Hearing loss is a partial or total inability to hear. Hearing loss may be present at birth or acquired at any time afterwards. Hearing loss may occur in one or both ears. In children, hearing problems can affect the ability to acquire spoken language, and in adults it can create difficulties with social interaction and at work. Hearing loss can be temporary or permanent. Hearing loss related to age usually affects both ears and is due to cochlear hair cell loss. In some people, particularly older people, hearing loss can result in loneliness.
Usher syndrome, also known as Hallgren syndrome, Usher–Hallgren syndrome, retinitis pigmentosa–dysacusis syndrome or dystrophia retinae dysacusis syndrome, is a rare genetic disorder caused by a mutation in any one of at least 11 genes resulting in a combination of hearing loss and visual impairment. It is the most common cause of deafblindness and is at present incurable.
Pendred syndrome is a genetic disorder leading to congenital bilateral sensorineural hearing loss and goitre with euthyroid or mild hypothyroidism. There is no specific treatment, other than supportive measures for the hearing loss and thyroid hormone supplementation in case of hypothyroidism. It is named after Vaughan Pendred (1869–1946), the British doctor who first described the condition in an Irish family living in Durham in 1896. It accounts for 7.5% to 15% of all cases of congenital deafness.
Norrie disease is a rare X-linked recessive genetic disorder that primarily affects the eyes and almost always leads to blindness. It is caused by mutations in the Norrin cystine knot growth factor gene, also referred to as Norrie Disease Pseudoglioma (NDP) gene. Norrie disease manifests with vision impairment either at birth, or within a few weeks of life, following an ocular event like retinal detachment and is progressive through childhood and adolescence. It generally begins with retinal degeneration, which occurs before birth and results in blindness at birth (congenital) or early infancy, usually by 3 months of age.
Doda district is an administrative district of the Jammu division of Indian-administered Jammu and Kashmir in the disputed Kashmir region. Doda covers 2,625 square kilometers.
Bhaderwah or Bhadarwah is a town, tehsil, and sub-district in the Doda district of Jammu Division of Jammu and Kashmir, India. Bhaderwah Valley is famous for it's beauty, nature. Bhaderwah valley is known as 'Land Of Snakes'. Bhaderwah is also a land of fairs and festivals like Mela Pat, Subar Dhar Mela, Kud dance, Pahari folk songs and music. It also has heritage sites like the Fort, hundered years old mosque and an ancient Vasuki Nag Temple.
Nonsyndromic deafness is hearing loss that is not associated with other signs and symptoms. In contrast, syndromic deafness involves hearing loss that occurs with abnormalities in other parts of the body. Nonsyndromic deafness constitutes 75% of all hearing loss cases, and an estimated 100 genes are thought to be linked to this condition. About 80% are linked to autosomal recessive inheritance, 15% to autosomal dominant inheritance, 1-3% through the X chromosome, and 0.5-1% are associated with mitochondrial inheritance.
Kata Kolok, also known as Benkala Sign Language and Balinese Sign Language, is a village sign language which is indigenous to two neighbouring villages in northern Bali, Indonesia. The main village, Bengkala, has had high incidences of deafness for over seven generations. Notwithstanding the biological time depth of the recessive mutation that causes deafness, the first substantial cohort of deaf signers did not occur until five generations ago, and this event marks the emergence of Kata Kolok. The sign language has been acquired by at least five generations of deaf, native signers and features in all aspects of village life, including political, professional, educational, and religious settings.
Pendrin is an anion exchange protein that in humans is encoded by the SLC26A4 gene . Pendrin was initially identified as a sodium-independent chloride-iodide exchanger with subsequent studies showing that it also accepts formate and bicarbonate as substrates. Pendrin is similar to the Band 3 transport protein found in red blood cells. Pendrin is the protein which is mutated in Pendred syndrome, which is an autosomal recessive disorder characterized by sensorineural hearing loss, goiter and a partial organification problem detectable by a positive perchlorate test.
The 2006 Doda massacre was a mass murder of 57 Hindu civilians in two separate incidents, allegedly carried out by Lashkar-e-Taiba militants in the Doda district of Jammu and Kashmir, India on 30 April 2006.
Gap junction beta-3 protein (GJB3), also known as connexin 31 (Cx31) — is a protein that in humans is encoded by the GJB3 gene.
Alpha-tectorin is a protein that in humans is encoded by the TECTA gene.
Otoferlin is a protein that in humans is encoded by the OTOF gene. It is involved in vesicle membrane fusion, and mutations in the OTOF gene are associated with a genetic form of deafness.
A series of massacres of Hindus in May–August 2001 by Islamic militants took place in the erstwhile Doda district of Jammu and Kashmir, India, wherein 43 Hindus were killed. The massacres took place at villages and temporary summer camps called dhoks in remote meadows used by local shepherds. The massacres were committed by members of Lashkar-e-Toiba, a Pakistan-based terrorist group. One massacre is believed to have been instigated by local Muslim shepherd tenants involved in a dispute over the pastures. The massacres triggered tensions across the Jammu region.
Chenab Valley is a term refers to parts of the Jammu Division of Jammu and Kashmir, India. It is used to refer to the present-day districts of Doda, Kishtwar, Ramban. These three districts used to be part of a single former district called Doda, which was created in 1948 out of the eastern parts of Udhampur district of the princely state of Jammu and Kashmir, and are sometimes collectively referred to as the Doda belt. The term is seen to be aimed at a communal break-up of the Jammu Division and iteration of Kashmiri Muslim irredentism.
Thathri is a town and a notified area committee in Doda district in the Indian union territory of Jammu and Kashmir. Thathri valley is located in the foothills of the Himalayan mountains, about 85 km from Batote. Apart from having ample forests, the town contains many small streams flowing through its various parts. It is located on the banks of the Chenab River and the town area is spread over 1.50 sq. Km².
Village Defence Guards (VDGs) formerly known as Village Defence Committees is a civilian militia first established in the mid-1990s in Indian-administered Jammu and Kashmir for the self-defence of locals, especially minorities such as Hindus, sikhs and vulnerable section of Muslims in remote hilly villages against militancy. It consist of villagers as well as police officers.
Chinta Valley is a valley and a village located 20 kilometres northeast of Bhaderwah town on Bhaderwah-Jai road. It is covered with thick coniferous forests from all sides with a stream flowing through it known as Chinta Nallah. A village called Thuba divides the valley from Bhaderwah. Chinta Valley is known for it's beauty and it is famous place for tourist.
Kahara is a village and tehsil in the Doda district of the Jammu division of the Indian union territory of Jammu and Kashmir. It is situated between mountainous cliffs 13 kilometres (8.1 mi) from Thathri on the Thathri-Gandoh road.
Wolfram-like syndrome is a rare autosomal dominant genetic disorder that shares some of the features shown by those affected with the autosomal recessive Wolfram syndrome. It is a type of WFS1-related disorder.
