Purpose
This article combines the results of 3 studies that were presented at the HeAL 2018 Conference in Lake Como, Italy, in June 2018. Each study involved electrocochleography (ECochG), a neurodiagnostic evaluation that has been used clinically for over 80 years but whose applications continue to expand. The 1st study describes recent research wherein ECochG was recorded from asymptomatic subjects who were siblings or offspring of patients with a confirmed diagnosis of Ménière's disease (MD). Our results provide evidence that ECochG may be helpful in not only diagnosing MD but also predicting it as well. Second, case studies are described where ECochG was important in both diagnosing superior semicircular canal dehiscence and monitoring the repair of this condition during surgery. Finally, although ECochG has been practiced clinically for over 8 decades, the protocols for recording, measuring, and interpreting the electrocochleogram continue to lack standardization among clinicians and scientists. We thus present normative data for some of these features based on noninvasive recordings made from the tympanic membrane from 100 normal hearing subjects.
Conclusions
Although the primary use of ECochG continues to be in the diagnosis of MD, we report on 2 additional clinical applications for this important test of inner ear/auditory nerve function. First, a preliminary study on a small sample of subjects indicates that ECochG may also be useful in predicting MD prior to the onset of symptoms in individuals who may be genetically predisposed to developing it. Second, through a series of case studies, we demonstrate how ECochG is used to help diagnose superior semicircular canal dehiscence and monitor the status of the inner ear during the surgical repair of this condition. Finally, normative values for clinically important components of the electrocochleogram based on tympanic membrane recordings have been established from a large sample of subjects.
A Third Labyrinthine Window: An Overview of Perilymph and Labyrinthine Fistulae and Superior Semicircular Canal Dehiscence
