Cholesteatoma Is Associated With Pediatric Progressive Sensorineural Hearing Loss

Abstract

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 cholesteatoma-associated 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.

Figure 1.

Linear regression analyses (panel A) comparing the log frequency of diagnostic codes between a progressive hearing loss cohort and stable hearing loss control group. The residuals from the linear regression (panel B) were used to identify outlier diagnostic codes that occurred more frequently in the progressive hearing loss cohort (high outliers). Various cholesteatoma diagnostic codes were identified as high, positive-residual outliers (highlighted in blue in Panel B). Analyses shown are for CHOP subjects. Comparable analyses were completed separately for VUMC patients (data not shown).

Figure 1.

Linear regression analyses (panel A) comparing the log frequency of diagnostic codes between a progressive hearing loss cohort and stable hearing loss control group. The residuals from the linear regression (panel B) were used to identify outlier diagnostic codes that occurred more frequently in the progressive hearing loss cohort (high outliers). Various cholesteatoma diagnostic codes were identified as high, positive-residual outliers (highlighted in blue in Panel B). Analyses shown are for CHOP subjects. Comparable analyses were completed separately for VUMC patients (data not shown).

Figure 2.

The number of subjects with cholesteatoma-associated progressive SNHL (blue bars) and stable SNHL (orange bars) as a function of hearing loss severity based on masked BC PTA4 for the first test (panel A), last test (panel B), and the degree of BC PTA4 shift (panel C).

Figure 2.

The number of subjects with cholesteatoma-associated progressive SNHL (blue bars) and stable SNHL (orange bars) as a function of hearing loss severity based on masked BC PTA4 for the first test (panel A), last test (panel B), and the degree of BC PTA4 shift (panel C).

Figure 2.

The number of subjects with cholesteatoma-associated progressive SNHL (blue bars) and stable SNHL (orange bars) as a function of hearing loss severity based on masked BC PTA4 for the first test (panel A), last test (panel B), and the degree of BC PTA4 shift (panel C).

Figure 3.

The number of subjects with cholesteatoma-associated progressive SNHL (blue bars) and stable SNHL (orange bars) as a function of the number of mastoidectomy surgeries.

Figure 4.

PTA4 BC and AC threshold shifts between the first and last tests for 68 ears with cholesteatoma that meet criteria for progressive hearing loss. BC threshold shifts (blue bars) indicate that all ears have shifts to poorer hearing of at least 10 dB. AC thresholds shifts (red bars) indicate ears with improved hearing for the last test (left), more stable hearing (middle), and poorer hearing for the last test (right). Note the differences in identifying progressive hearing loss between BC and AC thresholds.