This post is sponsored by Lurie Children’s Hospital, but all of the opinions within are those of The Everymom editorial board.
"Welcoming a new addition to the family brings boundless joy, but it can also bring in unexpected hurdles as well. Parenthood is a journey of unique experiences that can lead us to feel a bit lost at times, especially when it comes to your little one’s health. And if you’ve recently been told that your infant has or may have hearing loss, you might be wondering, “Where do I even start?” As we all know, every child is so different, and addressing their health needs requires a lot of individualized understanding and treatment. So, we are here to shed light on crucial insights and expert advice to empower parents to navigate the diagnosis of hearing loss.
To help you navigate your child’s next steps, we’ve built a resource to answer all your questions with our long-standing partners at Ann & Robert H. Lurie Children’s Hospital of Chicago. We asked their experts in pediatric hearing loss to share everything you need to know about hearing loss in infants, including the diagnosis and treatment process.
Meet the Expert
Nancy M. Young, MD, FACS, FAAP
Head, Section of Otology/Neurotology and Medical Director, Audiology & Cochlear Implant Programs, Lurie Children’s
Meet the Expert
Joy Ringger, AuD, CCC-A
Clinical Coordinator, Diagnostic Audiology
In this article
What is infant hearing loss, and how do I know if my infant has it?
Infant hearing loss is a condition in which a baby has difficulty hearing sounds, and while in some cases the condition is temporary, there are cases where hearing loss can be permanent. Infant hearing loss can affect one or both ears, and the degree of loss can range from mild to profound.
Because infants can’t communicate with us, even the most profound loss can be difficult for parents to recognize in their infant. For this reason, newborn hearing screening is done across the United States and in many other countries to help identify infant hearing loss with tools and expertise that parents do not have access to in the home setting. Most infants who do not pass the first newborn hearing screening have temporary hearing loss due to fluid in the middle ear. It is common for newborns to retain fluid in the ear after birth, but in most cases, it resolves itself. However, the fluid can still impact the results of the first and even second hearing screenings. If both screenings are not passed, it is recommended to visit an audiologist.
Some signs of hearing loss vary depending on the age of the infant, the degree of loss, and whether the loss is in one or both ears. Infants with profound hearing loss in both ears babble just like normal hearing infants during the first six months of life, therefore babbling early in life does not guarantee hearing is normal. Hearing loss is often not suspected in even profoundly deaf children until they are 18 to 24 months of age, when speech does not develop or is very limited. When the loss is only in one ear or in both ears and in the mild to moderate range, the loss is rarely suspected during infancy. The invisible nature of hearing loss is why newborn hearing screening is important. The following may be observed in infants with major hearing loss in both ears:
- Not startling to loud sounds
- Not turning toward a sound after they turn 6 months old
- Seeming to hear some sounds but not others
What does the evaluation process look like for infant hearing loss?
Treatment of hearing loss depends on the type and severity of hearing loss and whether the condition is temporary or permanent. During the evaluation process, experts will determine which two major types of hearing loss an infant may have: conductive hearing loss or sensorineural hearing loss.
Conductive Hearing Loss
This type of hearing loss is caused by a physical blockage that disrupts the flow of sound to the auditory nerve. Conductive hearing loss is usually temporary and, in most of these temporary cases, is due to fluid in the middle ear. In utero, a fetus is surrounded by fluid, and then, after the baby is delivered, they cry and their lungs become filled with air. Just like the lungs, the middle ear is also filled with fluid in utero, and once a baby enters the world, the fluid should be naturally pushed out and the space should be filled with air so that the pathway for sound to travel in is open. If fluid remains, the pathway will be blocked and the baby will likely fail the hearing screening.
Permanent causes of conductive hearing loss include more structural, anatomic abnormalities in the pathway that sound travels in the ear. One example of this is the improper development of the ear canal. Another example, although very rare, could be a problem with the middle ear bones, which are responsible for bringing sound to the inner ear.
Sensorineural Hearing Loss
This is the most common type of permanent hearing loss. It is usually caused by an abnormality of hair cells, the special sensory cells in the inner ear. In some cases, it is due to the lack of normal development of the nerve of hearing. Of the newborns that have sensorineural hearing loss, 10 percent have an uncommon type of loss called auditory neuropathy. With auditory neuropathy, the inner ear hair cells, the nerve of hearing, or the connections between the two are absent or abnormal.
Ideally, when an infant does not pass the first hearing screening, they will be rescreened before 1 month old. Most infants pass the second screening if fluid in the middle ear is the cause of the loss. For infants who do not pass the second screening, their pediatrician will refer them to an audiologist. When it comes to diagnosis and management, an audiologist will be best equipped to determine the degree and type of permanent hearing loss and whether hearing aids are necessary.
Experienced pediatric audiologists can often diagnose hearing loss in infants between 3 and 6 months of age through auditory brain stem response testing. This testing method measures the ear and brain’s response to sounds at different degrees of loudness. This test doesn’t require a child’s participation, so in infants, it can sometimes be done while they are sleeping. In older infants, sedation may be necessary to ensure that the child is still to ensure test accuracy. Once a diagnosis is confirmed, audiologists will be able to enroll children in early intervention by 6 months old.
What causes sensorineural hearing loss in children?
Sensorineural hearing loss in both ears is caused by genetic factors in 50 to 60 percent of children. The family history is usually negative for childhood hearing loss in these children. Therefore, genetic testing and counseling are often advised. In contrast, the likelihood of a genetic cause is 20 percent when sensorineural loss is only present in one ear. Hearing loss may also be caused by CMV, a common virus that may cause sensorineural hearing loss when a child’s infection happens during pregnancy. This is the most common non-genetic cause of childhood hearing loss. The loss may be present at birth or begin later. Infants who do not pass newborn hearing screening often undergo testing for CMV immediately after failing the first newborn hearing screening because only very early testing can determine if the infection happened before birth.
Genetic causes and CMV infection during pregnancy are also two of the most common causes of childhood sensorineural hearing loss beginning after birth. Another cause for hearing loss after birth is the use of medications that are toxic to the ear. Often these are medications used to treat a life-threatening condition. Another uncommon cause of hearing loss that’s important to note is bacterial meningitis. This serious infection can cause profound deafness and obstruct the inner ears. Urgent referral for cochlear implantation is necessary for children with bacterial meningitis because the obstruction may rapidly become so severe that implant surgery may not succeed.
What about gene therapy for sensorineural hearing loss?
Research to find medical treatments for sensorineural hearing loss has been underway for many years. According to Lurie Children’s experts, there have been exciting news reports about gene therapy for children with OTOF-related genetic hearing loss—this is a very rare cause of hearing loss. The initial results of gene therapy have demonstrated improvement in hearing. Although exciting, it remains to be determined if the improvement is permanent and whether the quality of the hearing enables understanding of spoken language. In addition, many other types of genetic hearing loss are associated with abnormal inner ear anatomy that may not be treatable after birth like OTOF-related hearing loss.
It’s important for parents to be aware of advances in medical treatment for hearing loss, but experts do not recommend waiting to begin early intervention when these genetic treatments are not yet available. Early intervention is necessary to stimulate the brain and help infants learn to understand speech and eventually talk. Waiting to cure the ear without immediately improving hearing will prevent a child’s brain from getting what it needs to understand the meaning of sounds.
However, early intervention to stimulate the brain is necessary for understanding speech and learning to talk. Curing the abnormality inside the ear later in childhood is unlikely to result in normal hearing and language development.
What are the treatment options after receiving a diagnosis?
There are several treatment options for infant hearing loss, depending on your child’s type and degree of loss.
- Hearing aid: Hearing aids are a nonsurgical way of improving most permanent hearing loss. They are recommended when the ear with hearing loss does not have the ability to hear all the sounds in spoken language. They amplify or make sounds louder and therefore capable of being heard. They can be put on and taken off like a pair of glasses. Special types of hearing aids have been developed for children without ear canals.
- Cochlear implant: For children with so much sensorineural hearing loss in one or both ears that hearing aids do not enable the sounds of spoken language to be heard, cochlear implants may be the only treatment to improve hearing. A cochlear implant is a surgical device that stimulates the nerve of hearing from inside the inner ear. Lurie Children’s Cochlear Implant Program is one of the largest and most experienced pediatric programs in the world. Their surgeons have performed more than 2,000 cochlear implant procedures since the program began in 1991 under the leadership of Dr. Nancy Young.
- Early intervention: Maximizing hearing has a positive impact on the development of spoken language and literacy, so after hearing devices are implemented, it’s important that children get connected with therapists who specialize in helping children develop their listening skills to get the most out of their hearing devices. This type of therapy also helps children who are learning sign language.
Hearing aids for long-term hearing loss
What is a hearing aid?
A hearing aid is a device that amplifies sound, meaning it makes sounds louder. It consists of a microphone that picks up sound, an amplifier that makes sounds louder, and a speaker (also known as a receiver) that delivers the sound to the ear. Any child who has a hearing loss that may interfere with speech and language development or who has a loss that interferes with their classroom performance may benefit from a hearing aid. However, hearing aids are not very effective when hearing loss is severe to profound. Amplification from hearing aids is not enough for spoken language to be clearly heard when this great degree of loss is present.
How will I know if my child needs a hearing aid?
The first step in determining your child’s needs is a complete hearing evaluation. Infants, young children, and children who are difficult to test because of additional medical and behavioral challenges benefit from the expertise of a pediatric audiologist so that an accurate diagnosis will be made. Your child also will need to be further evaluated by an otologist—an Ear, Nose, and Throat physician who further specializes in the treatment of ear disease. The otologist will work with the audiologist to determine if the hearing loss can be treated medically with a hearing device or surgically. They will also look for other medical problems that may relate to or be the cause of hearing loss.
Once an accurate diagnosis is made, infants can be fitted with hearing aids within the first few months of life. Early hearing aid use increases the chances that listening and spoken language skills will develop normally.
If a hearing aid is needed, what is the process of getting one that’s right for them?
If it is determined that your child is a good candidate for a hearing aid, several appointments will be made for your child within an audiology program. First, the audiologists will determine which hearing aid is best for your child. Another session will be scheduled for the fitting of the hearing aid. Instruction regarding the proper care, use, and maintenance of hearing aids will be discussed with you and your child during the fitting. Periodic evaluations will be necessary to ensure that your child is benefiting from the aid.
Cochlear implants
What is a cochlear implant, and which children may be candidates?
Cochlear implants are needed to improve sensorineural hearing loss present at birth or later on when a hearing aid is not enough. A cochlear implant bypasses damaged portions of the inner ear to stimulate the auditory nerve that connects the ear to the hearing part of the brain, enabling children to hear the sounds around them and to hear spoken language.
Early referral to an experienced pediatric cochlear implant program is essential to minimize the time the child’s brain is not being stimulated by hearing. Since the cochlear implant is a surgically implanted device, pediatric cochlear implant teams include audiologists and surgeons with specialized knowledge and expertise. Cochlear implant surgery can be done safely in infants and young children. While a child who has profound loss in both ears is waiting for cochlear implant evaluation and implantation, it’s important for the child to be fitted for hearing aids in the meantime. While this may not be a permanent solution, the use of hearing aids during this waiting period will help to preserve the hearing part of the brain until the cochlear implant evaluation and procedure is complete.
What is the process to evaluate a child for cochlear implantation?
At Lurie Children’s, once an accurate diagnosis of the type and degree of loss has been made, children with loss that is not adequately helped by a hearing aid will begin the cochlear implant evaluation. The evaluation includes imaging (usually an MRI of the brain and ears) and additional hearing evaluations and counseling by an otologist and audiologist members of the cochlear implant team. The evaluation also includes an evaluation by a speech pathologist who is a listening and spoken language specialist (LSLS). Depending on the additional needs of the child, other referrals may be suggested.
What is the post-operation recovery process?
During cochlear implant surgery, tiny electrodes are threaded into the inner ear. After likely returning home the same day and returning to school the following week, a post-operative visit will be done with the surgeon one to two weeks after that. After two to four weeks, your child will return to see their cochlear implant audiologist to turn the implant on and to be fitted with the external components of the implant system. The cochlear implant audiologist will customize the cochlear implant’s computer programming to fit your child’s needs and during follow-up visits, they will continue to fine-tune the device and evaluate hearing.
Achieving individualized, ongoing programming, rather than a one-time, one-size-fits-all approach, requires a level of expertise that comes from experience and training in pediatric hearing loss. Additional visits are needed to fine-tune the device and evaluate hearing. Communication between you, your child’s therapist, the school program, and the implant team is important to achieve the best outcome.
How successful are cochlear implants?
With early diagnosis, appropriate treatment, and ongoing support, the long-term outlook for infants with hearing loss is generally very good. The sooner hearing loss is identified and treated, the better the chances of achieving positive results in language and social development. Many children who receive a cochlear implant develop spoken language, especially if implantation is not delayed. While early intervention in the form of hearing aid fitting, initiation of listening and spoken language therapy, and timely implantation offer the best results, children who have additional conditions that may interfere with language development still benefit from implantation. Because hearing helps our children to connect to others and promotes their safety through environmental awareness, the goal of cochlear implantation is to optimize this ability to listen as much as possible.
Are hearing aids or cochlear implants short or long-term solutions?
Hearing aids and cochlear implants are considered long-term solutions for hearing loss. With appropriate therapies and follow-up appointments with audiologists, they can provide significant improvement in hearing ability for a lifetime.
Should children with hearing loss learn sign language?
Whether a child learns sign language or not is ultimately up to the parent’s choice. It is best determined based on a child’s and family’s individual needs. Before cochlear implants became available in the 1990s, sign language was crucial for most children with severe to profound hearing loss in both ears to develop language. However, research shows that most deaf children who used sign language before the cochlear implant was available developed significantly reduced language and literacy compared to hearing children. Fortunately, improving hearing with cochlear implants has a positive impact on sign language learning and literacy, as well as spoken language.
What are parents’ most common fears about infant hearing loss?
Parents may feel overwhelmed when they learn that their child is experiencing hearing loss. It is common to be fearful about the child missing key developmental milestones, having problems with socialization and learning, or feeling responsible for the condition. With early intervention, many children with hearing loss thrive. Fortunately, there are many support groups, community resources, and networks for children with hearing loss to connect and share their experiences. You are not alone!
About Ann & Robert H. Lurie Children’s Hospital of Chicago
Led by a team of world-renowned pediatrics experts, Lurie Children’s is the #1 pediatric hospital in Illinois and an institution we trust for all things kids’ health. With 140 years of clinical expertise, research, and community engagement, the team at Lurie Children’s is committed to putting children and their families at the center of everything they do. We are honored to partner with Lurie Children’s to help educate our readers about how we can help our kids lead a healthier future. Learn more about Lurie Children’s and find a doctor near you today!
This post was in partnership with Ann & Robert H. Lurie Children’s Hospital of Chicago, but all of the opinions within are those of The Everymom editorial board.