Abnormal Cochlear Potentials in Friedreich's Ataxia Point to Disordered Synchrony of Auditory Nerve Fiber Activity

Neuro-degenerative Diseases
Rosamaria SantarelliPietro Scimemi

Abstract

Friedreich's ataxia (FRDA) is a degenerative disorder caused by mutations of the FXN gene. Sensorineural hearing loss is one of the clinical features of FRDA, and the majority of hearing-impaired patients have shown evidence of auditory neuropathy. This study characterizes the cochlear receptor and auditory nerve potentials in a patient with FRDA who had the clinical profile of auditory neuropathy. The aim was to investigate the site of the lesion and the pathophysiological mechanisms behind the hearing dysfunction. Using transtympanic electrocochleography, both receptor (cochlear microphonic, CM, and summating potential, SP) and auditory nerve potentials were recorded in response to trains of clicks with stimulation intensities from 60 to 120 dB SPL. The results were compared with recordings obtained from two groups of subjects, i.e. 20 normally hearing controls and 19 subjects with cochlear hearing loss. The results showed that the synchronized neural response seen in both normally hearing and hearing-impaired subjects was lacking in our patient, replaced by a prolonged, low-amplitude negative potential that decreased in both amplitude and duration for rapid stimulation rates, consistent with adaptation of neural sources. CMs...Continue Reading

Citations

Oct 16, 2015·Brain : a Journal of Neurology·Gary Rance, Arnold Starr
May 29, 2018·Arquivos de neuro-psiquiatria·Bianca Simone ZeigelboimPaulo Breno Liberalesso
Feb 19, 2021·The Cerebellum·Nehzat KoohiDoris-Eva Bamiou

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