Audiometric assessment of pediatric patients with cystic fibrosis

Kreicher, Kathryn L.; Bauschard, Michael J.; Clemmens, Clarice S.; Riva, Concetta Maria; Meyer, Ted A.

doi:10.1016/j.jcf.2017.10.007

Cited by 22 publications

(15 citation statements)

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“…In contrast, all individuals in the control group had normal hearing from 0.25 to 8 kHz by the more conservative criterion. In a large retrospective study of hearing impairment, Kreicher et al (2018) reported 31.8% of individuals with CF (mean = 8.3 yr.) had hearing loss in at least one ear (>15 dB HL at any frequency from 0.25 to 8 kHz). Most ears with hearing loss showed a slight to mild loss (15%) with a smaller number of moderate to profound losses (3%).…”

Section: Discussionmentioning

confidence: 99%

“…The copyright holder for this preprint this version posted May 5, 2020. ; https://doi.org/10.1101/2020.04.29.20084848 doi: medRxiv preprint retrospective examination of the prevalence and type of hearing loss in the pediatric CF population, 31.8% had hearing loss in at least one ear in the SF region (Kreicher et al, 2018). Further analysis of the type of hearing loss (considering each ear separately) showed that 74.1% had normal hearing, 5.3% had conductive loss, 2.3% sensorineural loss, 5.1% mixed hearing loss, and 13.2%…”

Section: Introductionmentioning

confidence: 99%

“…Cystic fibrosis (CF) is the most common congenital life-threatening genetic disease in Caucasians occurring in 1/2500 births and affecting over 70,000 individuals worldwide (Cystic Fibrosis Foundation, 2017; Kreicher, Bauschard, Clemmens, Riva, & Meyer, 2018). Individuals with CF experience recurrent pulmonary infections that are commonly treated with intravenous aminoglycoside (IV-AG) and glycopeptide antibiotics (Barclay et al, 1996; Becker & Cooper, 2013; Jiang, Karasawa, & Steyger, 2017; O’Sullivan et al, 2017).…”

Section: Introductionmentioning

confidence: 99%

“…In mostly adults with CF, individuals with higher cumulative doses of IV-AGs were 4.5 times more likely to have hearing loss compared to individuals with lower cumulative doses (Garinis et al, 2017). In a recent retrospective examination of the prevalence and type of hearing loss in the pediatric CF population, 31.8% had hearing loss in at least one ear in the SF region (Kreicher et al, 2018). Further analysis of the type of hearing loss (considering each ear separately) showed that 74.1% had normal hearing, 5.3% had conductive loss, 2.3% sensorineural loss, 5.1% mixed hearing loss, and 13.2% undefined (Kreicher et al, 2018).…”

Section: Introductionmentioning

confidence: 99%

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Functional Impacts of Aminoglycoside Treatment on Speech Perception and Extended High-Frequency Hearing Loss in Cystic Fibrosis

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Hunter

Feeney

et al. 2020

Preprint

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Purpose: The purpose of this study is to better understand the prevalence of ototoxicity-related hearing loss and its functional impact on communication in a pediatric and young adult cohort with cystic fibrosis (CF) and individuals without CF (controls). Method: Observational, cross-sectional investigation of hearing function in children, teens, and young adults with CF (n = 57, mean = 15.0 yr.) who received intravenous aminoglycoside antibiotics and age- and gender-matched controls (n = 61, mean = 14.6 yr.). Participants completed standard and extended high frequency audiometry, middle ear measures, speech perception tests, and a hearing and balance questionnaire. Results: Individuals with CF were 3 to 4 times more likely to report issues with hearing, balance, and tinnitus and performed significantly poorer on speech perception tasks compared to controls. A higher prevalence of hearing loss was observed in individuals with CF (57%) compared to controls (37%). CF and control groups had similar proportions of slight and mild hearing losses, however individuals with CF were 7.6 times more likely to have moderate and greater degrees of hearing loss. Older participants displayed higher average EHF thresholds, with no effect of age on average SF thresholds. Although middle ear dysfunction has not previously been reported to be more prevalent in CF, this study showed that 16% had conductive or mixed hearing loss and higher rates of previous otitis media and pressure equalization (PE) tube surgeries compared to controls. Conclusions: Individuals with CF have a higher prevalence of conductive, mixed and sensorineural hearing loss, poorer speech-in-noise performance and higher rates of multiple symptoms associated with otologic disorders (tinnitus, hearing difficulty, dizziness, imbalance and otitis media) compared to controls. Accordingly, children with CF should be asked about these symptoms, receive baseline hearing assessment(s) prior to treatment with potentially ototoxic medications, and at regular intervals thereafter in order to provide otologic and audiologic treatment for hearing and ear-related problems to improve communication functioning.

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Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

See 2 more Smart Citations

Functional Impacts of Aminoglycoside Treatment on Speech Perception and Extended High-Frequency Hearing Loss in Cystic Fibrosis

Blankenship

Hunter

Feeney

et al. 2020

Preprint

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“…Audiological data analyses were adapted from previous AudGenDB studies . Pure‐tone air‐ and bone‐conduction audiometry and sound‐field testing were used to evaluate hearing outcomes.…”

Section: Methodsmentioning

confidence: 99%

Characteristics and progression of hearing loss in children with turner's syndrome

et al. 2019

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Objectives/Hypothesis: Evaluate patterns of hearing impairment in children with Turner's syndrome (TS) and determine factors influencing severity and progression.Study Design: Retrospective database review. Methods: Demographic, audiological, and medical data for children with TS were extracted from the Audiological and Genetic Database to analyze patterns of hearing loss with comorbidities, demographics, and interventions.Results: Two hundred seventy-two children with TS were identified; 213 had audiological data. Of these children, 72.3% (N = 154) had hearing loss in which 84.4% (N = 130) was bilateral. In individual ears, conductive hearing loss was most common (28.7%, n = 73), followed by mixed (22.0%, n = 93) and sensorineural (7.9%, n = 20). Otitis media (odds ratio [OR] = 2.7, 95% confidence interval [CI]: 1.2-6.5), eustachian tube dysfunction (OR = 9.5, 95% CI: 3.2-35.2), and aortic valve anomalies were also associated with higher rates of hearing loss (OR = 3.6, 95% CI: 1.3-11.5). Of ears with quantifiable severity, 16.3% (n = 40) had moderate or worse hearing loss. Aortic coarctation (36.3 vs. 21.3 dB, P < .001), seizures (40.6 vs. 21.3 dB, P = .006), facial anomalies (32.5 vs. 21.3 dB, P = .029), and hypertension (36.3 vs. 21.3 dB, P = .015) portended more severe loss.Conclusions: Children with TS have high rates of hearing loss. High rates of sensorineural loss were unexpected. Nonotologic comorbidities, including seizures, hypertension, and anomalies of the kidney and aorta were associated with greater prevalence, severity, and progression of loss. Children with TS, particularly with the above comorbidities, are at risk for poor hearing outcomes, and should be offered audiological intervention to efficiently direct patient care.

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Evaluation of otorhinolaryngologic, audiologic, and genetic findings in children with cystic fibrosis: A tertiary care experience

Nayır Büyükşahin,

Yalcın,

Gökırmak

et al. 2024

Pediatric Pulmonology

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ObjectivesTo evaluate otorhinolaryngologic findings and the relationship between aminoglycoside (AG) exposure and hearing loss in paediatric patients with cystic fibrosis (cwCF). We also aimed to investigate the genetic predisposition to AG ototoxicity by screening for m.1555A>G mutations.MethodsCwCF who underwent otorhinolaryngologic and audiologic examinations were retrospectively included. Clinical characteristics, ear‐nose‐throat related symptoms, and a history of ototoxic drug exposure were recorded. m.1555A>G mutations were retrospectively screened among patients with audiologic evaluations.ResultsTwo hundred thirty‐four cwCF were included in this study with a median age of 10.7 (range, 6.8–14.2) years. Nasal obstruction (14.1%) was the most common symptom. Fifty‐two (22.2%) patients had chronic rhinosinusitis (CRS) with nasal polyps (CRSwNP). There was a positive correlation between CRSwNP and the symptom of nasal obstruction (r:.234, p < .001), snoring (r:.179, p = .006), and sleeping with mouth open (r:.138, p = .034). One hundred forty‐nine (63.6%) patients had audiologic evaluations; 14 (9.4%) had hearing impairment. No statistical significance existed between ototoxicity and IV AG exposure (p = .90). Six (42.8%) of 14 patients did not receive ototoxic drugs. One hundred nineteen (50.8%) patients were screened for m.1555A>G mutations, and none were detected.ConclusionsAlmost a quarter of the study population had CRSwNP. Neither the relationship between AGs exposure and hearing loss nor the genetic predisposition to AG ototoxicity could be shown in cwCF.

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Audiometric assessment of pediatric patients with cystic fibrosis

Cited by 22 publications

References 37 publications

Functional Impacts of Aminoglycoside Treatment on Speech Perception and Extended High-Frequency Hearing Loss in Cystic Fibrosis

Functional Impacts of Aminoglycoside Treatment on Speech Perception and Extended High-Frequency Hearing Loss in Cystic Fibrosis

Characteristics and progression of hearing loss in children with turner's syndrome

Evaluation of otorhinolaryngologic, audiologic, and genetic findings in children with cystic fibrosis: A tertiary care experience

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