Crossed Signals

Question:   A 4 year old child is brought to your office after failing a school hearing screen. His parents bring an otoacoustic emissions test from an outside institution that shows intact cochlear outer hair cell function bilaterally. You obtain a behavioral audiogram which shows intact pure tone hearing but 0% word recognition in the left ear. Auditory brainstem response testing shows abnormal responses in the left ear.  What is your leading diagnosis? [Answer will be posted with next week's new question]

Answer to last week's question, Who Gives a Shrug...?! (May 18, 2015): 

The transverse process of the C1 vertebra can be used as a palpable landmark for finding the spinal accessory nerve in the neck.