-
Nature Reviews. Neurology Mar 2016Sensorineural hearing impairment is the most common form of hearing loss, and encompasses pathologies of the cochlea and the auditory nerve. Hearing impairment caused by... (Review)
Review
Sensorineural hearing impairment is the most common form of hearing loss, and encompasses pathologies of the cochlea and the auditory nerve. Hearing impairment caused by abnormal neural encoding of sound stimuli despite preservation of sensory transduction and amplification by outer hair cells is known as 'auditory neuropathy'. This term was originally coined for a specific type of hearing impairment affecting speech comprehension beyond changes in audibility: patients with this condition report that they "can hear but cannot understand". This type of hearing impairment can be caused by damage to the sensory inner hair cells (IHCs), IHC ribbon synapses or spiral ganglion neurons. Human genetic and physiological studies, as well as research on animal models, have recently shown that disrupted IHC ribbon synapse function--resulting from genetic alterations that affect presynaptic glutamate loading of synaptic vesicles, Ca(2+) influx, or synaptic vesicle exocytosis--leads to hearing impairment termed 'auditory synaptopathy'. Moreover, animal studies have demonstrated that sound overexposure causes excitotoxic loss of IHC ribbon synapses. This mechanism probably contributes to hearing disorders caused by noise exposure or age-related hearing loss. This Review provides an update on recently elucidated sensory, synaptic and neural mechanisms of hearing impairment, their corresponding clinical findings, and discusses current rehabilitation strategies as well as future therapies.
Topics: Animals; Auditory Pathways; Cochlear Nerve; Hair Cells, Auditory, Inner; Hearing Loss; Hearing Loss, Central; Humans; Synapses
PubMed: 26891769
DOI: 10.1038/nrneurol.2016.10 -
Cold Spring Harbor Perspectives in... Jan 2020Hidden hearing loss (HHL), a recently described auditory disorder, has been proposed to affect auditory neural processing and hearing acuity in subjects with normal... (Review)
Review
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.
Topics: Animals; Cochlea; Cochlear Nerve; Diagnosis, Differential; Disease Models, Animal; Hair Cells, Auditory, Inner; Hearing Loss; Humans
PubMed: 30617057
DOI: 10.1101/cshperspect.a035493 -
Hearing Research Jun 2017Common causes of hearing loss in humans - exposure to loud noise or ototoxic drugs and aging - often damage sensory hair cells, reflected as elevated thresholds on the... (Review)
Review
Common causes of hearing loss in humans - exposure to loud noise or ototoxic drugs and aging - often damage sensory hair cells, reflected as elevated thresholds on the clinical audiogram. Recent studies in animal models suggest, however, that well before this overt hearing loss can be seen, a more insidious, but likely more common, process is taking place that permanently interrupts synaptic communication between sensory inner hair cells and subsets of cochlear nerve fibers. The silencing of affected neurons alters auditory information processing, whether accompanied by threshold elevations or not, and is a likely contributor to a variety of perceptual abnormalities, including speech-in-noise difficulties, tinnitus and hyperacusis. Work described here will review structural and functional manifestations of this cochlear synaptopathy and will consider possible mechanisms underlying its appearance and progression in ears with and without traditional 'hearing loss' arising from several common causes in humans.
Topics: Animals; Auditory Perception; Auditory Threshold; Cochlear Nerve; Glutamic Acid; Hair Cells, Auditory, Inner; Hearing; Hearing Loss, Noise-Induced; Hearing Loss, Sensorineural; Humans; Nerve Degeneration; Noise; Risk Factors; Synapses; Synaptic Transmission
PubMed: 28087419
DOI: 10.1016/j.heares.2017.01.003 -
JPMA. the Journal of the Pakistan... Sep 2021Evoked Potentials are electrical potentials that occur in a group of neurons in response to stimulation of a sensory organ which can be recorded by surface electrodes.... (Review)
Review
Evoked Potentials are electrical potentials that occur in a group of neurons in response to stimulation of a sensory organ which can be recorded by surface electrodes. Testing evoked potentials is useful in assessing the integrity of neuronal pathways both at sensory and motor levels of neural control. Early auditory evoked potentials include cochlear and brainstem auditory-evoked potentials, popularly known as electrocochleogram, and auditory brainstem response. Evoked potential audiometry is a neurophysiogical test to assess auditory pathway function in response to auditory stimuli. Auditory brainstem response mainly assesses brainstem functions and integrity. These evoked potentials are widely used for assessment of the cochlear functions, auditory nerve and the brainstem. Most common indications for auditory evoked potentials include routine newborn hearing screening for auditory pathway deficits, detecting retrocochlear pathologies, intraoperative and intensive care monitoring, frequency-related measurement of auditory sensitivity and for diagnosing some demyelinating disorders in initial stages. The current narrative review was planned to highlight auditory brainstem response recording's basic principles, uses and methods of interpretation in health and disease phases.
Topics: Brain Stem; Cochlea; Cochlear Nerve; Evoked Potentials, Auditory, Brain Stem
PubMed: 34580520
DOI: 10.47391/JPMA.03-432 -
Balkan Medical Journal Sep 2017Morphologically congenital sensorineural hearing loss can be investigated under two categories. The majority of congenital hearing loss causes (80%) are membranous...
Morphologically congenital sensorineural hearing loss can be investigated under two categories. The majority of congenital hearing loss causes (80%) are membranous malformations. Here, the pathology involves inner ear hair cells. There is no gross bony abnormality and, therefore, in these cases high-resolution computerized tomography and magnetic resonance imaging of the temporal bone reveal normal findings. The remaining 20% have various malformations involving the bony labyrinth and, therefore, can be radiologically demonstrated by computerized tomography and magnetic resonance imaging. The latter group involves surgical challenges as well as problems in decision-making. Some cases may be managed by a hearing aid, others need cochlear implantation, and some cases are candidates for an auditory brainstem implantation (ABI). During cochlear implantation, there may be facial nerve abnormalities, cerebrospinal fluid leakage, electrode misplacement or difficulty in finding the cochlea itself. During surgery for inner ear malformations, the surgeon must be ready to modify the surgical approach or choose special electrodes for surgery. In the present review article, inner ear malformations are classified according to the differences observed in the cochlea. Hearing and language outcomes after various implantation methods are closely related to the status of the cochlear nerve, and a practical classification of the cochlear nerve deficiency is also provided.
Topics: Classification; Cochlea; Cochlear Nerve; Ear, Inner; Hearing Loss, Sensorineural; Humans; Osteogenesis; Temporal Bone; Tomography, X-Ray Computed
PubMed: 28840850
DOI: 10.4274/balkanmedj.2017.0367 -
Scientific Reports Nov 2023Acoustic overexposure can eliminate synapses between inner hair cells (IHCs) and auditory nerve fibers (ANFs), even if hair-cell function recovers. This synaptopathy has...
Acoustic overexposure can eliminate synapses between inner hair cells (IHCs) and auditory nerve fibers (ANFs), even if hair-cell function recovers. This synaptopathy has been extensively studied by confocal microscopy, however, understanding the nature and sequence of damage requires ultrastructural analysis. Here, we used focused ion-beam scanning electron microscopy to mill, image, segment and reconstruct ANF terminals in mice, 1 day and 1 week after synaptopathic exposure (8-16 kHz, 98 dB SPL). At both survivals, ANF terminals were normal in number, but 62% and 53%, respectively, lacked normal synaptic specializations. Most non-synapsing fibers (57% and 48% at 1 day and 1 week) remained in contact with an IHC and contained healthy-looking organelles. ANFs showed a transient increase in mitochondrial content (51%) and efferent innervation (34%) at 1 day. Fibers maintaining synaptic connections showed hypertrophy of pre-synaptic ribbons at both 1 day and 1 week. Non-synaptic fibers were lower in mitochondrial content and typically on the modiolar side of the IHC, where ANFs with high-thresholds and low spontaneous rates are normally found. Even 1 week post-exposure, many ANF terminals remained in IHC contact despite loss of synaptic specializations, thus, regeneration efforts at early post-exposure times should concentrate on synaptogenesis rather than neurite extension.
Topics: Mice; Animals; Cochlea; Noise; Hearing Loss, Noise-Induced; Hair Cells, Auditory; Hair Cells, Auditory, Inner; Synapses; Cochlear Nerve; Auditory Threshold
PubMed: 37945811
DOI: 10.1038/s41598-023-46859-6 -
Hearing Research Nov 2015In early tetrapods, it is assumed that the tympana were acoustically coupled through the pharynx and therefore inherently directional, acting as pressure difference... (Review)
Review
In early tetrapods, it is assumed that the tympana were acoustically coupled through the pharynx and therefore inherently directional, acting as pressure difference receivers. The later closure of the middle ear cavity in turtles, archosaurs, and mammals is a derived condition, and would have changed the ear by decoupling the tympana. Isolation of the middle ears would then have led to selection for structural and neural strategies to compute sound source localization in both archosaurs and mammalian ancestors. In the archosaurs (birds and crocodilians) the presence of air spaces in the skull provided connections between the ears that have been exploited to improve directional hearing, while neural circuits mediating sound localization are well developed. In this review, we will focus primarily on directional hearing in crocodilians, where vocalization and sound localization are thought to be ecologically important, and indicate important issues still awaiting resolution.
Topics: Alligators and Crocodiles; Animals; Behavior, Animal; Biological Evolution; Biophysical Phenomena; Birds; Cochlear Nerve; Ear; Evoked Potentials, Auditory, Brain Stem; Mammals; Reptiles; Sound Localization
PubMed: 26048335
DOI: 10.1016/j.heares.2015.05.009 -
ENeuro 2018Modeling is a useful tool for investigating various biophysical characteristics of neurons. Recent simulation studies of propagating action potentials (spike conduction)...
Modeling is a useful tool for investigating various biophysical characteristics of neurons. Recent simulation studies of propagating action potentials (spike conduction) along axons include the investigation of neuronal activity evoked by electrical stimulation from implantable prosthetic devices. In contrast to point-neuron simulations, where a large variety of models are readily available, Hodgkin-Huxley-type conductance-based models have been almost the only option for simulating axonal spike conduction, as simpler models cannot faithfully replicate the waveforms of propagating spikes. Since the amount of available physiological data, especially in humans, is usually limited, calibration, and justification of the large number of parameters of a complex model is generally difficult. In addition, not all simulation studies of axons require detailed descriptions of nonlinear ionic dynamics. In this study, we construct a simple model of spike generation and conduction based on the exponential integrate-and-fire model, which can simulate the rapid growth of the membrane potential at spike initiation. In terms of the number of parameters and equations, this model is much more compact than conventional models, but can still reliably simulate spike conduction along myelinated and unmyelinated axons that are stimulated intracellularly or extracellularly. Our simulations of auditory nerve fibers with this new model suggest that, because of the difference in intrinsic membrane properties, the axonal spike conduction of high-frequency nerve fibers is faster than that of low-frequency fibers. The simple model developed in this study can serve as a computationally efficient alternative to more complex models for future studies, including simulations of neuroprosthetic devices.
Topics: Action Potentials; Animals; Axons; Cochlear Nerve; Humans; Models, Neurological; Neural Conduction
PubMed: 30225348
DOI: 10.1523/ENEURO.0112-18.2018 -
Clinics in Perinatology Jun 2016Although hyperbilirubinemia is extremely common among neonates and is usually mild and transient, it sometimes leads to bilirubin-induced neurologic damage (BIND). The... (Review)
Review
Although hyperbilirubinemia is extremely common among neonates and is usually mild and transient, it sometimes leads to bilirubin-induced neurologic damage (BIND). The auditory pathway is highly sensitive to the effects of elevated total serum/plasma bilirubin (TB) levels, with damage manifesting clinically as auditory neuropathy spectrum disorder. Compared to full-term neonates, preterm neonates are more susceptible to BIND and suffer adverse effects at lower TB levels with worse long-term outcomes. Furthermore, although standardized guidelines for management of hyperbilirubinemia exist for term and late preterm neonates, similar guidelines for neonates less than 35 weeks gestational age are limited.
Topics: Cochlear Nerve; Cochlear Nucleus; Evoked Potentials, Auditory, Brain Stem; Hearing Loss, Central; Humans; Hyperbilirubinemia, Neonatal; Infant, Newborn; Infant, Premature; Kernicterus
PubMed: 27235210
DOI: 10.1016/j.clp.2016.01.006 -
Biological Cybernetics Oct 2016Lizard ears are coupled across the pharynx, and are very directional. In consequence all auditory responses should be directional, without a requirement for computation... (Review)
Review
Lizard ears are coupled across the pharynx, and are very directional. In consequence all auditory responses should be directional, without a requirement for computation of sound source location. Crocodilian ears are connected through sinuses, and thus less tightly coupled. Coupling may improve the processing of low-frequency directional signals, while higher frequency signals appear to be progressively uncoupled. In both lizards and crocodilians, the increased directionality of the coupled ears leads to an effectively larger head and larger physiological range of ITDs. This increased physiological range is reviewed in the light of current theories of sound localization.
Topics: Alligators and Crocodiles; Animals; Cochlear Nerve; Ear; Hearing; Lizards; Sound Localization
PubMed: 27734148
DOI: 10.1007/s00422-016-0698-2