188 p. (In Russ).
19. Bolezni ukha, gorla, nosa v detskom vozraste: National guidelines. Bogomilsky MR, ed. Moscow: GEOTAR-Media; 2021. 1010 p. (In Russ).
20. Pashkov AV, Savelyeva EE, Polunina TA, et al. Objective methods of diagnosing hearing impairment in children of the first years of life. Pediatricheskaya farmakologiya — Pediatric pharmacology. 2014;11(2):82–85. (In Russ). doi: https://doi.org/10.15690/pf.v11i2.963
21. Konstantinova N.P. Ototoxicity of Medications. RMJ. 2001;(16):715. (In Russ).
22. Garbaruk ES, Kalmykova IV, Vershinina EA. Sensonevral’naya tugoukhost’ i slukhovaya neiropatiya u nedonoshennykh detei. Rossiiskaya otorinolaringologiya = Russian Otorhinolaryngology. 2008;(S1): 228–232. (In Russ).
23. Boboshko MYu, Garbaruk ES, Markova TN, et al. Sensonevral’naya tugoukhost’ u detei: Clinical recommendations. St. Petersburg; 2016. 29 p. (In Russ).
24. Akinpelu OV, Waissbluth S, Daniel SJ. Auditory risk of hyperbilirubinemia in term newborns: a systematic review. Int J Pediatr Otorhinolaryngol. 2013;77(6):898–905. doi: https://doi.org/10.1016/j.ijporl.2013.03.029
25. Borkoski-Barreiro SA, Falcon-González JC, Liminana-Canal JM, Ramos-Macias A. Evaluation of very low birth weight (< 1,500 g) as a risk indicator for sensorineural hearing loss. Acta Otorrinolaringol Esp. 2013;64(6):403–408. doi: https://doi.org/10.1016/j.otorri.2013.05.002
26. Shapiro SM. Bilirubin toxicity in the developing nervous system. Pediatr Neurol. 2003;29(5):410–421. doi: https://doi.org/10.1016/j.pediatrneurol.2003.09.011
27. Rakhmanova IV, Dyakonova IN, Ishanova YuS. The hearing function in prematurely born children during the first year of life depending on the duration of gestation estimated by the otoacoustic technique.Vestnik otorinolaringologii = Russian Bulletin of Otorhinolaryngology. 2011;(6):20–23. (In Russ).
28. Savenko IV, Boboshko MYu. The hearing function in the prematurely infant. Vestnik otorinolaringologii = Russian Bulletin of Otorhinolaryngology. 2015;80(6):71–76. (In Russ).
29. Tavartkiladze GA, Markova TG, Tsygankova ER, et al. Diagnostika nasledstvennoi patologii v praktike vracha-surdologa: Tutorial guide. Moscow: RMAPO; 2011. 46 p. (In Russ).
30. Afanas’eva NA, Kaladze NN, Dosikova GV, Rybalko ON. Federal’nye klinicheskie rekomendatsii po okazaniyu meditsinskoi pomoshchi detyam s sindromom Vaardenburga. Simferopol; 2017. 13 p. (In Russ).
31. Markova TG, Geptner EN, Lalayants MR, et al. The clinical definition and etiology of Pendred syndrome (a review of the literature and clinical observations). Vestnik otorinolaringologii = Russian Bulletin of Otorhinolaryngology. 2016;(6):25–31. (In Russ). doi: https://doi.org/10.17116/otorino201681625-31
32. Tsukamoto K, Suzuki H, Harada D, et al. Distribution and frequencies of PDS (SLC26A4) mutations in Pendred syndrome and nonsyndromic hearing loss associated with enlarged vestibular aqueduct: a unique spectrum of mutations in Japanese. Eur J Hum Genet. 2003;11(12):916–922. doi: https://doi.org/10.1038/sj.ejhg.5201073
33. Brophy PD, Alasti F, Darbro BW, et al. Genome-wide copy number variation analysis of a Branchio-oto-renal syndrome cohort identifies a recombination hotspot and implicates new candidate genes. Hum Genet. 2013;132(12):1339–1350. doi: https://doi.org/10.1007/s00439-013-1338-8
34. Goderis J, De Leenheer E, Smets K, et al. Hearing loss and congenital CMV infection: a systematic review. Pediatrics. 2014;134(5):972–982. doi: https://doi.org/10.1542/peds.2014-1173
35. Andrade GM, Resende LM, Goulart EM, et al. Hearing loss in congenital toxoplasmosis detected by newborn screening. Braz J Otorhinolaryngol. 2008;74(1):21–28. doi: https://doi.org/10.1016/s1808-8694(15)30746-1
36. Westerberg BD, Atashband S, Kozak FK. A systematic review of the incidence of sensorineural hearing loss in neonates exposed to herpes simplex virus (HSV). Int J Pediatr Otorhinolaryngol. 2008;72(7):931–937. doi: https://doi.org/10.1016/j.ijporl.2008.03.001
37. Abdulkerimov KhT, Kartashova KI, Abdulkerimov ZKh, et al. The impact of an increased level of acoustic load on the functional state of the auditory analyzer of young people using audio players. Directions of development of preventive and corrective measures. Modern Medicine. 2019;(3):115–117. (In Russ).
38. Kirichenko IM. Kompleksnaya diagnostika kokhleovestibulyarnykh sindromov u bol’nykh rasseyannym sklerozom i vertebro-bazilyarnoi nedostatochnost’yu. [dissertation]. Moscow; 2012. 270 p. (In Russ).
39. С Savel’eva EE. Neirosensornaya tugoukhost’. In: Bolezni ukha, gorla, nosa v detskom vozraste: National guidelines. Bogomilsky MR, ed. Moscow: GEOTAR-Media; 2021. pp. 665–684. (In Russ).
40. Kryukov AI, Kunelskaya NL, Kulagina MI. Vozrastnye osobennosti issledovaniya slukha u detei. RMJ. 2011;19(6):386–389. (In Russ).
41. Guidelines for the early audiological assessment and management of babies referred from the newborn hearing screening programme. Brennan S, ed. BSA; 2021. 55 p. Available online: https://www.thebsa.org.uk/wp-content/uploads/2022/10/OD104-98-BSA-Practice-Guidance-Early-Assessment-Management.pdf. Accessed on August 01, 2025.
42. Tavartkiladze G.A. Rukovodstvo po klinicheskoi audiologii. Moscow: Medicine; 2013. 674 p. (In Russ).]
43. Year 2007 position statement: Principles and guidelines for early hearing detection and intervention programs. Pediatrics. 2007;120(4):898–921. doi: https://doi.org/10.1542/peds.2007-2333
44. Visual Reinforcement Audiometry: Recommended Procedure. BSA; 2024. 36 p. Available online: https://www.thebsa.org.uk/wp-content/uploads/2014/10/OD104-37-BSA-RP-VisualReinforcement-Audiometry.pdf. Accessed on August 01, 2025.
45. Tavartkiladze GA. Funktsional’nye metody issledovaniya slukhovogo analizatora. In: Otorinolaringologiya: National guidelines. Palchun VT, ed. 2nd edn. Moscow: GEOTAR-Media; 2016. pp. 118–155. (In Russ).
46. Garbaruk ES. Audiologicheskii skrining glubokonedonoshennykh detei metodom zaderzhannoi vyzvannoi otoakusticheskoi emissii. Rossiiskaya otorinolaringologiya = Russian Otorhinolaryngology. 2005;(1):47–50. (In Russ).
47. Bogomilsky MR, Rakhmanova IV, Sapozhnikov YaM, Lazarevich AA. Dynamics of acoustic function maturation in premature neonates according to evoked distortion product otoacoustic emission. Vestnik otorinolaringologii = Russian Bulletin of Otorhinolaryngology. 2008;3:4–7. (In Russ).
48. Rakhmanova IV, Sapozhnikov YaM, D’yakonova IN, et al. Metodika audiologicheskogo obsledovaniya nedonoshennykh detei razlichnogo gestatsionnogo vozrasta metodom registratsii vyzvannoi otoakusticheskoi emissii. Moscow; 2010. 30 p. (In Russ).
49. Daikhes NA, Yablonskii SV, Pashkov AV, Naumova IV. Universal’nyi audiologicheskii skrining novorozhdennykh i detei pervogo goda zhizni. Moscow; 2012. 33 p. (In Russ).
50. Savelyeva EE, Arefyeva NA. Hearing loss in children, options in diagnosis and rehabilitation. Meditsinskiy Sovet = Medical Council. 2014;(3):51–54. (In Russ). doi: https://doi.org/10.21518/2079-701X-2014-3-51-54
51. Gnezditskii VV, Korepina OS. Atlas po vyzvannym potentsialam mozga (prakticheskoe rukovodstvo, osnovannoe na analize konkretnykh klinicheskikh nablyudenii). Ivanovo: PresSto; 2011. 532 p. (In Russ).
52. Daikhes NA, Kerbabaev SE, Pashkov AV, et al. Osobennosti ob’ektivnoi otsenki funktsii slukhovogo analizatora u detei. Rossiiskaya otorinolaringologiya = Russian Otorhinolaryngology. 2003;(3):53–55. (In Russ).
53. Sapozhnikov YaM, Minasyan VS, Lazarevich AA. Hearing studies in newborns and infants. Meditsinskaya pomoshch’. 2005;(2):10–12. (In Russ).
54. Tavarkiladze GA. Klinicheskaya audiologiya: National guidelines: In 3 vol. Moscow: GEOTAR-Media; 2024 (In Russ).
55. Katz J. Handbook of clinical audiology. Philadelphia, PA: Williams & Wilkins; 1994. 839 p.
56. Pediatric Amplification: Clinical practice guidelines. American Academy of Audiology; 2013. 60 p. Available online: http://audiology-web.s3.amazonaws.com/migrated/PediatricAmplificationGuidelines.pdf_539975b3e7e9f1.74471798.pdf. Accessed on August 01, 2025.
57. Sapozhnikov YaM, Bogomil’skii MR. Sovremennye metody diagnostiki, lecheniya i korrektsii tugoukhosti i glukhoty u detei. Moscow: Ikar; 2001. 250 p. (In Russ).
58. Tavartkiladze GA, Gvelisiani TG. Klinicheskaya audiologiya. Moscow; 2003. 74 p. (In Russ).
59. Guidelines for the audiologic assessment of children from birth to 5 years of age. American Speech-Language-Hearing Association; 2024. Available online: http://www.infanthearing.org/coordinator_orientation/section2/10_asha_guidelines.pdf. Accessed on August 01, 2025.
60. Vishniakov VV, Sorokina MV. The transtympanic administration of steroids for the treatment of acute sensorineural hearing impairment. Vestnik otorinolaringologii = Russian Bulletin of Otorhinolaryngology. 2014;(4):55–58. (In Russ).
61. Stachler RJ, Chandrasekhar SS, Archer SM, et al. Clinical practice guideline: sudden hearing loss. Otolaryngol Head Neck Surg. 2012;146(3):S1–S35. doi: https://doi.org/10.1177/0194599812436449
62. Jachova Z, Ristovska L. Cochlear implantation in children with hearing impairment. Годишен зборник на Филозофскиот факултет = Annuaire de la Faculté de Philosophie. 2022;75:483–496. doi: https://doi.org/10.37510/godzbo2275483j
63. Warner-Czyz AD, Roland JT, Jr, Thomas D, et al. American Cochlear Implant Alliance Task Force guidelines for determining cochlear implant candidacy in children. Ear Hear. 2022;43(2): 268–282. doi: https://doi.org/10.1097/AUD.0000000000001087
64. Wolfe J. Cochlear implants: Audiologic management and considerations for implantable hearing devices. San Diego: Plural Publishing; 2020. 858 p.
65. Das A, Choudhary A, Hazra S, Sengupta A. Endoscope assisted versus microscopic cochlear implantation — a double blinded randomized trial. Eur Arch Otorhinolaryngol. 2025. doi: https://doi.org/10.1007/s00405-025-09290-3
66. Swain SK. Vertigo following cochlear implantation: a review. Int J Res Med Sci. 2022;10(2):572–577. doi: https://doi.org/10.18203/2320-6012.ijrms20220310
67. MedStar Health. Cochlear implants. 2022. Available online: https://www.medstarhealth.org/services/cochlear-implants. Accessed on August 01, 2025.
68. Banda González RI, Castillo Castillo S, Roque Lee G. Parámetros de programación del implante coclear. Bol Med Hosp Infant Mex. 2017;74(1):65–69 doi: https://doi.org/10.1016/j.bmhime.2017.11.016
69. Savelev ES, Popadyuk VI, Saveleva EE, et al. Cochlear implantation is an effective way to rehabilitate children with severe hearing loss. Meditsinskiy Sovet = Medical Council. 2025;19(5):60–66. (In Russ). doi: https://doi.org/10.21518/ms2025-094
70. Koroleva IV, Ogorodnikova EA, Levin SV,processor fitting in patients with cochlear implants. Vestnik otorinolaringologii = Russian Bulletin of Otorhinolaryngology. 2021;86(1):30–35. (In Russ). doi: https://doi.org/10.17116/otorino20218601130
71. Boboshko MYu, Riekhakainen EI. Rechevaya audiometriya v klinicheskoi praktike. St. Petersburg: Dialog; 2019. 80 p. (In Russ).
72. Klyachko DS, Pashkov AV, Gadaleva SV, Naumova IV. The electrically evoked compound action potential of the auditory nerve. Literature review. Rossiiskaya otorinolaringologiya = Russian Otorhinolaryngology. 2018;(4):99–120. (In Russ). doi: https://doi.org/10.18692/1810-4800-2018-4-99-120
73. Bakhshinyan VV. The current tendencies and prospects of the neural response telemetry in the rehabilitation of the patients after cochlear implantation. Vestnik otorinolaringologii = Russian Bulletin of Otorhinolaryngology. 2014;(2):21–25. (In Russ).
74. Yanov YuK, Pudov VI, Klyachko DS. ntraoperative stapedial reflexes usage for speech processors set up. Rossiiskaya otorinolaringologiya = Russian Otorhinolaryngology. 2012;(5):140–143. (In Russ).
75. Zhou H, Chen Z, Shi H, et al. Categories of Auditory Performance and Speech Intelligibility Ratings of Early-Implanted Children without Speech Training. PLoS One. 2013;8(1):e53852. doi: https://doi.org/10.1371/journal.pone.0053852]
76. Farag HM, Osman DM, Safwat RF. Language profle of children with cochlear implants: comparative study about the efect of age of cochlear implantation and the duration of rehabilitation. Eur Arch Otorhinolaryngol. 2024;281(8):4393–4399 doi: https://doi.org/10.1007/s00405-024-08689-8
77. Mosaed NAM, Mohamed ES, Youssif M, et al. Preoperative variables affecting outcome of cochlear implant. Egypt J Otolaryngol = EJO. 2024;40(1):113. doi: https://doi.org/10.1186/s43163-024-00563-y
78. Ramadhanti A, Lasminingrum L, Mahdiani S, et al. Level of Knowledge, Attitude, and Behavior among Specialists of Otorhinolaryngology-Head and Neck Surgery in West Java Towards Cochlear Implants. Althea Medical Journal. 2023;10(1):43–50. doi: https://doi.org/10.15850/amj.v10n1.2684
79. MEDEL Elektromedizinische Geräte GmbH. Conducting medical procedures on users of the MED-EL cochlear implant system. 32 p. (In Russ). Доступно по: https://www.medel.com/docs/default-source/isi-important-safetyinformation/languages/ru-ru-russian-russia/ci-cochlear-implants/aw41575_20_manual-medical-procedures-ci-abi-systems-rurussia-rus_web.pdf?auto=format&sfvrsn=9f669f42_6. Ссылка активна на 28.07.2025.
Review
For citations:
Popadyuk V.I., Savelev E.S., Saveleva E.E. Diagnosis and Rehabilitation Approaches in Children with Hearing Loss and Deafness in Russian Federation. Pediatric pharmacology. 2025;22(4):419-431. (In Russ.) https://doi.org/10.15690/pf.v22i4.2939
Views:
17
Email this article 