A previously unrecognized neuropathy was identified in Bulgarian gypsies, and was designated hereditary motor and sensory neuropathy-Lom (HMSNL) after the town where the initial cases were found. It was subsequently identified in other gypsy communities. The disorder, which is of autosomal recessive inheritance, was mapped to chromosome 8q24. It begins consistently in the first decade of life w...

Persons with auditory neuropathy/dys-synchrony display dys-synchronous peripheral neural function in the presence of intact outer hair cell function (e.g., Berlin et al., 1993; Starr et al., 1996). The majority of patients also report hearing difficulty characterized by difficulty understanding speech, particularly in background noise. Patients with AN/AD show variation along several dimensions...

• Auditory neuropathy is an uncommon presentation of elevated ICP, possibly due to stretch of the vestibulocochlear nerve from the increased ICP. • Petrous apex cephalocele and meningoceles are uncommon radiologic features of elevated ICP. • Ischemic central retinal vein occlusion is a rare ophthalmologic manifestation of elevated ICP that may cause irreversible vision loss if the increased ICP...

Smoorenburg GF: Normative findings of electrically evoked compound action potential measurement using the neural response telemetry of the Nucleus CI24M cochlear implant system. of autonomic and sensory neuropathy and the impairment of visual and auditory evoked potentials in type-1 diabetes. Is there a

TRAUMATIC SOUND EXPOSURE CAUSES sensorineural hearing loss (SNHL) by destroying or damaging the neural elements of the cochlea (Young 2011). Until recently, the focus of SNHL research was on the hair cells (HCs), the transducer cells in the cochlea (Fig. 1). Severe acoustic trauma destroys HCs, leading to loss of function in damaged regions (Engström et al. 1966). Less severe trauma can cause s...

Arnold's nerve ear-cough reflex is recognised to occur uncommonly in patients with chronic cough. In these patients, mechanical stimulation of the external auditory meatus can activate the auricular branch of the vagus nerve (Arnold's nerve) and evoke reflex cough. This is an example of hypersensitivity of vagal afferent nerves, and there is now an increasing recognition that many cases of refr...

Perceptual abnormalities such as hyperacusis and tinnitus often occur after acoustic overexposure. Although such exposure can also result in permanent threshold elevation, some individuals with noise-induced hyperacusis or tinnitus show clinically normal thresholds. Recent work in animals has shown that a "neuropathic" noise exposure can cause immediate, permanent degeneration of the cochlear n...

This article presents information about developmental outcomes of children with auditory neuropathy/auditory dyssynchrony (AN). Colorado data on the number of children screened and the number of children identified with unilateral and bilateral AN will be described. Descriptive information about the percent of children with AN with cognitive disability and disabilities other than hearing loss w...

The Canadian Hearing and Auditory Research Translation (CHART) group is a newly formed taskforce to develop collaborative research initiatives. Initial discussions centered on diagnostic improvements for middle ear disease, auditory neuropathy spectrum disorder, tinnitus and presbycusis. Central to these discussions was the widely held view that the standard audiogram and its interpretation is ...