Verbal auditory agnosia with focal EEG abnormality: an unusual case of a child presenting to an ENT surgeon with "deafness".

Verbal auditory agnosia implies the failure to recognize sounds in a patient who is nevertheless not deaf. We present a child referred to the ENT outpatients for suspected hearing loss investigation. At one point she had grommets inserted on the basis of a flat tympanogram but with no effect. All hearing tests, including electrocochleography and distraction tests, revealed normal hearing thresholds. At the age of three years and three months, and on direct questioning of the parents, we discovered that the child responded remarkably well to music. Paediatric neurologists made the diagnosis of verbal auditory agnosia. The condition is very similar to another acquired language disorder called Landau-Kleffner syndrome. It is unusual for such a case to present primarily to an ENT surgeon.