Symptoms, Triggers, and Warning Signs

In Epilepsy with Auditory Features (EAF), seizures often begin as brief, unusual sensory experiences. Because these events occur in the part of the brain that processes sound, they are characterized by what you hear or how you understand language ^[1][2]. Understanding these “auras”—which are actually the first stage of a focal seizure—can help you and your family recognize a seizure before it progresses ^[3].

Hallmark Auditory Symptoms

The sounds experienced during an EAF seizure are not “hallucinations” in the psychiatric sense; they are the result of abnormal electrical activity in the lateral temporal lobe ^[1]. Patients typically describe three types of auditory symptoms:

• Simple Sounds: The most common experiences are basic noises like ringing (often described as bells), buzzing, humming, clicking, or hissing ^[3][4].
• Auditory Distortions: Instead of hearing a new sound, real sounds in your environment may suddenly change. They might seem much louder (volume increase), muffled as if you are underwater, or strangely “tinny” ^[3][5].
• Complex Sounds: Less frequently, patients may hear more structured sounds, such as a specific melody or the sound of voices ^[1][5].

Ictal Aphasia: When Words Lose Meaning

A key feature of EAF is ictal aphasia—a sudden disruption in language that occurs during the seizure ^[1]. Many patients specifically experience receptive aphasia, where you can hear people talking, but the words sound like a foreign language or meaningless noise ^[1][6]. In some cases, you may also find it impossible to speak or find the right words yourself ^[7]. These episodes are usually brief, but they can be very distressing if you are in the middle of a conversation.

How Seizures Progress

For many people, a seizure stays “focal,” meaning it remains in one small area of the brain and you stay fully aware of what is happening. However, the electrical activity can spread to other brain networks ^[8]:

1. Focal Aware Seizure (Aura): You hear the ringing or experience language difficulty but remain conscious and alert ^[7].
2. Focal Impaired Awareness Seizure: If the seizure spreads, you may experience “loss of contact.” You might stare into space, stop responding to others, or perform repetitive movements like smacking your lips or fumbling with your clothes (automatisms) ^[9][8].
3. Bilateral Tonic-Clonic Seizure: The activity can spread to both sides of the brain, leading to a convulsion with stiffening and jerking of the limbs ^[7][8]. In one study of patients with a specific RELN genetic form of EAF, about 88% had experienced at least one convulsion, often preceded by an auditory aura (though this figure does not predict an individual’s personal risk) ^[7].

Triggers: Sound and Sleep

While many seizures happen without a clear cause, some patients have specific “reflex” triggers.

• Environmental Noises: In some cases, specific sounds like a telephone ringing, a certain piece of music, or loud background noise can trigger a seizure ^[7][10]. This is called an audiogenic trigger and was reported by about 8% of patients in a RELN-mutated study cohort ^[7].
• Sleep: Seizures in EAF frequently occur during sleep or upon waking ^[11]. While the relationship is complex, seizures that occur primarily during sleep have been associated in some cohorts with being more difficult to control with medication ^[11].

Seizure Action Plan and First Aid

Every patient should work with their care team to develop a personalized seizure action plan, including when to use any prescribed rescue medication. Keep a one-page diary detailing the date, exact sound, awareness level, and duration of your events.

Basic First Aid for a Convulsion:

• Time the event immediately.
• Protect the person from injury (ease them to the floor, put something soft under their head).
• Turn them on their side to keep the airway clear.
• Do NOT restrain them or put anything in their mouth.
• Do not offer food or pills until they are fully awake.

Red Flags: Seek Emergency Medical Care If:

• Duration: A convulsive (shaking) seizure lasts longer than 5 minutes ^[12]. Prolonged focal seizures also warrant urgent medical assessment.
• Repetition: You have a second seizure shortly after the first without fully waking up or returning to normal in between ^[13].
• Breathing/Injury: The person has difficulty breathing, turns blue, or is seriously injured during the event ^[12].
• First Seizure: It is the person’s first ever suspected seizure.
• Pregnancy/Water: The seizure occurs in a pregnant person or while in the water.
• Persistent Confusion: A person remains unusually confused, aggressive, or unable to be woken up for an extended period after the shaking stops ^[14][15].