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
Introduction
Congenital deafness, also known as congenital hearing loss, represents a significant and widespread global health challenge, having a profound impact on the ability of individuals to communicate effectively and integrate into society[1].
The pathophysiology of congenital deafness encompasses a wide range of abnormalities in the auditory system, from structural irregularities in the ear to impairments along the auditory nerve and central pathways, which explains the complexity of this sensory deficit[2]. These physiopathological there are remain some spelling problem, such as hearing hypotheses may result from various factors such as genetic mutations, prenatal infections such as cytomegalovirus (CMV) or rubella, exposure to ototoxic substances during pregnancy or complications during childbirth, underscoring the multifaceted etiology of congenital deafness[3]. Genetic factors play an important role in both syndromic and non-syndromic forms of congenital deafness, with syndromic cases often accompanied by other medical or developmental problems, further complicating the management and treatment of the condition. Nongenetic conditions, including prenatal infections and maternal health conditions, add layers of complexity to the understanding of congenital deafness, requiring a holistic approach to diagnosis and management[4].
Highlights.
Congenital deafness represents a major global health challenge, impacting both communication skills and social integration.
Genetic factors play an important role in both syndromic and non-syndromic forms of congenital deafness, with syndromic cases often accompanied by other medical or developmental problems, further complicating the management and treatment of the condition.
The aim of congenital deafness care is to respond to specific communication needs and improve children’s general quality of life and social integration. This approach is personalized for each patient, depending on whether they are bilaterally or unilaterally hearing impaired, the severity of the impairment and the resources available.
The exploration of congenital deafness requires a multidisciplinary strategy, integrating audiological assessments, genetic testing, and imaging investigations, and the management strategies for congenital deafness include a wide range of interventions individually adapted to individual needs, from the use of hearing aids and cochlear implants to auditory-verbal therapy and sign language training, all aimed at optimizing communication skills and improving the overall quality of life for those affected by the disorder. The implementation of universal newborn hearing screening initiatives has greatly enhanced early detection efforts, enabling timely intervention and support for newborns with congenital deafness, but difficulties persist in ensuring equitable access to screening, diagnostic, and intervention services, underscoring the ongoing need for resource allocation and policy advocacy in this area[5]. In the area of future research, the focus should be on elucidating the genetic and environmental factors that contribute to congenital deafness, in order to advance our understanding of the disease and guide focused interventions[6]. In this literature review, we have reviewed the general aspects of congenital deafness in order to enable general practitioners to fully understand the subject with the aim of intervening early in newborns through the implementation of well-codified screening programs.
Epidemiology
Congenital deafness is a dynamic feature of global health, reflecting the diversity of human populations and the complex interaction of genetic and environmental factors. The prevalence of congenital deafness is generally estimated at 1–3 cases per 1000 live births, but actual prevalence can vary considerably from one region to another and from one demographic group to another[7].
Some populations may have higher rates of congenital deafness due to specific genetic predispositions or cultural customs. Neonatal programs were set up in North America from the end of the 21st century onward in Europe and other developing countries. Prevalence has been increased to around 2.83 per 1000 newborns, while in adults the incidence is around 3.5 per 1000 adults. The number of individuals affected has been attributed to a number of factors, which may be genetic, acquired, or constitutional.
Since language skills are still developing and are not abnormal at this age, diagnostic findings for some forms of hearing loss, such as Auditory Neuropathy Spectrum Disorder, are not always conclusive in infants; consequently, prevalence estimates vary considerably. The prevalence of neonatal hearing loss varies considerably from country to country in the absence of national screening programs, with rates as high as 19 per 1000 babies in sub-Saharan Africa and 24 per 1000 in South Asia. In addition to the diversity of diagnostic methods or threshold criteria for hearing loss, other factors come into play to explain differences in prevalence estimates between high- and low-income countries[8].
The existence of risk factors is one of the most important variables in determining prevalence rates. Disparities in prevalence rates underline how crucial it is to implement universal newborn hearing screening programs to ensure that newborns with hearing loss are identified and treated promptly. The clustering of deafness within communities with a history of consanguineous marriages highlights the genetic aspect of congenital deafness, where inherited mutations may be more prevalent. In addition, socioeconomic conditions, access to healthcare, and exposure to environmental toxins during pregnancy can all have an impact on prevalence rates. Recognizing these epidemiological trends is essential to developing tailored screening and intervention programs that meet the specific needs of affected populations[9].
Pathophysiology
Congenital deafness, which results from dysfunction in the auditory system involving the outer, middle, or inner ear, as well as the auditory nerve and central auditory pathways, is a complex condition with diverse causes. Genetic mutations play an important role in congenital deafness, as changes in genes essential to auditory development and function can disrupt the complex processes involved in sound transmission and perception[10].
In addition, prenatal infections such as CMV or rubella can present a substantial risk by having a direct impact on developing auditory structures during crucial phases of fetal growth. In addition, exposure to ototoxic drugs or environmental toxins, such as specific antibiotics (e.g. gentamicin), chemotherapy drugs (e.g. cisplatin), or heavy metals during pregnancy, can have adverse effects on the auditory system, leading to congenital hearing loss. The pathophysiology of congenital deafness varies depending on the principal cause, requiring a comprehensive diagnostic approach including audiological evaluations, genetic testing, and imaging studies to identify the specific anatomical and functional irregularities contributing to hearing impairment[11]. In Fig. 1, the authors show the relation between the pathological anatomical structure of the ear, cases of hearing impairment, and the occurrence of viral infections[12]. Two disease pathways are suggested: first, the impact of viruses on the peripheral auditory system, which encompasses the functions of the outer and middle ear: is a conductive hearing loss, and second, the influence of viruses on the central auditory system, which involves the auditory nerve and due to damage to the cochlea, or the pathways from the brain to the auditory cortex: is a sensorineural hearing loss[13].
Figure 1.
Mechanisms and pathophysiology related to neonatal hearing loss as a result of certain infection diseases leading to certain immune responses.
Etiology
The origin of congenital deafness involves a complicated interplay between genetic and nongenetic elements, contributing to its multifaceted character. Genetic factors have a significant impact on the development of congenital deafness, as mutations in various genes related to auditory function and its development are responsible for a significant part of the genesis of this disorder[14]. In Table 1, we list the various syndromic and non-syndromic etiologies according to the genes affected and the Online Mendelian Inheritance in Man.
Table 1.
The various syndromic and non-syndromic etiologies according to the genes affected and the online Mendelian inheritance in man.
| OMIM locus | Associated genes | Common findings | |
|---|---|---|---|
| Non-syndromic hearing loss | |||
| DFNB1 | 220 290 | GJB2, GJB6 | Congenital mild to profound autosomal recessive nonsyndromic hearing loss |
| DFNB16 | 603 720 | STRC | Bilateral mild to moderate congenital SNHL |
| Infertility in males | |||
| DFNA8/12 | 602 574 | TECTA | Often prelingual, often mild or high-frequency SNHL |
| DFNB21 | 602 574 | TECTA | Prelingual severe to profound SNHL |
| Mitochondrial hearing loss | 561 000 | MT-RNR1, 1555G>A | Maternally inherited nonsyndromic hearing loss |
| Syndromic hearing loss | |||
| Pendred syndrome recessive | 274 600 | SLC26A4 | Euthyroid goiter progressive |
| Often asymmetric mild to moderate SNHL | |||
| Alport syndrome X-linked recessive dominant | 301 050 | COL4A 5 | Progressive hearing loss, hematuria, anterior lenticonus |
| 203 780 | COL4A 3 | ||
| 104 200 | COL4A 4 | ||
| Branchiooto-kidney syndrome dominant | 601 653 | EYA1 | HL is generally congenital, ear anomalies may involve external, middle, and inner ear |
| 601 205 | SIX1 | ||
| 600 963 | SIX5 | ||
| Usher syndrome recessive | 276 900 | MYO7A | Vestibular dysfunction with vison problems |
| 276 904 | USH1C | ||
| 601 067 | CDH23 | ||
| 602 083 | PCDH15 | ||
In addition to genetic factors, nongenetic determinants also play a crucial role in the origin of congenital deafness. Prenatal infections which represent 30%, such as CMV, rubella, and the Zika virus, represent significant risks during critical periods of fetal development, potentially leading to structural abnormalities or functional deficits in the auditory system[15–17]. Maternal health conditions such as gestational diabetes and preeclampsia further increase the risk of congenital deafness by affecting normal physiological processes during pregnancy[18].
In addition, malformations of the middle and inner ear represent a major cause of congenital deafness. These malformations can be either Complete labyrinthine aplasia, Cochlear aplasia or Cochlear hypoplasia, Widened vestibular aqueduct syndrome, and incomplete partition which can be divided into three subtypes according to Mondini’s classification[19] (Table 2).
Table 2.
Classification of incomplete partition according to Mondini classification.
| Type of malformation | IP-I | IP-II | IP-III |
|---|---|---|---|
| Residual hearing | No | Yes | Yes |
| Progressivity | No | Yes | No |
| Type and degree of HL | Severe to profound SNHL | Normal, mild, moderate, severe, profound, CHL, MHL, SNHL | Severe to profound MHL |
| ABG | Absent | Usually present at low frequencies | Usually present at all frequencies except 2 kHz |
| Audiological intervention | CI or ABI | HA or CI | Only CI |
| Cochlear nerve | Normal, hypoplastic, aplastic | Normal | Normal |
| Vestibular aqueduct | Rarely enlarged | Almost always enlarged | Medially located with different shapes and varying degrees of dilatation |
IP-I, incomplete partition type I; IP-II, incomplete partition type II; IP-III, incomplete partition type III; SNHL, sensorineural hearing loss; CHL, conductive hearing loss; MHL, mixed hearing loss; CI, cochlear implantation; ABI, auditory brainstem implantation.
Understanding the various factors at the root of congenital deafness is essential for a comprehensive diagnostic evaluation and personalized intervention strategies, involving both genetic testing and screening for nongenetic risk factors.
Diagnostic evaluation
Accurate diagnosis of congenital deafness requires a multidisciplinary approach involving a multidisciplinary team of healthcare professionals, including hearing audiologists, otolaryngologists, pediatric specialists, speech therapists, and genetic consultants.
Audiological assessments are the cornerstone of diagnostic evaluation, providing valuable information on the type and severity of hearing deficiency (Fig. 2). These evaluations are based on tonal audiometry, which quantifies hearing thresholds at different frequencies, making it easier to classify hearing impairment, and tympanometry, which assesses middle ear function and helps identify conditions such as otitis media with effusion, which may contribute to conductive hearing loss, and the auditory brainstem response (ABR) test assesses the integrity of the auditory nerve and central auditory pathways by measuring neural responses to auditory stimuli, providing information on the function of the auditory system beyond the cochlea[20].
Figure 2.
Summaries of characteristics and classification employed for describing loss of hearing.
It is recommended that newborns be screened within the first month of life. This generally involves two noninvasive diagnostic methods, otoacoustic emission testing (OAE) and automated auditory brainstem response (aABR)[20,21]. OAE involves sending sound into the inner ear to stimulate the cochlear hairs and produce OAE, while aABR measures the response to sound using electrodes placed on the baby’s head. Genetic testing can help elucidate the underlying genetic mechanisms and inform counseling regarding inheritance patterns and recurrence risks. Imaging studies, such as computed tomography (CT) scans and magnetic resonance imaging (MRI), complement audiologic and genetic assessments by providing anatomical information about the auditory structures. CT scans are useful for visualizing bony structures of the inner ear and temporal bone, while MRI offers detailed images of soft tissue structures, including the cochlea and auditory nerve[22]. As do other tests such as CMV neonatal screening, and the evaluation included laboratory testing for congenital syphilis or rubella[23]. In Table 3, we summarize the investigations to be carried out according to etiology.
Table 3.
We summarize the investigations to be carried out according to etiology.
| Diagnostic studies | Etiology |
|---|---|
| CT temporal bones | Trauma or ear malformations |
| MRI of temporal bones, brain | Inner ear malformations associated with hearing loss |
| Urine, saliva, or blood PCR within first 3 weeks of life; DBS at birth or later | Congenital CMV |
| Viral antibodies, viral DNA serological testing | Congenital TORCH infections; postnatal infections |
| Genetic testing | Syndromic or nonsyndromic, genetic |
CMV, cytomegalovirus; CT, computed tomography; DBS, dried blood spot; MRI, magnetic resonance imaging; PCR, polymerase chain reaction; TORCH, toxoplasma, other (syphilis), rubella, cytomegalovirus, herpes.
Management
The aim of congenital deafness care is to respond to specific communication needs and improve children’s general quality of life and social integration. This approach is personalized for each patient, depending on whether they are bilaterally or unilaterally hearing impaired, the severity of the impairment, and the resources available. A number of advances have been made in this area, most notably with the advent of cochlear implants, which offer a highly sophisticated therapeutic option for people with severe to profound hearing loss, as they bypass the damaged hair cells in the cochlea to stimulate the auditory nerve directly and restore hearing function. Cochlear implants are composed of an internal device, which is surgically placed, consisting of a receiver and an electrode array that is linked directly to the cochlea, and an external device consisting of a microphone, a speech processor, and transmitter (Fig. 3). Current guidelines recommend cochlear implantation for children with bilateral profound sensorineural hearing loss between 9 and 24 months of age, as the earliest implantation of a cochlear implant has been shown to be safe and associated with a much greater likelihood of improvement in spoken language and school performance[24,25].
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]).
Combined with cochlear implants, hearing aid systems, represented mainly by bone-anchored hearing systems, offer an effective alternative for patients with specific forms of hearing loss, such as conductive or mixed hearing loss, as well as unilateral deafness. These systems comprise a titanium attachment (abutment) implanted in the skull bone behind the ear, to which an external sound processor is connected, and they transmit sound vibrations directly through the bone to the inner ear, bypassing obstacles in the middle ear (Fig. 4)[27–29]. This direct transmission path improves sound localization and speech understanding, particularly in difficult listening environments. This is in addition to traditional hearing aid systems that amplify sound through the external or middle ear (Fig. 5)[30,31].
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.
Design of the conventional hearing aid systems (with permission[26]).
Several published data on unilateral hearing loss have confirmed its adverse effects on speech and language development, academic difficulties, including school failure, and behavioral problems. For these reasons, hearing rehabilitation for this population, based on conventional hearing aids, frequency modulation systems, contralateral signal routing aids, bone conduction hearing aids, and cochlear implants, is highly recommended[32,33].
Screening
Universal newborn hearing screening programs have become a crucial element in the rapid identification and management of congenital deafness, transforming the field of pediatric audiology and improving outcomes for affected newborns. These screening efforts aim to detect hearing loss soon after birth, enabling early intervention to minimize its impact on developmental milestones. Typically, screening protocols use objective measures such as OAE and ABR tests, which are nonintrusive, reliable, and can be performed efficiently in the nursery or neonatal intensive care unit[34]. Children who do not pass the initial screening undergo additional diagnostic evaluations to confirm the presence and severity of hearing loss, facilitating the implementation of appropriate intervention approaches. Early referral to appropriate intervention services, such as audiology clinics, early intervention programs, and specialized pediatric care centers, ensures that affected newborns benefit from thorough evaluation and access to rehabilitation services[35]. Nevertheless, despite significant progress in universal newborn hearing screening, challenges remain in ensuring equitable access and effectiveness of screening programs, particularly in underserved communities with limited healthcare resources. Sustained efforts are therefore needed to address barriers to screening, including financial limitations, cultural beliefs, and geographic disparities, to ensure that all infants, regardless of history or socioeconomic status, have the same opportunity to benefit from early identification and intervention for congenital deafness[36,37]. By advocating and investing in the expansion and improvement of universal newborn hearing screening programs, healthcare providers and policymakers can make significant progress in improving infant outcomes and quality of life.
Challenges
The two main challenges facing health systems are early diagnosis and early intervention, with the aim of limiting the unfavorable evolution and social disintegration of these children.
As far as screening systems are concerned, we note a major difference in the application of screening programs, mainly in middle- and low-income countries such as India and Central Africa, versus European countries. For this reason, the Coalition for Global Hearing Health has proposed in a consensus paper the strategies to be followed to improve these screening strategies, classifying them into three types according to the country’s level of resources. They propose a 1–3–6-month strategy as a basic strategy to be applied in low-income countries, and the 1–2–3 months strategy as an advanced strategy to be applied in developed countries. These strategies involve first screening, then identification, followed by intervention and finally amplification provided[38] (Table 4).
Table 4.
The different screening and intervention strategies proposed by the Coalition for Global Hearing Health Hearing Care Pathways Working Group.
| Service level | Hearing screened | Hearing loss identified | Early intervention started | Initial amplification provided |
|---|---|---|---|---|
| Basic EHDI 1–3–6 | By 1 month | By 3 month | By 6 month | By 6 month |
| Intermediate EHDI 1–3–6 | By 1 month | By 3 month | By 3 month | By 6 month |
| Advanced EHDI 1–2–3 | By 1 month | By 2 month | By 3 month | By 3 month |
One of the challenges in managing deafness remains unequal access to cochlear implants. This can be explained by the fact that cost-effectiveness in low-resource countries is such a limiting factor in the expansion and implementation of cochlear implant programs, that health systems in these countries often neglect the importance of managing hearing loss, which ultimately increases the total burden of hearing loss.
Conclusion
Congenital deafness is a major public health challenge, requiring a collaborative effort from a wide range of actors in the field, including health professionals, researchers, educators, and policy-makers. Despite advances in diagnostic tools, genetic studies, management strategies, and screening programs, disparities persist in access to essential services for children with congenital hearing loss. These disparities underscore the importance of ongoing advocacy to improve education, early detection, and access to resources for screening, diagnosis, and intervention resources. Future research should focus on filling gaps in knowledge about the genetic and environmental aspects of congenital deafness, and exploring new methods of screening, intervention, and support. Ultimately, by working together towards a common goal, patients with congenital deafness can receive comprehensive care and support that enables them to thrive and lead satisfying lives.
Acknowledgments
Not applicable.
Footnotes
Sponsorships or competing interests that may be relevant to content are disclosed at the end of this article.
Published online 21 May 2025
Contributor Information
Saad Bouchlarhem, Email: bouchlagehmsaad@gmail.com.
Eabdenbitsen Adil, Email: Adil-EabdenbitseN@ump.ac.ma.
Lachkar Azeddine, Email: Lachkar.Azeddine@ump.ac.ma.
El Ayoubi El Idrissi Fahd, Email: Fahd.elayoubi@ump.ac.ma.
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Author’s contribution
S.B. and L.A. developed the project idea. S.A., B.D., E.A., L.A., and E.A.E.I.F. supervised the project. All authors contributed to the article and approved the submitted version.
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The authors state that they have no conflicts of interest for this report.
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Dr Saad Bouchlarhem.
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Data availability statement
The data underlying this article are available in the article.
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Data Availability Statement
The data underlying this article are available in the article.