Bilateral objective tinnitus in an infant with tuberous sclerosis

This is the first report of objective tinnitus presenting as audible spontaneous otoacoustic emission in a patient with tuberous sclerosis. The tinnitus was loud, continuous, and high pitched: 7757.8 and 6257.8 Hz. The auditory system may be dysfunctional in patients with tuberous sclerosis. Possible causes of tinnitus in these patients are abnormal myelination and dysfunctional axons and neurons associated with tuberous sclerosis. A disturbance of the outer hair cells or the MOC efferent fibers innervating the outer hair cells is considered to be the source of the loud spontaneous otoacoustic emission.