Adeno-associated virus (AAV) has been successfully used to deliver gene therapy to
improve auditory function in mouse models of hereditary hearing loss. Many forms of …

Damage and loss of hair cells in the inner ear is the most frequent cause of hearing loss
(HL), since mammalian hair cells are not replenished once lost. To date, the treatment of HL …

More than 28 million Americans suffer from various forms of hearing loss. The lack of
effective treatments for many forms of hearing disorders has prompted interest in the …

Gene therapy for genetic hearing loss is a nascent field with just a handful of studies
published to date that demonstrate proof‐of‐concept recovery of auditory function (reviewed …

Cochlear sensory hair cells (HCs) are crucial for hearing as mechanoreceptors of the
auditory systems. Clarification of transcriptional regulation for the cochlear sensory HC …

Impairments of the inner ear result in sensorineural hearing loss and vestibular dysfunction
in humans. A large proportion of these disorders are congenital, and involve both auditory …

Genetic hearing loss accounts for up to 50% of prelingual deafness worldwide, yet there are
no biologic treatments currently available. To investigate gene therapy as a potential …

Atoh1 encodes a basic helix‐loop‐helix (bHLH) transcription factor required for the
development of the inner ear sensory epithelia, the dorsal spinal cord, brainstem …

Ear development requires the transcription factors ATOH1 for hair cell differentiation and
NEUROD1 for sensory neuron development. In addition, NEUROD1 negatively regulates …

Usher syndrome is a syndromic form of hereditary hearing impairment that includes
sensorineural hearing loss and delayed-onset retinitis pigmentosa (RP). Type 1 Usher …