Auditory Neuropathy Spectrum Disorder (ANSD) Guidelines

In June 2008, a panel of experts met in Como, Italy at the NHS 2008 Conference to develop the Guidelines for the Identification and Management of Infants and Young Children with Auditory Neuropathy.

The panel consisted of Yvonne Sininger, PhD, Arnold Starr, MD, Christine Petit, MD, PhD, Gary Rance, PhD, Barbara Cone, PhD, Kai Uus, MD, PhD, Patricia Roush, AuD, Jon Shallop, PhD, and Charles Berlin, PhD.

The conference was sponsored by the Bill Daniels Center for Children’s Hearing at Children's Hospital Colorado. Deborah Hayes, PhD, Former Co-chair of the Bill Daniels Center for Children’s Hearing, moderated the conference.

Guidelines for diagnosing and managing infants and young children with auditory neuropathy

Following the conference, Dr.  Hayes and Dr. Sininger developed the guidelines based on input from the panel. The guidelines are specific to infants and young children and include recommendations for: 

• terminology
• diagnostic criteria
• audiological assessment
• comprehensive medical and developmental assessment
• amplification
• cochlear implants
• communication development
• screening newborns
• monitoring infants for transient disorder
• supporting parents

The following is a brief synopsis of the guidelines:

• Terminology: The panel recommends the use of auditory neuropathy spectrum disorder (ANSD) to describe the disorder characterized by evidence of normal cochlear outer hair cell (sensory) function and abnormal auditory nerve function.
• Diagnostic criteria: The (minimum) audiological test battery required to diagnose ANSD includes presence of normal or near-normal otoacoustic emissions (OAEs) or cochlear microphonics (CM) and absent or markedly abnormal auditory brainstem response (ABR). Specific requirements for measuring these responses in infants are described in the guidelines.
• Audiological assessment: Comprehensive audiological assessment and monitoring of infants and young children with ANSD includes measures of behavioral response to sound, middle ear function including middle ear muscle reflexes, speech reception thresholds and speech understanding in quiet and in noise (when developmentally appropriate) and OAEs. Because transient ANSD has been reported in some infants, frequent audiological monitoring is important, especially in the first two years of life.
• Comprehensive medical and developmental assessments: As with any infant with hearing loss, infants with ANSD should receive comprehensive medical and developmental assessments including genetic consultation. In addition, neurological consultation is recommended to identify other peripheral neuropathies, if any.
• Amplification: When reliable behavioral audiometry (conditioned response measures) indicates elevated thresholds for pure tones and speech, hearing aids should be considered for young children with this diagnosis. Hearing aids should be fit using standard pediatric fitting guidelines.
• Cochlear implants: Children with ANSD who do not make progress in language development should be considered for cochlear implantation regardless of behavioral pure-tone thresholds.
• Communication development: As with any child with hearing loss, parents should receive unbiased information about all communication development options and methodologies.
• Screening newborns for ANSD: Infants who receive newborn intensive care (for five days or more) should receive hearing screening by ABR not OAE. Any infant who fails newborn hearing screening by ABR should not be re-screened and passed by OAEs.
• Monitoring infants with transient ANSD: Infants who “recover” from ANSD should receive audiological and speech and language developmental assessments periodically during the first three years of life. Any infant who demonstrates developmental delay in language acquisition should receive speech and language intervention.
• Supporting parents: Because the developmental trajectory of each child with ANSD is unique, parents are faced with uncertainty regarding their child’s developmental potential. Families should receive ongoing guidance from professionals and from other parents of infants with this diagnosis to receive accurate information and emotional support.

• ANSD Age-Appropriate Auditory Response (.pdf)
• ANSD No Auditory Response (Deaf) (.pdf)
• ANSD Variable Response (.pdf)

How to request a copy of the ANSD Guidelines

A complete monograph of the publication, Identification and Management of Infants and Young Children with Auditory Neuropathy Spectrum Disorder (ANSD), is now available. You may download a complete copy of the guidelines (.pdf).

Please reference the appropriate citation when referencing any portion of this monograph publication; Bill Daniels Center for Children’s Hearing. Guidelines for Identification and Management of Infants and Young Children with Auditory Neuropathy Spectrum Disorder. Children’s Hospital Colorado, 2008. Print.

Please direct all questions and comments to the Bill Daniels Center for Children’s Hearing at BillDanielsCenter@childrenscolorado.org or by phone at 720-777-6531.

The ANSD guidelines development conference, monograph publication and a series of national and international conferences to disseminate the guidelines were supported by the generous financial contributions from the Bill Daniels Center for Children’s Hearing and the Kelley Family/Schlessman Family Scottish Rite Masons Chair in Childhood Language Disorders at Children's Hospital Colorado.