Guidelines for Aetiological Investigation into Auditory Neuropathy Spectrum Disorder in Children and Young Adults
British Association of Audiovestibular Physicians. (2018).
United Kingdom: British Association of Audiovestibular Physicians, 1-23.
This is a guideline from the British Association of Audiovestibular Physicians providing good practice guidance on assessing auditory neuropathy spectrum disorder (ANSD) for health practitioners involved in the management of children and adolescents with ANSD in one or both ears.
British Association of Audiovestibular Physicians
During the first visit when auditory neuropathy spectrum disorder (ANSD) is confirmed, "all children with ANSD should have a clinical vestibular examination as soon as possible after identification. This should be done within the first few weeks of life when ANSD is identified early. Consider further vestibular investigations if ANSD is permanent especially if imbalance or delayed motor milestones are part of the picture or if cochlear implant is considered" (Grade D Recommendation; p. 11).
When investigating the etiology of auditory neuropathy spectrum disorder (ANSD) in children and young adults, obtain a detailed case history at the first visit when ANSD is confirmed. The case history should include:
<ul>
<li>description of ANSD including onset, duration, and progression of symptoms;</li>
<li>antenatal, birth, and postnatal history;</li>
<li>developmental milestones;</li>
<li>family history; and</li>
<li>medical history (Grade C Recommendation).</li>
</ul>
"Following the identification of [auditory neuropathy spectrum disorder] ANSD after the newborn hearing screen: all children identified as having the findings of ANSD on initial [auditory brainstem response] ABR testing, which usually occurs at about 4 weeks corrected age, should be seen for aetiological investigation as soon as possible" (Ungraded Recommendation; p. 4).
For children and young adults diagnosed with auditory neuropathy spectrum disorder, referrals to a clinical geneticist or for a comprehensive pediatric neurological evaluation should be considered (Ungraded Recommendation).
