Late-Onset Auditory Neuropathy Spectrum Disorder in a 13-Year-Old: A Possible Association with Neonatal Jaundice.

Abstract

This report highlights the case of a 13-year-old female diagnosed with late-onset auditory neuropathy spectrum disorder (ANSD). While newborn hearing screening programs are effective in early identification of hearing impairments, this case underscores the potential for late-onset ANSD that may be overlooked in such screenings. The case provides insights into possible pathophysiological mechanisms and emphasizes the need for continued monitoring of auditory health beyond the neonatal period. The child was born with neonatal jaundice and underwent phototherapy in the neonatal intensive care unit (NICU) for 10 days. Despite this medical history, she passed the newborn hearing screening and exhibited typical developmental milestones in auditory, speech, language, and motor domains during early childhood. At the age of 13, following a four-day episode of high fever caused by a foodborne infection, the child began experiencing difficulty hearing and understanding speech, particularly in noisy environments. Comprehensive audiological evaluation confirmed a diagnosis of auditory neuropathy spectrum disorder, suggesting a late-onset manifestation of the condition. The history of neonatal jaundice treated with phototherapy may have predisposed the child to auditory vulnerability, although the condition did not manifest during the early developmental years. The triggering role of the episode of fever suggests a potential interaction between predisposing factors and subsequent environmental or medical events. This case suggests a possible association between neonatal jaundice and late-onset ANSD, but other contributing factors cannot be ruled out. It highlights the need to address potential risk factors during counselling in newborn hearing screening programs and emphasizes the importance of periodic audiologic assessments in individuals with such risk factors.