MANAGEMENT OF CONGENITAL HEARING LOSS: EARLY INTERVENTION STRATEGIES
DOI:
https://doi.org/10.56238/isevmjv5n1-005Keywords:
Congenital Hearing Loss, Congenital CMV Infection, Neonatal ScreeningAbstract
Congenital hearing loss affects approximately 1 to 2 per 1,000 live births, with more than 50% of cases presenting a hereditary etiology. Early recognition of these causes, combined with effective neonatal hearing screening through Transient Evoked Otoacoustic Emissions (TEOAE) and Automated Auditory Brainstem Response (AABR), allows not only timely detection of hearing impairment but also etiological characterization, with a direct impact on therapeutic decision-making, functional prognosis, and family counseling. In this context, advances in molecular genetics and the development of gene therapies have emerged as promising perspectives for the future treatment of monogenic forms of congenital hearing loss (Mey et al., 2025; Lieu et al., 2020; Taylor et al., 2021). Without appropriate treatment, congenital hearing loss can lead to deficits in the development of communication, behavior, and intellect (Lieu et al., 2020; Zhang et al., 2025). Considering this, the identification of congenital hearing loss through Transient Evoked Otoacoustic Emissions (TEOAE) and Automated Auditory Brainstem Response (AABR) in neonatal hearing screening programs is crucial to initiate early rehabilitation with hearing aids or cochlear implants (Mey et al., 2025).
References
Akil, O., et al. (2019). Restoration of hearing in the VGLUT3 knockout mouse using virally mediated gene therapy. Neuron, 75(2), 283–293.
Bollani, L., et al. (2022). Congenital toxoplasmosis: State of the art. Frontiers in Pediatrics, 10, 894573.
Brasil. Ministério da Saúde. (2012). Diretrizes de atenção da triagem auditiva neonatal. Brasília: Ministério da Saúde.
Chung, P. K., et al. (2024). Valganciclovir in infants with hearing loss and clinically inapparent congenital cytomegalovirus infection: A nonrandomized controlled trial. The Journal of Pediatrics, 268, 113–120.e3.
Ding, N., et al. (2021). Advances in genome editing for genetic hearing loss. Advanced Drug Delivery Reviews, 168, 118–133.
Gao, X., et al. (2018). Treatment of autosomal dominant hearing loss by in vivo delivery of genome editing agents. Nature, 553(7687), 217–221.
Gelfand, S. A., & Lentz, J. J. (2020). Regenerative strategies for sensorineural hearing loss. Hearing Research, 397, 107927.
Iyer, A. A., et al. (2024). Induction of hair cell fate by Atoh1 regeneration in the mature mammalian cochlea. Nature Communications, 15(1), 452.
Kelsen, J. R., et al. (2018). GJB2-related hearing loss: Clinical and molecular perspectives. Journal of Medical Genetics, 55(6), 375–382.
Lawrence, S. M., Goshia, T., Sinha, M., et al. (2024). Decoding human cytomegalovirus for the development of innovative diagnostics to detect congenital infection. Pediatric Research, 95, 532–542. https://doi.org/10.1038/s41390-023-02957-9
Lieu, J. E. C., et al. (2020). Hearing loss in children: A review. JAMA, 324(21), 2195–2205. https://doi.org/10.1001/jama.2020.17647
Litovsky, R. Y., et al. (2016). Bilateral cochlear implants in children: Effects of auditory experience and deprivation on auditory perception. Hearing Research, 338, 176–190.
McGovern, M. M., et al. (2024). Reprogramming with Atoh1, Gfi1, and Pou4f3 promotes hair cell regeneration in the adult organ of Corti. PNAS Nexus, 3(10), egae445.
Mey, K., et al. (2025). Congenital hearing loss in children. Seminars in Medical Practice, 187, 45–56.
Morton, C. C., & Nance, W. E. (2006). Newborn hearing screening—A silent revolution. New England Journal of Medicine, 354(20), 2151–2164.
Niparko, J. K., et al. (2010). Spoken language development in children following cochlear implantation. JAMA, 303(15), 1498–1506.
Nussbaum, R. L., McInnes, R. R., & Willard, H. F. (2016). Thompson & Thompson: Genética médica (8ª ed.). Rio de Janeiro: Elsevier.
Pesch, M. H., et al. (2021). Cytomegalovirus infection in pregnancy: Prevention, presentation, management and neonatal outcomes. Journal of Midwifery & Women’s Health, 66(5), 611–623.
Santos, R. M. S., et al. (2025). The effectiveness of congenital toxoplasmosis treatment in minimizing hearing loss: A systematic review. Science Progress, 108(2), 1–15.
Shearer, A. E., & Smith, R. J. H. (2015). Massively parallel sequencing for genetic diagnosis of hearing loss: The new standard of care. Otology & Neurotology, 36(3), 507–512.
Smith, R. J. H., Bale, J. F., & White, K. R. (2005). Sensorineural hearing loss in children. The Lancet, 365(9462), 879–890.
Taylor, C. M., et al. (2021). 16p11.2 recurrent deletion. In GeneReviews. University of Washington.
Wang, X., et al. (2025). Viral-mediated connexin 26 expression combined with dexamethasone rescues hearing in a conditional Gjb2 null mice model. Advanced Science, 12(29), e2406510.
World Health Organization. (2021). World report on hearing. Geneva: WHO.
Zhang, Y., et al. (2025). Hearing loss in infants and children with asymptomatic congenital cytomegalovirus infection: An update in diagnosis, screening and treatment. Diagnostics, 15(3), 412.
Zhu, C., et al. (2023). In vivo genome editing rescues hearing in a mouse model of autosomal dominant deafness. Science Translational Medicine, 15(690), eabq4821.
Downloads
Published
Issue
Section
License

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