Disease relevance of Hearing Loss, Sensorineural

• Mutations of the WFS1 gene are responsible for two hereditary diseases, Wolfram syndrome and low frequency sensorineural hearing loss [1].
• The tubby mouse is characterized by an autosomal recessive mutation which results in the development of maturity-onset obesity and sensorineural hearing loss and retinal degeneration [2].
• This could apply to autoimmune sensorineural hearing loss, as well as any hearing disorder for which steroid therapy is recommended [3].
• BACKGROUND: Low cerebrospinal fluid (CSF) glucose levels have been associated with sensorineural hearing loss (SNHL) in bacterial meningitis but systematic investigations are lacking [4].
• Mitochondrial DNA (mtDNA) mutation associated with sensorineural hearing loss (SNHL) has previously been described in MELAS (mitochondrial myopathy, encephalopathy, lactic acidosis and stroke-like episodes) and in aminoglycoside-induced deafness [5].

High impact information on Hearing Loss, Sensorineural

• We identified a human mutation that causes dilated cardiomyopathy and heart failure preceded by sensorineural hearing loss (SNHL) [6].
• ATP6V1B mutations have been associated with early sensorineural hearing loss (SNHL), whereas ATP6V0A4 mutations are classically associated with either late-onset SNHL or normal hearing [7].
• Sensorineural hearing loss associated with this infection is mediated by the release of an exotoxin, pneumolysin [8].
• 1. More than 120 mutations have been identified in WFS1 associated with AR WS, as well as autosomal dominant nonsyndromic low-frequency sensorineural hearing loss (LFSNHL) [9].
• Chronic depolarization enhances the trophic effects of brain-derived neurotrophic factor in rescuing auditory neurons following a sensorineural hearing loss [10].

Chemical compound and disease context of Hearing Loss, Sensorineural

• Dopamine (DA), released from the lateral olivocochlear (LOC) efferent terminals, the efferent arm of the short-loop feedback in the cochlea, is considered as a protective factor in the inner ear since it inhibits auditory nerve dendrite firing in ischemia- or noise-induced excitotoxicity leading to sensorineural hearing loss (SNHL) [11].
• PURPOSE: The pattern of sensorineural hearing loss (SNHL) after primary treatment for nasopharyngeal carcinoma (NPC) was studied, and the effect of cisplatin, radiotherapy does, and fractionation were evaluated [12].
• OBJECTIVE: The purpose of this study was to clarify whether higher doses of steroids improve the prognosis of idiopathic sensorineural hearing loss (ISHL) and the suitable dose of steroid hormone [13].
• HYPOTHESIS: Although the glucocorticoid prednisone is the standard therapy for autoimmune sensorineural hearing loss, what this hormone does in the ear to restore hearing is not known [14].
• Heterozygosity for mutations in the fibrillar collagen gene COL11A1 causes sensorineural hearing loss in patients with Stickler syndrome or Marshall syndrome [15].

Biological context of Hearing Loss, Sensorineural

• OBJECTIVE: To study nonsyndromic progressive sensorineural hearing loss (SNHL) with significant linkage to the DFNA2 locus on chromosome 1p in a Dutch kindred [16].
• OBJECTIVE: To determine the spectrum of connexin 26 (Cx26) mutations and their phenotypes in children with sensorineural hearing loss (SNHL) or mixed hearing loss (MHL) [17].
• Pharmacokinetics of coenzyme Q10 in recovery of acute sensorineural hearing loss due to hypoxia [18].
• This study examines the acoustic reflex threshold criteria, derived from a large group of patients with cochlear hearing loss proposed by Cohen & Prasher (1992), in order to evaluate their effectiveness in differentiating between cochlear and retro-cochlear lesions [19].
• Therefore, to identify a potential model for this field of research, this study examined the effects of prednisolone on auditory brain stem response thresholds in the MRL/lpr mouse model of autoimmune sensorineural hearing loss [20].

Anatomical context of Hearing Loss, Sensorineural

• The WFS1 gene, responsible for low frequency sensorineural hearing loss and Wolfram syndrome, is expressed in a variety of inner ear cells [21].
• Conflicting investigations regarding the potential protective effect of melanin against noise-induced sensorineural hearing loss have suggested that eumelanin and pheomelanin may have differing effects within the stria vascularis [22].
• The severe sensorineural hearing loss and degenerative changes of the cochlear structures indicate that cochlear structures, especially the outer hair cells and the intermediate cells of the stria vascularis, are vulnerable to the missing Mpv17 gene product [23].
• OBJECTIVE: To treat patients with sudden sensorineural hearing loss (SSNL) who failed oral prednisone therapy by using a round window membrane (RWM) microcatheter [24].

Gene context of Hearing Loss, Sensorineural

• We have now determined the GJB2[connexin 26 (Cx26)] mutation spectrum in 60 index patients from mostly large Turkish families with autosomal-recessive inherited non-syndromic sensorineural hearing loss (NSSHL) [25].
• The CRMP1 and EVC genes are located near WFS1, the Wolfram syndrome type 1 gene, in which mutations also cause low frequency sensorineural hearing loss (LFSNHL) [26].
• Dominant white spotting W/W(v) and W(v)/W(v) mice are well-known mutants that lack strial intermediate cells in their cochlea and manifest hereditary sensorineural hearing loss [27].
• Sensorineural hearing loss (SNHL) was observed in 13 patients with AS (59%), 17 patients with RA (65.4%), and 13 healthy controls (41.9%) without statistically significant differences [28].
• On the both these observations we based our hypothesis that low circulating NGF might lead to sensorineural hearing loss (SNHL) [29].

Analytical, diagnostic and therapeutic context of Hearing Loss, Sensorineural

• A 37-year-old male developed severe sensorineural hearing loss (SNHL) following drip intravenous pyelography with iohexol [30].
• Immunoblotting against bovine inner ear extracts has previously identified a 68 kd antigen reactive with 22% to 58% of sera of patients with rapidly progressive sensorineural hearing loss (PSNHL) of suspected autoimmune causation [31].

References