Targeted Allele Suppression Prevents Progressive Hearing Loss in the Mature Murine Model of Human TMC1 Deafness

Comprehensive Hearing Loss and Deafness Panel Plus

Deafness and Hereditary Hearing Loss Overview

Clinical characteristics. Hereditary hearing loss and deafness may be conductive, sensorineural, or a combination of both; syndromic (associated with malformations of the external ear or other organs or with medical problems involving other organ systems) or nonsyndromic (no associated visible abnormalities of the external ear or any related medical problems); and prelingual (before language de...

Conversion deafness presenting as sudden hearing loss.

Conversion deafness is a somatoform disorder characterized by hearing loss without an anatomic or pathophysiologic lesion. Clinically, discrepancies between behavior hearing thresholds and objective electrophysiologic examinations, such as impedance audiometry, otoacoustic emissions (OAE), and auditory brainstem response (ABR), will raise the suspicion of this disorder. It is judged to be due t...

Catastrophic progressive hearing loss in childhood.

We present a report on a 5-year-old child with a complex medical and audiologic history who exhibited catastrophic progression in hearing loss. Hearing loss was initially attributed to bacterial meningitis at age 3 months; progression was apparently related to perilymph fistula at age 8 years. Etiologies associated with progressive hearing loss in children as well as signs of progression and mo...

Diaphanous homolog 3 (Diap3) Overexpression Causes Progressive Hearing Loss and Inner Hair Cell Defects in a Transgenic Mouse Model of Human Deafness

We previously demonstrated that a mutation in the 5' untranslated region of Diaphanous homolog 3 (DIAPH3) results in 2 to 3-fold overexpression of the gene, leading to a form of delayed onset, progressive human deafness known as AUNA1 (auditory neuropathy, nonsyndromic, autosomal dominant, 1). To investigate the mechanism of deafness, we generated two lines of transgenic mice overexpressing Dia...