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Auditory Neuropathy Spectrum Disorder

Auditory neuropathy spectrum disorder (ANSD) is a condition in which sound isn't transmitted properly from the ear to the brain. Patients may be able to hear sound but may not be able to distinguish what the sound is. Infants born in the United States are routinely screened for hearing problems, including ANSD, at birth.

  1. What Is It?

    • Hearing problems caused by auditory neuropathy spectrum disorder are related to damage to the auditory nerve or to the path between the functional parts of the ear and the auditory nerve. This may cause sound to not reach the brain, or sound to be deciphered incorrectly in the brain.

    Risk Factors

    • Doctors don't know exactly what causes ANSD. However, there are some risk factors that increase the chance of a child having ANSD. These risk factors include being born prematurely, having illness as a newborn, being born with a low birth weight, head trauma or a genetic predisposition to ANSD.

    Diagnosis

    • Newborns are routinely screened for a variety of hearing problems. However, not all problems may be detected in these routine screenings. If you notice that your child doesn't startle at loud sounds; doesn't coo, babble or laugh by 8 months of age; or doesn't imitate sounds and actions or respond to simple commands by 12 months of age, your doctor may pursue further tests to rule out ANSD.

    Prognosis

    • ANSD can vary in severity. Some sufferers may be able to hear sound but have trouble distinguishing what it is. Others may have severe symptoms that cause all sound to be the same, like television static. For some, symptoms will improve with time. For others, intervention such as hearing aids or cochlear implants will make communication easier.

    Treatment

    • There is no cure for ANSD. However, a number of therapies and devices help to mitigate the symptoms. A speech-language pathologist can help a child with ANSD develop communication skills. Hearing aids in conjunction with frequency modulation systems or cochlear implants may improve hearing and comprehension.

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