The mechanism of hearing loss in Paget's disease of bone.

Objectives/Hypothesis The mechanism of hearing loss (HL) in Paget's disease of bone was investigated. The present study was a systematic, prospective, controlled set of clinical investigations to test the hypothesis that there is a general underlying mechanism of HL in Paget's disease of bone and to gain additional insights into the auditory and otologic dynamics of this disease. Specific questions were 1) whether the mechanism is cochlear or retrocochlear and 2) whether the bone mineral density of the cochlear capsule is related to hearing levels. Study Design Several double-blinded, cross-sectional, prospective, correlational studies were conducted in a population of elderly human subjects with skull involvement with Paget's disease versus a control population of elderly subjects free of Paget's disease. Demographic and clinical data were recorded. Longitudinal observations were made in subjects under treatment. Methods Subjects were recruited from a Paget's disease clinic. Pure-tone auditory thresholds, word recognition, and auditory brainstem responses (ABRs) were recorded. The dimensions of the internal auditory canals were measured using computed tomographic (CT) images and digital image analysis. The precision, accuracy, and temporal stability of methods to measure the bone mineral density of the cochlear capsule and an adjacent area of nonotic capsule bone were validated and applied. Correlations were sought between hearing levels and cochlear capsule bone mineral density. Results ABRs were recorded in 64 ears with radiographically confirmed Paget's disease involving the skull. Responses were absent in eight ears, all of which had elevated high pure-tone thresholds. ABRs were interpreted as normal in 56 ears; none were abnormal. The mid-length diameter and minimum diameter of the internal auditory canal of 68 temporal bones from subjects with Paget's disease were found to have no statistically significant relationship to hearing thresholds. The Pearson product-moment correlation coefficients (age- and sex-adjusted) in the group with Paget's disease involving the temporal bone were −0.63 for left ears and −0.73 for right ears for high-frequency air conduction pure-tone thresholds (mean of 1, 2, and 4 kHz) versus cochlear capsule density. Correlation coefficients (age- and sex-adjusted) between cochlear capsule density and air-bone gap (mean at 0.5 and 1 kHz) for the affected group were −0.67 for left ears and −0.63 for right ears. All correlations between hearing thresholds and cochlear capsule density in pagetic subjects were significant at P < .001. The regressions were consistent throughout the ranges of hearing level. There were no significant correlations between cochlear capsule mean density and hearing level in the volunteer subjects. Conclusions The evidence supports the existence of a general, underlying, cochlear mechanism of pagetic HL that is closely related to loss of bone mineral density in the cochlear capsule. This mechanism accounts well for both the high-frequency sensorineural HL and the air-bone gap. Early identification, radiographic diagnosis of temporal bone involvement, and vigorous treatment with third-generation bisphosponates are important to limit the development and progression of pagetic HL.