Auditory agnosia progressing to cortical deafness. Long-term follow up of a one-year-old child to age 37.

Abstract

Background: There are two types of central auditory disorders due to pathology of the bilateral auditory cortices in adult patients: auditory agnosia with residual hearing; cortical deafness with total hearing loss. However, long-term changes of hearing acuity over physical development time are unknown.

Objective: The aim of this case report was to illustrate the pathophysiology of auditory changes in a 1 year-3 months old child who was diagnosed with auditory agnosia as a sequel of herpes encephalitis and later developed cortical deafness during a 36-year follow-up.

Materials and methods: Brain MRI, hearing tests, ABR, DPOAE and developmental tests of speech/language were periodically performed.

Results: Brain imaging revealed extensive damage in the bilateral auditory cortices. His auditory speech perception was lost and his language development was seriously delayed. His hearing threshold progressively decreased over time from normal at first to profound hearing loss as a teenager. However, at 29 years of age his ABR was normal. He was able to communicate and converse by sign language or writing.

Conclusions and significance: Later developing cortical deafness with profound hearing loss could be caused by retrograde degeneration of the auditory radiation overtime to the bilateral medial geniculate bodies.