Auditory neuropathies and electrocochleography

Abstract

Auditory neuropathy (AN) is a hearing disorder characterized by disruption of temporal coding of acoustic signals in auditory nerve fibres resulting in impairment of auditory perceptions relying on temporal cues (). Mechanisms suggested include both pre-synaptic and post-synaptic disorders affecting inner hair cell (IHC) depolarization, neurotransmitter release from ribbon synapses, spike initiation in auditory nerve terminals, loss of nerve fibres and impaired conduction, all occurring in the presence of normal physiological measures of outer hair cell (OHC) activities (otoacoustic emissions (OAEs) and cochlear microphonic (CM)). Disordered synchrony of auditory nerve discharge has been suggested as the basis of both the profound alterations of auditory brainstem responses (ABRs) and impairment of speech perception (). We review how electrocochleography (ECochG) can provide detailed information to help define objectively the sites of auditory neural dysfunction as affecting IHC receptor summating potential (SP) and compound action potential (CAP), the latter reflecting disorders of ribbon synapsis and auditory nerve fibres.

Key words::

• cochlear potentials
• ribbon synapses
• inner hair cells
• auditory nerve fibers

Acknowledgements

The authors are deeply indebted to Pietro Scimemi and Roberta Rossi for their support in data analysis, and Fabio Saccomandi for technical assistance. The authors also thank the anaesthesiologists who performed general anaesthesia, and the nurses involved in child care.

Declaration of interest: The authors report no conflicts of interest. The authors alone are responsible for the content and writing of the paper.