Auditory Neuropathy Spectrum Disorder (ANSD)

ANSD stands for auditory neuropathy spectrum disorder. It is a type of hearing loss where sound enters the ear normally but it isn’t properly transmitted.

What is ANSD? ANSD is a type of hearing loss in which the inner ear (cochlea) receives sound normally but there is a problem in how that sound is transferred to the brain. This may be due to a breakdown in how the sound signals travel from the inner ear to the brain through the hearing nerve or there may be damage to the hearing nerve itself.

How is ANSD ANSD is identified by a series of tests. These include: identified?

Evoked Otoacoustic This test measures how the outer hair cells in your child’s cochlea work. A Emission Evaluation small probe is inserted into the ear canal and a sound is presented. (called the “EOAE”) Normally, an echo is produced in response to the sound. This echo response suggests the hair cells of the cochlea may be healthy.

Brainstem Auditory This test measures how the cochlea and hearing nerve respond to sound Evoked Response while your child is sleeping. When a child has normal EOAE (echo) test (called the “BAER”) results and absent or abnormal BAER responses, this may mean they have ANSD. Over time, the echo may disappear and your child’s echo test may become abnormal.

Other tests We may also assess how your child’s ears respond to loud sound or sounds using special tests such as acoustic reflexes and cochlear microphonic.

How can ANSD • Hearing loss may or may not be present affect hearing? • Hearing loss can range from mild to profound and can change in degree over time • Hearing loss can be in one ear or both ears • A child may have difficulty understanding speech sometimes or all of the time • Often the ability to understand speech may not match with routine hearing test results • Some children may have trouble hearing in noisy conditions and other children are not able to hear in any condition

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To Learn More Free Interpreter Services • Audiology • In the hospital, ask your nurse. 206-987-5173 • From outside the hospital, call the • Ask your child’s healthcare provider toll-free Family Interpreting Line, 1-866-583-1527. Tell the interpreter • seattlechildrens.org the name or extension you need.

Auditory Neuropathy Spectrum Disorder (ANSD)

What places a child • Premature birth at increased risk • Jaundice in newborns for ANSD? • Not getting enough oxygen at birth • Treatment with medications that can affect hearing (ototoxic medications) • Genetic syndromes such as Charcot-Marie-Tooth Disease, Friedreich’s Ataxia and other genetic conditions that affect the hearing nerve

How is ANSD The audiology team will work closely with you and your child’s healthcare managed? team to manage your child’s ANSD. This may include: • Closely monitoring your child’s speech and language development. • Evaluating and monitoring your child’s listening skills by an aural habilitation therapist. • Regular behavioral hearing tests every 3 to 4 months starting when your child is 7 to 9 months old. • Referral to early intervention or school based services, depending on your child’s age. • Recommendations for hearing devices or other assistive listening devices based on your child’s behavioral test results. • In some cases, a cochlear implant (CI) may be considered in children who demonstrate significant degrees of hearing loss.

Seattle Children’s offers interpreter services for Deaf, hard of hearing or non-English speaking patients, family members and legal representatives free of charge. Seattle Children’s will make this information available in alternate formats upon request. Call the 5/20 Family Resource Center at 206-987-2201. This handout has been reviewed by clinical staff at Seattle Children’s. However, your child’s PE2069 needs are unique. Before you act or rely upon this information, please talk with your child’s healthcare provider.

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