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Deaf-Blind Disorders

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The absence of both hearing and vision.

Also Known As:

Blindness-Deafness; Deaf-Blind Syndromes; Deaf-Blindness Disorders; Deaf-Mutism-Blind Disorders; Deafness-Blindness; Hearing and Vision Loss; Prelingual Deaf-Blind Disorders; Prelingual Deafness-Blindness; Vision and Hearing Loss; Blind Deaf Disorders; Blind-Deaf Disorder; Blindness Deafness; Deaf Blind Disorders; Deaf Blind Syndromes; Deaf Blindness Disorders; Deaf Mutism Blind Disorders; Deaf-Blind Disorder; Deaf-Blind Disorder, Prelingual; Deaf-Blind Disorders, Prelingual; Deaf-Blind Syndrome; Deaf-Blindness Disorder; Deaf-Mutism-Blind Disorder; Deafness Blindness; Deafness-Blindness, Prelingual; Deafness-Blindnesses, Prelingual; Disorder, Blind-Deaf; Disorder, Deaf-Blind; Disorder, Deaf-Blindness; Disorder, Deaf-Mutism-Blind; Disorder, Prelingual Deaf-Blind; Disorders, Blind-Deaf; Disorders, Deaf-Blind; Disorders, Deaf-Blindness; Disorders, Deaf-Mutism-Blind; Disorders, Prelingual Deaf-Blind; Prelingual Deaf Blind Disorders; Prelingual Deaf-Blind Disorder; Prelingual Deafness Blindness; Prelingual Deafness-Blindnesses; Syndrome, Deaf-Blind; Syndromes, Deaf-Blind; Blind-Deaf Disorders

Networked: 38 relevant articles (0 outcomes, 0 trials/studies)

Disease Context: Research Results

Related Diseases

1.	Cerebral Palsy (Spastic Diplegia)
2.	Usher Syndromes (Usher Syndrome)
3.	Retinopathy of Prematurity (Retrolental Fibroplasia)
4.	Infection
5.	Hemorrhage

Experts

1.	Ehrenkranz, Richard A: 3 articles (01/2010 - 01/2009)
2.	Halliday, Henry L: 3 articles (01/2010 - 01/2009)
3.	Doyle, Lex W: 3 articles (01/2010 - 01/2009)
4.	Doyle, L W: 3 articles (01/2003 - 01/2003)
5.	Ehrenkranz, R A: 3 articles (01/2003 - 01/2003)
6.	Halliday, H L: 3 articles (01/2003 - 01/2003)
7.	Williams, David S: 2 articles (07/2011 - 05/2003)
8.	Gibbs, Daniel: 2 articles (07/2011 - 05/2003)
9.	Petit, Christine: 2 articles (10/2005 - 08/2005)
10.	El-Amraoui, Aziz: 2 articles (10/2005 - 08/2005)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Deaf-Blind Disorders:

1.	ROPIBA 01/01/2005 - "Infants were divided into 3 groups: (A) normal development (n = 35; 48%), (B) mild-to-moderate impairment (IQ 68-83, or mild to moderate hearing loss or visual problems, or mild/moderate CP or ADHD) (n = 25; 36%) and (C) severe impairment (IQ < 68, deafness, blindness or severe CP) (n = 13; 18%) The studied groups differ in respect to: gestational age (mean +/- SEM: 29.7 +/- 2.3 vs 28.1 +/- 2.5 vs 28.5 +/- 2.3 wks; p = 0.03), prevalence of retinopathy (ROP) (17 vs 28 vs 69%; p < 0.01), bronchopulmonary dysplasia (BPD) (31 vs 17 vs 62%; p = 0.03) and periventricular leukomalacia (PVL) (3 vs 0 vs 23%; p < 0.02). " 01/01/2009 - "Data regarding clinical outcomes including mortality, CLD (including need for home oxygen, or need for late rescue with corticosteroids), death or CLD, failure to extubate, complications in the primary hospitalisation (including infection, hyperglycaemia, glycosuria, hypertension, echodensities on ultrasound scan of brain, necrotising enterocolitis (NEC), gastrointestinal (GI) bleeding, GI perforation, intraventricular hemorrhage (IVH), severe retinopathy of prematurity (ROP), and long-term outcomes (including blindness, deafness, cerebral palsy and major neurosensory disability), were abstracted and analysed using RevMan 5 Nineteen RCTs enrolling a total of 1345 participants were eligible for this review. " 01/01/2003 - "Data regarding clinical outcomes including mortality, CLD (including need for home oxygen, or need for late rescue with corticosteroids), death or CLD, failure to extubate, complications in the primary hospitalisation (including infection, hyperglycaemia, glycosuria, hypertension, echodensities on ultrasound scan of brain, necrotising enterocolitis (NEC), gastrointestinal bleeding, intestinal perforation, and severe retinopathy of prematurity (ROP)), and long term outcome (including blindness, deafness, cerebral palsy and major neurosensory disability), were abstracted and analysed using RevMan 4.1. " 01/01/2003 - "Data regarding clinical outcomes including mortality, CLD (including late rescue with corticosteroids, or need for home oxygen therapy), death or CLD, failure to extubate, complications during the primary hospitalisation (including infection, hyperglycaemia, hypertension, hypertrophic cardiomyopathy, pneumothorax, severe intraventricular haemorrhage (IVH), necrotizing enterocolitis (NEC), gastrointestinal bleeding, and severe retinopathy of prematurity (ROP)), and long term outcome (including blindness, deafness, cerebral palsy and major neurosensory disability), were abstracted and analysed using RevMan 4.1. " 01/01/2010 - "Data regarding clinical outcomes including mortality, CLD (including late rescue with corticosteroids, and need for home oxygen therapy), death or CLD, failure to extubate, complications during the primary hospitalisation (including infection, hyperglycaemia, hypertension, pulmonary air leak, patent ductus arteriosus (PDA), severe intraventricular haemorrhage (IVH), periventricular leucomalacia (PVL), necrotising enterocolitis (NEC), gastrointestinal bleeding, intestinal perforation, severe retinopathy of prematurity (ROP), and long-term outcome (including blindness, deafness, cerebral palsy and major neurosensory disability) were abstracted and analysed using RevMan 5. Twenty-eight RCTs enrolling a total of 3740 participants were eligible for inclusion in this review. "
2.	OxygenIBA 01/01/2009 - "Data regarding clinical outcomes including mortality, CLD (including need for home oxygen, or need for late rescue with corticosteroids), death or CLD, failure to extubate, complications in the primary hospitalisation (including infection, hyperglycaemia, glycosuria, hypertension, echodensities on ultrasound scan of brain, necrotising enterocolitis (NEC), gastrointestinal (GI) bleeding, GI perforation, intraventricular hemorrhage (IVH), severe retinopathy of prematurity (ROP), and long-term outcomes (including blindness, deafness, cerebral palsy and major neurosensory disability), were abstracted and analysed using RevMan 5 Nineteen RCTs enrolling a total of 1345 participants were eligible for this review. " 01/01/2003 - "Data regarding clinical outcomes including mortality, CLD (including need for home oxygen, or need for late rescue with corticosteroids), death or CLD, failure to extubate, complications in the primary hospitalisation (including infection, hyperglycaemia, glycosuria, hypertension, echodensities on ultrasound scan of brain, necrotising enterocolitis (NEC), gastrointestinal bleeding, intestinal perforation, and severe retinopathy of prematurity (ROP)), and long term outcome (including blindness, deafness, cerebral palsy and major neurosensory disability), were abstracted and analysed using RevMan 4.1. " 01/01/2003 - "Data regarding clinical outcomes including mortality, CLD (including late rescue with corticosteroids, or need for home oxygen therapy), death or CLD, failure to extubate, complications during the primary hospitalisation (including infection, hyperglycaemia, hypertension, hypertrophic cardiomyopathy, pneumothorax, severe intraventricular haemorrhage (IVH), necrotizing enterocolitis (NEC), gastrointestinal bleeding, and severe retinopathy of prematurity (ROP)), and long term outcome (including blindness, deafness, cerebral palsy and major neurosensory disability), were abstracted and analysed using RevMan 4.1. " 01/01/2010 - "Data regarding clinical outcomes including mortality, CLD (including late rescue with corticosteroids, and need for home oxygen therapy), death or CLD, failure to extubate, complications during the primary hospitalisation (including infection, hyperglycaemia, hypertension, pulmonary air leak, patent ductus arteriosus (PDA), severe intraventricular haemorrhage (IVH), periventricular leucomalacia (PVL), necrotising enterocolitis (NEC), gastrointestinal bleeding, intestinal perforation, severe retinopathy of prematurity (ROP), and long-term outcome (including blindness, deafness, cerebral palsy and major neurosensory disability) were abstracted and analysed using RevMan 5. Twenty-eight RCTs enrolling a total of 3740 participants were eligible for inclusion in this review. " 01/01/2009 - "Data regarding clinical outcomes including mortality, CLD (including late rescue with corticosteroids, and need for home oxygen therapy), death or CLD, failure to extubate, complications during the primary hospitalisation (including infection, hyperglycaemia, hypertension, pulmonary air leak, patent ductus arteriosus (PDA), severe intraventricular haemorrhage (IVH), periventricular leucomalacia (PVL), necrotising enterocolitis (NEC), gastrointestinal bleeding, intestinal perforation, severe retinopathy of prematurity (ROP), and long-term outcome (including blindness, deafness, cerebral palsy and major neurosensory disability) were abstracted and analysed using RevMan 5. Twenty-eight RCTs enrolling a total of 3740 participants were eligible for inclusion in this review. "
3.	Adrenal Cortex Hormones (Corticosteroids)IBA 01/01/2009 - "Data regarding clinical outcomes including mortality, CLD (including need for home oxygen, or need for late rescue with corticosteroids), death or CLD, failure to extubate, complications in the primary hospitalisation (including infection, hyperglycaemia, glycosuria, hypertension, echodensities on ultrasound scan of brain, necrotising enterocolitis (NEC), gastrointestinal (GI) bleeding, GI perforation, intraventricular hemorrhage (IVH), severe retinopathy of prematurity (ROP), and long-term outcomes (including blindness, deafness, cerebral palsy and major neurosensory disability), were abstracted and analysed using RevMan 5 Nineteen RCTs enrolling a total of 1345 participants were eligible for this review. " 01/01/2003 - "Data regarding clinical outcomes including mortality, CLD (including need for home oxygen, or need for late rescue with corticosteroids), death or CLD, failure to extubate, complications in the primary hospitalisation (including infection, hyperglycaemia, glycosuria, hypertension, echodensities on ultrasound scan of brain, necrotising enterocolitis (NEC), gastrointestinal bleeding, intestinal perforation, and severe retinopathy of prematurity (ROP)), and long term outcome (including blindness, deafness, cerebral palsy and major neurosensory disability), were abstracted and analysed using RevMan 4.1. " 01/01/2003 - "Data regarding clinical outcomes including mortality, CLD (including late rescue with corticosteroids, or need for home oxygen therapy), death or CLD, failure to extubate, complications during the primary hospitalisation (including infection, hyperglycaemia, hypertension, hypertrophic cardiomyopathy, pneumothorax, severe intraventricular haemorrhage (IVH), necrotizing enterocolitis (NEC), gastrointestinal bleeding, and severe retinopathy of prematurity (ROP)), and long term outcome (including blindness, deafness, cerebral palsy and major neurosensory disability), were abstracted and analysed using RevMan 4.1. " 01/01/2010 - "Data regarding clinical outcomes including mortality, CLD (including late rescue with corticosteroids, and need for home oxygen therapy), death or CLD, failure to extubate, complications during the primary hospitalisation (including infection, hyperglycaemia, hypertension, pulmonary air leak, patent ductus arteriosus (PDA), severe intraventricular haemorrhage (IVH), periventricular leucomalacia (PVL), necrotising enterocolitis (NEC), gastrointestinal bleeding, intestinal perforation, severe retinopathy of prematurity (ROP), and long-term outcome (including blindness, deafness, cerebral palsy and major neurosensory disability) were abstracted and analysed using RevMan 5. Twenty-eight RCTs enrolling a total of 3740 participants were eligible for inclusion in this review. " 01/01/2009 - "Data regarding clinical outcomes including mortality, CLD (including late rescue with corticosteroids, and need for home oxygen therapy), death or CLD, failure to extubate, complications during the primary hospitalisation (including infection, hyperglycaemia, hypertension, pulmonary air leak, patent ductus arteriosus (PDA), severe intraventricular haemorrhage (IVH), periventricular leucomalacia (PVL), necrotising enterocolitis (NEC), gastrointestinal bleeding, intestinal perforation, severe retinopathy of prematurity (ROP), and long-term outcome (including blindness, deafness, cerebral palsy and major neurosensory disability) were abstracted and analysed using RevMan 5. Twenty-eight RCTs enrolling a total of 3740 participants were eligible for inclusion in this review. "
4.	myosin VIIaIBA 08/01/2005 - "Defects in myosin VIIa, the PDZ-domain-containing protein harmonin, cadherin 23, protocadherin 15, and the putative scaffolding protein sans, underlie five genetic forms of Usher syndrome type I (USH1), the most frequent cause of hereditary deafness-blindness in humans. " 08/01/1999 - "Two types of Usher syndrome, a blindness-deafness disorder, result from mutations in the myosin VIIa gene. " 10/15/2005 - "Defects in myosin VIIa, the PDZ-domain-containing protein harmonin, cadherin 23 and protocadherin 15 (two cadherins with large extracellular regions), and the putative scaffolding protein Sans underlie five genetic forms of Usher syndrome type I (USH1), the most frequent cause of hereditary deafness-blindness in humans. " 05/27/2003 - "Mutations in the myosin VIIa gene (MYO7A) cause Usher syndrome type 1B (USH1B), a major type of the deaf-blind disorder, Usher syndrome. " 01/01/1997 - "Recently, mutations in the gene for an unconventional myosin, myosin-VIIa, were found to be the basis for the deafness and vestibular dysfunction observed in shaker-1 (sh1) mice and for a human deafness-blindness syndrome, Usher syndrome type 1B. "
5.	Retinaldehyde (Retinal)IBA 07/01/2011 - "Mutations in the MYO7A gene cause a deaf-blindness disorder, known as Usher syndrome 1B. In the retina, the majority of MYO7A is in the retinal pigmented epithelium (RPE), where many of the reactions of the visual retinoid cycle take place. We have observed that the retinas of Myo7a-mutant mice are resistant to acute light damage. " 07/01/1995 - "Also the syndrome with encephalopathy, deafness, blindness and ataxia in young women is attributed to microangiopathy with small brain infarcts and retinal infarcts. " 09/01/1996 - "Blindness, deafness, quadriparesis, and a retinal malformation: the ravages of neurofibromatosis 2." 01/01/2013 - "Mutations of BM proteins from worms to humans are either embryonic lethal or result in severe diseases, including muscular dystrophy, blindness, deafness, kidney defects, cardio-vascular abnormalities or retinal and cortical malformations. "
6.	Norrie diseaseIBA 10/01/1992 - "A candidate gene for Norrie disease, an X-linked disorder characterized by blindness, deafness and mental disturbances, was recently isolated and found to contain microdeletions in numerous patients. " 06/01/2002 - "Norrie disease is an X-linked recessive syndrome of blindness, deafness, and mental retardation. " 12/01/1993 - "The X-lined gene for Norrie disease, which is characterized by blindness, deafness and mental retardation has been cloned recently. "
7.	Protein Isoforms (Isoforms)IBA 12/15/2015 - "Mutations in this gene disrupt its various isoforms and lead to non-syndromic deafness, blindness and deaf-blindness. " 01/01/2014 - "The 10 different genes associated with the deaf/blind disorder, Usher syndrome, encode a number of structurally and functionally distinct proteins, most expressed as multiple isoforms/protein variants. "
8.	Proteins (Proteins, Gene)IBA 01/01/2014 - "The 10 different genes associated with the deaf/blind disorder, Usher syndrome, encode a number of structurally and functionally distinct proteins, most expressed as multiple isoforms/protein variants. " 01/01/2013 - "Mutations of BM proteins from worms to humans are either embryonic lethal or result in severe diseases, including muscular dystrophy, blindness, deafness, kidney defects, cardio-vascular abnormalities or retinal and cortical malformations. "
9.	Myosins (Myosin)IBA 04/01/2014 - "Usher syndrome 1B (USH1B) is a severe, autosomal recessive, deaf-blind disorder caused by mutations in myosin 7A (MYO7A). " 01/01/1997 - "Recently, mutations in the gene for an unconventional myosin, myosin-VIIa, were found to be the basis for the deafness and vestibular dysfunction observed in shaker-1 (sh1) mice and for a human deafness-blindness syndrome, Usher syndrome type 1B. "
10.	DNA (Deoxyribonucleic Acid)IBA 02/01/2012 - "To investigate whether DNA damage can contribute to age-related hearing and vision loss, we investigated hearing and vision in Ercc1(δ/-) mutant mice, which are deficient in DNA repair of helix-distorting DNA lesions and interstrand DNA crosslinks. " 04/15/2003 - "These observations explain the phenotype in the mild form of Yemenite deaf-blind syndrome caused by specific SOX10 mutations in the HMG box that abrogate DNA binding without disrupting association with Pax3."

Therapies and Procedures

1.	Prostheses and Implants (Prosthesis) 12/01/2005 - "Sensory cortical prostheses have potential to aid people suffering from blindness, deafness and other sensory deficits. " 03/01/1977 - "Other rubella consequences such as blindness, deafness, and cardiac and neuromuscular defects remained present except as modified by operations and prostheses. "
2.	Length of Stay 01/01/2015 - "Survival free from neurodevelopmental disability at 2 years' corrected age, where neurodevelopmental disability is defined as cerebral palsy, blindness, deafness, developmental delay (standardised score more than 1 SD below the mean on the cognitive, language or motor subscales of the Bayley Scales of Infant Development Edition 3), or Gross Motor Function Classification System score ≥ 1. Growth, from birth to 36 weeks' corrected gestational age, at neonatal intensive care discharge and at 2 years' corrected age; body composition at 36 to 42 weeks' corrected postmenstrual age and at 2 years' corrected age; neonatal morbidity, including length of stay; nutritional intake. " 05/07/2014 - "Secondary and safety outcomes included invasive candidiasis, liver function, bacterial infection, length of stay, intracranial hemorrhage, periventricular leukomalacia, chronic lung disease, patent ductus arteriosus requiring surgery, retinopathy of prematurity requiring surgery, necrotizing enterocolitis, spontaneous intestinal perforation, and neurodevelopmental outcomes-defined as a Bayley-III cognition composite score of less than 70, blindness, deafness, or cerebral palsy at 18 to 22 months corrected age. "
3.	Activities of Daily Living (ADL) 06/01/2013 - "We conducted a secondary analysis of the 2006 American Community Survey using a representative sample of older Filipinos (2,113 males; 3,078 females) to measure functional limitations, limitations in activities of daily living, blindness/deafness and memory/learning problems. " 05/01/1999 - "This paper examines the moderating effects of three psychological attributes (neuroticism, self-efficacy expectancies and mastery) on the association between functional limitations (motor and cognitive limitation, vision and hearing loss) and disability ((instrumental) activities of daily living, role function and social function) in a sample of 624 community-dwelling older persons. "
4.	Neonatal Intensive Care 01/01/2015 - "Survival free from neurodevelopmental disability at 2 years' corrected age, where neurodevelopmental disability is defined as cerebral palsy, blindness, deafness, developmental delay (standardised score more than 1 SD below the mean on the cognitive, language or motor subscales of the Bayley Scales of Infant Development Edition 3), or Gross Motor Function Classification System score ≥ 1. Growth, from birth to 36 weeks' corrected gestational age, at neonatal intensive care discharge and at 2 years' corrected age; body composition at 36 to 42 weeks' corrected postmenstrual age and at 2 years' corrected age; neonatal morbidity, including length of stay; nutritional intake. "
5.	Nursing Care 09/01/2012 - "The Self-Assessment Instrument of Competencies for Cross-Cultural Nursing Care to Disabled People was constructed, specifically deafness, blindness or low sight, physical impairment and mental impairment, regarding greeting, accepting, helping, knowing and advocating. "