MORE ON THIS TOPIC FOR:

Parents Kids Teens

Auditory Processing Disorder

Ear Injuries

Ears
KidsHealth / For Parents / Auditory Neuropathy Spectrum Disorder (ANSD)
Speech-Language Therapy

► WHAT'S IN THIS ARTICLE? Cochlear Implants

Delayed Speech or Language

Listen Development

Your Child's Checkup: Newborn

About ANSD
Hearing loss is a common problem in newborns. Some cases are due Hearing Evaluation in Children
to auditory neuropathy spectrum disorder (ANSD), a problem in the
transmission of sound from the ear's innermost part (the inner ear) to
the brain.

PARTNER MESSAGE

The causes of ANSD are unknown, but children who are born
prematurely or have a family history of the condition are at higher risk
for it. Symptoms can develop at any age, but most kids with ANSD are
born with it and diagnosed in the first months of life.

As ANSD becomes better understood, it is diagnosed more frequently

and now accounts for about 10% to 15% of cases of hearing loss.

Fortunately, kids with ANSD can develop strong language and

communication skills with the help of medical devices, therapy, and
visual communication techniques. Proper diagnosis and early
intervention are essential, so if you suspect that your child has any
difficulty hearing, talk with your doctor as soon as possible.

How Hearing Works

To understand ANSD, it's helpful to review how the ear hears. Hearing
begins when sound waves that travel through the air reach the outer
ear, or pinna, the part of the ear that's visible. The sound waves go
through the ear canal into the middle ear, which includes the eardrum
(a thin layer of tissue) and three tiny bones called ossicles. The sound
causes the eardrum to vibrate. The ossicles amplify these vibrations
and carry them to the inner ear.

The inner ear is made up of a snail-shaped chamber called the

cochlea, which is filled with fluid and lined with four rows of tiny hair
cells. When the vibrations move through this fluid, the outer hair cells
contract back and forth and amplify the sound. When the vibrations
are big enough, the inner hair cells translate them into electrical nerve
impulses in the auditory nerve, which connects the inner ear to the
brain. When the nerve impulses reach the brain, they are interpreted as
sound.

How ANSD Affects Hearing

When someone has ANSD, sound enters the ear normally, but because
of damage to the inner row of hair cells or synapses between the inner
hair cells and the auditory nerve, or damage to the auditory nerve
itself, sound isn't properly transmitted from the inner ear to the brain.

As a result, the sound that arrives at the brain isn't organized in a way
that the brain can understand. It is disorganized and in some cases
the sound never even makes it to the brain. In other cases, ANSD is
due to a problem with the auditory nerve.

ANSD has only been understood and diagnosed in recent years. As a

result, many questions remain about it. Not all newborn hearing
screening programs can identify ANSD, so many kids and adults might
have it but not know it.

The symptoms of ANSD can range from mild to severe. Some kids
with ANSD hear sounds but have trouble determining what those
sounds are. For others, all sounds seem the same, like static or white
noise. For example, a voice might sound the same as water running, a
dog barking might sound the same as a car horn, or a bird chirping
might sound the same as a pan banging.
For some people, ANSD improves over time. For others, it remains the
same or gets worse.

Causes
The causes of ANSD aren't known. But some factors do put a child at
risk, including:

premature birth
illness in newborns such as jaundice
low birth weight
anoxia and hypoxia
family history of ANSD
head trauma

Symptoms
Even if a child passes a newborn hearing screening, symptoms of
hearing problems might only be noticed over time. Talk with your
for Parents for Kids
doctor if you think that your child is having trouble hearing or if you for Teens
for Educators
notice any of these symptoms:

your infant doesn't startle when there are loud or sudden noises
or doesn't turn toward sound
your baby isn't cooing, babbling, or laughing by 8 months
your child is not trying to imitate sounds and actions by 12
months or isn't responding to simple commands

If hearing problems seem likely, your doctor might refer you to an
audiologist (someone who specializes in diagnosing and treating
hearing loss and balance problems). Your doctor also might have your
child see an otolaryngologist (also called an ears, nose, and throat
[ENT] doctor).

Diagnosis
A series of tests can help diagnose ANSD and rule out other hearing
problems. Many of these are part of routine hearing screenings given
to newborns. They're also used to diagnose the condition in older kids.

The tests won't cause pain or discomfort and in most cases do not
require a hospital stay.

Middle ear muscle reflex (MEMR): The MEMR tests how well the ear
responds to loud sounds. In a healthy ear, loud sounds trigger a reflex
and cause the muscles in the middle ear to contract. In a child with
ANSD, loud sounds don't trigger the reflex or much louder sounds are
needed to trigger it.

For the MEMR (also called an acoustic reflex test), a soft rubber tip is
placed in the ear canal. A series of loud sounds are sent through the
tips into the ears and a machine records whether the sound has
triggered a reflex. Sometimes the test is done while the child is
sleeping.

Otoacoustic emission (OAE): This test measures how well the outer
hair cells in the cochlea function. It's done when the child is lying still
or asleep, either naturally or through mild sedation. A tiny probe that
contains a special microphone is placed in the ear canal, pulsing
sounds are sent through it, and a machine measures what kind of
echo the sound causes in the outer hair cells.

Auditory brainstem response (ABR): This test measures whether the

auditory nerve transmits sound from the inner ear to the lower part of
the brain and how loud sounds have to be for the brain to detect them.
If the brain is not receiving the information in a clear way, this test can
show that. During the ABR the child is asleep, either naturally or
through sedation. The audiologist places tiny earphones in the ear and
sends sounds through them while electrodes (which look like tiny
stickers) placed on the child's head measure brain activity.

Most newborn hearing screening programs use both OAE and ABR to
test a baby's hearing. Some only use OAE, which cannot diagnose
ANSD alone. That's why it's important to tell your doctor if you suspect
your child is not hearing well, even if he or she passed the newborn
hearing screening.

Other Tests
If these tests show that a child has ANSD, additional testing will be
needed. In kids older than 1, these tests can determine the severity of
the hearing problem. They include: 

Magnetic resonance imaging (MRI) to see if the auditory nerve

is present in both ears and if there are any inner ear
abnormalities.

Genetic testing to see if ANSD is caused by a genetic condition,

and if so, what treatments may be helpful.

Neurologic testing by a neurologist to look for any other nerve-

related problems.

Ongoing speech and language testing. A child with ANSD

needs regular visits with a speech-language-pathologist, who
will monitor speech and language development to make sure
the child is on track. Pathologists also can determine if
treatment isn't working well and suggest alternatives.

Opthalmology visit. Hearing loss can be associated with vision

loss, so it's important for kids with ANSD to have vision
screenings by an eye doctor.

Treatment
While there is no known cure for ANSD, assistive listening devices
(ALDs) can help kids with ANSD make sense of sounds and develop
language skills. You'll work with a medical team to determine which
devices are right for your child. Treatment for ANSD depends on how
severe it is and the child's age at diagnosis.

An important part of making any device effective is ongoing therapy

with a speech-language pathologist, who help kids with hearing loss
develop speaking and hearing skills.
ALDs include:

Frequency modulation (FM) system: An FM system helps reduce

background noise and makes a speaker's voice louder so the child can
understand it. The speaker wears a tiny microphone and a transmitter,
which sends an electrical signal to a wireless receiver that the child
wears either on the ear or elsewhere on the body. It's portable and can
be helpful in classroom settings.

Hearing aid: A hearing aid amplifies sounds coming into the ear. Often
it can help when the outer hair cells don't work as they should and
can't amplify sound. In some cases of ANSD, hearing aids help when
they're used with an FM system. In most cases, hearing aids used
alone do not help kids with ANSD because they only make the
disorganized sound louder.

Cochlear implant: A cochlear implant is a device that has internal and

external parts and is designed to replace the portions of the ear that
aren't working properly. Parts of the implant are placed inside the skull
during a surgical procedure. Another part of the implant is worn on the
outside, behind the ear. With training and therapy, a child with a
cochlear implant can learn to hear and speak well.

Cochlear implants usually aren't an option until after kids are at least 1

year old and have tried other ALDs without success. Cochlear
implants have helped many people with ANSD, but ongoing therapy
after the surgery is crucial for them to be effective. Not all kids with
ANSD are candidates for the implants.

Communication
In addition to assistive listening devices, kids with ANSD often can
benefit from learning visual communication skills. American Sign
Language (ASL), Cued Speech, and Signed Exact English are three
types of visual communication techniques. You'll work with the
medical team to determine which is best for your child.

ASL is the best known form of visual communication. A distinct

language with a grammar, vocabulary, and syntax all its own, ASL is
meant to be used in place of spoken language.

Cued Speech and Signed Exact English are visual communication

techniques that directly translate what's being said. Both are meant to
be used with spoken language to help people understand anything
they can't comprehend through lip reading.

Cued Speech is a technique in which different hand shapes are placed

in various positions around the face to convey — or cue — different
consonants and vowel sounds. It involves a limited number of hand
positions and shapes, which may make it easier to learn than other
methods. Cued speech can be used in any language and can be very
useful for bilingual families.

Signed Exact English involves using certain hand gestures to

represent sounds and suffixes (like "-ing") and prefixes (like "re-").
By learning as much as they can about communication techniques
and working closely with the medical team, parents can play an
important role in helping their kids manage ANSD and learn to
communicate.

Reviewed by: Thierry Morlet, PhD

Date reviewed: September 2014

About Us | Contact Us | Partners | Editorial Policy | Permissions Guidelines | Privacy Policy & Terms of
Use | Notice of Nondiscrimination

Note: All information on KidsHealth® is for educational purposes only. For specific medical advice, diagnoses, and
treatment, consult your doctor.
© 1995-2018 The Nemours Foundation. All rights reserved.
Images provided by The Nemours Foundation, iStock, Getty Images, Veer, Shutterstock, and Clipart.com.