Reviewed by Danielle Leisner, AuD, CCC-A, Sarah McKay, AuD, CCC-A
What is hearing loss?
About 2 to 3 out of every 1,000 children have hearing loss in one ear (unilateral) or both ears (bilateral) at birth. Early detection and management of hearing loss are very important and will help a child stay on track with developmental milestones.
The hearing and listening parts of the brain depend on consistent access to sounds and voices in the environment in order for a child to best develop speech and language, learn, and socialize. Hearing loss can be caused by conditions that affect any part of the ear and can interrupt or limit the brain’s access to sound and ability to understand speech. Learn more about how we hear.
What are the types of hearing loss?
The ear is divided into three major parts:
- Outer ear - pinna (the part that can be seen) and ear canal
- Middle ear - eardrum, ossicles (three bones) and the Eustachian tube (narrow tube that connects the middle ear to the throat)
- Inner ear - cochlea (hearing organ), and balance system
Conductive – hearing problem caused by condition affecting the outer and/or middle ear. This type of hearing loss is more likely to respond to medical or surgical treatment and may be temporary or permanent.
Sensorineural – hearing problem caused by condition affecting the inner ear. This type of hearing loss is usually permanent.
Mixed – hearing problem caused by a condition affecting both the outer and/or middle ear and the inner ear.
Neural – hearing problem where the ear detects sound normally but has difficulty sending the information to the brain.
What are the possible causes of hearing loss (present at birth or acquired)?
- Family history of permanent childhood hearing loss
- Genetic syndrome associated with hearing loss
- Anatomic differences in development of part(s) of the ear
- Craniofacial abnormality
- Infections at or before birth, such as cytomegalovirus (CMV)
- Hyperbilirubinemia
- Hypoxic events (brain does not receive enough oxygen)
- History of extracorporeal membrane oxygenation (ECMO) therapy
- History of ototoxic medications
- Conditions requiring a neonatal intensive care stay of greater than five days
- Head trauma
- Meningitis
- Exposure to loud sounds — especially through earphones
- Diseases of the middle or inner ear
What are the signs and symptoms of hearing loss?
You might be concerned that your child has a hearing loss if they:
- Does not respond to sounds and voices — or stops responding consistently
- Is not meeting age-appropriate speech and language milestones
- Has frequent middle ear infections and/or middle ear fluid
- Turns up the volume of music or television
- Says “what?” a lot and needs information or requests repeated
How do you test for and diagnose hearing loss?
At Children’s Hospital of Philadelphia, audiologists conduct comprehensive hearing evaluations for children from birth until early adulthood. Learn more about each of the hearing tests your child may receive and what to expect at your appointment. The results of a child’s hearing test are typically recorded on an audiogram. An audiogram is a graph of the softest level that a child can hear different pitches or frequencies (Hz). Your child’s audiologist will explain the results of your child’s hearing test at their appointment and will discuss if further testing or treatment is recommended.
What are the treatments for hearing loss?
If a hearing loss is found, a multidisciplinary team of specialists will provide you with appropriate resources, intervention services, and ongoing follow-up care. This team includes audiologists and speech-language pathologists to evaluate communication skills, as well as integrated psychosocial specialists to support healthy adjustment to hearing loss. Otolaryngologists (ear, nose and throat specialists) will check your child’s ears to make sure that other conditions such as ear wax or middle ear fluid are not contributing to your child’s hearing loss. The doctor may order other tests to see if the cause of the hearing loss can be found. The ENT doctor will also need to sign a medical clearance form if your child is getting a hearing device.
Your audiologist may also recommend evaluations with the following specialists:
- Genetics: A genetics specialist can look for the cause of a child’s hearing loss — there are more than 150 genes whose mutations can cause hearing loss — and if there are any other related medical conditions. They may also suggest that other family members have their hearing tested. If your child has an otoferlin gene (OTOF)-mediated hearing loss, they may be eligible for a gene therapy CHOP is offering as part of an experimental therapy trial. Learn more about this breakthrough treatment.
- Ophthalmology (eye specialist): Children with hearing loss rely on their eyesight to help them. It is important to have your child’s eyes checked. If they have vision problems, the specialist will suggest ways to improve this.
- Developmental skills: A psychologist or developmental and behavioral pediatrician can assess your child’s development and school performance. The results will help you to understand your child’s ability to learn and is useful in creating your child’s educational plan.
- Early Intervention (EI): If your child is younger than 5 years of age, they should be evaluated by your local early intervention program. This program is available to your child in your community. They may test your child and recommend services to stimulate speech and language development. They may simply want to monitor your child and track their development.
- Other: Some children also benefit from seeing a neurologist or a physical or occupational therapist. Sometimes families see a social worker to help them find resources or other support for their child. Some children with hearing loss also have dizziness or balance problems. Your child may be referred for a comprehensive multidisciplinary evaluation with CHOP's Balance and Vestibular Program.
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How We Hear
Narrator: Where is my phone? Sound is all around us all the time. Some are background sounds that you may not even notice, and other sounds you use for everyday communication and learning. Some sounds, like music, you choose for enjoyment, and other sounds, like the cellphone you can't find, tell you something is happening.
Have you ever wondered how we hear all of these sounds? Sure, you know we hear with our ears, but how exactly? Let's think about that cellphone. When it pings, it creates sound waves that travel through the air. Not all sound waves are the same, and in fact, they are all different, much like the ripples created in a pond would be very different if you threw a bowling ball into it versus a penny.
Back to the ear. The outer portion of your ear is often called the pinna. It helps to catch the sound waves and direct them down into your ear canal. You may have noticed that sometimes this part of your ear can have earwax. Don't worry, a bit of earwax is normal and is actually there to protect your ear.
At the very end of your ear canal is your tympanic membrane, also known as your eardrum. When the sound waves hit your eardrum, it vibrates. The sound journey is not over yet. We have just gotten started. On the other side of the eardrum is a place called the middle ear. There are three little bones in your middle ear called ossicles, and fun fact: these bones, the malleus, incus, and stapes, are the tiniest bones in your body.
The malleus is attached to the eardrum on one side and the incus on the other, and the incus attaches to the stapes. What do you think happens when the eardrum vibrates? You guessed it. Those three little bones move back and forth. Sound waves, which were invisible in the air, are now turned into mechanical energy, like a little machine.
All of that energy gets transferred to the inner ear by the stapes through a space called the oval window. On the other side of this window is a very important part of the inner ear, the cochlea. The cochlea looks like a snail shell and is the size of a pea. Unlike the other areas of the ear which are filled with air, the cochlea is filled with fluid.
It is also lined with thousands of tiny hairs that are actually cells. These hair cells create electrical impulses when they move. As sound energy is pushed through the oval window, it makes waves in the fluid in your cochlea. Kind of like those sound waves from outside your ear. Different hair cells respond to different sound waves.
Some respond to waves from low-pitched sounds like a tuba, and some respond to waves from high-pitched sounds like a flute. What is really cool is that different hair cells in the cochlea respond all at the same time as complex sounds made up of a variety of different pitches, like a person talking or a song on the radio enter your ear.
So at this point in the journey, sound is now being sent by electrical impulses from the hair cells of the cochlea to the hearing nerve, cochlear nerve. The hearing nerve sends the impulses up to your brain. Your ears help you determine where the sound is coming from, and your brain allows you to recognize the sounds, and in some cases, learn new sounds.
I know. This sounds like a very long journey, but it all happens in the blink of an eye. Speaking of my brain recognizing sounds, where is that cell phone? Oh, there it is.
Hearing devices for children
Some children who are diagnosed with hearing loss benefit from being fit with a hearing device (such as a hearing aid, bone conduction hearing device or cochlear implant). Learn more about hearing devices for children with hearing loss.
Resources to help
Hearing Loss in Children Resources
Department of Audiology Resources
We have created resources to help you find answers to your questions about hearing loss and assistive devices so you can feel confident in the care you are providing your child.
Meet your team
Each of our audiologists holds a Master or a Doctor of Audiology degree. Our staff provides medical care, emotional counseling and more. Our educator, psychologist, and social worker ensure your family gets the support you need.
Patient stories
