Document Detail

Paget disease and sensorineural hearing loss associated with spiral ligament degeneration.
MedLine Citation:
PMID:  15891639     Owner:  NLM     Status:  MEDLINE    
HYPOTHESIS: Previously unreported cystic degeneration of the spiral ligament in cases of Paget disease. BACKGROUND: About 70% of cases of Paget disease involve the skull, with hearing affected in approximately 50% of these. The hearing impairment may be sensorineural, mixed, or, rarely, only conductive. The etiology and pathogenesis of the hearing loss are not yet understood, and reports in the literature are inconsistent regarding the pathologic changes responsible for sensorineural hearing loss. Of six pairs of temporal bones from patients with Paget disease in the temporal bone collection of a research institution, two pairs have abnormalities not previously associated with sensorineural hearing loss or Paget disease. We report the histopathologic findings in these temporal bones. METHODS: The temporal bones were fixed in formalin, decalcified in ethylenediaminetetraacetic acid, embedded in celloidin, and sectioned in the horizontal plane at a thickness of 20 microm. Every 10th section was stained with hematoxylin-eosin and mounted on glass slides. The sections were examined by light microscopy. RESULTS: Cystic degeneration of the spiral ligament, primarily in the basal segment, was found in both cases. Endolymphatic hydrops and a small endolymphatic sac with calcification of the perisaccular tissue were found in one case. CONCLUSIONS: Cystic degeneration of the spiral ligament has not been previously reported and may be unique to Paget disease. This is consistent with recent literature showing a previously unsuspected role of the spiral ligament in sensorineural hearing loss.
Karen Borne Teufert; Fred Linthicum
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Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  Otology & neurotology : official publication of the American Otological Society, American Neurotology Society [and] European Academy of Otology and Neurotology     Volume:  26     ISSN:  1531-7129     ISO Abbreviation:  Otol. Neurotol.     Publication Date:  2005 May 
Date Detail:
Created Date:  2005-05-13     Completed Date:  2005-08-17     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  100961504     Medline TA:  Otol Neurotol     Country:  United States    
Other Details:
Languages:  eng     Pagination:  387-91; discussion 391     Citation Subset:  IM    
Histopathology Department, House Ear Institute, Los Angeles, California 90057, USA.
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MeSH Terms
Calcinosis / etiology
Cochlear Diseases / pathology
Cochlear Duct / pathology*
Cysts / pathology
Endolymphatic Hydrops / etiology,  pathology
Endolymphatic Sac / pathology
Fatal Outcome
Hearing Loss, Sensorineural / etiology*,  pathology*
Osteitis Deformans / complications*
Saccule and Utricle
Temporal Bone / pathology
Vestibular Diseases / etiology

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