Electrical Status Epilepticus in Slow Wave Sleep: Prospective Case Study of a Cortical Hearing Impairment

Abstract

AbstractThe 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. Abbreviations: ABR = auditory brainstem response, CPA = conditioned play audiometry, CSWS = continuous spikes and waves during slow wave sleep, EEG = electroencephalography, ESES = electrical status epilepticus of slow wave sleep, LKS = Landau-Kleffner syndrome, MRI = magnetic resonance imaging, MRL = minimal response level, REM = rapid eye movement, TEOAEs = transient evoked otoacoustic emissions, VRA = visual reinforcement audiometry