Ten children who were diagnosed with auditory neuropathy were matched with ten children who were diagnosed with other etiologies. All twenty children received cochlear implants at the Mayo Clinic in Rochester, Minnesota. Various measures were used to compare the outcomes for the two groups. We compared the children's unaided and aided audiograms, and measures of threshold and comfort levels. Pe...

UNLABELLED The brainstem auditory evoked potential (BAEP) is being extensively used as a method for the evaluation of cochlear function in individuals with diagnosis of auditory neuropathy/auditory dyssynchrony (AN/AD). In the absence of otoacoustic emissions, many cases of AN/AD have been diagnosed by the presence of CM identified in the BAEP. AIM to demonstrate the clinical applicability of...

UNLABELLED The electrical stimulation generated by the Cochlear Implant (CI) may improve the neural synchrony and hence contribute to the development of auditory skills in patients with Auditory Neuropathy/Auditory Dyssynchrony (AN/AD). AIM Prospective cohort cross-sectional study to evaluate the auditory performance and the characteristics of the electrically evoked compound action potential...

Zeng, Fan-Gang, Ying-Yee Kong, Henry J. Michalewski, and Arnold Starr. Perceptual consequences of disrupted auditory nerve activity. J Neurophysiol 93: 3050–3063, 2005. First published December 22, 2004; doi:10.1152/jn.00985.2004. Perceptual consequences of disrupted auditory nerve activity were systematically studied in 21 subjects who had been clinically diagnosed with auditory neuropathy (AN...

Auditory neuropathy spectrum disorder (ANSD) is a specific form of hearing loss characterized by the presence of normal or near-normal Otoacoustic Emissions (OAEs) but the absence of normal middle ear muscle reflex and severely abnormal or completely absent auditory brainstem response (ABRs). Amplification is considered a dilemma in such disorder with a lot of debates. This article will review ...

A child was referred for an audiologic evaluation, to include auditory brainstem evoked response testing, due to inconsistent responses to sound and delayed speech and language development. Results were characteristic of auditory neuropathy. In view of subsequent decline in motor function, a genetics evaluation was conducted, revealing a mitochondrial disorder. A brief overview of mitochondrial...