Em. Monsell et al., HEARING-LOSS IN PAGETS-DISEASE OF BONE - EVIDENCE OF AUDITORY-NERVE INTEGRITY, The American journal of otology, 16(1), 1995, pp. 27-33
Auditory brainstem responses (ABRs) were recorded in 64 years with rad
iographically confirmed Paget's disease involving the skull. Responses
were absent in eight ears, all of which had elevated high pure-tone t
hresholds. Auditory brainstem responses were interpreted as normal in
56 ears; none were abnormal. Computed tomography and digital image ana
lysis were used to quantify internal auditory canal (IAC) dimensions.
The midlength diameter and minimum diameter of the IAC of 68 temporal
bones from subjects with Paget's disease were found to have no statist
ically significant relation to hearing thresholds. Increased IAC lengt
h showed a limited relation to reduced hearing level in pagetic subjec
ts, possibly consistent with bossing adjacent to the porus acusticus.
Findings support the principle that hearing loss in Paget's disease of
bone is generally associated with intact auditory nerve function and
also support a cochlear site of lesion.