Head trauma as eliciting event in transient deterioration of sensorineural hearing loss and vertigo in Pendred/EVA syndrome

Summary 

A patient with Pendred/enlarge vestibular aqueduct (EVA) syndrome with a 26-year audiometric follow-up is presented, showing childhood onset of sensorineural hearing loss, characterized by several episodes of well-documented sudden transient deterioration in sensorineural hearing loss and vertigo elicited by head trauma. This report is unique in its detailed follow-up of hearing loss around the time of head trauma events, providing new evidence to show a causative relation between head trauma and sudden hearing loss in EVA/Pendred syndrome.

Keywords: Pendred syndrome, SLC26A4 gene, Enlarged vestibular aqueduct (EVA), Head trauma, Sensorineural hearing loss, Vertigo