Auditory neuropathies in infants

Abstract Auditory neuropathy is a sensorineural disorder characterized by absent or abnormal auditory evoked potentials, and normal cochlear outer hair cell function. It is believed that a variety of processes are involved in its pathophysiology and their influence on hearing can differ. We describe the diagnostic sequence and management of two new cases of auditory neuropathy in infants. The first case was a girl with no risk factors for hearing loss. Her absence of response to sounds was compared with her twin's reactions. Otoacoustic emissions were present but no auditory evoked potential response was detected. Her evolution was characteristic of deep sensorineural hearing loss without a hearing aid. At present she is awaiting a cochlear implant. The second case was a boy who underwent hearing loss screening for hyperbilirubinemia. Examinations were repeated three months later because the mother suspected hearing loss. Objective tests showed a pattern of auditory neuropathy. The boy's evolution was different: in spite of the presence of destructured auditory evoked potentials; it was evident that the boy conserved some hearing and had thresholds suggestive of moderate hearing loss.