Moderate to severe sensorineural hearing impaired child: Analysis of etiology, intervention, and outcome

Ruben, Robert J.; Levine, R. A.; Baldinger, Esther; Silver, Marilayn; Umano, Helen; Fishman, Gary; Feldman, W E; Stein, Myra; Kruger, Barbara

doi:10.1288/00005537-198201000-00009

Cited by 34 publications

(1 citation statement)

References 7 publications

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“…The question “Will my child’s hearing loss get worse over time?” presents a particular challenge for hearing healthcare professionals as changes in hearing over time are difficult to predict. Progressive sensorineural hearing loss (SNHL) is reported in 4% to 48% of pediatric patients with SNHL (Ruben et al 1982; Böhme 1985; Parving 1985; Newton & Rowson 1988; Brookhouser et al 1994; Berrettini et al 1999; Barreira-Nielsen et al 2016). Important considerations related to progressive SNHL in pediatric patients are (i) identifying the underlying etiologies, (ii) identifying a sufficient number of patients to understand characteristics, and (iii) defining hearing loss progression.…”

Section: Introductionmentioning

confidence: 99%

Cholesteatoma Is Associated With Pediatric Progressive Sensorineural Hearing Loss

et al. 2021

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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.

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Section: Introductionmentioning

confidence: 99%

Cholesteatoma Is Associated With Pediatric Progressive Sensorineural Hearing Loss

et al. 2021

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Screening for Auditory Dysfunction in Infants by Evoked Oto-Acoustic Emissions

Bonfils

Uziel²,

Pujol³

1988

Archives of Otolaryngology - Head and Neck Surgery

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Calculation of recurrence risks for heterogeneous genetic disorders

2000

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We present general formulae for several common situations in the genetic counseling of heterogeneous disorders. The occurrence or not of parental consanguinity is taken into account, since it distorts significantly the prior probabilities favoring the different mechanisms. Nonsyndromic deafness is used as a numerical application, since it can be produced by any type of monogenic inheritance and can be mixed with variable proportions of environmental cases. Recurrence risks are calculated including or not including environmental factors in the origin of the defect. In underdeveloped countries the proportion of environmentally determined cases of deafness is significantly higher than in first-world countries. Therefore, when environmental causes cannot be excluded, recurrence risks are always higher in developed than in developing countries. On average, when parental consanguinity is present there is a significant increase in recurrence risks for deafness, whether environmental factors are included or not.

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Moderate to severe sensorineural hearing impaired child: Analysis of etiology, intervention, and outcome

Cited by 34 publications

References 7 publications

Cholesteatoma Is Associated With Pediatric Progressive Sensorineural Hearing Loss

Cholesteatoma Is Associated With Pediatric Progressive Sensorineural Hearing Loss

Screening for Auditory Dysfunction in Infants by Evoked Oto-Acoustic Emissions

Calculation of recurrence risks for heterogeneous genetic disorders

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