Sensorineural hearing impairment is the most common form of hearing loss, and
encompasses pathologies of the cochlea and the auditory nerve. Hearing impairment …

The pathogenic mutation in Friedreich's ataxia (FRDA) is a homozygous guanine-adenine-
adenine (GAA) trinucleotide repeat expansion on chromosome 9q13 that causes a …

Test results and management data are summarized for 260 patients with diagnoses of
Auditory Neuropathy Spectrum Disorder (ANSD). Hearing aids were tried in 85 of these …

Cochlear implantation has become the standard‐of‐care for adults and children with severe
to profound hearing loss. There is growing evidence that qualitative as well as quantitative …

One hundred and fifty years since Nikolaus Friedreich's first description of the degenerative
ataxic syndrome which bears his name, his description remains at the core of the classical …

The effects of inner ear abnormality on audibility have been explored since the early 20th
century when sound detection measures were first used to define and quantify 'hearing loss' …

Hearing deficits associated with cognitive impairment have attracted much recent interest,
motivated by emerging evidence that impaired hearing is a risk factor for cognitive decline …

Auditory neuropathy spectrum disorder (ANSD) refers to a range of hearing impairments
characterized by deteriorated speech perception, despite relatively preserved pure-tone …

Friedreich ataxia is the most frequent hereditary ataxia, with an estimated prevalence of 3–4
cases per 100,000 individuals. This autosomal-recessive neurodegenerative disease is …

Friedreich ataxia, the most common hereditary ataxia, affects approximately 1 per 29,000
white individuals. In about 98% of these individuals, it is due to homozygosity for a GAA …