raremaprare-disease reference indexOrphadata 2026-06-23 · HPO 2026-09-01
ORPHA:3213

Deafness-opticoacoustic nerve atrophy-dementia syndrome

inactive · moved to ORPHA:52368Mohr-Tranebjaerg syndrome

Synonyms: Jensen syndrome · hearing loss-opticoacoustic nerve atrophy-dementia syndrome

Type
Disease
Group
Disorder
Status
inactive
Flag
Deprecated entity

This entity has been excluded from the Orphanet nomenclature of rare diseases and moved to Mohr-Tranebjaerg syndrome

1

Names in Orphadata languages

12 of 12 languages
LangNameSynonyms
enDeafness-opticoacoustic nerve atrophy-dementia syndromeJensen syndrome · hearing loss-opticoacoustic nerve atrophy-dementia syndrome
frSyndrome de surdité-atrophie optique-démenceSyndrome de Jensen
deSchwerhörigkeit - Optikusatrophie - DemenzJensen-Syndrom
esSíndrome de sordera-atrofia del nervio óptico y acústico-demenciaSíndrome de Jensen · Síndrome de hipoacusia-atrofia del nervio óptico y acústico-demencia
itSordità - atrofia ottica - demenzaSindrome di Jensen
nlDoofheid - atrofie van oog- en gehoorzenuw - dementie-syndroomSyndroom van Jensen
ptSíndrome de surdez-atrofia do nervo óptico e acústico-demênciaSíndrome Jensen · Síndrome de perda auditiva-atrofia dos nervos óptico e acústico-demência
plGłuchota - atrofia nerwu wzrokowego i słuchowego - demencjaZespół Jensena
csSyndrom zahrnující hluchotu, atrofii optického a akustického nervu a demenciJensenův syndrom
ukСиндром глухоти - атрофії оптико-акустичного нерва - деменціїСиндром втрати слуху - атрофії оптико-акустичного нерва - деменції · Синдром Дженсена
zh耳聋-视听神经萎缩-痴呆综合征听力丧失-视听神经萎缩-痴呆综合征
trSağırlık-optikoakustik sinir atrofi-demans sendromuJensen sendromu · işitme kaybı-optikoakustik sinir atrofi-demans sendromu
2

Cross-references

qualifier = Orphanet mapping relation
SourceCodeQualifierICD relationValidation
UMLSC1839564Evalidated

E exact · NTBT this ORPHAcode is narrower than the target · BTNT broader than the target · ND not decided.

3

Classification

Not placed in any Orphanet classification in this release.

No children.

4

Genes

0 direct

Orphanet records no gene directly on this code.

5

Prevalence and incidence

0 records

No prevalence record in this release.

6

HPO phenotypes

Orphanet has no HPO annotation for this code in this release.

7

Sources for this card