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hidden hearing loss: Causes, Current Knowledge, and Future Directions

International Journal of Otolaryngology and Head & Neck Surgery 2023年第3期12卷 107-123页

作者： Alexandra Jalkh Department of Otolaryngology Eye and Ear Hospital Naccach-Dbayeh Lebanon Department of Audiology Escula de Formacio Superior SAERA Castellon de la Plana Spain

Introduction: hidden hearing loss (HHL) is a type of auditory disorder that affects the auditory neural processing and hearing sensitivity in subjects with normal hearing thresholds. Unlike central auditory processing disorders, HHL happens when the cochlea (the peripheral auditory organ) is affected. There are several known risk factors to HHL which includes noise exposure, ototoxic drugs, and peripheral neuropathies, and age. Recent studies have shed light on this type of hearing loss, its etiology, prevalence, and how it can affect the auditory acuity in humans. Methods: This paper covers the current research regarding HHL, its causes, the different mechanisms involved in this disorder, and the diagnosis and potential treatments related to it. We will delve deeply into different researches concerning HHL. 4 articles from 285 were selected focusing on normal hearing individuals with bad speech intelligibility were discussed in this paper. In addition, articles discussing the effects of noise exposure on hearing impaired individuals were not considered as this study solely aims to focus on normal hearing sensitivity individuals with HHL, resulting in 4 articles from 285. Results: Numerous literatures over the decades have suggested that HHL is due to the degeneration of cochlear ribbon synapses, or hair cells synapses without hair cell damage. Their association with HHL was noted several times through this study, whether we were studying the effect of noise exposure, of age, or of ototoxicity. In all cases, no significant hair cell damage was observed, and normal thresholds were recovered. However, a decline in the amplitude of Auditory Brainstem Response (ABR) peak I from auditory nerve (AN) responses in noise exposed subjects and a decline in compound action potential (CAP) was measured when certain drugs were applied to the round window of Guinea pigs. Conclusion: Most studies, have proven that cochlear synaptophysin is the major contributor to noise induced, ag

关键词： hidden hearing loss Speech Testing Speech Intelligibility Noise Induced hearing loss Ototoxicity Schwan’s Cells Ablation

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hidden hearing loss:current perspectives and potential therapies

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Journal of Bio-X Research 2019年第2期2卷 62-67页

作者： Diyan Chen Gaogan Jia Yusu Ni Yan Chen ENT Institute and Otorhinolaryngology Department Affiliated Eye and ENT Hospital of Fudan University Key Laboratory of hearing Medicine of National Health and Family Planning Commission ShanghaiChina

hidden hearing loss(HHL),an auditory dysfunction that has gained much recent attention,has the hallmarks of speech discrimination and intelligibility deficits with normal or near-normal hearing *** pathological mechanisms of HHL are complicated and are not yet fully *** can be resulted from disorders of the central nervous system such as auditory cortex,and/or pathological changes of inner *** far,2 pathological phenomena,synaptopathy and auditory nerve demyelination,have been reported as underlying causes of otogenic ***,we review the clinical and physiological characteristics of HHL as well as the molecular pathological mechanisms of otogenic HHL and aim to allude to potential therapy targets for clinical applications in the future.

关键词： auditory cochlear hair cells demyelination hearing loss hidden hearing loss Schwann cells spiral ganglion neurons synapse synaptopathy

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Cochlear Synaptopathy Following Noise Exposure in Guinea Pigs: Its Electrophysiological and Histological Assessments

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Sound & Vibration 2020年第3期54卷 163-177页

作者： Parvane Mahdi Akram Pourbakht Vahid Pirhajati Mahabadi Alireza Karimi Yazdi Mahtab Rabbani Anari Mohammad Kamali Rehabilitation Research Center Department of AudiologySchool of Rehabilitation SciencesIran University of Medical SciencesTehran15459-13487Iran Department of Neurosciences School of MedicineIran University of Medical SciencesTehran14496-14535Iran Department of Otorhinolaryngology Tehran University of Medical SciencesTehran14197-33141Iran Department of Rehabilitation Management Iran University of Medical SciencesTehran15459-13487Iran

Exposure to high level of noise,may cause the permanent cochlear synaptic *** present study,a model of noise induced cochlear synaptopathy was established and the electrophysiological and histological metrics for its assessment was designed.6 guinea pigs were subjected to a synaptopathic noise(octave band of 4 kHz at 104 dB SPL,for 2-h).The amplitude growth curve of Auditory Brainstem Response(ABR)wave-I and wave-III latency shift in presence of noise were *** indexes were considered in pre-exposure,1 day post exposure(1DPE),1 week post exposure(1WPE)and 1 month post exposure(1MPE)to ***,the samples were histologically *** wave-I amplitude was different between pre and 1DPE(p-value≤0.05).However,at 1WPE,it was recovered at low intensities but at 70 dB SPL and above,the differences persisted even till *** masked ABR,the latency shift of wave-III was different between pre and 3 post exposure assessments(p-value≤0.05).Electro-microscopic analysis confirmed the synaptic degeneration,as the ribbons were larger than normal,hollow inside,and spherical and irregular in shape,also,the post synaptic density was abnormally thick and missed its flat *** data revealed that noise at level below that can produce permanent hearing loss,can incur synaptic ***,noise is considered to be more damaging than previously ***,designing tools for clinical assessment of synaptopathy is beneficial in comprehensive auditory evaluation of those with history of noise exposure and also in hearing protection planning.

关键词： Cochlear synaptopathy noise induced hearing loss hidden hearing loss excitotoxicity hearing protection

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hidden cochlear impairments

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Journal of Otology 2018年第2期13卷 37-43页

作者： Guang-Di Chen Center for hearing and Deafness State University of New York at Buffalo

Pure tone audiometry is a routine clinical examination used to identify hearing loss. A normal pure tone audiogram is usually taken as evidence of normal hearing. Auditory deficits detected in subjects with normal audiograms, such as poor sound discrimination and auditory perceptual disorders, are generally attributed to central problems. Does the pure tone audiogram truly reflect cochlear status？ Recent evidence suggests that individuals with normal audiogram may still have reduced peripheral auditory responses but normal central responses, indicating that the pure tone audiometry may not detect some types of cochlear injuries. In the cochlea, the outer hair cells （OHCs）, inner hair cells （IHCs）, and the spiral ganglion neurons that synapse with IHCs are the 3 key cochlear components in transducing acoustical vibrations into the neural signals. This report reviews three types of cochlear damage identified in laboratory animals that may not lead to overt hearing loss. The first type of cochlear impairment, such as missing a certain proportion of IHCs without damage to OHCs, may reduce the cochlear output and elevate response threshold; however, the reduced peripheral auditory sensitivity may be restored along the auditory pathway via central gain enhancement. The second type of cochlear impairment, such as selective damage to the synapses of the high-threshold thin auditory nerve fibers （ANFs）, reduces cochlear output at high stimulation levels with no effect on response threshold. In this case the reduced cochlear output may be compensated along the auditory pathway as well. The third type of cochlear impairment, such as missing a certain number of OHCs without damage to others, may not even affect cochlear function at all. These ＂hidden＂ cochlear impairments do not result in overt hearing loss, but they may increase the vulnerability of the cochlea to traumatic exposure and lead to disrupted central auditory processing.

关键词： hidden hearing loss OHC damage IHC damage Synaptopathy

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