It Is Not Just the Eyes - a Description of Abnormal Vestibulocochlear Anatomy in Duane’s Retraction Syndrome (DRS)

This case study presents a 2-month old male with Duane’s type I syndrome and bilateral severe to profound sensorineural hearing loss. The patient was referred to audiology after failing newborn hearing screen. Audiology testing revealed elevated thresholds, particularly in the left ear. Subsequent imaging demonstrated abnormal cochlear and vestibular structures, indicating an incomplete partition type I. Genetic screening for common deafness-related genes, an electrocardiogram, and infection screening for cytomegalovirus were all negative. The patient was subsequently diagnosed with left Duane’s syndrome type I at 11 months. He had a normal perinatal period and birth, with the exception of a hematoma detected around 8 weeks into gestation, which resolved without further complications. The mother received a Coronavirus-disease (COVID) vaccine during her first trimester. Family history is unremarkable for auditory or ophthalmological conditions. Duane’s retraction syndrome (DRS) is a rare form of strabismus. There is limited literature on its association with hearing loss and accompanying auditory abnormalities. We hope that this can gain further insight and provide clues to the pathogenesis and aberrant embryogenesis in this condition. Auditory screening is also important especially in settings where newborn hearing evaluation is not routine.