Auditory neuropathy is a hearing disorder in which sound enters into the inner ear normally but the transmission of signals from the inner ear to auditory cortex is impaired due to the poor brain development. We report a case of 7 year male child presented with hard of hearing, deformed both external ears and inability to speak since birth. On examination bilateral bat ears, accessory auricles, hypertelorism, hyperactive attention deficit and from auditory brainstem response and otoacoustic emission reports it is diagnosed as auditory neuropathy and associated with various forms which is not included in ENT syndromes till date.