Mice as Models for Human Hereditary Deafness

2006 ◽  
pp. 247-296 ◽  
Author(s):  
Karen P. Steel ◽  
Alexandra Erven ◽  
Amy E. Kiernan
Keyword(s):  
Hereditary Deafness

Ethical And Cultural Considerations In Research On Hereditary Deafness

1992 ◽  
Vol 25 (5) ◽  
pp. 973-978
Author(s):  
Kenneth M. Grundfast ◽  
Jeffrey Rosen
Keyword(s):  
Hereditary Deafness ◽  
Cultural Considerations

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.

Hereditary Deafness in the Cat

ORL ◽  
1977 ◽  
Vol 39 (4) ◽  
pp. 241-246 ◽  
Author(s):  
Hans H. Elverland ◽  
Iain W.S. Mair ◽  
Olav Borud
Keyword(s):  
Hereditary Deafness

Cochleo-Saccular Degeneration in One of Three Sisters with Hereditary Deafness, Absent Gastric Motility, Small Bowel Diverticulitis and Progressive Sensory Neuropathy

ORL ◽  
1981 ◽  
Vol 43 (1) ◽  
pp. 4-16 ◽  
Author(s):  
Makoto Igarashi ◽  
Duncan MacRae ◽  
Toshiaki O-Uchi ◽  
Bobby R. Alford
Keyword(s):  
Small Bowel ◽  
Gastric Motility ◽  
Sensory Neuropathy ◽  
Hereditary Deafness ◽  
Three Sisters

Regulatory Mutations in Human Hereditary Deafness

2012 ◽  
pp. 137-168
Author(s):  
Jonathan E. Bird ◽  
Thomas B. Friedman
Keyword(s):  
Hereditary Deafness ◽  
Regulatory Mutations

scholarly journals In-Silico Prediction of Candidate SNPs in TRIOBP, TMC1 and EYA4 Genes Causing Hereditary Deafness in Three Sudanese Patients Using Next Generation Sequencing

2014 ◽  
Vol 03 (02) ◽  
pp. 1-10
Author(s):  
Mahmoud Eltayeb Koko Musa ◽  
Yousuf Hasan Yousuf Bakhit ◽  
Ashraf Yahia Osman Mohamed ◽  
Asaad Tageldein Idris ◽  
Ashraf Ahmed Mohamed Ahmed Fadul ◽  
...  
Keyword(s):  
Next Generation Sequencing ◽  
In Silico ◽  
Candidate Snps ◽  
In Silico Prediction ◽  
Next Generation ◽  
Hereditary Deafness ◽  
Generation Sequencing

Molecular Genetics Aspects of Hereditary Deafness

2014 ◽  
Vol 10 (1) ◽  
pp. 18
Author(s):  
S. Lalchev ◽  
B. Popov
Keyword(s):  
Molecular Genetics ◽  
Hereditary Deafness

Hereditary deafness in Kirov oblast: Estimation of the incidence rate and DNA diagnosis in children

2012 ◽  
Vol 48 (4) ◽  
pp. 455-462 ◽  
Author(s):  
R. A. Zinchenko ◽  
A. A. Osetrova ◽  
E. I. Sharonova
Keyword(s):  
Incidence Rate ◽  
Dna Diagnosis ◽  
Hereditary Deafness ◽  
Kirov Oblast

scholarly journals Perinatal Gjb2 gene transfer rescues hearing in a mouse model of hereditary deafness

2015 ◽  
Vol 24 (13) ◽  
pp. 3651-3661 ◽  
Author(s):  
Takashi Iizuka ◽  
Kazusaku Kamiya ◽  
Satoru Gotoh ◽  
Yoshinobu Sugitani ◽  
Masaaki Suzuki ◽  
...  
Keyword(s):  
Gene Transfer ◽  
Mouse Model ◽  
Gjb2 Gene ◽  
Hereditary Deafness
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