Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Review
. 2025 May 21;87(6):3236-3243.
doi: 10.1097/MS9.0000000000002637. eCollection 2025 Jun.

Congenital deafness: from screening to management

Saad Bouchlarhem  1   2   3 , Sbai Achraf  1   2   3 , Benfadil Drissia  1   2   3 , Eabdenbitsen Adil  1   2   3 , Lachkar Azeddine  1   2   3 , El Ayoubi El Idrissi Fahd  1   3   4
Affiliations
Review

Congenital deafness: from screening to management

Saad Bouchlarhem et al. Ann Med Surg (Lond). .

Abstract

Congenital deafness represents a major global health challenge, impacting both communication skills and social integration. The results of epidemiological observations show variable prevalence rates, influenced by genetic and environmental factors. The pathophysiological processes involve abnormalities of the auditory system resulting from genetic mutations, prenatal infections, and exposure to ototoxic substances. Diagnostic approaches are multidisciplinary, combining audiological, genetic, and imaging assessments. Management options include the use of hearing aids, cochlear implants, therapies, and educational assistance, with a strong focus on the importance of early intervention. The implementation of universal newborn hearing screening programs plays a crucial role in early detection, although there are disparities. Future research efforts should focus on understanding genetic and environmental contributions, as well as developing innovative screening and intervention strategies. Collaboration between healthcare professionals, researchers, policymakers, and educators is essential to ensure equal and adequate care and support for people with congenital deafness. This comprehensive review synthesizes the current state of the art on congenital deafness, covering topics such as epidemiology, pathophysiology, etiology, diagnostic methods, management strategies, screening procedures, and future directions.

Keywords: cochlear implants; congenital deafness; genetic testing; screening.

PubMed Disclaimer

Conflict of interest statement

Sponsorships or competing interests that may be relevant to content are disclosed at the end of this article. The authors state that they have no conflicts of interest for this report.

Figures

Figure 1.
Figure 1.
Mechanisms and pathophysiology related to neonatal hearing loss as a result of certain infection diseases leading to certain immune responses.
Figure 2.
Figure 2.
Summaries of characteristics and classification employed for describing loss of hearing.
Figure 3.
Figure 3.
Design of the Cochlear implants: the external device includes a microphone, speech processor, and transmitter, while the internal device consists of an array of electrodes for electrical stimulation and receiver (with permission[26]).
Figure 4.
Figure 4.
Design of the bone conduction hearing aid. The bone-conduction hearing aids bypass the middle ear and transmit sound through vibrations to the cochlear nerve by a processor (1) and a connector (2).
Figure 5.
Figure 5.
Design of the conventional hearing aid systems (with permission[26]).
See this image and copyright information in PMC

Similar articles

See all similar articles

References

    1. Arndt S, Findeis L, Wesarg T, et al. . Long-term outcome of cochlear implantation in children with congenital, perilingual, and postlingual single-sided deafness. Ear Hear 2024;45:316–28. - PubMed
    1. Culbertson SR, Dillon MT, Richter ME, et al. . Younger age at cochlear implant activation results in improved auditory skill development for children with congenital deafness. J Speech Lang Hear Res 2022;65:3539-3547. - PMC - PubMed
    1. Paul A, Bense F, Boithias Guerot C, et al. . Leading risk factors for congenital deafness in the context of universal neonatal screening: our observations in a four-year retrospective study. Int J Neonatal Screen 2024;10:11. - PMC - PubMed
    1. Yuan D, Tournis E, Ryan ME, et al. . Early-stage use of hearing aids preserves auditory cortical structure in children with sensorineural hearing loss. Cereb Cortex 2024;34:bhae145. - PMC - PubMed
    1. Feng Y, Hu S, Zhao S, et al. . Recent advances in genetic etiology of non-syndromic deafness in children. Front Neurosci 2023;17:1282663. - PMC - PubMed

LinkOut - more resources