Hidden Hearing Loss: A Disorder with Multiple Etiologies and Mechanisms

David C Kohrman, Guoqiang Wan, Luis Cassinotti, Gabriel Corfas, David C Kohrman, Guoqiang Wan, Luis Cassinotti, Gabriel Corfas

Abstract

Hidden hearing loss (HHL), a recently described auditory disorder, has been proposed to affect auditory neural processing and hearing acuity in subjects with normal audiometric thresholds, particularly in noisy environments. In contrast to central auditory processing disorders, HHL is caused by defects in the cochlea, the peripheral auditory organ. Noise exposure, aging, ototoxic drugs, and peripheral neuropathies are some of the known risk factors for HHL. Our knowledge of the causes and mechanisms of HHL are based primarily on animal models. However, recent clinical studies have also shed light on the etiology and prevalence of this cochlear disorder and how it may affect auditory perception in humans. Here, we review the current knowledge regarding the causes and cellular mechanisms of HHL, summarize information on available noninvasive tests for differential diagnosis, and discuss potential therapeutic approaches for treatment of HHL.

Copyright © 2020 Cold Spring Harbor Laboratory Press; all rights reserved.

Figures

Figure 1.
Figure 1.
Hidden hearing loss (HHL) caused by cochlear synaptopathy. (A) Auditory brainstem response (ABR) recordings of control mice and mice 2 weeks after noise exposure (8–16 kHz, 100 dB sound pressure level [SPL], 2 hours). Noise exposure causes reduction of ABR peak I (P1) amplitudes without changes in threshold and latency. (B) The same HHL-causing noise exposure used in A results in inner hair cell (IHC) ribbon synapse loss in the base of the cochlea (e.g., 32 kHz region of the cochlea) but does not affect node of Ranvier structures. (C) Model for HHL caused by cochlear synaptopathy. Noise exposure or aging result in synaptic degeneration of low spontaneous rate (SR) AN fibers, which over time progresses to spiral ganglion neuron (SGN) loss. (Panels A and B modified from Wan and Corfas 2017; courtesy of Creative Commons Attribution 4.0 International License.)
Figure 2.
Figure 2.
Hidden hearing loss (HHL) caused by cochlear demyelination. (A) Auditory brainstem response (ABR) recordings from control mice and mice 4 months after transient demyelination caused by Schwann cell ablation. Mice present with clear signs of HHL, that is, ABR P1 amplitude reduction, latency increase, but no threshold elevation. (B) Demyelination causes persistent heminode pathology without loss of ribbon synapses. (C) Model for HHL caused by transient demyelination. After auditory nerve (AN) demyelination, remyelination takes place but the heminode structures do not recover. (Panels A and B modified from Wan and Corfas 2017; courtesy of Creative Commons Attribution 4.0 International License.)
Figure 3.
Figure 3.
Noninvasive diagnosis of hidden hearing loss (HHL) in rodents and humans. Auditory brainstem response (ABR), envelope following response (EFR), and middle ear muscle reflex (MEMR) recordings are the primary assays for HHL diagnosis. Alterations in the pattern of ABR traces can be used for differential diagnosis of HHL, including peak I amplitude, summating potential (SP)/ABR peak I (AP) ratio, peak V latency in masking noise, threshold in noise, and slope of the sound intensity to peak I amplitude relationship.
Figure 4.
Figure 4.
Neurotrophin 3 (NT-3) as a potential therapeutic for treatment of hidden hearing loss (HHL). (A) NT-3 transgenic overexpression in adult mouse cochlea promotes recovery of auditory brainstem response (ABR) peak 1 amplitudes after an HHL-inducing noise exposure (8–16 kHz, 100 dB, 2 hours). (B) NT-3 transgenic overexpression promotes synaptic regeneration after noise exposure. (C) Examples of synaptic immunostainings showing NT-3 transgene or protein promotes synaptic regeneration in both mouse and guinea pig exposed to noise. RW, Round window; inner hair cell (IHC) nucleus, dashed oval; glutamate receptors, black arrows; CtBP2-containing ribbons, white arrows. (Figure panels created from data in Wan et al. 2014, Sly et al. 2016, and Suzuki et al. 2016.)
https://www.ncbi.nlm.nih.gov/pmc/articles/instance/6938658/bin/cshperspectmed-COC-035493_UF1.jpg

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