title: In search of pathologic correlates for hearing loss and vertigo in Paget's disease. A clinical and histopathologic study of 26 temporal bones.
abstract: Mixed sensorineural and conductive hearing loss is a common clinical manifestation of Paget's disease of the temporal bone, and while there are numerous clinical and pathologic reports on the condition, none have identified a consistent pathologic explanation for the hearing loss. We performed histologic studies on 26 temporal bones exhibiting Paget's disease from 16 persons, of whom 7 had audiometric testing performed. Contrary to common opinion, the conductive hearing loss is not caused by ossicular fixation; in fact, no cause could be found in the seven ears with documented conductive hearing losses. While the sensorineural hearing losses were greater than normal for age, we could not identify cochlear disorders that could be attributed to Paget's disease. It is concluded that the hearing losses in Paget's disease are caused by changes in bone density, mass, and form that serve to dampen the finely tuned motion mechanics of the middle and inner ears.
authors.affiliation: Department of Otology and Laryngology, Harvard Medical School, Boston, Massachusetts.
authors.forename: U
authors.initials: U
authors.lastname: Khetarpal
authors.forename: H F
authors.initials: HF
authors.lastname: Schuknecht
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D001299:Audiometry; D001844:Bone Conduction; D001846:Bone Development; D001862:Bone Resorption; D003051:Cochlea / Q000473:pathology; D005260:Female; D006319:Hearing Loss, Sensorineural / Q000209:etiology* / Q000473:pathology; D006801:Humans; D008297:Male; D008875:Middle Aged; D009456:Neurofibromatosis 1 / Q000150:complications; D010001:Osteitis Deformans / Q000150:complications* / Q000473:pathology; D010014:Osteolysis / Q000473:pathology; D013701:Temporal Bone / Q000473:pathology*; D014717:Vertigo / Q000209:etiology* / Q000473:pathology