A child born with auditory neuropathy spectrum disorder (ANSD) has difficulty distinguishing one sound from another and understanding speech clearly. Nemours audiologists and other specialists can help kids with ANSD develop strong language and communication skills through the use of medical devices and therapy.
Auditory neuropathy spectrum disorder (ANSD) is a broad term that describes any of a variety of problems involving “misfirings” of the auditory (hearing) nerve, or problems affecting the nerve’s connection with the cochlea (inner ear). The result of ANSD is the disordered transmission of sound from the inner ear that makes sound disorganized when it reaches the brain.
The causes of auditory neuropathy spectrum disorder 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.
Proper diagnosis and early intervention are important, so if you think that your child has any difficulty hearing, talk with your doctor as soon as possible. Children with auditory neuropathy spectrum disorder benefit from specialized testing that is often different from what is offered in a newborn hearing screening. As ANSD becomes better understood, it is diagnosed more frequently and now accounts for about 10% to 15% of cases of hearing loss in children.
In some cases, auditory neuropathy spectrum disorder causes only mild hearing difficulties and is only a problem in noisy situations. In the majority of cases, however, it leads to significant hearing loss.
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Note: A parent or legal guardian must be with a child for a first visit.
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Auditory Neuropathy Spectrum Disorder (ANSD) occurs when sound enters the inner ear normally, but the transmission of signals from the inner ear to the brain is impaired. Knowing that ANSD is the cause of your child’s hearing loss allows us to approach the problem from the correct perspective.
Working Together to Help Your Child and Family
As with other types of hearing loss, we believe that a comprehensive team approach to treating auditory neuropathy spectrum disorder is best.
Our unique ANSD program brings together a team of experts including:
- pediatric audiologists: specialists who diagnose, evaluate, and treat
- pediatric otologist: an ENT doctor who specializes in abnormalities of
- speech and language pathologists: specialists who diagnose, evaluate, and treat pediatric communication problems
- an educator of the deaf and educational consultant
- a linguistic researcher: a specialist who studies language and speech sounds, formation, and structure
- an auditory neurophysiologist: a specialist who studies how the nerves related to hearing work
- consultants from various other departments including neurology, ophthalmology, and genetics
Reaching Out to the Community and Beyond
Our auditory neuropathy spectrum disorder team not only manages your child’s treatment plan, but is also actively involved in educating the community regarding the diagnosis and management of children with ANSD. Our goal is to use our specialized knowledge about ANSD to create a plan for treatment that we can share with other facilities throughout the country.
During the process of your child’s auditory neuropathy spectrum disorder evaluation, members of our team with meet with your family. Our team also meets on a regular basis to review cases.
Audiological testing includes an evaluation of the otoacoustic emissions (OAEs) of the inner ear — measures the function of the outer hair cells, or the “natural amplifiers” of the inner ear — middle ear muscle reflexes (MEMR), and an auditory brainstem response (ABR). The ABR test, performed when your child is either asleep naturally or sedated, measures whether the auditory nerve transmits sound from the inner ear to the brain and how loud sounds have to be for the brain to detect them.
Once the diagnosis of ANSD has been made a full evaluation follows. This evaluation includes a genetics consultation, a speech and language evaluation, imaging studies, and monitoring by our ANSD team.
Testing for auditory neuropathy spectrum disorder is appropriate when:
- Parents report their child is having difficulty understanding speech clearly, especially in the presence of background noise.
- Behavioral testing reveals absent middle ear muscle reflexes and decreased speech discrimination testing in quiet and noise.
- Speech and language development is a concern — children with this ANSD can have speech that ranges from mildly distorted to absent.
While there is no known cure for auditory neuropathy spectrum disorder, hearing aids and assistive listening devices (ALDs) can help kids with ANSD make sense of sounds and develop language skills. Our team approach allows us to collaborate to determine which devices and therapies are right for your child.
A crucial part of making any device effective is ongoing therapy with a speech-language pathologist, who helps children with hearing loss develop speaking and hearing skills.