A spontaneous homozygous deletion affecting the copy number of the cadherin 23 (Cdh23) gene in a line of laboratory mice gave rise to a new murine model with hereditary hearing loss and vestibular alterations.
Patients with congenital hearing loss could benefit from a gene therapy currently in development. Although there are approaches that could reverse the process in children and young people before it becomes severe, so far, adults do not have any treatment that prevents the progressive deterioration of auditory sensory cells caused by this disease.
It is known that >70% of the genes involved in pathogenic hearing loss are involved in autosomal recessive nonsyndromic hearing loss (ARNSHL), which accounts for 1 case for every 166 individuals in Iran due to the high rate of consanguineous marriage in the country.
Shionogi & Co. Ltd. has entered into an option agreement with Cilcare Dev SAS to acquire the exclusive license for the global development, manufacturing and commercialization of hearing loss treatment drug candidates CIL-001 and/or CIL-003.
One in every 500 newborns worldwide are affected by nonsyndromic hearing loss (NSHL). Whole-exome sequencing may help in identifying the genetic cause of NSHL.
It is known that CX3C chemokine receptor 1 (CX3CR1) expressed on macrophages plays a crucial role in inner hair cell ribbon synaptic repair and spiral ganglion neuron survival after synaptopathic noise-induced trauma.
The most common genetic cause of hereditary deafness in humans are mutations in the GJB2 gene, especially the 35delG and 235delC mutations. At the recent ARO meeting, researchers from Ear Nose and Throat Hospital of Fudan University presented the creation of a novel mouse model for studying the pathogenic mechanisms of hereditary deafness resulting from these mutations.
Noise-induced hearing loss is still the most common cause of acquired hearing loss nowadays. The mechanisms behind this may be explained through inflammatory responses in the cochlea after acoustic trauma.
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