What Is Congenital Hearing Loss?

What Is Congenital Hearing Loss?

Congenital hearing loss is hearing loss that is present when a baby is born. It can affect one or both ears and range from mild to profound. Some babies have difficulty hearing certain sounds, while others have little or no access to sound through hearing alone.

Congenital hearing loss may be caused by genetic changes, infections during pregnancy, differences in how the ear develops, or certain complications around birth. Sometimes the cause remains unknown even after testing.

Identifying hearing loss early is important because babies begin developing communication and language skills from birth. With appropriate support, children who are deaf or hard of hearing can develop meaningful language, communication, learning, and social skills.

The most important step for parents: Make sure your baby receives newborn hearing screening and any recommended follow-up testing. A baby who does not pass a hearing screening needs further evaluation, even if they appear to respond to some sounds.

How Common Is Congenital Hearing Loss?

Hearing loss is among the conditions routinely screened for in newborns in the United States.

The estimated number of babies born with hearing loss depends on the population studied, the degree of hearing loss included, and how cases are identified.

Approximately 1 to 3 babies per 1,000 are identified with hearing loss through newborn hearing screening and follow-up programs, although estimates vary.

Some children have hearing loss that is not detected immediately after birth. Others develop hearing loss later because of genetic conditions, infections, or additional medical factors.

For this reason, passing a newborn hearing screening does not eliminate the need to monitor hearing and communication development throughout childhood.

What Causes Congenital Hearing Loss?

Congenital hearing loss has several possible causes. These are often grouped into genetic and non-genetic factors.

In some children, more than one factor may contribute.

1. Genetic Causes

Genetic changes are responsible for a substantial proportion of congenital hearing loss cases.

Genes provide instructions for the development and function of the inner ear and other structures involved in hearing.

Changes in certain genes can affect these structures and cause hearing loss before birth.

Genetic hearing loss may occur even when neither parent has hearing difficulties.

Several inheritance patterns are possible:

• Autosomal recessive: A child inherits disease-causing variants from both parents, who may have normal hearing.
• Autosomal dominant: A disease-causing variant in one copy of a gene may be sufficient to cause hearing loss.
• X-linked: Hearing loss is associated with a gene located on the X chromosome.
• Mitochondrial: Certain genetic variants affecting hearing are inherited through mitochondrial DNA.

Some genetic hearing loss occurs as part of a syndrome involving other medical findings. Other forms affect hearing without additional recognized health conditions.

Genetic counseling and testing may help some families understand the cause, possible progression, and implications for relatives.

However, genetic testing does not identify a cause in every child.

For more information, see the CDC’s Parent Guide to Genetics and Hearing Loss.

2. Congenital Cytomegalovirus (CMV)

Cytomegalovirus is an important infectious cause of hearing loss in children.

Congenital CMV occurs when the virus passes to a baby during pregnancy.

Some babies with congenital CMV have hearing loss at birth. Others develop hearing loss months or years later, even if they passed their initial newborn hearing screening.

Hearing loss associated with congenital CMV may affect one or both ears and can change over time.

Important testing window: Testing for congenital CMV generally needs to be performed within the first 21 days after birth to distinguish infection acquired before birth from infection acquired afterward.

Parents whose newborn does not pass a hearing screening should ask the healthcare team promptly whether congenital CMV testing is indicated.

Some infants with symptomatic congenital CMV may benefit from specialist-directed antiviral treatment. These medications can have significant side effects, and treatment decisions require individualized medical assessment.

Babies with confirmed congenital CMV should receive ongoing hearing monitoring.

Learn more from the CDC’s guide to congenital CMV and hearing loss.

3. Other Infections During Pregnancy

Other infections during pregnancy may affect fetal development and contribute to hearing loss.

Examples include:

• Rubella
• Toxoplasmosis
• Syphilis
• Certain other congenital infections

The likelihood and severity of complications depend on the specific infection, timing, and other medical factors.

Vaccination, prenatal care, infection prevention, and appropriate treatment during pregnancy can help reduce some risks.

4. Differences in Ear Development

Some babies are born with structural differences involving the outer ear, middle ear, inner ear, or auditory nerve.

Examples include an absent or narrowed ear canal, differences in the middle-ear bones, and abnormalities in cochlear development.

These conditions may cause conductive, sensorineural, or mixed hearing loss.

Depending on the findings, an ear, nose, and throat (ENT) specialist may recommend additional testing or imaging.

5. Complications Around Birth

Certain serious complications around birth are associated with an increased risk of hearing loss.

These include severe oxygen deprivation, significant neurological injury, and dangerously elevated bilirubin levels.

Severe hyperbilirubinemia may damage auditory pathways. This risk is different from the common mild jaundice seen in many newborns.

Blood-group incompatibility can contribute to severe jaundice in some infants, but incompatibility alone does not mean a baby will develop hearing loss.

Babies who require prolonged neonatal intensive care or have other significant medical risk factors may need additional hearing monitoring.

What Are the Types of Congenital Hearing Loss?

Healthcare professionals classify hearing loss partly according to which structures of the hearing system are affected.

Conductive Hearing Loss

Conductive hearing loss occurs when sound does not travel efficiently through the outer or middle ear.

In congenital cases, possible causes include structural differences involving the ear canal or middle-ear bones.

Some conductive hearing conditions can be treated medically or surgically, while others require hearing technology or longer-term support.

Middle-ear fluid may also cause conductive hearing loss during infancy or childhood, although fluid that develops after birth is not necessarily congenital.

Sensorineural Hearing Loss

Sensorineural hearing loss involves the inner ear or auditory nerve.

It may result from genetic conditions, infections, structural abnormalities, or other causes.

Permanent sensorineural hearing loss generally cannot be reversed with current standard medical treatment.

Hearing aids, cochlear implants, and other supports may improve access to sound when appropriate.

Mixed Hearing Loss

Mixed hearing loss includes both conductive and sensorineural components.

For example, a child may have an inner-ear condition along with a separate problem affecting the middle ear.

Management depends on identifying and addressing each component appropriately.

What Are the Signs of Hearing Loss in Babies?

Some babies with hearing loss respond to certain sounds but not others. For that reason, observing a reaction to a loud noise does not guarantee normal hearing.

Possible signs worth discussing with a pediatrician include:

• Not startling or reacting to loud sounds
• Not turning toward sound as expected for their developmental stage
• Responding when someone is visible but not when called from out of sight
• Appearing to hear some sounds but missing others
• Not developing expected speech or language milestones
• Having limited responses to familiar voices

These signs do not establish a diagnosis. Babies develop at different rates, and hearing concerns should be evaluated professionally.

For example, the CDC identifies not turning toward the source of a sound after approximately six months as a possible warning sign.

Concern about hearing at any age is a reason to request an evaluation, even if the child previously passed a screening.

How Is Congenital Hearing Loss Diagnosed?

Newborn hearing screening is the first step in identifying babies who may have hearing loss.

However, screening is not the same as a complete diagnostic evaluation.

Newborn Hearing Screening

Most babies in the United States receive hearing screening before leaving the birth hospital.

Two common screening methods are:

• Automated auditory brainstem response (AABR): Measures electrical responses associated with sound transmission through portions of the auditory system.
• Otoacoustic emissions (OAE): Measures responses generated by outer hair cells in the cochlea.

These tests are generally quick and painless. Babies can often be screened while sleeping or resting quietly.

For more information, visit the CDC’s newborn hearing screening guide.

What If Your Baby Does Not Pass?

A baby who does not pass a newborn hearing screening does not necessarily have permanent hearing loss.

Temporary factors such as ear canal debris, middle-ear fluid, or testing conditions may influence screening results.

However, the result should never be ignored.

The healthcare team should arrange appropriate follow-up screening or a comprehensive diagnostic hearing evaluation according to the applicable newborn hearing screening protocol.

Parents should not wait until speech delays develop before completing the recommended follow-up.

The 1-3-6 Hearing Screening Benchmarks

The CDC recommends the following Early Hearing Detection and Intervention (EHDI) benchmarks:

• By 1 month: Complete newborn hearing screening.
• By 3 months: Complete diagnostic hearing evaluation when indicated.
• By 6 months: Enroll infants with confirmed hearing loss in appropriate early intervention services.

Some programs work toward an accelerated 1-2-3 timeline where practical.

These benchmarks help families and healthcare professionals avoid unnecessary delays.

See the CDC’s EHDI 1-3-6 benchmarks.

Diagnostic Hearing Tests

If further evaluation is needed, a pediatric audiologist may use diagnostic ABR, OAE testing, tympanometry, and age-appropriate behavioral methods.

Diagnostic ABR testing can help estimate hearing sensitivity in infants who cannot reliably respond to conventional hearing-test instructions.

Additional assessments may be recommended to determine the type, degree, and possible cause of hearing loss.

Why Does Early Identification Matter?

Early access to a fully accessible language is important for communication, learning, and social development.

Hearing loss can reduce access to spoken language when appropriate supports are not available.

However, spoken language is not the only valid route to language development.

Children may communicate through spoken language, signed language, or a combination of approaches depending on their needs and family choices.

The central goal is consistent access to language and opportunities for interaction from the earliest possible age.

Speech and Language Development

Children who use spoken language may benefit from appropriately selected hearing technology, speech-language services, and family-centered support.

Children who use signed language need regular access to fluent language models and meaningful signed communication.

Families should receive accurate information about available communication approaches so they can make informed decisions.

Learning and Social Development

Without appropriate access and accommodations, hearing loss can affect classroom communication, social participation, and learning.

Early intervention services can help families identify communication goals and access educational resources.

Children who are deaf or hard of hearing have diverse abilities, experiences, and developmental outcomes.

Hearing status alone does not determine a child’s intelligence, academic potential, or quality of life.

The Developing Brain

Early childhood is an important period for language learning and the development of neural pathways involved in communication.

The brain can change in response to experience, a property known as neuroplasticity.

This supports the importance of early and ongoing language access, whether through speech, sign language, or both.

For background, read our guide to what neuroplasticity is and how it works.

What Treatments and Support Are Available?

Management depends on the type and degree of hearing loss, medical findings, the child’s needs, and the family’s communication goals.

No single intervention is appropriate for every child.

Pediatric Hearing Aids

Hearing aids can make sounds more accessible for many infants and children with hearing loss.

They are individually selected and programmed by pediatric hearing professionals.

Children with different degrees of hearing loss may benefit from hearing aids, including some with severe hearing loss.

Proper fitting, verification, and follow-up are important because a child’s ears and listening needs change during growth.

Hearing aids do not reverse permanent sensorineural hearing loss or guarantee normal hearing.

Cochlear Implants

Cochlear implants may be an option for selected children with significant sensorineural hearing loss who receive insufficient benefit from appropriately fitted hearing aids.

Unlike hearing aids, cochlear implants use electrical stimulation to provide auditory information through the hearing nerve.

Candidacy depends on hearing levels, speech and language development, medical findings, device indications, and other factors.

Some FDA-approved cochlear implant systems have pediatric indications beginning at nine months of age for qualifying children. Other devices and indications differ.

Early evaluation may be appropriate when a child could benefit, but implantation at a particular age is not automatically recommended for every child.

Cochlear implantation requires surgery, rehabilitation, and ongoing programming. Outcomes vary, and implants do not restore normal hearing.

Families should receive balanced information about benefits, limitations, risks, and available communication approaches.

Treatment for Conductive Hearing Loss

Some conductive hearing conditions may improve with medical or surgical treatment.

For example, persistent middle-ear fluid may sometimes require tympanostomy tubes when clinical criteria are met.

Congenital structural differences involving the ear canal or middle-ear bones may require other approaches, including reconstructive surgery or selected bone-conduction hearing devices.

The timing and suitability of surgery depend on the child’s anatomy, medical findings, and expected benefits and risks.

Early Intervention and Communication Support

Early intervention services can support children and families in developing communication and language skills.

Depending on family goals and the child’s needs, these services may include:

• Pediatric audiology services
• Speech-language support
• Sign language instruction and access
• Family education and coaching
• Educational accommodations
• Deaf and hard-of-hearing specialist services
• Assistive listening technology

Families may also benefit from connecting with other parents and deaf or hard-of-hearing adults who can share experiences and communication perspectives.

Can Congenital Hearing Loss Get Worse?

Yes. Some forms of congenital hearing loss remain stable, while others progress or fluctuate.

Progression can occur with certain genetic conditions and congenital infections, including CMV.

Hearing may change in one or both ears over time.

Regular audiological monitoring is important so hearing devices, communication plans, and educational supports can be adjusted when needed.

A child who passed newborn screening may still require additional testing if new hearing concerns arise.

How Can Parents Support a Child With Congenital Hearing Loss?

Parents and caregivers play an important role in helping children access language and communication.

Helpful actions include:

• Complete newborn hearing screening and recommended follow-up tests.
• Keep appointments with pediatric audiologists and other specialists.
• Ask questions about the type and degree of hearing loss.
• Explore available spoken and signed language approaches.
• Provide frequent, meaningful communication and interaction.
• Use hearing technology consistently when it is part of the child’s care plan.
• Request early intervention services promptly.
• Monitor hearing and communication development over time.
• Connect with family support and deaf or hard-of-hearing community resources.

Families should not feel pressured to choose a particular technology or communication method without understanding the alternatives.

The goal is to help the child develop accessible language, meaningful relationships, and the skills needed to participate fully in daily life.

When Should You Request Another Hearing Evaluation?

Contact your child’s pediatrician or pediatric audiologist if you notice new concerns about hearing, communication, or language development.

Possible warning signs include inconsistent responses to sound, unexpected speech or language delays, difficulty following spoken directions, or concerns raised by caregivers or teachers.

These signs require individualized assessment and may have several explanations.

For a broader discussion, see our article on early warning signs of cognitive and hearing changes.

Is Congenital Hearing Loss the Same as Auditory Processing Disorder?

No. Congenital hearing loss means hearing loss is present at birth.

Auditory processing disorder (APD) involves difficulties processing auditory information within the central auditory nervous system.

Some children with APD have normal conventional hearing thresholds, although listening difficulties can have several possible explanations.

APD is not automatically caused by congenital hearing loss, and a newborn hearing screening does not diagnose APD.

Learn more in our guide to auditory processing disorder (APD).

Frequently Asked Questions

Can congenital hearing loss be cured?

It depends on the cause. Some conductive hearing problems can improve with medical or surgical treatment. Permanent sensorineural hearing loss generally cannot be reversed with current standard treatments, but appropriate technology and communication support can improve access and participation.

Is congenital hearing loss always inherited?

No. Genetic causes are important, but hearing loss can also result from congenital infections, developmental differences, and certain complications during pregnancy or birth. Some cases remain unexplained.

Can a baby have hearing loss in only one ear?

Yes. Congenital hearing loss may affect one or both ears. Hearing loss in one ear can still affect communication and sound localization, and professional follow-up is important.

Can a baby pass newborn hearing screening and still have hearing loss?

Yes. Some hearing conditions develop or become detectable later, including certain genetic conditions and congenital CMV-related hearing loss. Parents should request evaluation whenever hearing or communication concerns arise.

Does a baby who fails hearing screening definitely have hearing loss?

No. A screening result indicates that additional testing is needed. It does not establish the diagnosis or cause. Completing recommended follow-up is essential.

When should a child receive hearing aids?

When hearing aids are appropriate, fitting should generally proceed promptly after confirmation of hearing loss and individualized evaluation. Families should not wait until a child reaches a particular preschool age simply to begin exploring hearing technology.

At what age can a child receive a cochlear implant?

Some cochlear implant systems have FDA-approved indications beginning at nine months of age for eligible children. Candidacy depends on the specific device, hearing assessment, medical findings, and individual circumstances.

Can children with congenital hearing loss learn sign language?

Yes. Sign languages are complete natural languages. Early access to an accessible language, including signed language, can support communication and development. Families may choose signed language, spoken language, or combined approaches according to their child’s needs.

Will congenital hearing loss affect a child’s intelligence?

Hearing loss itself does not determine intelligence. Children who are deaf or hard of hearing have a wide range of abilities. Accessible language, appropriate educational support, and attention to any additional medical or developmental needs are important.

How often should a child with congenital hearing loss have hearing tests?

The schedule depends on age, hearing-loss type, stability, risk factors, and the child’s treatment plan. Infants and young children may need relatively frequent follow-up, particularly when hearing loss could progress or hearing devices require adjustment.

The Bottom Line

Congenital hearing loss is hearing loss present at birth. It can have genetic, infectious, structural, or other medical causes and may affect one or both ears.

Early screening, accurate diagnosis, and timely access to communication and intervention services are important for healthy language and social development.

Families have several options, including hearing aids, cochlear implants when appropriate, spoken language services, signed language, and educational support.

If your baby does not pass newborn hearing screening or you have concerns about hearing at any age, arrange professional follow-up rather than waiting for symptoms to become more obvious.