Panel presentation of Current Approach to Auditory Neuropathy Spectrum Disease

Background: Approximately 10% of children born with a permanent hearing loss have auditory neuropathy. Implementation of universal newborn hearing screening has led to early detection and intervention. An increased understanding of the long-term achievements of children with auditory neuropathy is essential to developing a comprehensive account of expected outcomes of children for improving counselling and clinical management.

Objective. This study examined language and speech outcomes in a population-based cohort of 9-year-old children with hearing loss and auditory neuropathy, and investigated factors influencing outcomes.

Method. Receptive and expressive language skills, speech output accuracy, and a diverse set of cognitive, demographic and audiological, variables (including age at device fitting) were evaluated at 3-, 5- and 9-years of age using direct assessment and caregiver report. Multiple regression analyses were used to address two questions: 1) Do language and speech outcomes at earlier ages predict language and speech outcomes at 9 years of age? 2) Which cognitive, demographic and audiological variables measured at 9 years of age predict concurrent language and speech outcomes after controlling for early language and speech outcomes?

Result and Discussion. The influence of cognitive, demographic and audiological variables, including auditory neuropathy, on 9-year outcomes was quantified. The implications of results on clinical best-practice management of children with hearing loss and auditory neuropathy will be discussed.