There are two main types of hearing loss: nerve hearing loss and conductive hearing loss. Children who have nerve hearing loss are usually born with the condition, which is also called sensorineural hearing loss (SNHL). SNHL occurs when there are problems of the inner ear that cause a hearing problem. Conductive hearing loss occurs when there is a problem conducting sound waves anywhere along the route through the ear canal, tympanic membrane (eardrum), middle ear or one of the three hearing bones (ossicles).
Causes of SNHL in children include:
- Congenital deafness. Most cases of congenital deafness are caused by genetics. Some cases are caused by infections or other congenital syndromes.
- Vestibular schwannoma. Typically first occurring in the teenage years, this condition occurs when a benign (harmless) tumor forms on the nerve of the inner ear. Children with this condition often have unilateral hearing loss (deafness in only one ear) and ringing in the ears (tinnitus).
Possible causes of conductive hearing loss include (in order from the most common to the least common):
- Otitis media/middle ear fluid. The eustachian tube allows air to pass from the back of the nose to the middle ear to equalize pressure to the outside world (i.e., pop the ear). In young children, this tube may not function efficiently, leading to fluid accumulation and infections in the middle ear.
- Eardrum perforation/hole in the eardrum. This condition occurs when there is bursting of the eardrum from trauma, infection or poor eustachian tube function. It can also occur after placement of ear tubes. Children with eardrum perforation can have drainage from the ear and often have a history of ear tube surgery.
- Cholesteatoma. This condition occurs when there is an abnormal skin growth in the middle ear.
- Hearing bone abnormalities. Congenital ossicular malformation can occur when a child is born with hearing bones that have not formed properly. Children born with ossicular malformation have a normal looking ear. Ossicular abnormalities can also occur as a result of cholesteatoma or another chronic ear condition.
- Congenital atresia of the ear canal. This condition occurs when a child is born without an ear canal.
Symptoms of any of the above types of hearing loss include trouble hearing or non-responsiveness to sound.
Diagnosis of Hearing Loss
All newborns should receive a newborn hearing screen—also called an otoacoustic emissions test—before leaving the hospital at birth. If this test comes back abnormal, your child must be re-tested, as there is a chance your child may have nerve hearing loss. Approximately 1 in 1,000 children are born with profound deafness.
Testing is a painless, noninvasive experience for children, incorporating games and activities designed to evaluate children on their terms. Results from your child’s evaluation are often shared with you the same day.
Doctors at Riley at IU Health can identify the type and severity of a child’s hearing loss and make recommendations for amplification, communication evaluations and speech therapy. If necessary, they will coordinate consultations with ear, nose and throat specialists. If a diagnostic evaluation shows that your child has a hearing loss that is appropriate for hearing aids, your family will be referred to a hearing aid audiologist.
Additional testing used to diagnose hearing loss includes:
- Tympanograms. Tests such as a tympanogram may be ran to measure the mobility of the eardrum. In a tympanogram, if the eardrum does not move well, then there is likely fluid in the ear.
- Physical exam. The doctor can diagnose fluid in the ear that can cause conductive hearing loss, which can be treated medically. The ear exam may be normal even if your child has nerve hearing loss.
- Auditory brainstem response (ABR). This test is used to diagnose nerve hearing loss. An ABR tests the entire circuit of hearing from the ear to the brain, giving the doctor information about the function of the inner ear and the brain pathways used for hearing. The ABR can help the doctor determine the degree of hearing loss and if it is affecting both ears. This test can be conducted in young children, as it does not require a response on the part of the child and can be done while the child is sleeping.
- Audiometry. If your child is old enough to respond to an audiogram, audiometry can be used to diagnose nerve hearing loss or conductive hearing loss. This test is conducted in a sound booth and requires your child to respond to sound by pointing to a toy or some other form of response.
- Magnetic resonance imaging (MRI). Your child’s doctor may use a MRI to determine if the hearing nerve is present and if the cochlea has developed normally. In addition, MRI can be used to diagnose a vestibular schwannoma, a rare cause of nerve hearing loss.
If your child is diagnosed with nerve hearing loss, his or her doctor will likely recommend a hearing aid. This is the first course of treatment for nerve hearing loss. Children referred for a hearing aid at Riley at IU Health will meet with a hearing aid audiologist. At that time, your child's care team will discuss amplification options and help your family select appropriate technology. Once the equipment arrives, your child will return for a hearing aid fitting and training to learn how to care for and use the hearing aids.
If the nerve hearing loss is profound and a hearing aid trial has been done, then your child may be considered for a cochlear implant.
Children with vestibular schwannoma often require surgery to remove the tumor. Depending on the size of the growth and the level of your child’s hearing loss, the surgeon may try to save the hearing or—if the tumor is too large—the tumor will be removed and the hearing will be lost.
If your child has conductive hearing loss, there could be several causes and each has a unique treatment option. Treatment options for each cause of conductive hearing loss include:
- Otitis media. Otitis media (fluid and infection of the middle ear) can be treated with observation and/or antibiotics. For recurrent episodes of otitis media, the doctor may recommend surgical drainage with ear tube placement. Resolution or removal of the fluid should lead to improved hearing.
- Eardrum perforation. For children with eardrum perforation, the doctor may choose to correct this with a tympanoplasty, a surgery used to reconstruct the eardrum.
- Cholesteatoma. Children with cholesteatoma typically require surgery to remove the abnormal skin growth, because the cholesteatoma can continue to grow.
- Ossicular malformation. Children with ossicular malformation may elect to have ossicular chain reconstruction (OCR), a surgery that reconstructs the damaged or missing bone. Another potential option is amplification with a hearing aid.
- Congenital atresia of the ear canal. Congenital atresia of the ear canal may be treated with an osteo-integrated implement or atresiaplasty. An osteo-integrated implement is a prosthesis that conducts sound through the skull directly to the inner ear, bypassing the abnormal ear canal. Atresiaplasty is a surgical procedure in which a surgeon builds an ear canal and new eardrum.
Key Points to Remember
Key Points to Remember
- The two main types of hearing loss are nerve hearing loss and conductive hearing loss.
- Most children with nerve hearing loss have a congenital condition, meaning that they were born with the hearing loss.
- The first step in treatment for nerve hearing loss in children is a hearing aid. If that is ineffective, the child will be considered for a cochlear implant.
- For children with conductive hearing loss, hearing may be improved through surgery or with a hearing aid device.
Support Services & Resources
Support Services & Resources
Find more information on pediatric hearing loss from these trusted organizations
Riley at IU Health offers a broad range of supportive services to make life better for families who choose us for their children's care.
This website from the Hearing Loss Association of America offers support and resources for parents of children with hearing loss.
This national organization provides guidance for families of children who have hearing loss.
Hearing Loss Research
Hearing Loss Research
The Ear, Nose & Throat Department at Riley at IU Health participates in a number of ongoing research studies and clinical trials for pediatric hearing loss. Investigators from the department engage in a variety of auditory neuroscience studies, including the creation of auditory tissue in a lab using stem cells. The department is also conducting clinical trials to study cochlear implant outcomes in children. Ask your child's doctor for more information about participation in research studies and clinical trials at Riley at IU Health.