Temporal bone findings in a case of susac's syndrome

Howard W. Francis, Chadi Makary, Christopher Halpin, Benjamin T. Crane, Saumil N. Merchant

Research output: Contribution to journalArticle

Abstract

OBJECTIVE: To describe the histopathologic findings in the temporal bones of a patient with Susac's syndrome (SS). BACKGROUND: The key clinical features of SS consist of symptoms of encephalopathy, visual defects due to occlusion of branches of the retinal artery, and sensorineural hearing loss. The otopathology in SS has not been described. MATERIALS AND METHODS: A 51-year-old woman was hospitalized with severe headache, rapidly progressive encephalopathy, and bilateral low-frequency sensorineural hearing loss. Magnetic resonance imaging showed lesions of the corpus callosum. Fluorescein angiography of the eyes showed focal areas of irregular retinal artery caliber and leakage from small vessels. SS was diagnosed. She died of a pulmonary embolus 1 month after onset of symptoms. Both temporal bones were prepared in celloidin and examined using light microscopy. RESULTS: Findings were nearly identical in both temporal bones. The apical halves of both cochleae showed widespread atrophy of structures of the cochlear duct (inner and outer hair cells, tectorial membranes, striae vasculares, spiral ligaments, and spiral limbi). The apical parts of both cochleae also showed apparent occlusion of capillaries within the stria vascularis and related areas of the cochlear duct. Cochlear neurons were present in normal numbers. There was no endolymphatic hydrops. The vestibular sense organs were normal for age. CONCLUSION: This first reported otopathologic case of SS with hearing loss showed atrophy and degeneration involving the apical halves of the cochlear duct without inflammation or infection. The findings were consistent with capillary occlusion as being responsible for the atrophy.

Original languageEnglish (US)
Pages (from-to)1198-1204
Number of pages7
JournalOtology and Neurotology
Volume32
Issue number8
DOIs
StatePublished - Oct 1 2011
Externally publishedYes

Fingerprint

Susac Syndrome
Temporal Bone
Cochlear Duct
Cochlea
Atrophy
Sensorineural Hearing Loss
Brain Diseases
Outer Auditory Hair Cells
Inner Auditory Hair Cells
Tectorial Membrane
Spiral Ligament of Cochlea
Endolymphatic Hydrops
Sense Organs
Stria Vascularis
Retinal Artery Occlusion
Retinal Artery
Collodion
Fluorescein Angiography
Corpus Callosum
Embolism

Keywords

  • Otopathology
  • Sensorineural hearing loss
  • Susac's syndrome
  • Temporal bone histopathology

ASJC Scopus subject areas

  • Otorhinolaryngology
  • Sensory Systems
  • Clinical Neurology

Cite this

Francis, H. W., Makary, C., Halpin, C., Crane, B. T., & Merchant, S. N. (2011). Temporal bone findings in a case of susac's syndrome. Otology and Neurotology, 32(8), 1198-1204. https://doi.org/10.1097/MAO.0b013e31822e9665

Temporal bone findings in a case of susac's syndrome. / Francis, Howard W.; Makary, Chadi; Halpin, Christopher; Crane, Benjamin T.; Merchant, Saumil N.

In: Otology and Neurotology, Vol. 32, No. 8, 01.10.2011, p. 1198-1204.

Research output: Contribution to journalArticle

Francis, HW, Makary, C, Halpin, C, Crane, BT & Merchant, SN 2011, 'Temporal bone findings in a case of susac's syndrome', Otology and Neurotology, vol. 32, no. 8, pp. 1198-1204. https://doi.org/10.1097/MAO.0b013e31822e9665
Francis HW, Makary C, Halpin C, Crane BT, Merchant SN. Temporal bone findings in a case of susac's syndrome. Otology and Neurotology. 2011 Oct 1;32(8):1198-1204. https://doi.org/10.1097/MAO.0b013e31822e9665
Francis, Howard W. ; Makary, Chadi ; Halpin, Christopher ; Crane, Benjamin T. ; Merchant, Saumil N. / Temporal bone findings in a case of susac's syndrome. In: Otology and Neurotology. 2011 ; Vol. 32, No. 8. pp. 1198-1204.
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