Archives of oto-rhino-laryngology

, Volume 242, Issue 1, pp 71–76 | Cite as

The endolymphatic sac in the Mondini disorder

  • Ruth Gussen
Article

Summary

The endolymphatic sacs are described in temporal bone specimens from a 31-year-old man with bilateral Mondini disorder. The ducts and sacs are thin-walled, cyst-like structures with complete absence of loose vascular perisac tissue, and are directly apposed to the bone of the vestibular aqueduct. Histological evidence of severe bone erosion is present in these specimens and is most marked in the intermediate and distal portions of the vestibular aqueduct. It is also present in the foveal region of the posterior temporal bone surface underlying the sac. Erosion of the bony wall of the paravestibular canaliculus (PVC) is demonstrable, with incorporation of the vein of the PVC inside the margin of the widened vestibular aqueduct. These findings suggest a causal relationship between pressure within the endolymphatic duct and sac and erosion of the surrounding bone. The absence of endolymphatic hydrops of the cochlea and vestibular organs in the Mondini disorder constrast significantly with the endolymphatic hydrops seen in Meniere's disease.

Key words

Mondini Vestibular aqueduct Endolymphatic sac Endolymphatic hydrops 

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References

  1. 1.
    Beal DD, Davey PR, Lindsay JR (1967) Inner ear pathology of congenital deafness. Arch Otolaryngol 85:134–142Google Scholar
  2. 2.
    Fraser JS (1927) A case of congenital deafness showing malformation of the bony and membraneous labyrinth on both sides. J Laryngol Otol 42:315Google Scholar
  3. 3.
    Gussen R (1968) Mondini type of genetically determined deafness. J Laryngol Otol 82:41–55Google Scholar
  4. 4.
    Gussen R (1972) Abnormalities of the endolymphatic sac system. Ann Otol Rhinol Laryngol 81:235–240Google Scholar
  5. 5.
    Gussen R (1982) Vascular mechanisms in Meniere's disease. Arch Otolaryngol 108:544–549Google Scholar
  6. 6.
    Paparella MM (1980) Mondini's deafness. A review of histopathology. Ann Otol Rhinol Laryngol [Suppl 67] 89:1–10Google Scholar
  7. 7.
    Peron DL, Schuknecht HF (1975) Congenital cholesteatoma with other anomalies. Arch Otolaryngol 102:498–505Google Scholar
  8. 8.
    Schuknecht HF (1980) Mondini dysplasia. A clinical and pathological study. Ann Otol Rhinol Laryngol [Suppl 65] 89:1–23Google Scholar
  9. 9.
    Secretan JP (1944) A propos d'ectasie de l'oreille interne. Pract Oto-Rhino-Laryngol 6:281Google Scholar
  10. 10.
    Sugiura S, Nomura Y (1979) Histological findings in a case of Mondini anomaly of the inner ear. Ann Otol Rhinol Laryngol 79:1139–1142Google Scholar

Copyright information

© Springer-Verlag 1985

Authors and Affiliations

  • Ruth Gussen
    • 1
  1. 1.Division of Head and Neck Surgery and The Department of Pathology, 31-24 Rehabilitation CenterUCLA School of MedicineLos AngelesUSA

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