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[Congenital cerebrospinal fluid pressure labyrinth]
O Michel1, J Breunsbach, R Matthias
1Klinik und Poliklinik für Hals-Nasen-Ohren-Heilkunde, Universität zu Köln.
HNO
|December 1, 1991
Summary
Three children with congenital mixed deafness likely have an X-linked syndrome involving stapes fixation and perilymph gusher. Surgical intervention for gusher was successful, preserving hearing.
Area of Science:
- Otolaryngology
- Genetics
- Pediatric Audiology
Background:
- Congenital mixed deafness can have complex etiologies.
- Fixed stapes footplate and perilymph gusher are rare but significant findings.
- Cerebrospinal fluid (CSF) fistulas can be associated with hearing loss.
Observation:
- Audiometric and CT scan data from three children with bilateral congenital mixed deafness were analyzed.
- Two children presented with a fixed stapes footplate, experiencing a perilymph gusher during tympanotomy.
- Enlarged auditory canal fundus and absent bony barrier to the vestibule suggested potential CSF fistulas in two brothers.
Findings:
- A perilymph gusher, occurring during platinotomy in cases of stapes fixation, was successfully managed with fat grafts, maintaining hearing.
- No patent or abnormal cochlear aqueduct was identified in any of the three cases.
- The clinical presentation suggests a potential X-linked mixed deafness syndrome characterized by stapes fixation and perilymph gusher.
Implications:
- This study proposes a classification for congenital perilymph-CSF shunts.
- Early identification and management of stapes fixation and perilymph gusher are crucial for hearing preservation.
- Understanding this X-linked syndrome may aid in genetic counseling and future research.