Auditory steady state response in auditory neuropathy

Review: Auditory neuropathy is a disorder characterised by preservation of outer hair cell function, with normal otoacoustic emissions and/or cochlear microphonics, but an absent or distorted auditory brainstem response. Purpose: This study aimed to objectively assess hearing thresholds in patients with auditory neuropathy, using the auditory steady state response. Materials and methods: Thirteen patients with auditory neuropathy and 15 normal hearing subjects were examined. Audiological evaluation included basic audiological tests, otoacoustic emissions, auditory brainstem response and auditory steady state response. Results: In the auditory neuropathy patients, the auditory brainstem response was absent in 11 patients, while the auditory steady state response was absent in only three. Conclusion: The auditory steady state response may serve as a valuable objective measure for assessing the hearing threshold across different frequencies in patients with auditory neuropathy. We recommend that auditory steady state response be used to complete the evaluation of patients with auditory neuropathy.