Variation in Auditory Neuropathy Spectrum Disorder: Implications for Evaluation and Management

ABSTRACT

Auditory neuropathy spectrum disorder (ANSD) has an effect, either directly or indirectly, on the neural processing of auditory stimuli. Physiological measures are needed to accurately identify and assess ANSD. Sound processing in patients with ANSD is highly variable, and relationships between hearing sensitivity and ability to process speech do not follow the typical hearing loss rules. Auditory function may show changes over time, with progression or fluctuation, or may remain stable. Considerable variation in presentation is observed across patients of all ages and includes milder forms of ANSD. Based on these observations, management should proceed with thorough assessment of individual capabilities. It is important to distinguish between detection (sensitivity) and discrimination of sound, especially in noise, when evaluating function and benefit from various forms of management. Visual information is important for most patients and cochlear implants provide significant benefit when that option is selected. As the understanding of ANSD and the inherent variation among patients advances, the knowledge and methods that we bring to our clinical practices should allow us to more accurately characterize function and implement appropriate management.

KEYWORDS

Auditory neuropathy - auditory dys-synchrony - auditory neuropathy spectrum disorder - cochlear hair cell response - auditory neural response

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