title: [Congenital cerebrospinal fluid pressure labyrinth].
abstract: The audiograms and CT scans of three children with a bilateral congenital mixed deafness are presented. Two children underwent an exploratory tympanotomy revealing a fixed stapes footplate: a perilymph gusher arose during platinotomy in both cases. The gusher was controlled successfully with a large fat graft in both children, and hearing remained unchanged. Two of the children were brothers: they had no other deformities except an enlarged fundus of the auditory canal on CT scans, and no clearly defined bony barrier to the vestibule, suggesting a cerebrospinal fluid fistula. Neither a patent nor an abnormal cochlear aqueduct could be detected in all three cases. It is likely that the three patients present an X-linked mixed deafness syndrome with fixation of the stapedial foot plate and perilymph gusher. A classification of congenital perilymph-CSF shunts is proposed.
authors.affiliation: Klinik und Poliklinik für Hals-Nasen-Ohren-Heilkunde, Universität zu Köln.
authors.forename: O
authors.initials: O
authors.lastname: Michel
authors.forename: J
authors.initials: J
authors.lastname: Breunsbach
authors.forename: R
authors.initials: R
authors.lastname: Matthias
mesh_terms: D002558:Cerebrospinal Fluid Otorrhea / Q000235:genetics* / Q000503:physiopathology / Q000601:surgery*; D015170:Cerebrospinal Fluid Pressure / Q000502:physiology*; D002648:Child; D003638:Deafness / Q000235:genetics* / Q000601:surgery*; D005260:Female; D008040:Genetic Linkage / Q000235:genetics; D006801:Humans; D010498:Perilymph / Q000502:physiology*; D012729:Sex Chromosome Aberrations / Q000235:genetics; D013199:Stapes / Q000002:abnormalities; D013201:Stapes Surgery; D013577:Syndrome; D014057:Tomography, X-Ray Computed; D014960:X Chromosome
vernacular_title: Das angeborene Liquordrucklabyrinth.