While inner ear disorders are common, our ability to intervene and recover their sensory
function is limited. In vitro models of the inner ear, like the organoid system, could aid in …

From the first identified non-syndromic hearing loss gene in 1995, to those discovered in
present day, the field of human genetics has witnessed an unparalleled revolution that …

Sound signal is detected by sensory hair cells located in the cochlear region of the inner ear,
and transmitted to the central nervous system by the spiral ganglion neurons (SGNs). These …

Incomplete partition type III (IP-III) is a relatively rare inner ear malformation that has been
associated with a POU3F4 gene mutation. The IP-III anomaly is mainly characterized by …

Hereditary hearing loss is genetically heterogeneous, with a large number of genes and
mutations contributing to this sensory, often monogenic, disease. This number, as well as …

POU3F4 mutations (DFNX2) are the most prevalent among non-syndromic X-linked hearing
loss (HL) identified to date. Clinical manifestations of DFNX2 usually comprise congenital …

X-linked deafness (DFNX) is estimated to account for up to 2% of cases of hereditary
hearing loss and occurs in both syndromic and non-syndromic forms. POU3F4 is the gene …

Objectives/Hypothesis To determine the frequency of the incomplete partition type III
anomaly and the genetic and clinical features associated with POU3F4 mutations in children …

Hearing loss (HL) is the most common sensory defect and affects 450 million people
worldwide in a disabling form. Pathogenic sequence alterations in the POU3F4 gene, which …

The cochlea consists of diverse cellular populations working in harmony to convert
mechanical stimuli into electrical signals for the perception of sound. Otic mesenchyme cells …