Electrical status epilepticus in slow wave sleep: prospective case study of a cortical hearing impairment
C G Fowler1, J E Kile, K E Hecox
1Department of Communicative Disorders, University of Wisconsin-Madison, 53706, USA.
Insights
A rare childhood disorder, electrical status epilepticus in slow wave sleep, caused central hearing impairment in a young girl. Treatment with anticonvulsants and steroids improved cognitive and social skills, with ongoing gains in communication.
Area of Science:
- Pediatric Neurology
- Neurodevelopmental Disorders
- Auditory Processing
Background:
- Mosaic Down syndrome, leukemia, and chemotherapy are risk factors for hearing impairment.
- Early and regular hearing assessments are crucial for identifying auditory processing issues.
- Electrical status epilepticus in slow wave sleep (ESES) is a rare pediatric neurological disorder.
Observation:
- A young girl presented with central hearing impairment, lack of responsiveness to auditory stimuli, and regression in speech, language, and cognitive skills.
- Abnormal electroencephalographic (EEG) activity was observed bilaterally.
- The patient had a history of mosaic Down syndrome, leukemia, and chemotherapy.
Findings:
- Diagnosis of electrical status epilepticus in slow wave sleep (ESES) was established.
- ESES was associated with significant auditory processing deficits and developmental regression.
- EEG monitoring confirmed bilateral abnormalities characteristic of ESES.
Implications:
- Early diagnosis and intervention for ESES are critical for managing associated neurodevelopmental deficits.
- Treatment with anticonvulsant medications and steroids can ameliorate EEG abnormalities and improve cognitive and social functioning.
- While communication skills may improve more slowly, the case highlights the potential for significant recovery with appropriate management.
Abstract:
The development of a central hearing impairment is described in a young girl with risk factors for hearing impairment that included mosaic Down syndrome, leukemia, and chemotherapy. This case is unusual in the prospective regularity with which hearing was assessed from birth. The diagnosis is electrical status epilepticus in slow wave sleep, a rare childhood disorder, which was associated with lack of responsiveness to auditory signals, regression of emerging speech and language and other cognitive skills, and abnormal electroencephalographic (EEG) activity in both hemispheres. Treatment of the disorder with anticonvulsant medications and steroids has ameliorated the condition by suppressing the abnormal EEG activity and allowing substantial improvements in cognitive and social skills, although communication skills are improving more slowly.
