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Ontology Browser

Term:
Opticocochleodentate Degeneration (DOID:9002585)
Annotations: Rat: (0) Mouse: (0) Human: (0) Chinchilla: (0) Bonobo: (0) Dog: (0) Squirrel: (0) Pig: (0)
Parent Terms Term With Siblings Child Terms
Deafness +     
Muscle Spasticity +     
quadriplegia +     
speech disorder +     
3-methylglutaconic aciduria with deafness, encephalopathy, and Leigh-like syndrome  
Absence of Tibia with Congenital Deafness 
Absent Eyebrows and Eyelashes with Mental Retardation 
AGAT deficiency  
Albinism Deafness Syndrome 
Alexander Disease  
aphasia +   
Arthrogryposis Multiplex with Deafness, Inguinal Hernias, and Early Death 
articulation disorder +   
Atonic-Astatic Syndrome of Foerster 
autosomal dominant mental retardation 20  
autosomal dominant nonsyndromic deafness 65  
Ayazi Syndrome 
Branchiogenic-Deafness Syndrome 
Burn-McKeown syndrome  
Canavan disease  
Cardioauditory Syndrome of Sanchez Cascos 
Cerebrocortical Degeneration of Infancy 
Cerebroretinal Microangiopathy with Calcifications and Cysts +   
childhood spinal muscular atrophy +   
Childhood-Onset Spasticity with Hyperglycinemia  
chromosome 17p13.1 deletion syndrome 
Cockayne syndrome +   
COLOBOMA, OSTEOPETROSIS, MICROPHTHALMIA, MACROCEPHALY, ALBINISM, AND DEAFNESS  
Congenital Deafness and Familial Myoclonic Epilepsy 
Congenital Deafness, and Onychodystrophy, Autosomal Dominant  
Congenital Deafness, with Vitiligo and Achalasia 
Congenital Emphysema, with Deafness, Penoscrotal Web, and Mental Retardation 
Congenital Muscular Dystrophy with Severe Central Nervous System Atrophy and Absence of Large Myelinated Fibers 
Congenital Myopathy with Neuropathy and Deafness  
Coxoauricular Syndrome 
Davenport Donlan Syndrome 
Deaf-Blind Disorders +   
Deafness Hyperuricemia Neurologic Ataxia 
Deafness with Anhidrotic Ectodermal Dysplasia 
Deafness, Autosomal Dominant, due to Mutation In Myo1a 
Deafness, Cataract, Retinitis Pigmentosa, And Sperm Abnormalities 
Deafness, Congenital Onychodystrophy, Recessive Form 
Deafness, with Smith-Magenis Syndrome  
Diaminopentanuria 
Dislocated Elbows, Bowed Tibias, Scoliosis, Deafness, Cataract, Microcephaly, and Mental Retardation 
dystonia 9  
Dystonia Musculorum Deformans +   
echolalia 
Encephalopathy, Spastic Tetraparesis, and Hypogonadism 
Epiphyseal Dysplasia of Femoral Head, Myopia, and Deafness 
Familial Amyloid Polyneuropathies +   
familial encephalopathy with neuroserpin inclusion bodies  
Fine-Lubinsky Syndrome 
Fitzsimmons Walson Mellor Syndrome 
Focal Epilepsy with Speech Disorder and with or without Mental Retardation  
Fountain Syndrome 
Gerstmann-Straussler-Scheinker syndrome  
Gilles de la Tourette syndrome +   
Hereditary Central Nervous System Demyelinating Diseases +   
Hereditary Optic Atrophies +   
hereditary sensory neuropathy +   
hereditary spastic paraplegia 24 
Herrmann Syndrome 
high myopia-sensorineural deafness syndrome  
Hirschsprung Disease with Polydactyly, Renal Agenesis, and Deafness 
Huntington's disease +   
Huntington's disease-like 2  
Huntington's Disease-Like Syndrome  
Hyperlipoproteinemia Type II, and Deafness 
HYPERTELORISM, PREAURICULAR SINUS, PUNCTAL PITS, AND DEAFNESS 
Ichthyosis, Spastic Quadriplegia, and Mental Retardation  
ITM2B-related cerebral amyloid angiopathy 2  
Johnson Neuroectodermal Syndrome 
Jones Syndrome 
Keipert syndrome  
Kennedy's disease  
Keratitis-Ichthyosis-Deafness Syndrome +   
Konigsmark Knox Hussels Syndrome 
Koone Rizzo Elias Syndrome 
Lafora disease  
Leukoencephalomyelopathy  
Lipodystrophy with Congenital Cataracts and Neurodegeneration  
locked-in syndrome 
Lynch Lee Murday syndrome 
Mandibular Hypoplasia, Deafness, Progeroid Features, and Lipodystrophy Syndrome  
Maxillofacial Dysostosis 
Mental Retardation, X-Linked +   
Mental Retardation-Hypotonic Facies Syndrome, X-Linked, 1  
Methylenetetrahydrofolate Reductase Deficiency  
Meyenburg-Altherr-Uehlinger Syndrome 
Microcephaly Deafness Syndrome 
Microcephaly with Spastic Quadriplegia 
mitochondrial DNA depletion syndrome 6  
motor peripheral neuropathy +   
Muckle-Wells syndrome  
Multiple Synostoses Syndrome 1  
mutism  
Myoclonic Epilepsy, Congenital Deafness, Macular Dystrophy, and Psychiatric Disorders 
Myoclonus, Cerebellar Ataxia, and Deafness 
myotonia congenita +   
myotonic dystrophy type 1 +   
Nathalie Syndrome 
Nephrosis Deafness Urinary Tract Digital Malformation 
neuroacanthocytosis +   
NEURODEVELOPMENTAL DISORDER WITH BEHAVIORAL ABNORMALITIES, ABSENT SPEECH, AND HYPOTONIA  
NEURODEVELOPMENTAL DISORDER WITH CEREBELLAR HYPOPLASIA AND SPASTICITY  
NEURODEVELOPMENTAL DISORDER WITH EPILEPSY, SPASTICITY, AND BRAIN ATROPHY  
Neurodevelopmental Disorder with Impaired Speech and Hyperkinetic Movements  
Neurodevelopmental Disorder with Microcephaly, Cortical Malformations, and Spasticity  
NEURODEVELOPMENTAL DISORDER WITH SPASTICITY AND POOR GROWTH  
neurofibromatosis +   
neurogenic scapuloperoneal syndrome Kaeser type  
neuronal ceroid lipofuscinosis +   
Non-Lissencephalic Cortical Dysplasia 
Noninsulin-Dependent Diabetes Mellitus with Deafness  
nonsyndromic deafness +   
Okur-Chung Neurodevelopmental Syndrome  
Opticocochleodentate Degeneration 
pantothenate kinase-associated neurodegeneration +   
PCWH syndrome  
Peripheral Neuropathy, Ataxia, Focal Necrotizing Encephalopathy, and Spongy Degeneration of Brain 
Perrault syndrome +   
Popov-Chang Syndrome 
Powell Venencie Gordon syndrome 
Premature Atherosclerosis with Deafness, Nephropathy, Diabetes Mellitus, Photomyoclonus, and Degenerative Neurologic Disease 
primary cerebellar degeneration +   
Progressive Psychomotor Deterioration  
Ramos Arroyo Clark Syndrome 
Ribbonlike Corneal Degeneration with Deafness 
Richards-Rundle Syndrome 
Rolandic Epilepsy, Mental Retardation, and Speech Dyspraxia, X-Linked  
Schimke X-Linked Mental Retardation Syndrome 
Schlegelberger Grote Syndrome 
Secretory Diarrhea, Myopathy, and Deafness 
Seemanova Lesny Syndrome 
Severe Mental Retardation, with Spasticity and Pigmentary Tapetoretinal Degeneration 
SIDDIQI SYNDROME  
Sinoatrial Node Dysfunction and Deafness  
spastic ataxia +   
Spastic Ataxia with Congenital Miosis 
Spastic Quadriplegia, Retinitis Pigmentosa, and Mental Retardation 
Speech Disturbance - Use of Faulty Phrasing and Unrelated Words 
speech-language disorder-1  
spinal muscular atrophy with lower extremity predominance +   
Spongiform Encephalopathy with Neuropsychiatric Features  
syndromic microphthalmia 10 
syndromic X-linked intellectual disability Turner type  
temtamy preaxial brachydactyly syndrome  
Tietz syndrome  
tuberous sclerosis +   
Unverricht-Lundborg syndrome +   
Webb-Dattani Syndrome  
Wells Jankovic Syndrome 
Wilson disease +   
Wright Dyck Syndrome 
X-linked Alport syndrome  
X-linked intellectual disability-psychosis-macroorchidism syndrome  
X-Linked Mental Retardation Gustavson Type 
X-Linked Retinitis Pigmentosa, and Sinorespiratory Infections, with or without Deafness  
Yemenite Deaf-Blind Hypopigmentation Syndrome 

Synonyms
Primary IDs: MESH:C563002 ;   RDO:0012467

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RGD is funded by grant HL64541 from the National Heart, Lung, and Blood Institute on behalf of the NIH.