Abnormal Brain Stem Auditory Evoked Potentials in a Girl with the Central Alveolar Hypoventilation Syndrome

Abstract

Brainstem auditory evoked potentials were recorded in a 3-year-old girl with the central alveolar hypoventilation syndrome (Ondine's syndrome). Abnormal findings were seen at the level of the mid to upper brain stem (wave III), which was not reproducibly recordable on the left side. This electrophysiologic abnormality is consistent with a previous finding in a patient with sleep apnea.

Congenital central hypoventilation syndrome: An update

“…Based on the initial premise that a centrally located defect is present in CCHS, it is not surprising that over the years multiple attempts, albeit unsuccessful, have been made to identify structural central nervous system abnormalities. Earlier reports of hypoplasia of the arcuate nucleus in one patient with CCHS, 39 and the presence of abnormal evoked potential responses to auditory stimuli [40][41][42] have further suggested that a brainstem lesion may be present. In addition, central hypoventilation syndrome has been reported in occasional patients with cerebrovascular malformations, [43][44][45][46][47] and in patients with central nervous system infections.…”

Breath-Holding Spells May be Associated With Maturational Delay in Myelination of Brain Stem

“…In the presence of pathologic processes affecting the brain stem auditory pathways, a prolongation of latencies or loss of these potentials related to the affected level are expected (Jewett et al, 1970;Moller and Jannetta, 1982). Previous studies have reported that the BAEP are influenced by various diseases affecting the central nervous system, particularly in the presence of brain stem involvement (Litscher et al, 1996;Prasher and Bannister, 1986). Increases in absolute and IPL, increases in duration of the waves, and decreases in amplitude of the waves suggest axonal demyelization or asynchronization at the axonal or synaptic levels (Rosenhall et al, 2003).…”

Temporary central hypoventilation following craniopharyngioma resection

“…Neurologic causes of hypoventilation have been well described. Most cases of central hypoventilation are congenital, present in infancy, and are felt to be the result of primary defects of the brainstem respiratory centers (1)(2)(3)(4). Acquired cases of central hypoventilation have also been reported, but predominantly in the setting of insults to the brain stem, including: hemorrhage (5), neoplasm (6)(7)(8), enlargement of the fourth ventricle (9), encephalitis (10), ischemia (11), and infarction (12).…”

“…Hypoventilation from neurologic causes (central hypoventilation) has been well described (1)(2)(3)(4). While most cases are congenital in nature, numerous reports of acquired central hypoventilation exist.…”