Patient 1 is male, firstborn of healthy parents; pregnancy and delivery were uneventful. Neonatal period was complicated by a group B meningococcal sepsis. Seizures occurred from the age of fourteen months, and he developed an epileptic encephalopathy. He suffers from myoclonic seizures of the left arm, atonic seizures, and tonic seizures. The latter were provoked by sudden sounds or unexpected visual stimuli. Neurological examination revealed no focal abnormalities; there is a psychomotor developmental delay with severe learning disabilities. Extended diagnostic work-up, including magnetic resonance (MR) imaging and MR spectography, and neurometabolic and genetic evaluation were all normal. Three seizure types have coexisted since the onset of epilepsy. He was not a suitable candidate for epilepsy surgery. At the age of 5 years, a vagal nerve stimulator was implanted, and a revision took place after two years because of dysfunction and increase in seizure frequency. Ketogenic diet had no effect. At age 10, his epilepsy was still refractory, with seizure frequency varying from several seizures a day to 30 seizures a day, depending on triggering factors. He used lamotrigine and clorazepam at the time of referral to the audiological team. Treatment alternative with sound generators was initiated after counseling both the boy and his parents. During a follow-up period of a year at the child neurology outpatient clinic, his parents reported that he continuously removed his retrainers. As there was no change in seizure frequency, they stopped the sound generator therapy.
Patient 2 is the third son of healthy parents, born after an uneventful pregnancy and delivery. At age three months, he suffered from pneumococcal meningitis. He developed a spastic right-sided hemiplegia and localization-related complex partial seizures over a period of two years. He became seizure-free and did not use antiepileptic drugs for several years until he developed startle seizures at age 10. A sudden sound resulted in a tonic seizure, sometimes accompanied by urinary or fecal incontinence. Seizure frequency was variable, depending on triggering factors such as a pencil falling in the classroom or a school bell ringing. He had at least 2-3 seizures a day; they did not respond to antiepileptic drugs. He attends a normal school. His MR imaging showed an encephalomalacia of the left hemisphere. Epilepsy surgery was considered, but his parents preferred the noninvasive treatment alternative with sound generators. He and his parents were counseled by our audiological team, and therapy with sound generators was initiated with considerable success. Over a period of a few years, seizure frequency was reduced to one per month. He was even able to watch fireworks and to set one off himself when wearing his sound generators.