Auditory Neuropathy Spectrum Disorder (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.

ANSD is identified by a series of tests explained below.

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

This test measures how the cochlea and hearing nerve respond to sound while your child is sleeping. When a child has normal EOAE (echo) test 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.

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.

• Hearing loss may or may not be present.
• 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.

• Premature birth
• Jaundice in newborns
• 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

The audiology team will work closely with you and your child’s healthcare 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.