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Autosomal dominant epilepsy with auditory features
Reviewed by Medicinsk Expert•Last updated: 2026-10-22•Condition

Autosomal dominant epilepsy with auditory features

Autosomal dominant epilepsy with auditory features (ADEAF) is an uncommon, inherited form of focal epilepsy characterized by sensory symptoms, particularly auditory hallucinations, during seizures.

Overview & Symptoms

Autosomal dominant epilepsy with auditory features (ADEAF) is an uncommon form of epilepsy that runs in families. People with this condition typically hear sounds (auditory features), such as buzzing, humming, or ringing, during seizures. Some people hear more complex sounds, like specific voices or music, or changes in the volume of sounds. Some people with ADEAF suddenly become unable to understand language before losing consciousness during a seizure. This inability to understand speech is known as receptive aphasia. Less commonly, seizures may cause visual hallucinations, a disturbance in the sense of smell, a feeling of dizziness or spinning (vertigo), or other symptoms that affect the senses.

ADEAF is called a focal epilepsy because the seizures start in one part of the brain, rather than involving the entire brain from the beginning. Most people with ADEAF have focal aware seizures, which do not cause a loss of consciousness. These seizures are thought to begin in a part of the brain called the lateral temporal lobe. In some people, seizure activity may spread from the lateral temporal lobe to affect other regions of the brain. If seizure activity spreads to the entire brain, it causes a loss of consciousness, muscle stiffening, and rhythmic jerking. Episodes that begin as focal seizures and spread throughout the brain are known as secondarily generalized seizures.

Seizures associated with ADEAF usually begin in adolescence or young adulthood. They may be triggered by specific sounds, such as a ringing telephone or speech, but in most cases the seizures do not have any recognized triggers. In most affected people, seizures are infrequent and effectively controlled with medication.

Frequency

ADEAF appears to be uncommon, although its prevalence is unknown.

Causes & Genetic Factors

Variants (also called mutations) in the LGI1 gene or RELN gene are the most common cause of ADEAF. Variants in other genes have been identified in small numbers of affected families, and the genetic cause in many families is unknown.

The LGI1 gene provides instructions for making a protein called Lgi1 or epitempin, which is found primarily in nerve cells (neurons). Although the precise role of epitempin remains uncertain, researchers suggest it may play a role at the junction of neurons (synapses) where cell-to-cell communication takes place.

The RELN gene provides instructions for making a protein called reelin. Reelin is produced in the brain both during brain development and after birth. It appears to play several roles at synapses, including controlling communication between neurons.

Variants in the LGI1 or RELN gene may impair the formation or function of synapses. Abnormal communication between neurons can lead to seizure activity in the brain.

Associated Genes

  • DEPDC5
  • LGI1
  • RELN
  • MICAL1

Inheritance

This condition is inherited in an autosomal dominant pattern, which means one copy of the altered gene in each cell is sufficient to raise the risk of developing epilepsy. About two-thirds of people who inherit an LGI1 gene variant will develop seizures. Some people who have the altered gene never develop the condition, which is known as reduced penetrance. The likelihood of developing seizures in people with a variant in RELN or other associated genes is unknown. Most people with ADEAF have one affected parent and other relatives with the condition.

Other Names for This Condition

  • ADEAF
  • ADLTE
  • ADPEAF
  • Autosomal dominant lateral temporal lobe epilepsy
  • Autosomal dominant partial epilepsy with auditory features
  • Epilepsy, partial, with auditory features
  • ETL1

Frequently Asked Questions

Most patients with ADEAF respond exceptionally well to standard anti-seizure medications that target focal seizures. First-line treatments typically include carbamazepine, oxcarbazepine, or lamotrigine. Unlike refractory epilepsies, a majority of individuals with ADEAF achieve complete seizure freedom on modest doses of monotherapy, provided medication adherence is maintained.
DEAF is primarily evaluated using targeted gene sequencing or multi-gene epilepsy panels that look for mutations in the LGI1 gene, as well as rarer genes like RELN or MICAL1. Because the condition is inherited in an autosomal dominant pattern, an affected parent has a 50 percent chance of passing the variant to each child. However, the condition displays incomplete penetrance, meaning around 30 to 40 percent of individuals who inherit the altered gene never develop seizures. Genetic counseling helps family members understand individual risk versus actual clinical expression.
uditory features in ADEAF are sudden, brief (typically lasting seconds to a couple of minutes), repetitive, and highly stereotyped, such as a consistent tone, ringing, or distorted volume. In contrast, psychiatric auditory hallucinations are usually persistent, emotionally complex, and lack electroencephalogram (EEG) abnormalities. Inner ear disorders, like tinnitus or Meniere disease, cause continuous or prolonged ringing often paired with measurable hearing loss, whereas ADEAF episodes are paroxysmal and frequently accompanied by receptive aphasia, dreamy states, or progression to motor seizures.
Yes, a subset of patients has reflex seizures triggered by sudden, patterned, or resonant sounds, such as a ringing telephone, radio static, specific musical tones, or background chatter. Management involves identifying these precise acoustic triggers through a seizure diary, adjusting sound settings on phones and household appliances, using noise-dampening ear protection in loud environments, and optimizing anti-seizure medication to raise the seizure threshold.
Source: MedlinePlus, National Library of Medicine

MedlinePlus brings together authoritative health information from the National Library of Medicine (NLM), the National Institutes of Health (NIH), and other government agencies and health-related organizations.

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