Startle Epilepsy and Tinnitus Masking
LR is a 9-year-old girl with a history of epilepsy. Specifically, LR has a type of seizure that is evoked by loud or startling sounds. These seizures are known as startle-provoked epileptic seizures (SPES). Although she exhibited normal development prior to age 3, LR’s mother reports a multiple-hour seizure at age 3, and LR now has global developmental delays and is nonverbal. She can follow some simple commands from her mother (finds some body parts); answers some yes/no questions with head shakes or nods; and loves to dance though her mobility is slightly impaired and restricted because of seizure activity.
For most patients, the occurrence of epileptic seizures is unforeseeable and, most of the time, sporadic. Manford et al estimated that, in about 5% of patients, there are specific triggers for epilepsy. The most common being photosensitivity. With SPES, the trigger/stimuli can be sensory stimuli of various modalities, but most commonly this stimulus is noise. Manford et al also reported that same “noise” stimuli, if the patient is forewarned, will have no effect. Cases of SPES are considered rare.