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Objectives: This study identified an association between cholesteatoma and progressive sensorineural hearing loss using a large pediatric longitudinal audiologic dataset. Cholesteatoma is a potential sequela of chronic otitis media with effusion, a commonly observed auditory pathology that can contribute to hearing loss in children. The purpose of this report is to (i) describe the process of identifying the association between cholesteatoma and progressive sensorineural hearing loss in a large pediatric dataset and (ii) describe the audiologic data acquired over time in patients identified with cholesteatoma-associated progressive sensorineural hearing loss.Design: Records of patients included in the Audiologic and Genetics Database (n = 175,215 patients) were examined using specified criteria defining progressive hearing loss. A linear regression model examined the log frequency of all diagnostic codes in the electronic health record assigned to patients for a progressive hearing loss cohort compared with a stable hearing loss group. Based on findings from the linear regression analysis, longitudinal audiometric air (AC) and bone conduction (BC) thresholds were extracted for groups of subjects with cholesteatomaassociated progressive (n = 58 subjects) and stable (n = 55 subjects) hearing loss to further analyze changes in hearing over time. Results:The linear regression analyses identified that diagnostic codes for cholesteatoma were associated with progressive sensorineural hearing loss in children. The longitudinal audiometric data demonstrated within-subject changes in masked BC sensitivity consistent with progressive sensorineural hearing loss in children diagnosed with cholesteatoma. Additional analyses showed that mastoidectomy surgeries did not appear to contribute to the observed progressive hearing loss and that a high number of cholesteatoma patients with progressive hearing loss had normal-hearing thresholds at their first test. Conclusions:The statistical analyses demonstrated an association between cholesteatoma and pediatric progressive sensorineural hearing loss. These findings inform clinical management by suggesting that children with cholesteatoma diagnoses may be at increased risk for progressive sensorineural hearing loss and should receive continued monitoring even after a normal masked BC baseline has been established.

The middle-ear system relies on a balance of mass and stiffness characteristics for transmitting sound from the external environment to the cochlea and auditory neural pathway. Phase is one aspect of sound that, when transmitted and encoded by both ears, contributes to binaural cue sensitivity and spatial hearing. The study aims were (i) to investigate the effects of middle-ear stiffness on the auditory brainstem neural encoding of phase in human adults with normal pure-tone thresholds and (ii) to investigate the relationships between middle-ear stiffness-induced changes in wideband acoustic immittance and neural encoding of phase. The auditory brainstem neural encoding of phase was measured using the auditory steady-state response (ASSR) with and without middle-ear stiffness elicited via contralateral activation of the middle-ear muscle reflex (MEMR). Middle-ear stiffness was quantified using a wideband acoustic immittance assay of acoustic absorbance. Statistical analyses demonstrated decreased ASSR phase lag and decreased acoustic absorbance with contralateral activation of the MEMR, consistent with increased middle-ear stiffness changing the auditory brainstem neural encoding of phase. There were no statistically significant correlations between stiffness-induced changes in wideband acoustic absorbance and ASSR phase. The findings of this study may have important implications for understanding binaural cue sensitivity and horizontal plane sound localization in audiologic and otologic clinical populations that demonstrate changes in middle-ear stiffness, including cochlear implant recipients who use combined electric and binaural acoustic hearing and otosclerosis patients.

Purpose: Until recently, there has been little investigation on the effects of cochlear implantation on the transmission of acoustic stimuli through the middle-ear system. Recent studies have shown that cochlear implantation decreases low-frequency acoustic absorbance, consistent with a stiffer middle-ear system postsurgery. The objectives of this study are (a) to investigate the time course of changes in acoustic absorbance post–cochlear implantation in the implanted ear and (b) to compare changes in acoustic absorbance between implanted and nonimplanted ears over time. Method: Seventeen adult cochlear implant (CI) recipients within 6 months of device activation participated in this study. Wideband acoustic absorbance was measured in both ears at one to six different time points from pre-implantation up to 6-month postactivation. Analyses examined (a) changes in acoustic absorbance as compared to pre-implantation and (b) differences in acoustic absorbance between implanted and nonimplanted ears over time. Results: Acoustic absorbance in the implanted ear decreased postsurgery for frequencies lower than 1.5 kHz and persisted through at least 6-month postactivation. We also observed that the spectral range of decreased acoustic absorbance in the implanted ear decreased with longer time postsurgery. Differences in acoustic absorbance between implanted and nonimplanted ears occurred over a broad spectral range at the activation time point and persisted through at least 3-month postactivation, though for a narrower spectral range at the later time point. Conclusions: Cochlear implantation increased middle-ear stiffness as indicated by decreased acoustic absorbance of low-frequency acoustic power. The findings of this study are consistent with those of previous studies and may have important implications toward understanding spatial hearing and programming of acoustic components for CI-combined electric and binaural acoustic stimulation patients.