Individuals with lesions that affect the auditory nerve show poor performance with CIs, likely because neural transmission of the electrical signal from the CI is affected. Reviewed data show that individuals with lesions that primarily affect the cochlear sensory system and the synapse, which are bypassed by the CI, have optimal CI outcomes. In this review, we examine the precise mechanisms of genetic lesions causing ANSD and the effect of these lesions on CI outcomes. Auditory neuropathy spectrum disorder (ANSD) is characterized by dysfunctional transmission of sound from the cochlea to the brain due to defective synaptic function or neural conduction.
There is growing evidence that qualitative as well as quantitative deficits in the auditory nerve may affect cochlear implant (CI) outcomes. However, no tests are available now to decide whether a child with ANSD might benefit more from a hearing aid or cochlear implant.Cochlear implantation has become the standard‐of‐care for adults and children with severe to profound hearing loss. Some children benefit more from a cochlear implant than from hearing aids. Remote microphone systems can improve listening in noisy backgrounds. Some professionals recommend the use of remote microphone systems. Hearing aids must be set carefully to prevent damage to the parts of the ear that receive sounds normally. However, many children get limited hearing aid benefit or no benefit at all. What is the Current Treatment for ANSD?Ĭurrently, there is no medical treatment for ANSD. At this time, no tests can tell us if a child’s hearing will change. Other infants stay the same or even get worse. They may start to hear and speak within a year or two. Some newborn babies who have been diagnosed with ANSD improve over time. It may also show problems with hearing in noisy backgrounds. Ongoing testing may show a poor understanding of speech.
Parents and doctors may not suspect hearing loss right away if an infant’s response to sound changes from day to day.Īs children get older, more hearing information becomes available. This may slow the identification of ANSD. They can be used in both newborn infants and older children.ĭepending upon what test is used for newborn hearing screening, some children with ANSD may pass the first hearing screening in the hospital. These tests include auditory brainstem response (ABR), acoustic reflex testing and otoacoustic emissions (OAE). Neurological disorders such as Charcot-Marie-Tooth syndrome or Friedreich’s AtaxiaĪudiologists use a combination of tests to diagnose ANSD.Severe jaundice during the newborn period.Many risk factors have been linked to ANSD in children. It can be inherited genetically or caused by trauma or disease. The condition likely has more than one cause. Most children with ANSD are diagnosed in the first months of life. The child acts as if they have a hearing loss.The child’s hearing appears to change daily or even hourly.
This is especially true in a noisy place. The child may have a hard time understanding speech.The child has inconsistent responses to speech.Hearing loss can vary greatly in children with ANSD, from normal hearing to profound hearing loss. This can be very frustrating for parents and can make a getting final diagnosis difficult. A child with ANSD may appear to hear one day and not hear the next. Children with ANSD may have hearing responses that vary or worsen over time.
The sounds may fade in and out or are "out of sync". They may also have trouble understanding speech clearly. Someone with ANSD may have trouble telling one sound from another. Auditory Neuropathy Spectrum Disorder (ANSD) is a hearing problem in which the ear detects sound normally, but has a problem sending it to the brain.
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