The pattern and progression of hearing loss in Marfan Syndrome: A study of children and young adults.

OBJECTIVE: (s): To describe the prevalence, type, severity, and progression of hearing loss (HL) in children and young adults with Marfan Syndrome (MFS), and to evaluate the influence of comorbidities on HL. STUDY DESIGN: Retrospective database analysis. RESULTS: Of 70 patients with MFS, 52.8% (N = 37) had HL. Within the HL group, 75.7% (N = 28) had bilateral HL, while 24.3% (N = 9) had unilateral HL. 21.4% (N = 15) had moderate or more severe HL. The overall prevalence of CHL was 40.5% (N = 15), SNHL 13.5% (N = 5), and mixed HL 37.8% (N = 14). The mean initial age of HL was 8.4 years (range 0.8-24.0). HL was more prevalent in patients with MFS who also experienced chronic otitis media (ES 1.00, 95% CI, 0.32-1.68) and skull anomalies (ES 0.75, 95% CI, 0.07-1.44) as well as for patients with hypertension (ES 2.17, 95% CI, -1.29-5.64). CONCLUSIONS: Children and young adults with Marfan syndrome have a high likelihood of hearing loss, with high rates of CHL, chronic otitis media, and Eustachian tube dysfunction. SNHL is also prevalent in this syndrome; hypertension increased the likelihood of SNHL. Early audiologic screening is needed to ascertain type of HL and to efficiently direct patient care in this population.

Vitamin D Deficiency, Hypocalcemia, and Hearing Loss in Children.

OBJECTIVE: Characterize relations between vitamin D deficiency (VDD), hypocalcemia, and hearing loss (HL) in children. STUDY DESIGN: Retrospective review. SETTING: Tertiary referral hospital. PATIENTS: Children in the Audiological and Genetic Database with a diagnosis of VDD, rickets, or osteomalacia. INTERVENTION: None. MAIN OUTCOME MEASURES: Prevalence, type, severity (4-tone pure-tone average, PTA), and progression of HL. HL was defined as greater than 15 dB HL at any threshold by pure tone, greater than 20 dB HL by sound field audiometry, or greater than 25 dB in infants less than 1 year of age. RESULTS: Of 888 children with VDD, 474 (53.4%) had HL, with 17% having moderate-profound HL. Compared with an age-matched cohort of 13,320 children drawn from the same database, children with VDD were significantly more likely to have sensorineural HL (SNHL) (adjusted odds ratios [aOR] 1.26 [95% confidence interval [CI] 1.01-1.58]). Among children with VDD, children with femur fracture had a significantly higher rate of HL (81% versus 53%, p = 0.008) and children with hypocalcemia had a significantly higher rate of moderate-profound HL (36% versus 18%, p = 0.016). Additionally, hypocalcemia with and without VDD was associated with SNHL (aOR 2.30 [1.07-4.56]). CONCLUSIONS: Both vitamin D deficiency and hypocalcemia were found to be independently associated with SNHL, a type of HL that is less likely to improve over time. Recognition of VDD and hypocalcemia as independent risk factors for the development of SNHL could allow for better evaluation and treatment of this patient population. Routine audiological evaluation should be considered in this population.

Subjective vs Computerized Assessment of Surgeon Skill Level During Mastoidectomy.

This pilot study examines the use of surgical instrument tracking and motion analysis in objectively measuring surgical performance. Accuracy of objective measures in distinguishing between surgeons of different levels was compared to that of subjective assessments. Twenty-four intraoperative video clips of mastoidectomies performed by junior residents (n = 12), senior residents (n = 8), and faculty (n = 4) were sent to otolaryngology programs via survey, yielding 708 subjective ratings of surgical experience level. Tracking software captured the total distance traveled by the drill, suction irrigator, and patient's head. Measurements were used to predict surgeon level of training, and accuracy was estimated via area under the curve (AUC) of receiver operating characteristic curves. Key objective metrics proved more accurate than subjective evaluations in determining both faculty vs resident level and senior vs junior resident level. The findings of this study suggest that objective analysis using computer software has the potential to improve the accuracy of surgical skill assessment.

Impact of early gastroesophageal reflux disease on childhood otologic outcomes.

OBJECTIVE: Recent evidence has suggested that children with gastroesophageal reflux disease (GERD) face increased risk for otologic disease. This study aims to evaluate otologic outcomes in children with GERD using a large pediatric hearing database. METHODS: Data pertaining to children with GERD in the first year of life were extracted from the Audiological and Genetic Database. Subgroups included children with uncomplicated GERD, complicated GERD (with esophagitis), and GERD requiring surgery (fundoplication or fundoplasty). Main outcome measures included hearing loss (HL) prevalence, type, severity, and progression. Children without GERD in the first year of life served as controls. RESULTS: 5747 children were diagnosed with GERD in the first year of life. Evidence of HL was present in 40.9% children with uncomplicated GERD, 63.1% in children with complicated GERD, 75% in children with GERD requiring surgical intervention, and 43.3% in controls (p < 0.001). Children with GERD requiring surgery demonstrated the highest odds of developing any HL (aOR 3.02 [1.40-7.26]), moderate-to-profound HL (aOR 3.44 [1.56-7.29]), and mixed HL (aOR 4.58 [2.07-9.65]) relative to children with uncomplicated or complicated GERD. GERD requiring surgery was independently associated with Eustachian tube dysfunction (aOR 2.41 [1.17-5.20]) and tympanostomy tube placement (aOR 6.23 [3.05-13.01]). CONCLUSION: GERD diagnosed within the first year of life is associated with pediatric hearing impairment and otologic issues, including otitis media, Eustachian tube dysfunction, and need for tympanostomy tube placement. GERD requiring surgical intervention is particularly associated with hearing loss in this population. Children with GERD and HL deserve close audiologic follow-up.

Hearing outcomes in children of diabetic pregnancies.

OBJECTIVE: Children of diabetic pregnancies (CDPs) face numerous risk factors for hearing loss (HL). The objective of this study was to investigate the hearing outcomes of CDPs on a population scale. METHODS: Using the Audiological and Genetic Database, the prevalence, severity, and progression of HL in CDPs was compared against children of non-diabetic pregnancies (CNDPs) who served as controls. RESULTS: Among 311 CDPs, 71.1% demonstrated evidence of HL compared to 45.5% in CNDPs (p < 0.001). The mean age at which CDPs received audiograms was 3.6 years compared to 5.4 years for CNDPs (p < 0.001). Compared to CNDPs, CDPs were similarly affected by common otologic conditions such as acute otitis media (25.7%), chronic otitis media (38.3%), and Eustachian tube dysfunction (41.8%) (all p > 0.05). CDPs were more likely to have bilateral HL (81%) and sensorineural hearing loss (SNHL) (8%) relative to CNDPs (p < 0.001 and p = 0.004, respectively). Rates of conductive HL and mixed HL were not significantly different between groups (p = 0.952 and p = 0.058, respectively). CDPs were at significant risk for the development of HL (aOR 1.66 [1.28-2.17], SNHL (aOR 1.63 [1.01-2.52], and high-frequency HL (aOR 1.32 [1.03-1.68]). Of the comorbidities evaluated, CDPs with hyperbilirubinemia (aOR 1.85 [1.18-2.84]), perinatal asphyxia (aOR 1.90 [1.06-3.16]), or congenital heart disease (aOR 1.21 [1.07-1.37]) demonstrated higher risk of SNHL. CONCLUSION: Children of diabetic pregnancies face increased risks of developing HL, particularly bilateral and sensorineural hearing loss. Given these findings, we recommend close audiologic follow-up for these children, especially those with complicated birth histories or additional medical problems.

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

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. LEVEL OF EVIDENCE: NA Laryngoscope, 130:1540-1546, 2020.

Effect of Malnutrition on Hearing Loss in Children.

OBJECTIVE: To characterize the relation between protein-calorie malnutrition (PCM) and hearing loss (HL) in children. STUDY DESIGN: Retrospective review. SETTING: Tertiary referral hospital. PATIENTS: Children in the Audiological and Genetic Database with a diagnosis of protein-calorie malnutrition, marasmus, and/or kwashiorkor. INTERVENTIONS: None. MAIN OUTCOME MEASURES: Prevalence, type, severity (4-tone pure-tone average, PTA), and progression of HL. RESULTS: Of 770 children with PCM, 57.8% had HL, compared to 45.5% of children without PCM (p < 0.001). Severely malnourished children had significantly higher odds of moderate-profound HL (aOR 2.27, 95% CI 1.47-3.43), high-frequency HL (aOR 1.82, 95% CI 1.21-2.75), and sensorineural or mixed HL (aOR 1.60, 95% CI 1.05-2.41) compared to children without PCM. Severely malnourished children had significantly worse initial (35.0 dB vs 25.0 dB, p < 0.001), and final median PTA (31.3 dB vs 20.0 dB, p < 0.001) compared to children without PCM. Additionally, HL in children who were moderately and severely malnourished was significantly less likely to improve (aOR 0.47, 95% CI 0.25-0.82 and aOR 0.4, 95% CI 0.2-0.9) when compared to those without PCM. CONCLUSIONS: Given the greater prevalence and severity of hearing loss, children with PCM should be considered an at-risk group for poor audiological outcomes, and clinical practice should focus on early treatment and intervention for malnourished children. Routine audiological evaluation should be considered in this population.