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X-linked mixed deafness with congenital fixation of the stapedial footplate and perilymphatic gusher.

An X-linked syndrome characterized in males by profound mixed deafness, vestibular abnormalities, congenital fixation of the stapes and perilymphatic otorrhea on attempted stapedectomy has been documented in a large Caucasian kindred. Female heterozygotes have similar but milder audiologic abnormalities. The syndrome of congenital fixation of the stapes with perilymphatic gusher may be a relatively common form of X-linked deafness and is an important clinical entity because affected males may be significantly benefited by sound amplification.

Audiometry↗

[Local pO2 measurements in the cochlear perilymph of guinea pigs in oxygen and oxygen/carbon dioxide breathing (author's transl)].

After breathing a mix of 10% CO2 and 90% O2 or hyperbaric O2 (2 bar absolute) an appreciable rise of pO2 can be demonstrated by D.C. polarography in the scala tympani of the guinea pig chochlea. When test animals are made to breathe 100% pure O2 at normal pressure the rise of pO2 is significantly less pronounced. The observations made in these animal experiments suggest that the inhalation of CO2O2 currently constitutes the only valid inhalation treatment in acute inner ear deafness. Further compression of hyperbaric O2 is associated with a clear-cut rise of pO2. It should, however, be remembered that hyperbaric oxygen may produce toxic effects in various body tissues.

Animals↗