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Frequency-Specific Electrocochleography Indicates that Presynaptic and Postsynaptic Mechanisms of Auditory Neuropathy Exist

McMahon, Catherine M.1; Patuzzi, Robert B.2; Gibson, William P. R.3,4; Sanli, Halit4

doi: 10.1097/AUD.0b013e3181662c2a
Research Articles

Objectives: The physiological mechanisms underlying auditory neuropathy (AN) remain unclear and it is likely that the multiple disruptions are classified under the broadly defined term. Cochlear implantation is being more widely used in this population to bypass the suspected site-of-lesion although a number of cases have been identified within the Sydney Cochlear Implant Centre where this management strategy has been unsuccessful. It is likely that this relates to the different physiological mechanisms underlying AN.

Design: To investigate the site-of-lesion in AN, frequency-specific round window electrocochleography (ECochG) was used to assess local hair-cell, dendritic, and axonal currents generated within the cochlea in 14 subjects with AN and compared with responses from two normally hearing subjects. ECochG results were then compared with electrically evoked auditory brain stem response (EABR) measured after cochlear implantation.

Results: The results of this study demonstrate that two dominant patterns of ECochG waveforms (produced by a high-frequency alternating tone burst) can be identified in this population of AN subjects: (a) gross waveform showing a prolonged summating potential (SP) latency that, in most cases, is followed by a small compound action potential; and (b) gross waveform showing a normal latency SP waveform followed by a broad negative potential [assumed to reflect the dendritic potential (DP) identified in anaesthetized guinea-pigs]. This study demonstrates that in most subjects (n = 7) with a prolonged latency SP but no DP, normal morphology EABR waveforms were elicited for all electrode channels. On the other hand, all subjects (n = 7) who showed a normal latency SP followed by a broad negative DP, showed EABR waveforms that were absent or having poor wave V morphology. The authors' interpretation of this is that ECochG results may provide a classification of AN into pre- and postsynaptic lesions.

Conclusions: We suggest that a presynaptic and postsynaptic type of AN exist that may have implications for the fitting of cochlear implants.

Auditory neuropathy is characterized by normal cochlear mechanical function but absent or severely disrupted synchronous neural activity. Because of the broad classification system used, AN may encompass multiple sites-of-lesion including inner hair cells, the primary afferent synapse or the auditory brainstem. By comparing frequency-specific electrocochleography (ECochG) waveforms before cochlear implantation with electrically-evoked auditory brainstem responses (EABR) measured after implantation, we demonstrate here that EcochG can be used to differentially diagnose pre-synaptic and post-synaptic types of auditory neuropathy. This has potential implications for the fitting of cochlear implants in individuals with AN.

1Speech, Hearing and Language Research Centre, Department Linguistics, Macquarie University, NSW, Australia; 2Physiology, School of Biomedical and Chemical Sciences, University of Western Australia, Australia; 3Faculty of Medicine, University of Sydney, NSW, Australia; and 4Sydney Cochlear Implant Centre, NSW, Australia.

Portions of this work were presented at the XIX International Evoked Response Audiometry Study Group, Cuba 2005.

Address for correspondence: Catherine M. McMahon, PhD, C5A 517 Audiology, Department Linguistics, Macquarie University, NSW 2109, Australia. E-mail:

Received August 1, 2006; accepted September 12, 2007.

© 2008 Lippincott Williams & Wilkins, Inc.