Auditory neuropathy is a hearing disorder in which the inner ear successfully detects sound, but has a problem with sending sound from the ear to the brain. It can affect people of all ages, from infancy through adulthood. The number of people affected by auditory neuropathy is not known, but current information suggests that auditory neuropathies play a substantial role in hearing impairments and deafness.
An auditory neuropathy (AN) is roughly defined anatomically as a hearing loss caused by damage or aplasia of the auditory portion of the eighth nerve (6 on the picture to the left), which is located between the inner ear (cochlea-D) and the brainstem. (see item 6 on figure above-right). It is an overlap syndrome between otology and neurology, as it causes symptoms of interest to otologists (hearing loss), but is caused by a disease of interest to neurologists (nerve damage). A related acronym, ANSD — auditory neuropathy spectrum disorder — is a less well defined version of AN.
When their hearing sensitivity is tested, people with auditory neuropathy may have normal hearing or hearing loss ranging from mild to severe. They always have poor speech-perception abilities, meaning that they have trouble understanding speech clearly. People with auditory neuropathy have greater impairment in speech perception than hearing health experts would predict based upon their degree of hearing loss on a hearing test. For example, a person with auditory neuropathy may be able to hear sounds, but would still have difficulty recognizing spoken words. Sounds may fade in and out or seem out of sync for these individuals.
What causes auditory neuropathy?
Researchers report several causes of auditory neuropathy. In some cases, the cause may involve damage to the inner hair cells—specialized sensory cells in the inner ear that transmit information about sounds through the nervous system to the brain. In other cases, the cause may involve damage to the auditory neurons that transmit sound information from the inner hair cells to the brain. Other possible causes may include inheriting genes with mutations or suffering damage to the auditory system, either of which may result in faulty connections between the inner hair cells and the auditory nerve (the nerve leading from the inner ear to the brain), or damage to the auditory nerve itself. A combination of these problems may occur in some cases.
What Are the Signs & Symptoms of ANSD?
Symptoms can start at any age, but most kids with ANSD are born with it. Hearing loss can be mild to severe. Some kids hear sounds normally but still have trouble understanding speech clearly.
Even if a child passes a newborn hearing screening, hearing problems might be noticed over time. Talk to the doctor if your child does not reach these hearing milestones in the first year of life:
- For newborns, startling or “jumping” to sudden loud noises.
- By 3 months, recognizing a parent’s voice.
- By 6 months, turn the eyes or head toward a new sound and repeat sounds (like “ooh” and “aah”).
- By 12 months, making babbling sounds, responded to their name, imitating words, and saying a few words, such as “mama” or “bye-bye.”
As your child gets older, signs of a hearing problem may include:
- limited, unclear, or no speech
- not seem to pay attention or follow directions
- not responding to conversation-level speech or answering inappropriately
- being easily frustrated when there’s a lot of background noise
- needing a higher TV volume
- learning problems
Talk to your doctor if you have concerns about your child’s hearing.
How Is ANSD Diagnosed?
ANSD is diagnosed by an audiologist. An audiologist specializes in testing and helping people with hearing loss. Hearing tests that can help diagnose ANSD include:
- An otoacoustic emission (OAE): This test measures how well the cochlea works. It’s done when the child is lying still or asleep. Tiny earphones are placed in the ear canal. Clicking sounds or tones are made and electrodes (tiny stickers with wires attached) pick up the response from the inner ear (cochlea).
- Auditory brainstem response (ABR): This test measures how well the hearing nerve sends sound from the inner ear to the brainstem (the lower part of the brain). This test can show if the brain is not receiving the information in a clear way. Tiny earphones are placed in the ear canal. Clicking sounds or tones are made and electrodes measure the hearing nerve and brain’s response to the sounds. ANSD is likely when the OAE is normal and there is little or no response during the ABR.
- Middle ear muscle reflex (MEMR) test: Inside the ear is a tiny muscle that tightens when we hear a loud noise. This is called the middle ear muscle reflex (MEMR). For this test, a soft rubber probe tip goes in the ear canal. A series of loud sounds are sent through the tips into the ears. A machine records how well the middle ear muscle reflex reacts to the sounds. In a child with ANSD, loud sounds don’t trigger the reflex or sounds need to be louder to trigger it.
Newborn hearing screening programs may use a simple version of OAE and/or the ABR to test a baby’s hearing. The complete version of these tests is used to diagnose ANSD.
Children diagnosed with ANSD may need more testing, including:
- MRI: to look for inner ear problems and to see if there are hearing nerves in both ears
- genetic testing: to see if ANSD is part of a genetic condition
- neurologic testing: to look for any other nerve problems
- vision testing: Hearing and vision problems often go together, so it’s important for kids with ANSD to see an eye doctor.
Health professionals—including otolaryngologists (ear, nose, and throat doctors), pediatricians, and audiologists—use a combination of methods to diagnose auditory neuropathy. These include tests of auditory brainstem response (ABR) and otoacoustic emissions (OAE). The hallmark of auditory neuropathy is an absent or very abnormal ABR reading together with a normal OAE reading. A normal OAE reading is a sign that the outer hair cells are working normally.
An ABR test uses electrodes placed on a person’s head and ears to monitor brain wave activity in response to sound. An OAE test uses a small, very sensitive microphone inserted into the ear canal to monitor the faint sounds produced by the outer hair cells in response to auditory stimulation. ABR and OAE testing are painless and can be used for newborn babies and infants as well as older children and adults. Other tests may also be used as part of a comprehensive evaluation of an individual’s hearing and speech-perception abilities.
Treatment
Most kids with ANSD benefit from an assistive listening device. These work best when combined with ongoing speech-language therapy to help improve speaking and hearing skills. Assistive listening devices include:
- Frequency modulation (FM) systems: An FM system helps reduce background noise and makes a speaker’s voice louder. The person talking (like a teacher) wears a tiny microphone and a transmitter. The transmitter sends an electrical signal to a wireless receiver that the child wears either on the ear or directly in a hearing aid. It’s portable, and can be helpful in classrooms and other loud settings.
- Hearing aid: A hearing aid amplifies sounds coming into the ear. Hearing aids can help some children with ANSD, especially when used with an FM system. Hearing aids used alone do not help kids with ANSD because they only make the disorganized sound louder.
- Cochlear implant: A cochlear implant is an option for some kids with ANSD. This surgically placed device bypasses the parts of the ear that are not working properly. It stimulates the hearing nerve directly. With training and therapy, kids with a cochlear implant can learn to hear and speak well.
What Else Should I Know?
Besides using assistive listening devices, kids with ANSD may learn visual communication skills, such as:
- Sign Language is a special language with a grammar, vocabulary, and syntax all its own. It is meant to be used in place of spoken language.
- Cued Speech is a technique where the speaker makes hand gestures around their mouth. The person watching the speaker uses these hand cues to tell the difference between lip movements that look the same (such as “b” and “p”). Cued speech can be used in any language and can be very useful for bilingual families.
- Signing Exact English (SEE) is a sign system that matches exactly what is spoken in English. It includes sign language, but its sentence structure and grammar are taken from English.
What are the roles of the outer and inner hair cells?
Outer hair cells help amplify sound vibrations entering the inner ear from the middle ear. When hearing is working normally, the inner hair cells convert these vibrations into electrical signals that travel as nerve impulses to the brain, where the brain interprets the impulses as sound.
Although outer hair cells—hair cells next to and more numerous than inner hair cells—are generally more prone to damage than inner hair cells, outer hair cells seem to function normally in people with auditory neuropathy.
Are there risk factors for auditory neuropathy?
There are several ways that children may acquire auditory neuropathy. Some children diagnosed with auditory neuropathy experienced particular health problems before or during birth or as newborns. These problems include inadequate oxygen supply during or prior to birth, premature birth, jaundice, low birth weight, and dietary thiamine deficiency. In addition, some drugs used to treat pregnant women or newborns may damage the baby’s inner hair cells, causing auditory neuropathy. Adults may also develop auditory neuropathy along with age-related hearing loss.
Auditory neuropathy runs in some families, and in some cases, scientists have identified genes with mutations that compromise the ear’s ability to transmit sound information to the brain. Thus, inheritance of mutated genes is also a risk factor for auditory neuropathy.
Some people with auditory neuropathy have neurological disorders that also cause problems outside of the hearing system. Examples of such disorders are Charcot-Marie-Tooth syndrome and Friedreich’s ataxia.
Does auditory neuropathy ever get better or worse?
Some newborn babies who have been diagnosed with auditory neuropathy improve and start to hear and speak within a year or two. Other infants stay the same, while some get worse and show signs that the outer hair cells no longer function (abnormal otoacoustic emissions). In people with auditory neuropathy, hearing sensitivity can remain stable, get better or worse, or gradually worsen, depending on the underlying cause.
What treatments, devices, and other approaches can help people with auditory neuropathy to communicate?
Researchers are still seeking effective treatments for people with auditory neuropathy. Meanwhile, professionals in the hearing field differ in their opinions about the potential benefits of hearing aids, cochlear implants, and other technologies for people with auditory neuropathy. Some professionals report that hearing aids and personal listening devices such as frequency modulation (FM) systems are helpful for some children and adults with auditory neuropathy. Cochlear implants (electronic devices that compensate for damaged or nonworking parts of the inner ear) may also help some people with auditory neuropathy. No tests are currently available, however, to determine whether an individual with auditory neuropathy might benefit from a hearing aid or cochlear implant.
Debate also continues about the best ways to educate and improve communication skills in infants and children who have hearing impairments such as auditory neuropathy. One approach favors sign language as the child’s first language. A second approach encourages the use of listening skills—together with technologies such as hearing aids and cochlear implants—and spoken language. A combination of these two approaches may also be used. Some health professionals believe it may be especially difficult for children with auditory neuropathy to learn to communicate only through spoken language because their ability to understand speech is often severely impaired. Adults with auditory neuropathy and older children who have already developed spoken language may benefit from learning how to speechread (also known as lip reading).
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