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Introduction The role of elastin in tympanic retractions and chronic otitis media is not well established. Williams Syndrome (WS), a pathology related to a mutation in the elastin gene, could generate tympanic retractions. Objective To compare the prevalence of tympanic retractions among patients with WS and controls. Methods WS patients ( n = 43 ears) and controls ( n = 130 ears) were evaluated by digital otoscopic examination and the degree of tympanic membrane retraction was classified by 2 blinded experienced otolaryngologists. Results The agreement rate between the evaluators was 71.1% for pars tensa and 65% for pars flaccida retraction ( p < 0.001). The pars tensa and pars flaccida retractions are present in patients with WS after an adjusted residue of respectively - 2.8 and - 2.6 ( p = 0.011 and p = 0.022) compared with controls. Conclusions Tympanic membrane retractions are not more common in the WS group when compared with controls.
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Abstract Introduction The role of elastin in tympanic retractions and chronic otitis media is not well established. Williams Syndrome (WS), a pathology related to a mutation in the elastin gene, could generate tympanic retractions. Objective To compare the prevalence of tympanic retractions among patients with WS and controls. Methods WS patients (n= 43 ears) and controls (n= 130 ears) were evaluated by digital otoscopic examination and the degree of tympanic membrane retraction was classified by 2 blinded experienced otolaryngologists. Results The agreement rate between the evaluators was 71.1% for pars tensa and 65% for pars flaccida retraction (p< 0.001). The pars tensa and pars flaccida retractions are present in patients with WS after an adjusted residue of respectively - 2.8 and - 2.6 (p= 0.011 and p= 0.022) compared with controls. Conclusions Tympanic membrane retractions are not more common in the WS group when compared with controls.
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OBJECTIVE: To compare the degree of sensorineural hearing loss in patients with Ménière's disease (MD) with and without hyperinsulinism by different methods of assessment. STUDY DESIGN: Historical cohort study. SETTING: Ménière's Disease Care and Research Clinics of Hospital de Clinicas de Porto Alegre, a tertiary care university hospital in Southern Brazil. PATIENTS: Patients with a definite diagnosis of MD based on the American Academy of Otolaryngology-Head and Neck Surgery (AAO-HNS) guidelines. INTERVENTION: Patients were assessed by glucose overload tests (5-h glucose and insulin curves) and under baseline physiological conditions (Homeostasis Model Assessment/Insulin Resistance [HOMA-IR], Quantitative Insulin Sensitivity Check Index [QUICKI], and glucose/insulin ratio). These patients underwent annual pure-tone audiometry and were analyzed using 4-tone average (FTA), that is, arithmetic mean of 500, 1,000, 2,000, and 3,000 Hz, during the third, fourth, and fifth years of disease progression. MAIN OUTCOME MEASURE: Hearing loss assessed by FTA and classified in Stages I to IV (AAO-HNS). RESULTS: Forty-nine (76.6%) patients were defined as hyperinsulinemic and 15 (23.4%) as normoinsulinemic. Impairment on FTA was higher in the hyperinsulinemic group (52.04 ± 17.5 versus 39.75 ± 9.20, p = 0.027) when assessed by the 5-hour insulin curve. Hyperinsulinemic subjects were 3.5 times more likely to develop hearing damage greater than 40 dB (i.e., Stages III and IV) than normoinsulinemic subjects (OR = 3.52; 95% CI, 1.05-11.76). A moderate correlation between the insulin curve and the HOMA-IR was found (r = 0.524, p = 0.001). CONCLUSION: Hyperinsulinism in MD is associated with greater clinical hearing damage.