autosomal dominant epilepsy with auditory features
Findings
No curated finding names autosomal dominant epilepsy with auditory features yet. Everything below is reference data from Mondo, HPO and GenCC, not evidence about what affects it.
Definition
A rare, genetic, familial partial epilepsy disease characterized by focal seizures associated with prominent ictal auditory symptoms, and/or receptive aphasia, presenting in two or more family members and having a relatively benign evolution.
Definition from the Mondo Disease Ontology (MONDO:0010898), read 2026-09-29. CC BY 4.0.
Features
17 features
What the disease is reported to present with, and how often among people who have it — never how often a feature means the disease. HPO, annotations 2026-09-02.
- Auditory hallucinationHPOHP:0008765
- Very frequent (80% to 99% of cases)
- EEG with focal epileptiform dischargesHPOHP:0011185
- Very frequent (80% to 99% of cases)
- Focal-onset seizureHPOHP:0007359
- Very frequent (80% to 99% of cases)
- AphasiaHPOHP:0002381
- Frequent (30% to 79% of cases)
- Interictal epileptiform activityHPOHP:0011182
- Frequent (30% to 79% of cases)
- Abnormal autonomic nervous system physiologyHPOHP:0012332
- Occasional (5% to 29% of cases)
- Focal autonomic seizureHPOHP:0011154
- Occasional (5% to 29% of cases)
- Focal aware seizureHPOHP:0002349
- Occasional (5% to 29% of cases)
- Nocturnal seizuresHPOHP:0031951
- Occasional (5% to 29% of cases)
- Visual hallucinationHPOHP:0002367
- Occasional (5% to 29% of cases)
- Atypical behaviorHPOHP:0000708
- Very rare (1% to 4% of cases)
- Bilateral tonic-clonic seizureHPOHP:0002069
- Very rare (1% to 4% of cases)
Reported absent (2)
- Brain imaging abnormalityHPOHP:0410263
- Intellectual disabilityHPOHP:0001249
Show the remaining 5
- Bilateral tonic-clonic seizure with focal onsetHPOHP:0007334
- Very rare (1% to 4% of cases)
- DepressionHPOHP:0000716
- Very rare (1% to 4% of cases)
- Generalized-onset seizureHPOHP:0002197
- Very rare (1% to 4% of cases)
- ImpulsivityHPOHP:0100710
- Very rare (1% to 4% of cases)
- MigraineHPOHP:0002076
- Very rare (1% to 4% of cases)
Genes
3 genes
Germline variants in the gene encoding each protein, as classified by GenCC submitters, each in their own words. Limited, disputed and refuted submissions are listed too.
- LGI1HGNC:6572
- Definitive · ClinGen · Autosomal dominant · 2020
- Strong · PanelApp Australia · Autosomal dominant · 2025
- Supportive · Orphanet · Autosomal dominant · 2021
- MICAL1HGNC:20619
- Moderate · PanelApp Australia · Autosomal dominant · 2025
- RELNHGNC:9957
- Supportive · Orphanet · Autosomal dominant · 2021
Where it sits
Other names
8 names
Resolves to: autosomal dominant epilepsy with auditory features
- Also called
- ADEAFADLTEadolescent/adult onset autosomal dominant epilepsy with auditory featuresADPEAFautosomal dominant lateral temporal lobe epilepsyautosomal dominant partial/lateral temporal epilepsy with auditory featurespartial epilepsy with auditory aurapartial epilepsy with auditory features