Paget disease and sensorineural hearing loss associated with spiral ligament degeneration.
OBJECTIVE
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.
Teufert
Karen Borne
KB
Histopathology Department, House Ear Institute, Los Angeles, California 90057, USA. kteufert@hei.org
Linthicum
Fred
F
D000368:Aged; D002114:Calcinosis / Q000209:etiology; D015834:Cochlear Diseases / Q000473:pathology; D003053:Cochlear Duct / Q000473:pathology*; D003560:Cysts / Q000473:pathology; D018159:Endolymphatic Hydrops / Q000209:etiology / Q000473:pathology; D004712:Endolymphatic Sac / Q000473:pathology; D017809:Fatal Outcome; D006319:Hearing Loss, Sensorineural / Q000209:etiology* / Q000473:pathology*; D006801:Humans; D008297:Male; D010001:Osteitis Deformans / Q000150:complications*; D012444:Saccule and Utricle; D013701:Temporal Bone / Q000473:pathology; D015837:Vestibular Diseases / Q000209:etiology