Auditory neuropathy/dyssynchrony (AN/AD) is a relatively, recently described diagnostic term. It describes a disorder characterised by detectable otoacoustic emissions and/or cochlear microphonics, combined with abnormal or absent auditory brainstem responses and deficient speech understanding. Consequently, the patient's cognitive responses are inconsistent with a behavioural tone audiogram. This review describes the epidemiology, pathogenesis, diagnosis, and treatment of AN/AD. We have focused on the diagnostic pathway, because diagnosis is sometimes difficult, and recent research has provided important insights into the aetiopathology of the disease. The diagnostic tools for AN/AD currently used in our department will be demonstrated in a clinical case. It is very important to recognise AN/AD and provide patient counselling and support as early as possible, due to the potentially major influence of this disorder on a patient's social life and language acquisition.