By Breanna Benson

Auditory Neuropathy Spectrum Disorder (ANSD) is a form of hearing loss in which: * outer hair cells in the cochlea function normally * the auditory nerve carries unsynchronized electrical signals to the brain WHAT IS ANSD?

ANSD typically does not present itself in newborn hearing screenings. * Otoacoustic emissions (OAE) are used, and in ANSD, the OAE appear normal. * Sometimes they are identified at birth if there is another hearing loss present. * Infants with ANSD exhibit no auditory brain stem response (ABR). - This, however, is not always associated with deafness. People who have ANSD do not exhibit any middle ear muscle response during evaluations. IDENTIFICATION

There are several medical history factors that can be used to help identify ANSD. * Prematurity (25-36 weeks) * Hyperbilirubinemia (jaundice) - Not all jaundice cases indicate ANSD. * Perinatal asphyxia * Genetic (recessive and dominant genes) IDENTIFICATION

Up to 15% of children who have a permanent hearing loss have ANSD. Individuals with ANSD have difficulty recognizing speech, especially when there is background noise in the environment. Levels of hearing for people with ANSD range from normal hearing to profound hearing loss. Usually, children who have ANSD have lower speech perception than children who have similar degrees of sensorineural hearing loss. PREVALENCE AND PRESENTATION

Symptoms and side effects vary greatly by individual, depending on: * Medical history * Additional hearing loss * Therapy/treatment * Assistive listening devices The one common side effect between everyone affected by ANSD is difficulty with speech perception and sound recognition. SYMPTOMS AND SIDE EFFECTS

There are several different options for therapy for individuals with ANSD. * Visual cues * Hearing aids - Children can develop functional speech perception skills when it is quiet. * Cochlear implants - 85% success out of 260 children in a 2010 study - Electrical stimulation may help synchronize impaired portions of the brain THERAPY AND CARE

*Auditory brainstem implant trial - Children are only eligible if they also have cochlear nerve deficiency (20% of kids with ANSD). There are some implications for therapy and care of ANSD. * Some individuals never progress in speech and language development, regardless of good intervention and consistent amplification. * ANSD reversal, or normalization, can occur in some children. * Sometimes the dyssynchrony becomes worse over time. THERAPY AND CARE

ANSD is a unique disorder that is different for each individual it affects. An analogy featured on the Supporting Success for Children with Hearing Loss website says: “ You are driving between two cities a considerable distance apart. You have a talk radio station playing on the car radio. As you leave, the range of the radio station behind the signal develops static. You can still hear the speech, but it is much harder to understand. Turning up the radio volume does little to improve your understanding of the speech presented on the talk radiostation. For children with ANSD, the amount of dyssynchrony, or ‘static’ interferes with understanding especially if there is ANY background noise present.” AUDITORY NEUROPATHY SPECTRUM DISORDER

ANSD - Auditory Neuropathy/Dyssynchrony Spectrum Disorder - Success For Kids With Hearing Loss. (2016, January). Retrieved February 16, 2016, from Beck, D. L. (2013, January 3). Reflections on Auditory Neuropathy Spectrum Disorder: Retrieved February 16, 2016, from disorder Maxon, H. (2015, December 29). SPED 585 Module 2 Section 2 Inner Ear. Retrieved February 21, 2016, from v=JYPbBPLUlQwhttps:// SOURCES