Laboratory mice (Mus musculus) have become the major model species for inner ear
research. The major uses of mice include gene discovery, characterization, and …

Cerebellar ataxia, neuropathy and vestibular areflexia syndrome (CANVAS) typically
presents in middle life with a combination of neuropathy, ataxia and vestibular disease, with …

Dizziness and hearing loss are among the most common disabilities. Many forms of
hereditary balance and hearing disorders are caused by abnormal development of …

Approximately 3 in 1000 children in the US under 4 years of age are affected by hearing
loss. Currently, cochlear implants represent the only line of treatment for patients with severe …

Hearing loss (HL) is the most common sensory deficit in humans and is frequently
accompanied by peripheral vestibular loss (PVL). While often overlooked, PVL is an …

Mutations in voltage-gated potassium channel KCNE1 cause Jervell and Lange-Nielsen
syndrome type 2 (JLNS2), resulting in congenital deafness and vestibular dysfunction. We …

Aim: The aim of this study was to comprehensively evaluate vestibular function in the largest
series presymptomatic as well as symptomatic p. P51S variant carriers, to determine which …

Cerebellar and vestibular disorders leading to dizziness or episodic vertigo may show
overlapping clinical features. A deep phenotyping including a complete familial history is a …

Background: A considerable amount of research has been published about genetic hearing
impairment. Fifty to sixty percent of hearing loss is thought to have a genetic cause. Genes …

Our understanding of the genetic basis of Ménière's disease (MD) is still limited. Although
the familial clustering and the geographical and racial differences in incidence strongly …