Global Variome shared LOVD
PSEN1 (presenilin 1)
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Phenotypes for disease #05103 (deafness (deafness))
Legend
Please note that a short description of a certain column can be displayed when you move your mouse cursor over the column's header and hold it still. Below, a more detailed description is shown per column.
Phenotype details
: additional information on the phenotype of the individual, preferably use HPO terms only (http://www.human-phenotype-ontology.org/)
Diagnosis/Initial
: initial diagnosis, before molecular testing
Diagnosis/Definite
: phenotype individual after molecular testing (OMIM abbreviation)
Inheritance
: Indicates the inheritance of the phenotype in the family; unknown, familial (autosomal/X-linked, dominant/ recessive), paternal (Y-linked), maternal (mitochondrial), isolated (sporadic) or complex
All options:
Unknown
Familial
Familial, autosomal dominant
Familial, autosomal recessive
Familial, X-linked
Familial, X-linked dominant
Familial, X-linked dominant, male sparing
Familial, X-linked recessive
Paternal, Y-linked
Maternal, mitochondrial
Isolated (sporadic)
Di-genic
Complex
- = Not applicable
Age/Examination
: age at which the individual was examined.
35y = 35 years
04y08m = 4 years and 8 months
00y00m01d12h = 1 day and 12 hours
18y? = around 18 years
30y-40y = between 30 and 40 years
>54y = older than 54
? = unknown
Age/Diagnosis
: age diagnosis was confirmed
35y = 35 years
04y08m = 4 years and 8 months
00y00m01d12h = 1 day and 12 hours
18y? = around 18 years
30y-40y = between 30 and 40 years
>54y = older than 54
? = unknown
Age/Onset
: Age first symptoms disease appeared in individual:
35y = 35 years
04y08m = 4 years and 8 months
00y00m01d12h = 1 day and 12 hours
18y? = around 18 years
30y-40y = between 30 and 40 years
>54y = older than 54
? = unknown
Phenotype/Onset
: individual's phenotype at Age/Onset described using HPO
Protein
: result from protein staining
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all entries beginning with 'p.(Arg'
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Date
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Date
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Date
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Date
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Date
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Date
2019|2020 <2020-03
all entries in 2019 or 2020, and before March, 2020
Numeric
23
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Numeric
23|24
all entries exactly matching 23 or 24
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Numeric
!23
all entries not exactly matching 23
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all entries lower than 23
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all entries lower than, or equal to, 23
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Numeric
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all entries higher than, or equal to, 23
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Numeric
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all entries with values from 20 to 29, but not equal to 23
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all entries containing 'Asian', 'asian', including 'Caucasian', 'caucasian', etc.
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all entries containing 'Asian' or 'African', but not containing 'Caucasian'
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all entries containing 'South Asian', but not containing 'South East Asian'
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765 entries on 8 pages. Showing entries 1 - 100.
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Phenotype ID
Phenotype details
Diagnosis/Initial
Diagnosis/Definite
Inheritance
Age/Examination
Age/Diagnosis
Age/Onset
Phenotype/Onset
Protein
Owner
Individual ID
0000041990
-
-
-
Unknown
-
-
-
-
-
Johan den Dunnen
00055335
0000041993
-
-
-
Unknown
-
-
-
-
-
Johan den Dunnen
00055338
0000041995
-
-
-
Unknown
-
-
-
-
-
Johan den Dunnen
00055340
0000041998
-
-
-
Unknown
-
-
-
-
-
Johan den Dunnen
00055343
0000042004
-
-
-
Unknown
-
-
-
-
-
Johan den Dunnen
00055349
0000060262
-
-
-
Familial, autosomal recessive
-
-
-
-
-
Mieke Wesdorp
00080031
0000061069
-
-
-
Unknown
-
-
-
-
-
Johan den Dunnen
00080061
0000061070
-
-
-
Unknown
-
-
-
-
-
Johan den Dunnen
00080018
0000061071
-
-
-
Familial, autosomal recessive
-
-
-
-
-
Mieke Wesdorp
00080020
0000061072
-
-
-
Familial, autosomal recessive
-
-
-
-
-
Mieke Wesdorp
00080030
0000061073
-
-
-
Familial, autosomal recessive
-
-
-
-
-
Mieke Wesdorp
00080032
0000061074
-
-
-
Familial, autosomal recessive
-
-
-
-
-
Mieke Wesdorp
00080048
0000061081
-
-
-
Familial, autosomal recessive
-
-
-
-
-
Mieke Wesdorp
00080017
0000061082
-
-
-
Familial, autosomal recessive
-
-
-
-
-
Mieke Wesdorp
00080021
0000087948
X-linked nonsyndromic progressive hearing loss
-
-
Familial, X-linked
-
02y
02y
-
-
N Cobbler
00112384
0000092473
-
-
-
Familial, autosomal recessive
08y
-
-
-
-
David Baux
00117226
0000092474
-
-
-
Isolated (sporadic)
07y
-
-
-
-
David Baux
00117227
0000092475
-
-
-
Familial, autosomal recessive
04y
-
00y
-
-
David Baux
00117228
0000092476
-
-
-
Isolated (sporadic)
10y
-
00y
-
-
David Baux
00117229
0000092477
-
-
-
Familial, autosomal recessive
03y
-
00y
-
-
David Baux
00117230
0000092478
-
-
-
Familial, autosomal recessive
28y
-
00y
-
-
David Baux
00117231
0000092479
-
-
-
Isolated (sporadic)
10y
-
00y
-
-
David Baux
00117232
0000092480
-
-
-
Isolated (sporadic)
03y
-
00y
-
-
David Baux
00117233
0000092481
-
-
-
Isolated (sporadic)
03y
-
-
-
-
David Baux
00117234
0000092482
-
-
-
Isolated (sporadic)
05y
-
00y
-
-
David Baux
00117235
0000092483
-
-
-
Familial, autosomal recessive
13y
-
00y
-
-
David Baux
00117236
0000092484
-
-
-
Familial, autosomal recessive
03y
-
00y
-
-
David Baux
00117237
0000092485
-
-
-
Isolated (sporadic)
13y
-
<10y
-
-
David Baux
00117238
0000092486
-
-
-
Familial, autosomal recessive
02y
-
-
-
-
David Baux
00117239
0000092487
-
-
-
Familial, autosomal recessive
02y
-
-
-
-
David Baux
00117240
0000092488
-
-
-
Isolated (sporadic)
09y
-
<10y
-
-
David Baux
00117241
0000092489
-
-
-
Familial, autosomal recessive
18y
-
-
-
-
David Baux
00117242
0000092490
-
-
-
Isolated (sporadic)
04y
-
00y
-
-
David Baux
00117243
0000092491
-
-
-
Isolated (sporadic)
01y
-
00y
-
-
David Baux
00117244
0000092492
-
-
-
Isolated (sporadic)
03y
-
00y
-
-
David Baux
00117245
0000092493
-
-
-
Familial, autosomal recessive
02y
-
00y
-
-
David Baux
00117246
0000092494
-
-
-
Isolated (sporadic)
02y
-
00y
-
-
David Baux
00117247
0000092495
-
-
-
Isolated (sporadic)
25y
-
-
-
-
David Baux
00117248
0000092496
-
-
-
Familial, autosomal recessive
28y
-
00y
-
-
David Baux
00117249
0000092497
-
-
-
Isolated (sporadic)
11y
-
00y
-
-
David Baux
00117250
0000092498
-
-
-
Isolated (sporadic)
06y
-
00y
-
-
David Baux
00117251
0000092499
-
-
-
Isolated (sporadic)
31y
-
-
-
-
David Baux
00117252
0000092500
-
-
-
Familial, autosomal recessive
03y
-
00y
-
-
David Baux
00117253
0000092501
-
-
-
Familial, autosomal recessive
08y
-
00y
-
-
David Baux
00117254
0000092502
-
-
-
Familial, autosomal recessive
03y
-
00y
-
-
David Baux
00117255
0000092503
-
-
-
Isolated (sporadic)
03y
-
00y
-
-
David Baux
00117256
0000092504
-
-
-
Isolated (sporadic)
04y
-
<10y
-
-
David Baux
00117257
0000092505
-
-
-
Familial, autosomal recessive
33y
-
<10y
-
-
David Baux
00117258
0000092506
-
-
-
Isolated (sporadic)
01y
-
00y
-
-
David Baux
00117259
0000092507
-
-
-
Isolated (sporadic)
32y
-
00y
-
-
David Baux
00117260
0000092508
-
-
-
Isolated (sporadic)
06y
-
-
-
-
David Baux
00117261
0000092509
-
-
-
Isolated (sporadic)
02y
-
00y
-
-
David Baux
00117262
0000092510
-
-
-
Isolated (sporadic)
06y
-
<10y
-
-
David Baux
00117263
0000092511
-
-
-
Isolated (sporadic)
01y
-
00y
-
-
David Baux
00117264
0000092512
-
-
-
Isolated (sporadic)
04y
-
<03y
-
-
David Baux
00117265
0000092513
-
-
-
Isolated (sporadic)
06y
-
<10y
-
-
David Baux
00117266
0000092514
-
-
-
Isolated (sporadic)
27y
-
-
-
-
David Baux
00117267
0000092515
-
-
-
Familial, autosomal recessive
04y
-
<03y
-
-
David Baux
00117268
0000092516
-
-
-
Isolated (sporadic)
10y
-
<10y
-
-
David Baux
00117269
0000092517
-
-
-
Familial, autosomal recessive
01y
-
00y
-
-
David Baux
00117270
0000092518
-
-
-
Isolated (sporadic)
02y
-
00y
-
-
David Baux
00117271
0000092519
-
-
-
Isolated (sporadic)
25y
-
00y
-
-
David Baux
00117272
0000092520
-
-
-
Isolated (sporadic)
04y
-
00y
-
-
David Baux
00117273
0000092521
-
-
-
Isolated (sporadic)
01y
-
00y
-
-
David Baux
00117274
0000092522
-
-
-
Isolated (sporadic)
03y
-
-
-
-
David Baux
00117275
0000092523
-
-
-
Familial, autosomal recessive
25y
-
<05y
-
-
David Baux
00117276
0000092524
-
-
-
Isolated (sporadic)
12y
-
<06y
-
-
David Baux
00117277
0000092525
-
-
-
Familial, autosomal recessive
08y
-
00y
-
-
David Baux
00117278
0000092526
-
-
-
Familial, autosomal dominant
61y
-
-
-
-
David Baux
00117279
0000092527
-
-
-
Familial, autosomal dominant
08y
-
-
-
-
David Baux
00117280
0000092528
-
-
-
Familial, X-linked recessive
02y
-
00y
-
-
David Baux
00117281
0000092529
-
-
-
Isolated (sporadic)
09y
-
00y
-
-
David Baux
00117282
0000092530
-
-
-
Isolated (sporadic)
11y
-
00y
-
-
David Baux
00117283
0000092531
-
-
-
Isolated (sporadic)
03y
-
00y
-
-
David Baux
00117284
0000092532
-
-
-
Isolated (sporadic)
12y
-
-
-
-
David Baux
00117285
0000092533
-
-
-
Isolated (sporadic)
05y
-
-
-
-
David Baux
00117286
0000092534
-
-
-
Isolated (sporadic)
01y
-
00y
-
-
David Baux
00117287
0000092535
-
-
-
Isolated (sporadic)
03y
-
00y
-
-
David Baux
00117288
0000092536
-
-
-
Isolated (sporadic)
25y
-
00y
-
-
David Baux
00117289
0000092537
-
-
-
Isolated (sporadic)
01y
-
00y
-
-
David Baux
00117290
0000092538
-
-
-
Familial, autosomal recessive
13y
-
<10y
-
-
David Baux
00117291
0000092539
-
-
-
Isolated (sporadic)
23y
-
>10y
-
-
David Baux
00117292
0000092540
-
-
-
Isolated (sporadic)
23y
-
<03y
-
-
David Baux
00117293
0000124335
-
Sensorineural deafness, nonsyndromic
DFNX-1
Unknown
-
-
-
-
-
Johan den Dunnen
00151972
0000124336
-
Sensorineural deafness, nonsyndromic
DFNX-1
Unknown
-
-
-
-
-
Johan den Dunnen
00151973
0000124337
post-lingual, bilateral, progressive deafness, normal speech development, received bilateral cochlear implants
nonsyndromic sensorineural hearing loss
DFNX-1
Familial, X-linked recessive
-
-
-
-
Reduced enzymatic activity
Giulia Soldà
00151974
0000124338
post-lingual progressive bilateral hearing impairment, characterized by down-sloping audiometric profile, with moderate HL at low and middle frequencie, severe hearing impairment at higher frequencies; neurological evaluation displayed sign and symptoms of peripheral neuropathy, with both sensory and motor deficits
sensorineural hearing loss
DFNX-1
Familial, X-linked recessive
-
-
-
-
Reduced enzymatic activity
Giulia Soldà
00151975
0000124348
post-lingual progressive bilateral hearing impairment, characterized by down-sloping audiometric profile, with moderate HL at low and middle frequencies and a severe hearing impairment at higher frequencies; neurological evaluation displayed sign and symptoms of mildly symptomatic peripheral neuropathy, with prevalent sensory involvement (i.e. Romberg positivity, absent deep tendon reflexes; showed chronic denervation at EMG evaluation, and mild axonal neuropathy affecting only motor nerves
sensorineural hearing loss
DFNX-1
Familial, X-linked recessive
-
-
-
-
Reduced enzymatic activity
Giulia Soldà
00151985
0000124349
-
Sensorineural deafness, nonsyndromic
DFNX-1
Unknown
-
-
-
-
-
Johan den Dunnen
00151986
0000124350
-
Sensorineural deafness, nonsyndromic
DFNX-1
Unknown
-
-
-
-
-
Johan den Dunnen
00151987
0000128163
-
deafness, non-syndromic (DFN)
-
-
-
-
-
-
-
Anne-Françoise Roux
00163026
0000128164
-
deafness, non-syndromic (DFN)
-
-
-
-
-
-
-
Anne-Françoise Roux
00163027
0000128165
-
deafness, non-syndromic (DFN)
-
-
-
-
-
-
-
Anne-Françoise Roux
00163028
0000128166
-
deafness, non-syndromic (DFN)
-
-
-
-
-
-
-
Anne-Françoise Roux
00163029
0000128167
-
deafness, non-syndromic (DFN)
-
-
-
-
-
-
-
Anne-Françoise Roux
00163030
0000128168
-
deafness, non-syndromic (DFN)
-
-
-
-
-
-
-
Anne-Françoise Roux
00163031
0000128169
-
deafness, non-syndromic (DFN)
-
-
-
-
-
-
-
Anne-Françoise Roux
00163032
0000128170
-
deafness, non-syndromic (DFN)
-
-
-
-
-
-
-
Anne-Françoise Roux
00163033
0000128171
-
deafness, non-syndromic (DFN)
-
-
-
-
-
-
-
Anne-Françoise Roux
00163034
0000128172
-
deafness, non-syndromic (DFN)
-
-
-
-
-
-
-
Anne-Françoise Roux
00163035
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