Assessment of cochlear synaptopathy by electrocochleography to low frequencies in a preclinical model and human subjects.

Cochlear synaptopathy is the loss of synapses between the inner hair cells and the auditory nerve despite survival of sensory hair cells. The findings of extensive cochlear synaptopathy in animals after moderate noise exposures challenged the long-held view that hair cells are the cochlear elements most sensitive to insults that lead to hearing loss. However, cochlear synaptopathy has been difficult to identify in humans. We applied novel algorithms to determine hair cell and neural contributions to electrocochleographic (ECochG) recordings from the round window of animal and human subjects. Gerbils with normal hearing provided training and test sets for a deep learning algorithm to detect the presence of neural responses to low frequency sounds, and an analytic model was used to quantify the proportion of neural and hair cell contributions to the ECochG response. The capacity to detect cochlear synaptopathy was validated in normal hearing and noise-exposed animals by using neurotoxins to reduce or eliminate the neural contributions. When the analytical methods were applied to human surgical subjects with access to the round window, the neural contribution resembled the partial cochlear synaptopathy present after neurotoxin application in animals. This result demonstrates the presence of viable hair cells not connected to auditory nerve fibers in human subjects with substantial hearing loss and indicates that efforts to regenerate nerve fibers may find a ready cochlear substrate for innervation and resumption of function.

Low-Frequency Hearing Preservation With Long Electrode Arrays: Inclusion of Unaided Hearing Threshold Assessment in the Postoperative Test Battery.

Purpose The goal of this work was to evaluate the low-frequency hearing preservation of long electrode array cochlear implant (CI) recipients. Method Twenty-five participants presented with an unaided hearing threshold of ≤ 80 dB HL at 125 Hz pre-operatively in the ear to be implanted. Participants were implanted with a long (31.5-mm) electrode array. The unaided hearing threshold at 125 Hz was compared between the preoperative and postoperative intervals (i.e., initial CI activation, and 1, 3, 6, 9, and 12 months after activation). Results Eight participants maintained an unaided hearing threshold of ≤ 80 dB HL at 125 Hz postoperatively. The majority (n = 5) demonstrated aidable low-frequency hearing at initial activation, whereas 3 other participants experienced an improvement in unaided low-frequency hearing thresholds at subsequent intervals. Conclusions CI recipients can retain residual hearing sensitivity with fully inserted long electrode arrays, and low-frequency hearing thresholds may improve during the postoperative period. Therefore, unaided hearing thresholds obtained within the initial weeks after surgery may not reflect later hearing sensitivity. Routine measurement of postoperative unaided hearing thresholds-even for patients who did not demonstrate aidable hearing thresholds initially after cochlear implantation-will identify CI recipients who may benefit from electric-acoustic stimulation. Supplemental Material https://doi.org/10.23641/asha.11356637.