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Auditory Neuropathy/Dyssynchrony

Conditions

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Auditory neuropathy spectrum disorder (ANSD) happens when sound is received by the cochlea but nerve impulses within the auditory system are disrupted. Most often a child with ANSD has difficulty hearing and understanding speech.

Causes 

The exact cause of auditory neuropathy is not fully understood. Researchers believe it may result from more than one problem affecting how sound signals travel from the inner ear to the brain.

Possible causes include:

  • Damage to the inner ear cells that send sound information to the brain
  • Problems with the connection between the inner ear and the hearing nerve
  • Damage to the hearing nerve itself
  • Sometimes, a combination of these problems may occur.

Children with auditory neuropathy often have normal-functioning outer ear hair cells, but sound signals are not transmitted properly to the brain.

Some children with auditory neuropathy may have had health problems during the mother's pregnancy, at birth or shortly after birth that could have affected their hearing. However, a direct cause is not always known.

Risk Factors

  • Lack of oxygen at birth
  • Severe jaundice (high bilirubin levels) in the newborn period
  • Premature birth
  • Family history or genetic conditions associated with hearing loss
  • Neurological disorders, such as Charcot-Marie-Tooth disease or Friedreich's ataxia. 
  • Certain infections or illnesses, including mumps and other diseases that affect the nervous system
  • Use of medications that can damage the inner ear or hearing nerve
  • Head injury or trauma affecting the hearing system

Symptoms and Types

  • Have difficulty understanding speech, especially in noisy environments
  • Have trouble telling one sound from another
  • Appear to hear well one day but not another
  • Have hearing that seems to change or fluctuate over time
  • Respond inconsistently to sounds or speech
  • Experience sounds that seem distorted, faded, or out of sync
  • Show signs of speech, language or learning difficulties related to hearing challenges

Auditory neuropathy can range from mild to severe and may affect one or both ears.

Diagnosis and Tests

Your child may need hearing tests that include:

  • Tympanometry and acoustic reflex testing checks how well the middle ear is working and helps identify where the hearing problem may be located.
  • Otoacoustic emissions (OAE) measures how well the inner ear (cochlea) responds to sound.
  • Auditory brainstem response (ABR) checks how the hearing nerve and brain respond to sound.
  • Pure tone air and bone conduction testing finds the softest sounds your child can hear at different pitches.

Treatment and Care

Experts do not always agree on which hearing devices work best for children with auditory neuropathy. A treatment that helps one child may not help another.

Possible treatment options include:

  • Hearing aids to make sounds louder
  • FM or remote microphone systems that help children hear better in noisy places, such as classrooms
  • Cochlear implants, which are electronic devices that help send sound signals to the hearing nerve when the inner ear is not working properly

Currently, no test is available that can predict whether a child will benefit from hearing aids or a cochlear implant. Therefore, treatment decisions are based on the child's individual hearing, communication needs and progress.

There are two main ways to help children with auditory neuropathy learn to communicate:

  • Sign language approach: Children learn sign language as their primary language.
  • Listening and spoken language approach: Children learn to listen and speak using hearing technology, such as hearing aids or cochlear implants.

Some families choose to use both approaches together. However, because many children with auditory neuropathy have difficulty understanding speech, some professionals believe that learning through spoken language alone may be especially challenging.

It is important for parents to work with a team of health care and educational professionals to find the best approach for their child. Parents and caregivers can also support communication by spending time interacting with their child through talking, smiling, making eye contact, and responding to their attempts to communicate.

Living and Managing

Family involvement plays an important role in helping a child with auditory neuropathy succeed. Ways families can help include:

  • Being actively involved in their child's hearing, communication, and learning needs
  • Working closely with health care providers, therapists, and teachers
  • Creating fun and meaningful activities that encourage communication and interaction
  • Supporting their child's language development through everyday conversations, play, and family activities

With patience, support and teamwork, families can help their child build strong communication skills and reach their full potential.