scholarly journals Navigating Hereditary Hearing Loss: Pathology of the Inner Ear

2021 ◽  
Vol 15 ◽  
Author(s):  
Teresa Nicolson
Keyword(s):  
Hearing Loss ◽  
Animal Models ◽  
Inner Ear ◽  
Auditory System ◽  
Cranial Nerve ◽  
Hereditary Hearing Loss ◽  
Hearing Organ ◽  
Peripheral Auditory System ◽  
The Brain

Inherited forms of deafness account for a sizable portion of hearing loss among children and adult populations. Many patients with sensorineural deficits have pathological manifestations in the peripheral auditory system, the inner ear. Within the hearing organ, the cochlea, most of the genetic forms of hearing loss involve defects in sensory detection and to some extent, signaling to the brain via the auditory cranial nerve. This review focuses on peripheral forms of hereditary hearing loss and how these impairments can be studied in diverse animal models or patient-derived cells with the ultimate goal of using the knowledge gained to understand the underlying biology and treat hearing loss.

scholarly journals Deafness-in-a-dish: modeling hereditary deafness with inner ear organoids

2021 ◽  
Author(s):  
Daniel R. Romano ◽  
Eri Hashino ◽  
Rick F. Nelson
Keyword(s):  
Animal Models ◽  
Inner Ear ◽  
Auditory System ◽  
Hearing Aids ◽  
Molecular Mechanisms ◽  
Functional Disability ◽  
Experimental Studies ◽  
Hereditary Deafness ◽  
Human Auditory System ◽  
Human Inner Ear

AbstractSensorineural hearing loss (SNHL) is a major cause of functional disability in both the developed and developing world. While hearing aids and cochlear implants provide significant benefit to many with SNHL, neither targets the cellular and molecular dysfunction that ultimately underlies SNHL. The successful development of more targeted approaches, such as growth factor, stem cell, and gene therapies, will require a yet deeper understanding of the underlying molecular mechanisms of human hearing and deafness. Unfortunately, the human inner ear cannot be biopsied without causing significant, irreversible damage to the hearing or balance organ. Thus, much of our current understanding of the cellular and molecular biology of human deafness, and of the human auditory system more broadly, has been inferred from observational and experimental studies in animal models, each of which has its own advantages and limitations. In 2013, researchers described a protocol for the generation of inner ear organoids from pluripotent stem cells (PSCs), which could serve as scalable, high-fidelity alternatives to animal models. Here, we discuss the advantages and limitations of conventional models of the human auditory system, describe the generation and characteristics of PSC-derived inner ear organoids, and discuss several strategies and recent attempts to model hereditary deafness in vitro. Finally, we suggest and discuss several focus areas for the further, intensive characterization of inner ear organoids and discuss the translational applications of these novel models of the human inner ear.

Causes and Types of Sensorineural Hearing Loss. Clinical Manifestations and Basic Principles of Treatment

2020 ◽  
pp. 9-16
Author(s):  
Sergey Armakov
Keyword(s):  
Hearing Loss ◽  
Sensorineural Hearing Loss ◽  
Inner Ear ◽  
Clinical Manifestations ◽  
Sensorineural Hearing ◽  
Doppler Imaging ◽  
Comprehensive Treatment ◽  
Impaired Hearing ◽  
Temporal Bones ◽  
The Brain

Sensorineural hearing loss is a disorder associated with the damage to the inner ear structures: the cochlea (cortical organ), dysfunctioning of the vestibule-cochlear nerve or the central part of the auditory analyser (brain stem and cortical representation of the cortical temporal lobe). In recent years, there has been a steady increase in ensorineural hearing loss patients; they account for ca. 70% among the total patients with impaired hearing. The disease has numerous causes and a complex pathogenesis. Among the main factors contributing to hearing loss are genetic predisposition, perinatal pathology, including hypoxia at childbirth, exposure to infectious and toxic agents and metabolic disorders, injuries (mechanical, acoustic and altitude trauma). Vascular-rheological disorders in the vertebro-basilar system play an important part because blood is supplied to the inner ear from the anterior inferior cerebellar artery. There are sudden, acute and chronic sensorineural hearing loss. The ensorineural hearing loss isdiagnosed by examinations that allow to verify the diagnosis and to determine the sound analyser damage level. This complex includes audiometric examinations, including the tuning fork examination, speech audiometry, and acoustic impedancemetry. If necessary, ultrasound Doppler imaging of the main blood vessels of the brain, computed tomography of the temporal bones, and MRI of the brain are prescribed. The pattern of comprehensive treatment should include, first of all, the elimination of the disease cause and anti-hypoxic drugs, anti-oxidants and a number of physiotherapy procedures.

scholarly journals Mouse models to study inner ear development and hereditary hearing loss

2007 ◽  
Vol 51 (6-7) ◽  
pp. 609-631 ◽  
Author(s):  
Lilach M. Friedman ◽  
Amiel A. Dror ◽  
Karen B. Avraham
Keyword(s):  
Hearing Loss ◽  
Inner Ear ◽  
Mouse Models ◽  
Ear Development ◽  
Hereditary Hearing Loss ◽  
Inner Ear Development

scholarly journals HUBUNGAN ANTARA GANGGUAN PENDENGARAN DENGAN SERUMEN PADA LANSIA DI PUSKESMAS MEDAN JOHOR

2019 ◽  
Vol 1 (2) ◽  
pp. 41-47
Author(s):  
Alamsyah Lukito
Keyword(s):  
Hearing Loss ◽  
Mechanical Energy ◽  
Electrical Energy ◽  
Ear Canal ◽  
Cross Sectional ◽  
Hearing Organ ◽  
Human Ear ◽  
The Brain

The human ear is a hearing organ that captures and changes sound in the form of mechanical energy into electrical energy efficiently and is passed on to the brain to be realized and understood. Serum that collects and forms masses will clog the ear canal, causing interference with the sound that results in hearing loss. The research that will be conducted is a study with a cross-sectional method with a sample of 52 people. The majority of respondents were men with a majority of elderly with an average age of 67 years. The results showed that respondents who had serumen were as much as 59.6% and those who had hearing loss were 63.5%. This shows that there is a relationship between hearing loss and the presence of serumen.

Ménière Recognizes That Vertigo Can Originate from the Inner Ear

2016 ◽  
Author(s):  
Robert W. Baloh
Keyword(s):  
Hearing Loss ◽  
Inner Ear ◽  
Blood Vessels ◽  
Semicircular Canal ◽  
Semicircular Canals ◽  
Underlying Disease ◽  
The Brain ◽  
Benign Course

Prosper Ménière was the first clinician to conclude that vertigo can result from diseases of the inner ear. The symptom of vertigo originally fell under the rubric of apoplectiform cerebral congestion, a disorder thought to result from overfilling of blood vessels in the brain. Ménière noted that patients with vertigo and hearing loss associated with damage to the inner ear often have a benign course, and aggressive treatments such as bleeding can be more dangerous than the underlying disease. The first hint that the semicircular canals may be related to balance rather than hearing was provided by a Frenchman, Marie Jean Pierre Flourens. He systematically cut each semicircular canal in the pigeon and noted that the animal’s head and body tended to move in the plane of the damaged canal. The gyrations of the animals described by Flourens made Ménière think that vertigo in humans might be a similar phenomenon.

The inner ear

2009 ◽  
pp. 109-148
Author(s):  
Rogan Corbridge ◽  
Nicholas Steventon
Keyword(s):  
Hearing Loss ◽  
Inner Ear ◽  
Sudden Hearing Loss ◽  
Structure And Function ◽  
Noise Induced Hearing Loss ◽  
Hereditary Hearing Loss ◽  
Syndromic Hearing Loss ◽  
And Function

Structure and function of the inner ear 110 Hearing loss 114 Presbyacusis 116 Noise-induced hearing loss 118 Idiopathic sudden hearing loss 120 Autoimmune ear disease 122 Ototoxicity 124 Hereditary hearing loss 126 Syndromic hearing loss I 128 Syndromic hearing loss II 130 Non-organic hearing loss (NOHL) ...

Girl with accelerated growth, hearing loss, inner ear anomalies, delayed myelination of the brain, and del(22)(q13.1q13.2)

2000 ◽  
Vol 92 (3) ◽  
pp. 195-199 ◽  
Author(s):  
Yasuko Fujita ◽  
Daishi Mochizuki ◽  
Yosuke Mori ◽  
Natsue Nakamoto ◽  
Masaaki Kobayashi ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Inner Ear ◽  
Accelerated Growth ◽  
Ear Anomalies ◽  
The Brain ◽  
Delayed Myelination

scholarly journals Genetics of Peripheral Vestibular Dysfunction: Lessons from Mutant Mouse Strains

2014 ◽  
Vol 25 (03) ◽  
pp. 289-301 ◽  
Author(s):  
Sherri M. Jones ◽  
Timothy A. Jones
Keyword(s):  
Hearing Loss ◽  
Inner Ear ◽  
Mouse Models ◽  
Noise Exposure ◽  
Mutant Mouse ◽  
Vestibular Dysfunction ◽  
Mouse Strains ◽  
Neural Function ◽  
Hereditary Hearing Loss ◽  
New Genes

Background: A considerable amount of research has been published about genetic hearing impairment. Fifty to sixty percent of hearing loss is thought to have a genetic cause. Genes may also play a significant role in acquired hearing loss due to aging, noise exposure, or ototoxic medications. Between 1995 and 2012, over 100 causative genes have been identified for syndromic and nonsyndromic forms of hereditary hearing loss. Mouse models have been extremely valuable in facilitating the discovery of hearing loss genes and in understanding inner ear pathology due to genetic mutations or elucidating fundamental mechanisms of inner ear development. Purpose: Whereas much is being learned about hereditary hearing loss and the genetics of cochlear disorders, relatively little is known about the role genes may play in peripheral vestibular impairment. Here we review the literature with regard to genetics of vestibular dysfunction and discuss what we have learned from studies using mutant mouse models and direct measures of peripheral vestibular neural function. Results: Several genes are considered that when mutated lead to varying degrees of inner ear vestibular dysfunction due to deficits in otoconia, stereocilia, hair cells, or neurons. Behavior often does not reveal the inner ear deficit. Many of the examples presented are also known to cause human disorders. Conclusions: Knowledge regarding the roles of particular genes in the operation of the vestibular sensory apparatus is growing, and it is clear that gene products co-expressed in the cochlea and vestibule may play different roles in the respective end organs. The discovery of new genes mediating critical inner ear vestibular function carries the promise of new strategies in diagnosing, treating, and managing patients as well as predicting the course and level of morbidity in human vestibular disease.

The Effect of Continuous Monaural Noise on Loudness Matches to Tinnitus

1988 ◽  
Vol 31 (1) ◽  
pp. 98-102 ◽  
Author(s):  
M. J. Penner
Keyword(s):  
Hearing Loss ◽  
Sensorineural Hearing Loss ◽  
Auditory System ◽  
Noise Level ◽  
Sensorineural Hearing ◽  
Peripheral Auditory System

Data from two psychophysical tasks are presented. In the first, 8 subjects with sensorineural hearing loss and tinnitus adjusted the intensity of a continuous monaural noise to mask the tinnitus. In the second, in the presence of continuous monaural noise, the same subjects adjusted the intensity of a pulsed monaural tone to match the loudness of the tinnitus. The tone was either ipsilateral or contralateral to the noise. Although the noise level required to mask the tinnitus increased substantially, as did the level of the ipsilateral matching tone, the change in the level of the contralateral matching tone was minimal. One possible explanation of these findings is related to the functioning of the peripheral auditory system.

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