Recommended Procedure: Assessment and Management of Auditory Neuropathy Spectrum Disorder (ANSD) in Young Infants
British Society of Audiology Professional Guidance Group. (2019).
Bathgate (UK): British Society of Audiology, Retrieved September 26, 2019 from https://www.thebsa.org.uk/.
This guideline revises and updates the 2013 Newborn Hearing Screening Programme (NHSP) in England document "Newborn Hearing Screening and Assessment: Guidelines for the Assessment and Management of Auditory Neuropathy Spectrum Disorder in Young Infants." This current British Society of Audiology (BSA) guidance provides recommendations about the identification, assessment, diagnosis, and management of infants suspected of having auditory neuropathy spectrum disorder. The following summary highlights broad areas to consider. For detailed step-by-step directions for testing order and interpretation, see the BSA's full text document.
British Society of Audiology
Per BSA recommendation, this document on the assessment and management of auditory neuropathy spectrum disorder should be read in conjunction with the BSA "Recommended Procedure: Cochlear Microphonic Testing;" see Associated Article below.
Children with a stable and permanent auditory neuropathy spectrum disorder diagnosis resulting in severe-to-profound hearing loss and who receive no benefit from hearing aids should be considered for cochlear implantation.
Children with auditory neuropathy spectrum disorder who have residual speech recognition in quiet should be considered for a hearing assistive technology trial.
For children with auditory neuropathy spectrum disorder, language and communication development should be assessed and monitored by a speech-language pathologist and/or deaf educator.
For children with auditory neuropathy spectrum disorder, "ongoing and regular monitoring of auditory status (behavioural, functional, electrophysiological and middle ear) and hearing, speech, language and general development is required" (p. 18).
Children with auditory neuropathy spectrum disorder with reliably elevated behavioral hearing thresholds should be considered for a hearing aid trial.
For children with auditory neuropathy spectrum disorder, mode of communication (e.g., sign language, auditory-oral, total communication) should be decided by the needs of the family and the progress of the child.
<p>Children with auditory neuropathy spectrum disorder (ANSD) should be managed by a multidisciplinary team with high levels of experience and expertise in ANSD. The team should include:</p>
<ul>
<li>a pediatric audiologist;</li>
<li>appropriate medical professionals (e.g., otolaryngologist, neurologist);</li>
<li>a speech-language pathologist; and </li>
<li>a teacher of the deaf.</li>
</ul>
<p>In some cases, children should be referred to a center specializing in ANSD.</p>
<p>When assessing infants suspected of having auditory neuropathy spectrum disorder (ANSD), core assessment should include:</p>
<ul>
<li>auditory brainstem response (ABR) testing; </li>
<li>transient evoked otoacoustic emissions (TEOAE); </li>
<li>cochlear microphonics (CM); </li>
<li>tympanometry; </li>
<li>bone conduction assessment; and </li>
<li>stapedial reflex (SR) testing.</li>
</ul>
<p>Testing for ANSD should begin with ABR assessment. If the infant’s ABR is absent, CM and/or otoacoustic emissions (OAE) should be conducted. See the British Society of Audiology full text document for detailed description of testing order and interpretation (e.g., frequencies to use, scenarios for when OAE or CM are most appropriate).</p>
To distinguish between long-term auditory neuropathy spectrum disorder (ANSD) and maturational, or transient, ANSD, infants should repeat auditory brainstem response (ABR) testing approximately 2 months after initial ABR assessment. Additional ABR testing may be repeated at a later age to confirm long-term ANSD diagnosis.
Parents and caregivers of children with auditory neuropathy spectrum disorder should receive "ongoing communication, support, encouragement, and information … critical to successful management" (p. 17). Families may benefit from support groups, consultation, and counseling.
"Children with transient [auditory neuropathy spectrum disorder] ANSD should be monitored for their communication development at least until they reach school age" (p. 24). If there are no communication concerns, children should still be monitored annually by an audiologist.
Children demonstrating unilateral auditory neuropathy spectrum disorder should be managed with caution and referred for additional testing (e.g., MRI imaging).
