Auditory Neuropathy/Auditory Synaptopathy

Patients with auditory neuropathy (AN)/auditory synaptopathy (AS) present unique evaluation and management challenges. Communication ability using auditory stimuli varies among patients, with particular difficulty understanding speech in noise. Auditory physiologic responses are key to accurate identification and monitoring of patients with AN/AS. Management approaches should consider individual variation and the possibility of change over time. Many patients with accurately characterized AN/AS demonstrate success with cochlear implants. Areas of discovery, including understanding of synaptic and neural mechanisms, genotype/phenotype relationships, and use of cochlear and cortical evoked potentials, will promote accurate clinical evaluation and management of infants, children, and adults with AN/AS.