Severe sensorineural hearing impairment
Symptom Information:
| Symptom ID: | HPO:0008625 | ||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
| Synonyms: |
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| Cross references: |
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| Is a (Whole tree): |
HPO:
All(HPO:0000001) Phenotypic abnormality(HPO:0000118) Abnormality of the ear(HPO:0000598) Abnormality of the inner ear(HPO:0000359) Functional abnormality of the inner ear(HPO:0011389) Sensorineural hearing impairment(HPO:0000407) Severe sensorineural hearing impairment(HPO:0008625) Hearing abnormality(HPO:0000364) Hearing impairment(HPO:0000365) Sensorineural hearing impairment(HPO:0000407) Severe sensorineural hearing impairment(HPO:0008625) MedDRA: Ear and labyrinth disorders(MedDRA:10013993) Hearing disorders(MedDRA:10019243) Hearing impairment(HPO:0000365) Severe sensorineural hearing impairment(HPO:0008625) Congenital, familial and genetic disorders(MedDRA:10010331) Ear and labyrinthine disorders congenital(MedDRA:10013995) Hearing disorders congenital(MedDRA:10019244) Hearing impairment(HPO:0000365) Severe sensorineural hearing impairment(HPO:0008625) |
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| Database Frequency: | 150 / 7739 | ||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
| Resource: |
All diseases associated with this symptom:
| 1p36 deletion syndrome | (Orphanet:1606) |
| 2p15p16.1 microdeletion syndrome | (Orphanet:261349) |
| 2q37 microdeletion syndrome | (Orphanet:1001) |
| 8q21.11 microdeletion syndrome | (Orphanet:284160) |
| ALG11-CDG | (Orphanet:280071) |
| ATAXIA, DEAFNESS, AND CARDIOMYOPATHY | (OMIM:208750) |
| ATAXIA, SPASTIC, CHILDHOOD-ONSET, AUTOSOMAL RECESSIVE, WITH OPTICATROPHY AND MENTAL RETARDATION | (OMIM:270500) |
| AXENFELD-RIEGER ANOMALY WITH PARTIALLY ABSENT EYE MUSCLES, DISTINCTIVEFACE, HYDROCEPHALY, AND SKELETAL ABNORMALITIES | (OMIM:109120) |
| Acro-osteolysis-keloid-like lesions-premature aging syndrome | (Orphanet:363665) |
| Agenesis of the corpus callosum - intellectual deficit - coloboma - micrognathia | (Orphanet:52055) |
| Alpha-N-acetylgalactosaminidase deficiency type 2 | (Orphanet:79280) |
| Alpha-thalassemia - X-linked intellectual deficit syndrome | (Orphanet:847) |
| Alström syndrome | (Orphanet:64) |
| Arthrogryposis-like hand anomaly - sensorineural deafness | (Orphanet:1144) |
| Atherosclerosis - deafness - diabetes - epilepsy - nephropathy | (Orphanet:1192) |
| Autosomal dominant intermediate Charcot-Marie-Tooth disease type E | (Orphanet:93114) |
| Autosomal recessive spastic paraplegia type 44 | (Orphanet:320401) |
| BARTTER SYNDROME, TYPE 4A | (OMIM:602522) |
| BARTTER SYNDROME, TYPE 4B | (OMIM:613090) |
| BRANCHIOOTIC SYNDROME 1 | (OMIM:602588) |
| BRANCHIOOTORENAL SYNDROME 1 | (OMIM:113650) |
| BROWN-VIALETTO-VAN LAERE SYNDROME 1 | (OMIM:211530) |
| Björnstad syndrome | (Orphanet:123) |
| Blepharophimosis-intellectual deficit syndrome, Verloes type | (Orphanet:293725) |
| Branchiogenic deafness syndrome | (Orphanet:50815) |
| CARPENTER SYNDROME 1 | (OMIM:201000) |
| CEREBELLAR ATAXIA AND NEUROSENSORY DEAFNESS | (OMIM:212850) |
| CEREBROOCULOFACIOSKELETAL SYNDROME 1 | (OMIM:214150) |
| COCKAYNE SYNDROME A | (OMIM:216400) |
| COCKAYNE SYNDROME B | (OMIM:133540) |
| CODAS syndrome | (Orphanet:1458) |
| COENZYME Q10 DEFICIENCY, PRIMARY, 1 | (OMIM:607426) |
| CORNELIA DE LANGE SYNDROME 1 | (OMIM:122470) |
| Charcot-Marie-Tooth disease type 1E | (Orphanet:90658) |
| Charcot-Marie-Tooth disease type 4B2 | (Orphanet:99956) |
| Chudley-McCullough syndrome | (Orphanet:314597) |
| Coffin-Lowry syndrome | (Orphanet:192) |
| Corneal anesthesia - deafness - intellectual deficit | (Orphanet:1051) |
| Craniofacial-deafness-hand syndrome | (Orphanet:1529) |
| Craniosynostosis - anal anomalies - porokeratosis | (Orphanet:85199) |
| D-glyceric aciduria | (Orphanet:941) |
| DEAFNESS, AUTOSOMAL DOMINANT 1 | (OMIM:124900) |
| DEAFNESS, AUTOSOMAL DOMINANT 28 | (OMIM:608641) |
| DEAFNESS, AUTOSOMAL RECESSIVE 23 | (OMIM:609533) |
| DEAFNESS, AUTOSOMAL RECESSIVE 28 | (OMIM:609823) |
| DEAFNESS, AUTOSOMAL RECESSIVE 42 | (OMIM:609646) |
| DEAFNESS, AUTOSOMAL RECESSIVE 46 | (OMIM:609647) |
| DEAFNESS, AUTOSOMAL RECESSIVE 74 | (OMIM:613718) |
| DEAFNESS, AUTOSOMAL RECESSIVE 8 | (OMIM:601072) |
| DEAFNESS, AUTOSOMAL RECESSIVE 9 | (OMIM:601071) |
| DEAFNESS, MID-TONE NEURAL | (OMIM:124700) |
| DEAFNESS, NEURAL, CONGENITAL MODERATE | (OMIM:221500) |
| DEAFNESS, NEURAL, WITH ATYPICAL ATOPIC DERMATITIS | (OMIM:221700) |
| DEAFNESS, SENSORINEURAL, WITH PERIPHERAL NEUROPATHY AND ARTERIAL DISEASE | (OMIM:124950) |
| Deaf blind hypopigmentation syndrome, Yemenite type | (Orphanet:3214) |
| Deafness - small bowel diverticulosis - neuropathy | (Orphanet:3217) |
| Deafness - enamel hypoplasia - nail defects | (Orphanet:3220) |
| Deafness-infertility syndrome | (Orphanet:94064) |
| Developmental malformations - deafness - dystonia | (Orphanet:79107) |
| Disorder of sex development - intellectual deficit | (Orphanet:2983) |
| Distal monosomy 10q | (Orphanet:96148) |
| Distal monosomy 6p | (Orphanet:96125) |
| Donnai-Barrow syndrome | (Orphanet:2143) |
| Ectodermal dysplasia - sensorineural deafness | (Orphanet:1883) |
| Ermine phenotype | (Orphanet:999) |
| FACIOSCAPULOHUMERAL MUSCULAR DYSTROPHY 1 | (OMIM:158900) |
| FRIEDREICH ATAXIA, SO-CALLED, WITH OPTIC ATROPHY AND SENSORINEURALDEAFNESS | (OMIM:136600) |
| Familial cutaneous collagenoma | (Orphanet:53296) |
| Familial isolated dilated cardiomyopathy | (Orphanet:154) |
| Familial long QT syndrome | (Orphanet:768) |
| Fibrodysplasia ossificans progressiva | (Orphanet:337) |
| Fountain syndrome | (Orphanet:3219) |
| Galactose epimerase deficiency | (Orphanet:79238) |
| Goldenhar syndrome | (Orphanet:374) |
| Growth delay due to insulin-like growth factor type 1 deficiency | (Orphanet:73272) |
| HISTIDINURIA DUE TO A RENAL TUBULAR DEFECT | (OMIM:235830) |
| HYPOPHOSPHATEMIC RICKETS, AUTOSOMAL RECESSIVE, 1 | (OMIM:241520) |
| Hyperleucine-isoleucinemia | (OMIM:238340) |
| Hypermethioninemia encephalopathy due to adenosine kinase deficiency | (Orphanet:289290) |
| Hypoparathyroidism - deafness - renal disease | (Orphanet:2237) |
| ICHTHYOSIS, HYSTRIX-LIKE, WITH DEAFNESS | (OMIM:602540) |
| Infantile cerebellar-retinal degeneration | (Orphanet:313850) |
| Intellectual deficit, X-linked - Dandy-Walker malformation - basal ganglia disease - Seizures | (Orphanet:1568) |
| Jervell and Lange-Nielsen syndrome 1 | (OMIM:220400) |
| Kearns-Sayre syndrome | (Orphanet:480) |
| LEOPARD SYNDROME 1 | (OMIM:151100) |
| Leber congenital amaurosis 1 | (OMIM:204000) |
| Lenz-Majewski hyperostotic dwarfism | (Orphanet:2658) |
| Lipodystrophy - intellectual deficit - deafness | (Orphanet:50811) |
| MEGDEL syndrome | (Orphanet:352328) |
| MELAS | (Orphanet:550) |
| MENTAL RETARDATION, X-LINKED 46 | (OMIM:300436) |
| MERRF | (Orphanet:551) |
| MICROCEPHALY 3, PRIMARY, AUTOSOMAL RECESSIVE | (OMIM:604804) |
| Marshall syndrome | (Orphanet:560) |
| Maternally-inherited diabetes and deafness | (Orphanet:225) |
| NEUROENDOCRINE CARCINOMA OF SALIVARY GLANDS, SENSORINEURAL HEARINGLOSS, AND ENAMEL HYPOPLASIA | (OMIM:603641) |
| NEUROPATHY, HEREDITARY SENSORY AND AUTONOMIC, TYPE IA | (OMIM:162400) |
| NOONAN SYNDROME 1 | (OMIM:163950) |
| Nail-patella syndrome | (Orphanet:2614) |
| Nephronophthisis-like nephropathy 1 | (OMIM:613159) |
| Neurologic Waardenburg-Shah syndrome | (Orphanet:163746) |
| Neurological conditions associated with aminoacylase 1 deficiency | (Orphanet:137754) |
| Neutral lipid storage myopathy | (Orphanet:98908) |
| Norrie disease | (Orphanet:649) |
| OPITZ-KAVEGGIA SYNDROME | (OMIM:305450) |
| Oculootodental syndrome | (Orphanet:99806) |
| Oculopharyngodistal myopathy | (Orphanet:98897) |
| Otodental syndrome | (Orphanet:2791) |
| Otospondylomegaepiphyseal dysplasia | (Orphanet:1427) |
| PERIPHERAL NEUROPATHY, MYOPATHY, HOARSENESS, AND HEARING LOSS | (OMIM:614369) |
| PROGRESSIVE EXTERNAL OPHTHALMOPLEGIA WITH MITOCHONDRIAL DNA DELETIONS,AUTOSOMAL DOMINANT, 1 | (OMIM:157640) |
| Phosphoribosylpyrophosphate synthetase superactivity | (Orphanet:3222) |
| Pontine tegmental cap dysplasia | (Orphanet:269229) |
| RETINITIS PIGMENTOSA INVERSA WITH DEAFNESS | (OMIM:268010) |
| Renal coloboma syndrome | (Orphanet:1475) |
| Retinitis pigmentosa - intellectual deficit - deafness - hypogenitalism | (Orphanet:3085) |
| Rosaï-Dorfman disease | (Orphanet:158014) |
| STARGARDT MACULAR DEGENERATION, ABSENT OR HYPOPLASTIC CORPUS CALLOSUM,MENTAL RETARDATION, AND DYSMORPHIC FEATURES | (OMIM:612948) |
| STICKLER SYNDROME, TYPE I | (OMIM:108300) |
| Sensory ataxic neuropathy-dysarthria-ophthalmoparesis | (Orphanet:70595) |
| Sialidosis type 1 | (Orphanet:812) |
| Spinocerebellar ataxia with epilepsy | (Orphanet:254881) |
| Split hand - split foot - deafness | (Orphanet:71271) |
| Spondylocarpotarsal synostosis | (Orphanet:3275) |
| Sporadic Leigh syndrome | (Orphanet:255199) |
| Stickler syndrome type 2 | (Orphanet:90654) |
| Stickler syndrome type 3 | (Orphanet:166100) |
| Thiamine-responsive megaloblastic anemia syndrome | (Orphanet:49827) |
| Townes-Brocks syndrome | (Orphanet:857) |
| Upper limb defect - eye and ear abnormalities | (Orphanet:2489) |
| Vici syndrome | (Orphanet:1493) |
| WAARDENBURG SYNDROME, TYPE 2B | (OMIM:600193) |
| WAARDENBURG SYNDROME, TYPE 2E | (OMIM:611584) |
| WAARDENBURG SYNDROME, TYPE 4A | (OMIM:277580) |
| WAARDENBURG SYNDROME, TYPE 4B | (OMIM:613265) |
| WAARDENBURG SYNDROME, TYPE 4C | (OMIM:613266) |
| WT limb-blood syndrome | (Orphanet:3466) |
| Warsaw breakage syndrome | (Orphanet:280558) |
| Weissenbacher- Zweymuller syndrome | (Orphanet:3450) |
| Wolfram syndrome | (Orphanet:3463) |
| Wolfram syndrome 1 | (OMIM:222300) |
| Wolfram syndrome 2 | (OMIM:604928) |
| Wolfram syndrome, mitochondrial form | (OMIM:598500) |
| Woodhouse-Sakati syndrome | (Orphanet:3464) |
| X-linked Charcot-Marie-Tooth disease type 1 | (Orphanet:101075) |
| X-linked Charcot-Marie-Tooth disease type 4 | (Orphanet:101078) |
| X-linked Charcot-Marie-Tooth disease type 6 | (Orphanet:352675) |
| X-linked intellectual deficit, Najm type | (Orphanet:163937) |
| X-linked spinocerebellar ataxia type 3 | (Orphanet:85297) |