Autosomal-dominant Epilepsy With Auditory Features (ADEAF)

Reviewed by: HU Medical Review Board | Last updated: November 2022

Autosomal-dominant epilepsy with auditory features (ADEAF) is a rare form of epilepsy. The word "auditory" means "related to sound." People with ADEAF often hear buzzing, ringing, or humming sounds. Knowing ADEAF, its symptoms, and treatment options can help you and your loved ones better manage this syndrome.1

What causes ADEAF?

In years past, doctors thought that ADEAF was caused only by a genetic change (mutation). But doctors now think there might be many different factors that can lead to ADEAF. It is found both in people with a family history of the condition and people without such a history.2

The lateral (on the side) temporal lobe of the brain is the area that processes sound. Because people with ADEAF experience sound-related symptoms, doctors think that the syndrome originates in the temporal region of the brain. For this reason, ADEAF may also be called autosomal-dominant lateral temporal epilepsy (ADLTE). It is also sometimes called autosomal-dominant partial epilepsy with auditory features, or ADPEAF.1,2

Inheritance

For those with a genetic mutation, the inheritance is autosomal dominant. This means that if 1 parent has the mutation, there is a 50 percent chance of their child inheriting the disorder.3

But many times, ADEAF occurs without any family history. When this happens, the doctor may call the condition epilepsy with auditory features (EAF). This more generic term describes the symptoms without linking the condition to a genetic inheritance. New genetic mutations can appear even without a family history. This is known as a de novo mutation.2,3

Who gets autosomal-dominant epilepsy with auditory features?

Seizures from ADEAF usually begin when people are young adults or in their late teens.1,4

The exact number of people with ADEAF is not known. Less than 3 percent of people with epilepsy have a family history of any kind of epilepsy. And only part of that 3 percent have symptoms of ADEAF. So the number of people with ADEAF is presumed to be very low.1,4

Autosomal-dominant mutations happen on chromosomes that are not involved in determining a person's sex. So your sex does not affect how likely you are to have ADEAF.3

Symptoms

Most people with ADEAF have focal aware seizures. Sometimes these are known as "simple partial seizures." They begin on one side of the brain. A person experiencing a focal aware seizure does not lose consciousness and is aware of their surroundings.2,5,6

The most common sound-related symptoms of ADEAF include:1

  • Buzzing
  • Humming
  • Ringing

Less common sound symptoms include:1,2

  • Specific sounds, voices, or music
  • Changes in the tone or loudness of sounds

Some people with ADEAF lose their ability to understand language during a seizure. This loss is known as "receptive aphasia" and it occurs before the person loses consciousness. Losing consciousness and awareness means the person is having a focal impaired awareness seizure. These are sometimes called "complex partial seizures."1,5

Diagnosis

Genetic testing can help you find out if you have ADEAF or a different type of epilepsy that includes sound-related symptoms. Other tests your doctor will order to diagnose the syndrome and rule out others include:4

Your doctor will likely order other tests. Talk to your doctor about which ones might be needed.

Treatment

For most people, ADEAF seizures can be easily managed with traditional anti-seizure drugs. Few clinical studies have looked at which drug may be more effective. This is probably because most people find complete relief from their seizures with the most common, inexpensive anti-seizure drugs.4

Outlook

The overall outlook for those with ADEAF is positive. Most people with the syndrome control their symptoms with anti-seizure drugs. Many remain symptom-free for years or decades. No other problems with thinking or learning appear to be linked to the syndrome.4

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