Auditory Neuropathy Spectrum Disorder

History Of Auditory Neuropathy Spectrum Disorder

The diagnosis of Auditory Neuropathy (AN) started emerging in the late 1980’s with the addition of otoacoustic emissions testing to the battery of tests to diagnose hearing loss. At this time there was a unique group of hearing impaired patients with unusual test results. The otoacoutic emissions, signifying that the outer hair cells of the cochlea were functional, were found to be present in patients that, based on the booth and ABR testing, were hearing impaired . Audiologists were quite perplexed with this finding. In the early 1990’s a group of professionals gathered to discuss this unique group of patients (my son was one of them). The term auditory neuropathy was coined by Arnold Starr, MD, a neurologist at UCI believed that the nerve was damaged. In time, the concensus was that the nerve was mis-firing, or dys-synchronously firing so this disorder has also been called Auditory Dys-synchrony.

Because AN was not a typical hearing loss it is misunderstood by many audiologists, speech language pathologists, and teachers of the deaf. Many parents of children diagnosed with AN have received conflicting information and advice about diagnosis, management, and recommendations as to which mode of communication they should use with their children. Unfortunately, nearly 20 years later parents continue to recieve conflicting information. Because of this continued conflict a panel of experts came together at the International Newborn Hearing Screening Conference (June 19-21, 2008 in Como, Italy) for the purpose of developing guidelines for identification and management of infants with what had been called Auditory Neuropathy and/or Auditory Dys-synchrony. At the Guidelines Development Conference on the Identification and Management of Infants with Auditory Neuropathy the consensus was to adopt the term “auditory neuropathy spectrum disorder” (ANSD) to describe this long debated and multi-faceted disorder.

Hearing aid use with ANSD has been heavily debated topic among professionals from the beginning . Some facilities use hearing aids with AN children with limited success. There is current research stating that hearing aids are useful if about 50% of the cases but again, this is disputed among professionals. There is professional disagreement over the use of cochlear implants in patients with ANSD, however there has been a trend to implant these children based on poor language gains. As the number of successfully implanted AN children grows, there are more and more referrals for cochlear implantation evaluations being made. Seminars for all professionals and parents of ANSD children are being held throughout the world to promote and encourage understanding of the disorder and it’s management. As of 2008, there are several children with AN that now have bilateral cochlear implants that are progressing wonderfully with them.

As parents, it becomes difficult to decide which path to follow with our children, especially with all the disagreement among professionals. We must become case managers, advocates,and educators for our children. We must educate ourselves about this disorder called Auditory Neuropathy Spectrum Disorder so that we can educate others, including many professionals that work with hearing impaired children. Many parents have had to fight to obtain the services and equipment our children need because of lack of understanding of the disorder.