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Hearing Loss, Sensorineural
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Definition
: Hearing loss resulting from damage to the COCHLEA and the sensorineural elements which lie internally beyond the oval and round windows. These elements include the AUDITORY NERVE and its connections in the BRAINSTEM.
HEARING LOSS, MIXED CONDUCTIVE-SENSORINEURAL is also available
Examples
Hearing Loss, Central
;
Hearing Loss, Noise-Induced
;
Presbycusis
;
Usher Syndromes
Other names
Sensorineural Hearing Loss; Hearing Loss, Cochlear; Cochlear Hearing Loss
Substance
CAS Registry & name
Categories
Source
SeSAME syndrome
0
*Hearing Loss, Sensorineural
*Intellectual Disability
*Seizures.
Hittner Hirsch Kreh syndrome
0
*Abnormalities, Multiple
*Coloboma
*Hearing Loss, Sensorineural
*Heart Defects, Congenital
*Intellectual Disability
*Microphthalmos
Ear/abnormalities.
Histiocytosis with joint contractures and sensorineural deafness
0
*Contracture
*Hearing Loss, Sensorineural
*Histiocytosis.
Cataract ataxia deafness
0
*Ataxia
*Cataract/congenital
*Hearing Loss, Sensorineural
*Peripheral Nervous System Diseases.
Deafness, autosomal dominant nonsyndromic sensorineural 24
0
*Hearing Loss, Sensorineural.
Deafness, autosomal dominant nonsyndromic sensorineural 23
0
*Hearing Loss, Sensorineural.
Deafness, autosomal dominant nonsyndromic sensorineural 22
0
*Hearing Loss, Sensorineural.
Charcot-
Marie-
Tooth disease and deafness
0
*Charcot-Marie-Tooth Disease
*Hearing Loss, Sensorineural.
Deafness, autosomal dominant nonsyndromic sensorineural 17
0
*Hearing Loss, Sensorineural.
Deafness oligodontia syndrome
0
*Anodontia
*Hearing Loss, Sensorineural.
Boudhina Yedes Khiari syndrome
0
*Epilepsy
*Growth Disorders
*Hearing Loss, Sensorineural
*Intellectual Disability
*Microcephaly
*Skin Diseases.
Fitzsimmons Walson Mellor syndrome
0
*Glomerulonephritis, IGA
*Hearing Loss, Sensorineural
*Intellectual Disability
*Muscle Spasticity
*Spastic Paraplegia, Hereditary.
Barakat syndrome
0
*Hearing Loss, Sensorineural
*Hypoparathyroidism
*Nephrosis.
Pfeiffer Kapferer syndrome
0
*Abnormalities, Multiple
*Growth Disorders
*Hearing Loss, Sensorineural
*Intellectual Disability.
Nonsyndromic sensorineural hearing loss
0
*Hearing Loss, Sensorineural.
Axenfeld-
Rieger anomaly with cardiac defects and sensorineural hearing loss
0
*Abnormalities, Multiple
*Eye Abnormalities
*Hearing Loss, Sensorineural
*Heart Septal Defects, Atrial.
Gemignani syndrome
0
*Hearing Loss, Sensorineural
*Paraparesis, Spastic
*Spinocerebellar Ataxias.
Bjornstad syndrome
0
*Hair Diseases
*Hearing Loss, Sensorineural.
Stickler syndrome, type 1
0
*Arthritis
*Connective Tissue Diseases
*Hearing Loss, Sensorineural
*Retinal Detachment.
Griscelli syndrome type 1
0
*Hearing Loss, Sensorineural
*Pigmentation Disorders
*Piebaldism.
Gonadal dysgenesis XX type deafness
0
*Hearing Loss, Sensorineural
*Gonadal Dysgenesis, 46,XX.
Knuckle pads, leuconychia and sensorineural deafness
0
*Abnormalities, Multiple
*Hearing Loss, Sensorineural
*Keratoderma, Palmoplantar.
Optic atrophy 1 and deafness
0
*Hearing Loss, Sensorineural
*Optic Atrophies, Hereditary.
Brown-
Vialetto-
Van Laere syndrome
0
*Hearing Loss, Sensorineural
*Bulbar Palsy, Progressive.
Fechtner syndrome
0
*Hearing Loss, Sensorineural
*Nephritis, Hereditary
*Thrombocytopenia.
Flynn Aird syndrome
0
*Cataract
*Central Nervous System Diseases
*Dental Caries
*Hearing Loss, Sensorineural
*Joint Diseases
*Retinitis Pigmentosa
*Skin Diseases.
J Neurol Sci 2(2):161-82
Albinism ocular late onset sensorineural deafness
0
*Hearing Loss, Sensorineural
*Albinism, Ocular.
Townes-
Brocks syndrome
0
*Abnormalities, Multiple
*Anus, Imperforate
*Hearing Loss, Sensorineural
Thumb/abnormalities.
Tunglang Savage Bellman syndrome
0
*Hearing Loss, Sensorineural
*Pseudohypoaldosteronism
*Salivary Gland Diseases.
Winkelman Bethge Pfeiffer syndrome
0
*Dwarfism, Pituitary
*Hearing Loss, Sensorineural.
Pendred syndrome
0
*Goiter, Nodular
*Hearing Loss, Sensorineural.
Schaap Taylor Baraitser syndrome
0
*Cataract
*Growth Disorders
*Hearing Loss, Sensorineural
*Hypertrichosis.
Treft Sanborn Carey syndrome
0
*Ataxia
*Blepharoptosis
*Hearing Loss, Sensorineural
*Muscular Diseases
*Ophthalmoplegia
*Optic Atrophy.
Thiamine responsive megaloblastic anemia syndrome
0
*Anemia, Megaloblastic
*Diabetes Mellitus
*Hearing Loss, Sensorineural
Ketoglutarate Dehydrogenase Complex/deficiency.
Vohwinkel syndrome
0
*Abnormalities, Multiple
*Hand Deformities, Congenital
*Hearing Loss, Sensorineural
*Keratoderma, Palmoplantar.
Craniofacial deafness hand syndrome
0
*Abnormalities, Multiple
*Hearing Loss, Sensorineural
*Craniofacial Abnormalities.
Cowchock syndrome
0
*Charcot-Marie-Tooth Disease
*Hearing Loss, Sensorineural
*Mental Retardation, X-Linked.
Cochleosaccular degeneration of the inner ear and progressive cataracts
0
*Cataract
*Hearing Loss, Sensorineural.
Cleft palate, midfacial hypoplasia, triangular facies, and sensorineural hearing loss
0
*Abnormalities, Multiple
*Cleft Palate
*Hearing Loss, Sensorineural
*Facies
*Craniofacial Abnormalities.
Progressive hearing loss stapes fixation
0
*Hearing Loss, Conductive
*Hearing Loss, Sensorineural.
Nephropathy deafness hyperparathyroidism
0
*Hearing Loss, Sensorineural
*Hyperparathyroidism
*Kidney Failure, Chronic.
Donnai-
Barrow syndrome
0
*Hearing Loss, Sensorineural
*Myopia
*Proteinuria
*Agenesis of Corpus Callosum.
Ectodermal dysplasia, sensorineural hearing loss, and distinctive facial features
0
*Ectodermal Dysplasia
*Hearing Loss, Sensorineural
*Facies.
Keratoderma palmoplantar deafness
0
*Hearing Loss, Sensorineural
*Keratoderma, Palmoplantar.
Marshall syndrome
0
*Cataract
*Hearing Loss, Sensorineural
*Osteochondrodysplasias
*Craniofacial Abnormalities.
Deafness enamel hypoplasia nail defects
0
*Amelogenesis Imperfecta
*Hearing Loss, Sensorineural
*Nails, Malformed.
Coloboma, cleft lip/palate and mental retardation syndrome
0
*Cleft Lip
*Cleft Palate
*Coloboma
*Hearing Loss, Sensorineural
*Hematuria
*Intellectual Disability
*Microphthalmos.
Chitty Hall Baraitser syndrome
0
*Bone Diseases, Metabolic
*Growth Disorders
*Hearing Loss, Sensorineural
*Lacrimal Duct Obstruction
Femur/abnormalities.
Rosenberg Chutorian syndrome
0
*Hearing Loss, Sensorineural
*Optic Atrophy
*Polyneuropathies.
Robinson Miller Bensimon syndrome
0
*Ectodermal Dysplasia
*Hearing Loss, Sensorineural.
Congenital ectodermal dysplasia with hearing loss
0
*Contracture
*Ectodermal Dysplasia
*Hearing Loss, Sensorineural
Fingers/abnormalities.
Ermine phenotype
0
*Hearing Loss, Sensorineural
*Piebaldism.
Corneal dystrophy and perceptive deafness
0
*Corneal Dystrophies, Hereditary
*Hearing Loss, Sensorineural.
Chudley-
Mccullough syndrome
0
*Hearing Loss, Sensorineural
*Arachnoid Cysts
*Agenesis of Corpus Callosum.
Charcot-
Marie-
Tooth disease, Type 2J
0
*Charcot-Marie-Tooth Disease
*Hearing Loss, Sensorineural.
Arthrogryposis-
like hand anomaly and sensorineural deafness
0
*Arthrogryposis
*Hearing Loss, Sensorineural
*Craniofacial Abnormalities.
CAPOS syndrome
0
*Cerebellar Ataxia
*Foot Deformities, Congenital
*Hearing Loss, Sensorineural
*Optic Atrophy
*Reflex, Abnormal.
STRC protein, human
0
*Membrane Proteins
Hearing Loss, Sensorineural.
Kcnq4 protein, mouse
0
*KCNQ Potassium Channels
Hearing Loss, Sensorineural.
Kcnq4 protein, rat
0
*KCNQ Potassium Channels
Hearing Loss, Sensorineural.
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