• We are proposing that the evidence is sufficient to determine that cochlear implantation may be covered for treatment of bilateral pre- or post-linguistic, sensorineural, moderate-to-profound hearing loss in individuals who demonstrate limited benefit from amplification. (cms.gov)
  • Cochlear implanted children with prelinguistic sensorineural bilateral deafness of profound degree, using either the ACE or SPEAK coding strategy, were evaluated and compared. (cun.es)
  • Unilateral and Bilateral ear deafness. (who.int)
  • Clinically, testing of GJB2 and GJB6 plays a prominent role in diagnosis and genetic counseling because mutations in these genes account for more than 50% of severe-to-profound autosomal recessive nonsyndromic deafness in many world populations. (nature.com)
  • Detailed clinical histories were obtained for affected individuals (ages ranging from 6 to 40 years), who exhibited prelingual, profound, sensorineural hearing loss and independent ambulation delayed beyond 1.5 years of age. (bmj.com)
  • Human adenylate kinase 2 deficiency causes a profound hematopoietic defect associated with sensorineural deafness. (lu.se)
  • Sensorineural deafness and male infertility is a condition characterized by hearing loss and an inability to father children. (medlineplus.gov)
  • Affected individuals have moderate to severe sensorineural hearing loss, which is caused by abnormalities in the inner ear . (medlineplus.gov)
  • Sensorineural hearing loss or deafness occur due to problems with the auditory nerve or the part of the brain responsible for hearing. (umiamihealth.org)
  • In children with hearing loss or deafness, these problems are often caused by genetic conditions. (umiamihealth.org)
  • Sign language can help children with hearing loss and deafness communicate with others using their hands. (umiamihealth.org)
  • As the rate of acquired hearing loss secondary to environmental causes decreases and improvements in the diagnosis of abnormalities occur, the significance of genetic factors that lead to deafness increases. (nature.com)
  • Estimates of the different types of genetic deafness exceed 400, and to date, 60 genes for syndromic and nonsyndromic hearing loss have been identified. (nature.com)
  • The deafness segregating in family PKSN32 was not linked to any of the known recessive or dominant deafness loci (Hereditary Hearing Loss Homepage, http://dnalab-www.uia.ac.be/dnalab/hhh/ ). (bmj.com)
  • Sudden sensorineural hearing loss (SSNHL) is generally known as sudden deafness. (daijiworld.com)
  • In conclusion, Sudden sensorineural hearing loss is reversible. (daijiworld.com)
  • The major criteria are sensorineural hearing loss, iris pigmentary abnormality (two eyes different color or iris bicolor or characteristic brilliant blue iris), hair hypopigmentation (white forelock or white hairs at other sites on the body), dystopia canthorum (lateral displacement of inner canthi) and the presence of a first-degree relative previously diagnosed with WS. (bioline.org.br)
  • Cochlear Limited (ASX: COH), the global leader in implantable hearing solutions, introduces its next generation Cochlear™ Osia® System with the ability to have an MRI at 3.0 T, designed to improve hearing outcomes for people with conductive hearing loss, mixed hearing loss and single-sided sensorineural deafness (SSD). (cochlear.com)
  • Purpose of study: development and study of the therapeutic effect of combined optrotec- tant drugs, updating of molecular genetic and audiological diagnostics, as well as the establish- ment of prognostic factors for the effectiveness of hearing recovery in inflammatory diseases of the ear and sensorineural hearing loss (SNHL). (cnaa.md)
  • For instance, in August 2019, FDA approved MED-EL Cochlear Implant System for single-sided deafness and asymmetric hearing loss. (grandviewresearch.com)
  • The study took place over a period of 12 months from January 1, 2021, to December 31, 2021, and included patients with hearing loss and/or peripheral vertigo who underwent a pure-tone audiometry revealing deafness. (bvsalud.org)
  • A rare mitochondrial disease characterized by a variable phenotype comprising congenital sensorineural deafness intermittent or persistent hypoglycemia and hepatic and renal dysfunction potentially progressing to organ failure. (globalgenes.org)
  • Deafness in an animal is usually not a life-threatening disorder in the way of some renal disorders, and it is not a painful condition like hip dysplasia, but it does put an animal at risk from undetected dangers such as motor vehicles or predators, and deaf animals create their own liabilities and present non-trivial training challenges to their owners. (vin.com)
  • Molecular genetic tests are available for many types of syndromic and nonsyndromic deafness, although often only on a research basis. (nature.com)
  • The abnormal vestibular phenotype associated with ESPN mutations will be a useful clinical marker for refining the differential diagnosis of non-syndromic deafness. (bmj.com)
  • In human audiology deafness is also classified as syndromic (associated with other disorders) or nonsyndromic. (vin.com)
  • Genetic causes can be subdivided into syndromic (more than one symptom) and non-syndromic (deafness only). (batod.org.uk)
  • 2013). Non-syndromic sensorineural prelingual deafness: The importance of genetic counseling in demystifying parents' beliefs about the cause of their children's deafness . (up.pt)
  • odd) eyes, and several of these genes are not linked to masking, white coats or deafness (e.g. (wikipedia.org)
  • The signs and symptoms of sensorineural deafness and male infertility are related to the loss of multiple genes in this region. (medlineplus.gov)
  • Researchers are working to determine how the loss of additional genes in the deleted region affects people with sensorineural deafness and male infertility. (medlineplus.gov)
  • 1 Forty recessive deafness loci (DFNB) have been mapped and the genes responsible at 20 of these loci have been reported. (bmj.com)
  • Genetic deafness is caused by the baby's genes. (batod.org.uk)
  • Serious damage to the eardrum or the delicate bones inside the ear can cause sudden, permanent deafness. (prettylitter.com)
  • Congenital CMV (cCMV) causes about 10% to 20% of permanent deafness in children in the UK and is the leading cause of non-hereditary deafness. (ndcs.org.uk)
  • Sensorineural deafness and male infertility: a contiguous gene deletion syndrome. (medlineplus.gov)
  • Despite there being very little public awareness, CMV is more common than many other conditions associated with deafness such as Down's syndrome, spina bifida, toxoplasmosis and cystic fibrosis. (ndcs.org.uk)
  • Allport syndrome, which presents with both sensorineural deafness and kidney disease, is an example. (vin.com)
  • Table 1 indicates the other forms of deafness for which molecular genetic testing is currently available. (nature.com)
  • Because large numbers of deaf puppies and kittens get put down, it is important to identify animals affected by the hereditary forms of deafness B both unilaterally and bilaterally deaf B and remove them from the potential breeding pool to reduce the number of future deaf animals. (vin.com)
  • Forms of deafness can be sorted into three complimentary categories, each with two types. (vin.com)
  • The most commonly seen forms of deafness in companion animals are (1) hereditary congenital sensorineural deafness, (2) acquired later-onset sensorineural deafness, and (3) acquired later-onset conductive deafness. (vin.com)
  • 1 This structure is a major target of mechanisms producing deafness. (vin.com)
  • Acquired conductive deafness may result from otitis externa, otitis media, excess cerumen production, or congenital defects in the ossicles (rare). (vin.com)
  • A range of different diseases of the ear have the potential to cause deafness, for example, cholesteatoma, Meniere's disease, mastoiditis, and persistent otitis media with effusion (glue ear). (batod.org.uk)
  • Deafness depends on the cat's genotype (genetic make-up), and not its phenotype (physical appearance). (wikipedia.org)
  • Non-genetic deafness includes deafness caused by infections during pregnancy, infections the newborn baby has, trauma, prematurity, low birth weight, or hyperbilirubinemia. (batod.org.uk)
  • Genetic deafness can be passed on or inherited via DNA due to a mutated gene. (batod.org.uk)
  • Sensorineural deafness - This kind of deafness involves the hair-like nerve cells and other neural structures that send vibrations to the brain or even the brain itself, which interprets these signals as sound. (prettylitter.com)
  • Excessive exposure to loud noise, a head injury, or drugs that are toxic to nerve cells can all cause sensorineural deafness. (prettylitter.com)
  • Sensorineural (nerve) deafness is loss of auditory function because of loss of cochlear hair cells or auditory nerve neurons. (vin.com)
  • Please give me some medicine so that my nerve deafness should not worsen. (ndtv.com)
  • Severe sensorineural deafness after cerebral trauma by electroacupuncture and scalp acupuncture: a report of 19 cases]. (qigonginstitute.org)
  • Females with two chromosome 15 deletions in each cell have sensorineural deafness as their only symptom because the CATSPER2 gene deletions affect sperm function, and women do not produce sperm. (medlineplus.gov)
  • ESPN , a gene in the DFNB36 critical interval at 1p36.3, was a good positional candidate because a mutation of Espn is known to cause deafness and vestibular dysfunction in the jerker mouse. (bmj.com)
  • There may not be any known family history of deafness, depending on whether the gene is dominant or recessive. (batod.org.uk)
  • Deafness can occur in white cats with yellow, green or blue irises, although it is mostly likely in white cats with blue irises. (wikipedia.org)
  • Deafness may occur in cats for a variety of different reasons, either from birth or at any point in later life. (prettylitter.com)
  • Exclusion criteria: -Pateint having sensorineural deafness due to Traumatic injury. (who.int)
  • A maximum two-point LOD score of 4.1 (θ = 0) was obtained with marker D1S214 , defining a new recessive deafness locus, DFNB36 . (bmj.com)
  • Pro228Leu) variant and presenting with sensorineural deafness, developmental delay, and lactic acidosis. (bvsalud.org)
  • Detailed family history revealed that many of the family members had heterochromia iridis, premature canitis, white forelock, and sensory neural deafness in various combinations [Figure - 4] . (bioline.org.br)
  • The parents of an individual with sensorineural deafness and male infertility each carry one copy of the chromosome 15 deletion, but they do not show symptoms of the condition. (medlineplus.gov)
  • Males with two chromosome 15 deletions in each cell have sensorineural deafness and infertility. (medlineplus.gov)
  • We mapped a human deafness locus DFNB36 to chromosome 1p36.3 in two consanguineous families segregating recessively inherited deafness and vestibular areflexia. (bmj.com)
  • This is more common in children with asymptomatic (showing no other symptoms) cCMV, and some may develop deafness in the other ear later. (ndcs.org.uk)
  • He had been diagnosed with Alport during childhood in the context of a positive family history, sensorineural deafness, and progressive kidney dys-function. (aao.org)
  • it may actually be a condition called cat congenital sensorineural deafness. (prettylitter.com)
  • Noise-induced loss of hearing is an irreversible, sensorineural condition that progresses with exposure. (cdc.gov)
  • The cause of this disorder is not completely understood, but there is a correlation between the dog's coat color and deafness. (petmd.com)
  • Deafness is far more common in white cats than in those with other coat colours. (wikipedia.org)
  • In white cats with mixed-coloured eyes (odd-eyed cats), it has been found that deafness is more likely to affect the ear on the blue-eyed side. (wikipedia.org)
  • For example, solid white blue-eyed Foreign White or Ojos Azules cats are not linked to deafness. (wikipedia.org)
  • BAER-testing (Brainstem Auditory Evoked Response) is used to test deafness in cats. (wikipedia.org)
  • It's no coincidence that many (though not all) white cats suffer from congenital sensorineural deafness -- especially white cats with blue eyes. (prettylitter.com)
  • Half of all children with deafness caused by cCMV have a degree of deafness at birth and this may be picked up following newborn hearing screening. (ndcs.org.uk)
  • Half of the children whose deafness is caused by cCMV will have progressive or late onset deafness (becoming worse over time). (ndcs.org.uk)
  • It is estimated that outpatient department of Al-Khadra diagnoses and 7 (4%) had sensorineu- around 1 in 100 children between 5 Teaching Hospital where 25 to 30 chil- ral deafness (Table 1). (who.int)
  • In December 2019, the FDA approved the first active Osseointegrated steady-state implant which can be used for children 12 years and older with single-sided sensorineural deafness. (grandviewresearch.com)
  • While deafness does not directly affect a dog's quality of life, deaf Catahoula Leopard Dogs can be challenging to train and require an experienced home. (petmd.com)
  • The other half go on to develop deafness after birth and will have had no problems identified on their newborn hearing screen. (ndcs.org.uk)
  • Most deafness caused by cCMV develops during the first three years of life and so may affect speech and language development. (ndcs.org.uk)
  • Since the 2010s, researchers have been studying the molecular mechanisms underlying deafness of which certain types of so-called sensorineural deafness and. (pasteur.fr)
  • This results in eight possible combinations, such as acquired later-onset sensorineural deafness. (vin.com)