Gusher phenomenon

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A deformity of the inner ear in which the perilymph space of the inner ear is in further connection with the cerebrospinal fluid space is referred to as a Gusher phenomenon ( English gusher "spring source") or pressure labyrinth . The Gusher phenomenon is one of the malformations inherited via the X chromosome .

The pressure of the CSF on the fluid in the inner ear leads to an impairment of movement of the stapes footplate . In tone audiometry , conductive hearing loss or combined hearing loss can be found. The tone audiogram is very similar to that of otosclerosis . However, if the oval window and thus the inner ear is opened during otosclerosis surgery , a small amount of perilymph will not emerge as expected, but brain fluid in a surge. This (eponymous) event requires a corresponding sealing of the oval window and usually results in considerable damage to the inner ear function.

Indications of the presence of a Gusher phenomenon arise u. U. already from a particularly pronounced hearing impairment in the low frequency range and in particular from a preserved stapedius reflex . In contrast to otosclerosis, the history reveals a hearing loss that has existed since early childhood. Changes in the bony labyrinth can be seen in high-resolution computed tomography , in particular a greatly enlarged internal auditory canal .

literature

  • CW Cremers: How to prevent a stapes gusher. In: Adv. Otorhinolaryngol. 2007; 65, p. 278.
  • D. Plester et al.: Atlas of ear surgery. Kohlhammer, 1989.
  • B. Welleschik , E. Salomonowitz: Inner ear malformation. Otoliquorrhea in stapedectomy. In: Laryng. Rhinol. Otol. 65, 1986, p. 688.
  • RJ Gorlin, HV Toriello, MM Cohen: Hereditary hearing loss and its syndromes. Oxford University Press, USA 1995. (online)