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J Audiol Otol > Epub ahead of print
DOI: https://doi.org/10.7874/jao.2022.00192    [Epub ahead of print] Published online November 24, 2022.
Tone-Burst Auditory Brainstem Response and Cortical Potentials in Diagnosis of Syndromic Auditory Neuropathy Spectrum Disorder
Wafaa A. Kaf1  , Samantha Reiter1,2  , Amanda Brodeur3  , Letitia White-Minnis1  , William Deal4 
1Department of Communication Sciences and Disorders, Missouri State University, Springfield, MO, USA
2St. Cloud Veterans Affairs Health Care System, St. Cloud, MN, USA
3Department of Biomedical Sciences, Missouri State University, Springfield, MO, USA
4Department of Psychology, Missouri State University, Springfield, MO, USA
Correspondence  Wafaa A. Kaf ,Tel: +1-417-836-4456, Fax: +1-417-836-4242, Email: wafaakaf@missouristate.edu
Submitted: April 21, 2022  Accepted after revision: September 19, 2022
Abstract
In this study, we report our findings of comprehensive evaluation in a man with syndromic craniofacial features, cognitive impairment, and hearing loss. The patient underwent psychological and genetic testing and screening for 133 genetic mutations associated with hearing loss, as well as extensive audiological evaluation to assess the auditory pathway between the middle ear and the auditory cortex. Psychological testing showed moderate cognitive impairment. Genetic testing did not reveal a genetic mutation for hearing loss. Audiological evaluation revealed mixed hearing loss and signs of auditory neuropathy spectrum disorder (ANSD) despite absence of otoacoustic emissions and an absent click-evoked auditory brainstem response (ABR) without recording of cochlear microphonics (CM). ANSD was characterized by abnormal speech discrimination, bilateral robust CM to 2,000 Hz tone-burst (TB) ABR, and abnormal left thalamocortical and cortical pathways diagnosed based on auditory middle latency and cortical N1-P2 responses. These behavioral and electrophysiological findings suggest post-synaptic ANSD at the brainstem level. An abnormal left thalamocortical auditory pathway may be attributable to the combined effect of lack of neural synchrony secondary to ANSD mainly on the left and/or brain injury. The findings in this study support the use of TB ABR and auditory cortical potentials in the ANSD test protocol and in patients with craniofacial anomalies.
Keywords: Syndromic hearing loss; Tone-burst ABR; Cortical potentials; Genetic screening


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