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Craniofacial microsomia (CFM) represents a spectrum of craniofacial malformations, ranging from isolated microtia with or without aural atresia to underdevelopment of the mandible, maxilla, orbit, facial soft tissue, and/or facial nerve. The genetic causes of CFM remain largely unknown.We performed genome sequencing and linkage analysis in patients and families with microtia and CFM of unknown genetic etiology. The functional consequences of damaging missense variants were evaluated through expression of wild-type and mutant proteins in vitro.We studied a 5-generation kindred with microtia, identifying a missense variant in FOXI3 (p.Arg236Trp) as the cause of disease (logarithm of the odds = 3.33). We subsequently identified 6 individuals from 3 additional kindreds with microtia-CFM spectrum phenotypes harboring damaging variants in FOXI3, a regulator of ectodermal and neural crest development. Missense variants in the nuclear localization sequence were identified in cases with isolated microtia with aural atresia and found to affect subcellular localization of FOXI3. Loss of function variants were found in patients with microtia and mandibular hypoplasia (CFM), suggesting dosage sensitivity of FOXI3.Damaging variants in FOXI3 are the second most frequent genetic cause of CFM, causing 1% of all cases, including 13% of familial cases in our cohort.

Background Osteoporosis and low bone density (LBD) may be associated with higher risk of hearing loss, but findings are inconsistent and longitudinal data are scarce. Bisphosphonates may influence risk, but the relation has not been studied in humans. We longitudinally investigated associations of osteoporosis and LBD, bisphosphonate use, vertebral fracture (VF), hip fracture (HF), and risk of self-reported moderate or worse hearing loss. Design Longitudinal cohort study. Setting The Nurses' Health Study (NHS) (1982-2016) and Nurses' Health Study II (NHS II) (1995-2017). Participants Participants included 60,821 NHS women, aged 36-61 years at baseline, and 83,078 NHS II women, aged 31-48 years at baseline (total n = 143,899). Measurements Information on osteoporosis, LBD, bisphosphonate use, VF, HF, and hearing status was obtained from validated biennial questionnaires. In a subcohort (n = 3749), objective hearing thresholds were obtained by audiometry. Multivariable-adjusted Cox proportional hazards models were used to examine independent associations between osteoporosis (NHS), osteoporosis/LBD (NHS II), and risk of hearing loss. Results The multivariable-adjusted relative risk (MVRR, 95% confidence interval) of moderate or worse hearing loss was higher among women with osteoporosis or LBD in both cohorts. In NHS, compared with women without osteoporosis, the MVRR was 1.14 (1.09, 1.19) among women with osteoporosis; in NHS II, the MVRR was 1.30 (1.21, 1.40) among women with osteoporosis/LBD. The magnitude of the elevated risk was similar among women who did and did not use bisphosphonates. VF was associated with higher risk (NHS: 1.31 [1.16, 1.49]; NHS II: 1.39 [1.13, 1.71]), but HF was not (NHS: 1.00 [0.86, 1.16];NHS II: 1.15 [0.75,1.74]). Among participants with audiometric measurements, compared with women without osteoporosis/LBD, the mean multivariable-adjusted hearing thresholds were higher (i.e., worse) among those with osteoporosis/LBD who used bisphosphonates. Conclusion Osteoporosis and LBD may be important contributors to aging-related hearing loss. Among women with osteoporosis, the risk of hearing loss was not influenced by bisphosphonate use.

Microtia is a congenital malformation that encompasses mild hypoplasia to complete loss of the external ear, or pinna. Although the contribution of genetic variation and environmental factors to microtia remains elusive, Amerindigenous populations have the highest reported incidence. Here, using both transmission disequilibrium tests and association studies in microtia trios (parents and affected child) and microtia cohorts enrolled in Latin America, we map an ∼10-kb microtia locus (odds ratio = 4.7; P = 6.78e-18) to the intergenic region between Roundabout 1 (ROBO1) and Roundabout 2 (ROBO2) (chr3: 78546526 to 78555137). While alleles at the microtia locus significantly increase the risk of microtia, their penetrance is low (1%). We demonstrate that the microtia locus contains a polymorphic complex repeat element that is expanded in affected individuals. The locus is located near a chromatin loop region that regulates ROBO1 and ROBO2 expression in induced pluripotent stem cell–derived neural crest cells. Furthermore, we use single nuclear RNA sequencing to demonstrate ROBO1 and ROBO2 expression in both fibroblasts and chondrocytes of the mature human pinna. Because the microtia allele is enriched in Amerindigenous populations and is shared by some East Asian subjects with craniofacial malformations, we propose that both populations share a mutation that arose in a common ancestor prior to the ancient migration of Eurasian populations into the Americas and that the high incidence of microtia among Amerindigenous populations reflects the population bottleneck that occurred during the migration out of Eurasia.

Persistent tinnitus is common, disabling, and difficult to treat. High-dose aspirin may precipitate tinnitus, but longitudinal data on typical dose aspirin and other analgesics are scarce.To investigate independent associations of aspirin, NSAIDs, and acetaminophen and risk of incident persistent tinnitus.Longitudinal cohort study.Nurses' Health Study II (1995-2017).A total of 69,455 women, age 31-48 years, without tinnitus at baseline.Information on analgesic use and tinnitus obtained by biennial questionnaires.After 1,120,936 person-years of follow-up, 10,452 cases of incident persistent tinnitus were reported. For low-dose aspirin, the risk of developing persistent tinnitus was not elevated among frequent low-dose aspirin users. For moderate dose aspirin, frequent use was associated with higher risk of tinnitus among women aged60 years, but not among older women (p-interactionInformation on tinnitus and analgesic use was self-reported. Information on indications for analgesic use was not available. Studies in non-White women and men are needed.The risk of developing persistent tinnitus was not elevated among frequent low-dose aspirin users. Among younger women, frequent moderate-dose aspirin use was associated with higher risk. Frequent NSAID use and frequent acetaminophen use were associated with higher risk of incident persistent tinnitus among all women, and the magnitude of the risks tended to be greater with increasing frequency of use. Our results suggest analgesic users are at higher risk for developing tinnitus and may provide insight into the precipitants of this challenging disorder, but additional investigation to determine whether there is a causal association is needed.

When in-person experiences were taken away from medical students and residency applicants during the COVID-19 pandemic, institutions had to pivot to virtual experiences. We present here a comprehensive overview of virtual engagement for medical students. As we increasingly embrace virtual opportunities, it may be possible to continue utilizing these programs for many years to come.

We conducted a prospective study of dietary patterns and longitudinal change in audiometric hearing thresholds among 3,135 women (mean age = 59 years) in the Nurses’ Health Study II (2012–2018). Diet adherence scores for the Dietary Approaches to Stop Hypertension (DASH) and Alternate Mediterranean (AMED) diets and the Alternate Healthy Eating Index 2010 (AHEI-2010) were calculated using validated food-frequency questionnaires. Baseline and 3-year follow-up hearing sensitivities were assessed by pure-tone audiometry at 19 US sites. We used multivariable-adjusted logistic regression models to examine independent associations between diet adherence scores and risk of ≥5 dB elevation in the pure-tone average (PTA) of low-frequency (LPTA0.5,1,2 kHz), mid-frequency (MPTA3,4 kHz), and high-frequency (HPTA6,8 kHz) hearing thresholds. Higher adherence scores were associated with lower risk of hearing loss. Compared with the lowest quintile of DASH score, the multivariable-adjusted odds ratios for mid-frequency and high-frequency threshold elevation in the highest quintile were 0.71 (95% confidence interval (CI): 0.55, 0.92; P for trend = 0.003) and 0.75 (95% CI: 0.59, 0.96; P for trend = 0.02); for AMED and AHEI scores, for mid-frequency threshold elevation, they were 0.77 (95% CI: 0.60, 0.99; P for trend = 0.02) and 0.72 (95% CI: 0.57, 0.92; P for trend = 0.002). Nonsignificant inverse associations were observed for high-frequency threshold elevation. There were no significant associations between adherence scores and low-frequency threshold elevation. Our findings indicate that eating a healthy diet might reduce the risk of acquired hearing loss.

OBJECTIVE/HYPOTHESIS Mortality attribution can have significant implications for reimbursement, hospital/department rankings, and perceptions of safety. This work seeks to compare the accuracy of externally assigned diagnosis-related group (DRG)-based service line mortality attribution in otolaryngology to an internal review process that assigns mortality to the teams that cared for a patient during hospitalization. STUDY DESIGN Retrospective case series. METHODS Mortality events at Vanderbilt University Medical Center (VUMC) from 2012 to 2018 were compared. Included events were assigned to the otolaryngology service line (OSL) via the following methods: an external agency (Vizient) using DRG, utilization management assignment based on the service that provided care at admission (admission service), discharge (discharge service), or throughout hospitalization (major service line), or through the internal VUMC mortality review committee. Internal review was considered the standard for comparison. RESULTS Of the 28 mortality events assigned to OSL by the DRG-based external method, nine (32%) were actually attributable to OSL. Of the 23 total mortality events attributable to OSL at our institution, external DRG-based review captured nine (39%). The designation of major service during hospitalization was correct 95% of the time and captured 87% of mortality events. Differences between external and internal attribution methods were statistically significant (P

Objectives Tinnitus and hearing loss commonly coexist, however, the temporal relation between tinnitus and hearing loss is complex and not fully understood. Our objective was to examine the longitudinal association between persistent tinnitus, bothersome tinnitus, and 3-year elevation of audiometric hearing thresholds. Design We conducted a longitudinal cohort study among 3106 women (mean age 59 years) who were participants in the Nurses' Health Study II (2012-2018). Information on tinnitus was obtained from biennial questionnaires. Longitudinal changes in air conduction thresholds (0.5 to 8 kHz) were assessed by pure-tone audiometry conducted by licensed audiologists at 19 audiology testing sites across the United States. Logistic regression was used to estimate multivariable-adjusted odds ratios (MVORs, 95% confidence interval [CI]) and evaluate the relations of persistent tinnitus (several days per week or more), bothersome tinnitus (interferes with work, sleep, or daily activities), and risk of 3-year elevation of hearing thresholds. Results Persistent tinnitus was associated with higher risk of 3-year elevation of hearing thresholds across a broad range of frequencies. Compared with women without tinnitus, the MVORs (95% CI) for ≥5-dB threshold elevation among women with persistent tinnitus were 1.01 (0.81, 1.25) at 0.5 kHz, 1.45 (1.17, 1.81) at 1 kHz, 1.25 (1.00, 1.56) at 2 kHz, 1.34 (1.07, 1.69) at 3 kHz, 1.34 (1.06, 1.70) at 4 kHz, 1.49 (1.16, 1.91) at 6 kHz, and 1.63 (1.25, 2.12) at 8 kHz. The magnitudes of the associations for ≥10-dB threshold elevation were similar. The magnitudes of the associations were substantially greater among women with bothersome tinnitus. For example, compared with women without tinnitus, the MVORs (95% CI) for a ≥5- and ≥10-dB elevation of hearing thresholds at 4 kHz were 2.97 (1.50, 5.89) and 2.79 (1.38, 5.65), respectively. The risk was elevated even among women with tinnitus who had clinically normal hearing thresholds at baseline. In analyses that examined the association of tinnitus and elevation of low-, mid- and high-frequency pure-tone average (PTA) hearing thresholds, the results were similar. Compared with women without tinnitus, the MVORs (95% CI) for ≥5-dB PTA elevation among women with persistent tinnitus were 1.29 (0.99,1.67) for LPTA(0.5,1,2 kHz); 1.44 (1.16, 1.78) for MPTA(3,4 kHz); and 1.38 (1.11, 1.71) for HPTA(6,8 kHz). For ≥10-dB elevation, the MVORs were 2.85 (1.55, 5.23), 1.52 (1.10, 2.09), and 1.41 (1.10, 1.82), respectively. Conclusion Persistent tinnitus was associated with substantially higher risk of 3-year hearing threshold elevation, even among women with clinically normal baseline hearing. The magnitudes of the associations were greater among those with bothersome tinnitus. Monitoring hearing sensitivities may be indicated in patients with tinnitus, including those without audiometric evidence of hearing impairment.

This cohort study examines subjective patient-reported outcomes after nasal breathing surgery and identifies factors associated with patients’ perception of the procedure’s value.

BACKGROUND: Previous studies demonstrated higher risk of hearing loss among cigarette smokers, but longitudinal data on whether the risk is influenced by smoking cessation are limited. We prospectively investigated relations between smoking, smoking cessation and risk of self-reported moderate or worse hearing loss among 81,505 women in the Nurses’ Health Study II (1991-2013) METHODS: Information on smoking and hearing status was obtained from validated biennial questionnaires. Cox proportional hazards regression was used to estimate multivariable-adjusted relative risks (MVRR,95%CI). RESULTS: During 1,533,214 person-years of follow-up, 2760 cases of hearing loss were reported. Smoking was associated with higher risk of hearing loss and the risk tended to be higher with greater number of pack-years smoked. Compared with never smokers, the MVRR(95% CI) among past smokers with 20+ pack-years of smoking was 1.30(1.09,1.55) and 1.21(1.02,1.43) for current smokers. The magnitude of elevated risk diminished with greater time since smoking cessation. Compared with never smokers, the MVRR among smokers who quit

Menopause may be a risk factor for hearing loss, and postmenopausal hormone therapy (HT) has been proposed to slow hearing decline; however, there are no large prospective studies. We prospectively examined the independent relations between menopause and postmenopausal HT and risk of self-reported hearing loss.Prospective cohort study among 80,972 women in the Nurses' Health Study II, baseline age 27 to 44 years, followed from 1991 to 2013. Baseline and updated information was obtained from detailed validated biennial questionnaires. Cox proportional-hazards regression models were used to examine independent associations between menopausal status and postmenopausal HT and risk of hearing loss.After 1,410,928 person-years of follow-up, 18,558 cases of hearing loss were reported. There was no significant overall association between menopausal status, natural or surgical, and risk of hearing loss. Older age at natural menopause was associated with higher risk. The multivariable-adjusted relative risk of hearing loss among women who underwent natural menopause at age 50+ years compared with those aged less than 50 years was 1.10 (95% confidence interval [CI] 1.03, 1.17). Among postmenopausal women, oral HT (estrogen therapy or estrogen plus progestogen therapy) was associated with higher risk of hearing loss, and longer duration of use was associated with higher risk (P trend 0.001). Compared with women who never used HT, the multivariable-adjusted relative risk of hearing loss among women who used oral HT for 5 to 9.9 years was 1.15 (95% CI 1.06, 1.24) and for 10+ years was 1.21 (95% CI 1.07, 1.37).Older age at menopause and longer duration of postmenopausal HT are associated with higher risk of hearing loss.

OBJECTIVES Gastroesophageal reflux disease (GERD) is common and often treated with proton pump inhibitors (PPIs) or H2-receptor antagonists (H2-RAs). GERD has been associated with exposure of the middle ear to gastric contents, which could cause hearing loss. Treatment of GERD with PPIs and H2-RAs may decrease exposure of the middle ear to gastric acid and decrease the risk of hearing loss. We prospectively investigated the relation between GERD, use of PPIs and H2-RAs, and the risk of hearing loss in 54,883 women in Nurses' Health Study II. DESIGN Eligible participants, aged 41 to 58 years in 2005, provided information on medication use and GERD symptoms in 2005, answered the question on hearing loss in 2009 or in 2013, and did not report hearing loss starting before the date of onset of GERD symptoms or medication use. The primary outcome was self-reported hearing loss. Cox proportional hazards regression was used to adjust for potential confounders. RESULTS During 361,872 person-years of follow-up, 9842 new cases of hearing loss were reported. Compared with no GERD symptoms, higher frequency of GERD symptoms was associated with higher risk of hearing loss (multivariable adjusted relative risks

Aspirin, nonsteroidal antiinflammatory drugs (NSAID), and acetaminophen are commonly used. Frequent use of analgesics has been associated with a higher risk of hearing loss. However, the association between duration of analgesic use and the risk of hearing loss is unclear. We investigated the relationship between duration of analgesic use and self-reported hearing loss among 55,850 women in the Nurses’ Health Study. Cox proportional hazards regression was used to adjust for potential confounders. During 873,376 person-years of follow-up (1990–2012), longer durations of NSAID use (for >6 years of use compared with 6 years of use compared with 6 years of use compared with

Objective To develop and implement a streamlined, patient-centered service delivery model for patients referred for cochlear implantation (CI) at a high-volume academic center. Patients CI candidate adults. Interventions CI, implementation of new CI delivery model. Main outcome measures Referral-to-surgery time, patient travel burden. Results Data from 206 adults that underwent CI were used to develop a process map of the initial operational state from referral date to day of surgery (referral-to-surgery time). The initial referral-to-surgery time was 136 days on average, yet the average total work time by all involved providers was 17.6 hours. Prolonged wait times were associated with the following preoperative tasks: appointment scheduling, insurance approval, device ordering and shipment, and surgical scheduling. Patients traveled to the institution on at least two occasions for appointments. A new bundled, patient-centered CI delivery model was developed to address prolonged wait times, travel burden, and process inefficiencies. The new model implemented an interactive electronic medical record, coordinated appointments with same-day surgery, and stocked device inventory to reduce the referral-to-surgery time to 24 days-an improvement of 112 days. In the new model, new patient consultation and surgery were completed in one day, reducing the patient travel burden to the institution. Conclusions The new CI program demonstrates that delivery innovations can have a substantial impact on measures of patient convenience and experience, and that these results are achievable without new technologies or changes in medical management. With a focus on patient-centered design, health care delivery models can be augmented to increase value for patients.

AIMS/HYPOTHESIS: Type 2 diabetes mellitus has been implicated as a risk factor for hearing loss, with possible mechanisms including microvascular disease, acoustic neuropathy or oxidative stress. A few small studies have examined the longitudinal association between type 2 diabetes and hearing loss, but larger studies are needed. Our objective was to examine whether type 2 diabetes (including diabetes duration) is associated with incident hearing loss in two prospective cohorts: Nurses’ Health Studies (NHS) I and II. METHODS: We conducted a longitudinal study of 139,909 women to examine the relationship between type 2 diabetes and the risk of self-reported incident hearing loss. A physician-diagnosis of diabetes was ascertained from biennial questionnaires. The primary outcome was hearing loss reported as moderate or worse in severity (categorised as a ‘moderate or severe’ hearing problem, or ‘moderate hearing trouble or deaf’) on questionnaires administered in 2012 in NHS I and 2009 or 2013 in NHS II. Cox proportional hazards regression was used to adjust for potential confounders. RESULTS: During >2.4 million person-years of follow-up, 664 cases of moderate or worse hearing loss were reported among those with type 2 diabetes and 10,022 cases among those without type 2 diabetes. Compared with women who did not have type 2 diabetes, those with type 2 diabetes were at higher risk for incident moderate or worse hearing loss (pooled multivariable-adjusted HR 1.16 [95% CI 1.07, 1.27]). Participants who had type 2 diabetes for ≥8 years had a higher risk of moderate or worse hearing loss compared with those without type 2 diabetes (pooled multivariable-adjusted HR 1.24 [95% CI 1.10, 1.40]). CONCLUSIONS/INTERPRETATION: In this large longitudinal study, type 2 diabetes was associated with a modestly higher risk of moderate or worse hearing loss. Furthermore, longer duration diabetes was associated with a higher risk of moderate or worse hearing loss.

BACKGROUND: Specific nutrients have been associated with hearing status, but associations between healthful dietary patterns and risk of hearing loss have not been prospectively evaluated. OBJECTIVE: We sought to prospectively examine the relations between adherence to the Alternate Mediterranean diet (AMED), the Dietary Approaches to Stop Hypertension (DASH), and the Alternative Healthy Eating Index-2010 (AHEI-2010), and risk of hearing loss. METHODS: We conducted a longitudinal cohort study (1991–2013) of 81,818 women in the Nurses’ Health Study II, aged 27–44 y at baseline. We assessed diet every 4 y with the use of food frequency questionnaires and calculated AMED, DASH, and AHEI-2010 adherence scores. Baseline and updated information from validated biennial questionnaires was used in Cox proportional hazards regression models to examine independent associations between adherence scores and risk of self-reported moderate or worse hearing loss. RESULTS: During >1 million person-years of follow-up, 2306 cases of moderate or worse hearing loss were reported. Higher cumulative average AMED and DASH scores were significantly inversely associated with risk of hearing loss. For women with scores in the highest compared with the lowest quintile, the multivariable-adjusted relative risks (MVRRs) of hearing loss were 0.70 (95% CI: 0.60, 0.82) (P-trend

Background Higher intake of certain vitamins may protect against cochlear damage from vascular compromise and oxidative stress, thereby reducing risk of acquired hearing loss, but data are limited. Objective We prospectively examined the relation between carotenoids, vitamin A, vitamin C, vitamin E, and folate intake and risk of self-reported hearing loss in women. Design This prospective cohort study followed 65,521 women in the Nurses' Health Study II from 1991 to 2009. Baseline and updated information obtained from validated biennial questionnaires was used in Cox proportional hazards regression models to examine independent associations between nutrient intake and self-reported hearing loss. Results After 1,084,598 person-years of follow-up, 12,789 cases of incident hearing loss were reported. After multivariable adjustment, we observed modest but statistically significant inverse associations between higher intake of β-carotene and β-cryptoxanthin and risk of hearing loss. In comparison with women in the lowest quintile of intake, the multivariable-adjusted RR of hearing loss among women in the highest quintile was 0.88 (95% CI: 0.81, 0.94; P-trend Conclusion Higher intakes of β-carotene, β-cryptoxanthin, and folate, whether total or from diet, are associated with lower risk of hearing loss, whereas higher vitamin C intake is associated with higher risk.

Background: The extent to which surgeons understand costs associated with expensive operative procedures remains unclear. The goal of the study was to better understand surgeon cost awareness of operating room supplies and implants. Methods: This was a cross-sectional study of faculty (n = 24) and trainees (fellow and residents, n = 27) in the Department of Otolaryngology. Participants completed surveys to assess opinions on importance of cost and ease in accessing cost data and were asked to estimate the costs of operating room (OR) supplies and implants. Estimates within 20% of actual cost were considered correct. Analyses were stratified into faculty and trainee surgeons. Results: Cost estimates varied widely, with a low percentage of correct estimations (25% for faculty, 12% for trainees). Surgeons tended to underestimate the cost of high-cost items (55%) and overestimate the cost of low-cost items (77%). Attending surgeons were more accurate at correctly estimating costs within their own subspecialty (33% vs 16%, P < .001). Self-rated cost knowledge and years in practice did not correlate with cost accuracy ( P < .05). Conclusions: A majority of surgeons were unable to correctly estimate the costs of items/implants used in their OR. An opportunity exists to improve the mechanisms by which cost data are fed back to physicians to help promote value-based decision making.

Chronic excess alcohol intake has been associated with irreversible hearing loss and acute alcohol intake may temporarily impair auditory function; however, some evidence suggests that long-term moderate alcohol intake may be related to lower risk of hearing loss. This study prospectively examined the association between total alcohol and individual alcoholic beverage consumption and risk of hearing loss in women. Data were prospectively collected from 65,424 participants in the Nurses' Health Study II (NHS II), aged 27-44 years at baseline (follow-up 1991-2009). Alcohol consumption was assessed using a validated questionnaire every 4 years. An incident case was defined as a self-reported hearing problem that began after 1991. Cox proportional hazards multivariate regression was used to adjust for potential confounders. During 1,024,555 person-years of follow-up, 12,384 cases of hearing loss occurred. After multivariate adjustment, there was no significant association between total alcohol consumption and risk of hearing loss. In exploratory analyses, beer consumption was associated with increased risk and wine consumption was associated with reduced risk. No significant association was observed for consumption of liquor. Total alcohol consumption is not associated with risk of hearing loss in women. The modest associations observed for beer (direct) and wine (inverse) may be due to chance or residual confounding but merit further study.

Background: Acquired hearing loss is common and often disabling, yet limited prospective data exist on potentially modifiable risk factors. Evidence suggests that higher intake of fish and long-chain omega-3 (n−3) polyunsaturated fatty acids (PUFAs) may be associated with a lower risk of hearing loss, but prospective information on these relations is limited. Objective: We prospectively examined the independent associations between consumption of total and specific types of fish, long-chain omega-3 PUFAs, and self-reported hearing loss in women. Design: Data were from the Nurses’ Health Study II, a prospective cohort study. The independent associations between consumption of fish and long-chain omega-3 PUFAs and self-reported hearing loss were examined in 65,215 women followed from 1991 to 2009. Baseline and updated information was obtained from validated biennial questionnaires. Cox proportional hazards regression models were used to estimate multivariable-adjusted RRs and 95% CIs. Results: After 1,038,093 person-years of follow-up, 11,606 cases of incident hearing loss were reported. Consumption of 2 or more servings of fish per week was associated with a lower risk of hearing loss. In comparison with women who rarely consumed fish (

Microtia is a rare, congenital malformation of the external ear that in some cases has a genetic etiology. We ascertained a three-generation family with bilateral microtia and hearing loss segregating as an autosomal dominant trait. Exome sequencing of affected family members detected only seven shared, rare, heterozygous, nonsynonymous variants, including one protein truncating variant, a HOXA2 nonsense change (c.703C>T, p.Q235*). The HOXA2 variant was segregated with microtia and hearing loss in the family and was not seen in 6,500 individuals sequenced by the NHLBI Exome Sequencing Project or in 218 control individuals sequenced in this study. HOXA2 has been shown to be critical for outer and middle ear development through mouse models and has previously been associated with autosomal recessive bilateral microtia. Our data extend these conclusions and define HOXA2 haploinsufficiency as the first genetic cause for autosomal-dominant nonsyndromic microtia.

The mortality observed-to-expected (O:E) ratio is rapidly becoming the most important measured quality metric by allowing quantification and comparison of survival outcomes among different providers and institutions. Although the O:E ratio is monitored by external observers, the ratio is unfamiliar to individuals within most institutions.Retrospective chart review.Vanderbilt University Medical Center.Twenty-eight patients cared for by the Department of Otolaryngology died while in the hospital between January 2001 and December 2010. All patient charts were reviewed for indicators related to mortality. From January 2006 to December 2010, a standardized mortality O:E ratio had been available using the All Patient Refined-Diagnosis Related Group (APR-DRG) grouper from the United Healthcare Consortium (UHC). The O:E ratio can be monitored over time to measure and quantify the effect of various interventions.The otolaryngology O:E ratio quarterly results have varied from 1.1 to 0.29, based on a standard of 1.0. Internally, results have been primarily the result of mortalities of patients on the Head and Neck Service. Attention to common postoperative complications, accurate coding of comorbidities, and the compassionate use of palliative care consults have led to a significant decrease in the O:E ratio. Conversely, transfers from other hospitals have increased the ratio.The Department of Otolaryngology has reduced the O:E ratio by focusing attention on factors that have been shown to reduce mortality and to enhance compassionate terminal care.

Use of analgesics is common and is associated with increased risk of hearing loss in men; however, the relation has not been examined prospectively in women. The authors prospectively examined the relation between frequency of aspirin, ibuprofen, and acetaminophen use and risk of hearing loss among 62,261 women aged 31–48 years at baseline (1995) in Nurses’ Health Study II. The outcome was self-reported hearing loss (n= 10,012), and the follow-up period was 1995–2009. Cox proportional hazards regression was used to adjust for potential confounders. During 764,247 person-years of follow-up, ibuprofen use and acetaminophen use were independently associated with increased risk of hearing loss, but aspirin use was not. For ibuprofen, the multivariate-adjusted relative risk of hearing loss was 1.13 (95% confidence interval (CI): 1.06, 1.19) for use 2–3 days/week, 1.21 (95% CI: 1.11, 1.32) for use 4–5 days/week, and 1.24 (95% CI: 1.14, 1.35) for use ≥ 6d ays/ week (P-trend < 0.0001), compared with use less than once per week. For acetaminophen, the corresponding relative risks were 1.11 (95% CI: 1.02, 1.19), 1.21 (95% CI: 1.07, 1.37), and 1.08 (95% CI: 0.95, 1.22), respectively (P-trend = 0.0007). In this study, use of ibuprofen or acetaminophen (but not aspirin) 2 or more days per week was associated with an increased risk of hearing loss in women. acetaminophen; analgesics; anti-inflammatory agents, non-steroidal; aspirin; hearing loss; ibuprofen

Objective Hearing loss is a common and disabling sensory disorder, yet prospective data on potentially modifiable risk factors are limited. Previous studies suggest that alcohol consumption may influence the development of hearing loss, yet results have been inconsistent. The purpose of this study was to prospectively examine the relation between alcohol use and hearing loss in men. Design We examined prospectively the independent association between alcohol intake and self-reported professionally diagnosed hearing loss in 26,809 men aged 40 to 74 yrs at baseline in 1986. Study participants completed detailed questionnaires at baseline and every 2 yrs thereafter. Incident cases of hearing loss were defined as those professionally diagnosed after 1986. Cox proportional hazards multivariate regression was used to adjust for potential confounding factors. Results During 386,081 person-years of follow-up, 3447 incident cases of hearing loss were reported. Overall, there was no association between level of alcohol intake and risk of hearing loss. Compared with those who did not consume alcohol, the multivariate-adjusted hazard ratios (95% confidence interval) were 1.00 (0.89 to 1.12) for those who consumed 5.0 to 9.9 g/day, 1.08 (0.96 to 1.21) for 10.0 to 14.9 g/day, and 0.98 (0.85 to 1.13) for 30.0 to 49.9 g/day. The results did not differ by age group or folate intake. Among those with lower intake of vitamin B12, however, higher consumption of alcohol, specifically liquor, was associated with an increased risk of hearing loss. Conclusions Our data suggest that low or moderate alcohol consumption does not influence the risk of hearing loss in older men. A possible relation between vitamin B12 intake, alcohol consumption, and hearing loss merits further investigation.

We report a large Chinese family with X-linked postlingual nonsyndromic hearing impairment in which the critical linkage interval spans a genetic distance of 5.41 cM and a physical distance of 15.1 Mb that overlaps the DFN2 locus. Mutation screening of the PRPS1 gene in this family and in the three previously reported DFN2 families identified four different missense mutations in PRPS1. These mutations result in a loss of phosphoribosyl pyrophosphate (PRPP) synthetase 1 activity, as was shown in silico by structural analysis and was shown in vitro by enzymatic activity assays in erythrocytes and fibroblasts from patients. By in situ hybridization, we demonstrate expression of Prps1 in murine vestibular and cochlea hair cells, with continuous expression in hair cells and postnatal expression in the spiral ganglion. Being the second identified gene associated with X-linked nonsyndromic deafness, PRPS1 will be a good candidate gene for genetic testing for X-linked nonsyndromic hearing loss.

Portable video technology is a widely available new tool with potential to be used by pediatric otolaryngology practices for patient and family education. Podcasts are media broadcasts that employ this new technology. They can be accessed via the Internet and viewed either on a personal computer or on a handheld device, such as an iPod or an MP3 player. We wished to examine the feasibility of establishing a podcast-hosting Web site. We digitally recorded pediatric otologic procedures in the operating room and saved the digital files to DVDs. We then edited the DVDs at home with video-editing software on a personal computer. Next, spoken narrative was recorded with audio-recording software and combined with the edited video clips. The final products were converted into the M4V file format, and the final versions were uploaded onto our hospital's Web site. We then downloaded the podcasts onto a high-quality portable media player so that we could evaluate their quality. All of the podcasts are now on the hospital Web site, where they can be downloaded by patients and families at no cost. The site includes instructions on how to download the appropriate free software for viewing the podcasts on a portable media player or on a computer. Using this technology for patient education expands the audience and permits portability of information. We conclude that a home computer can be used to inexpensively create informative surgery demonstrations that can be accessed via a Web site and transferred to portable viewing devices with excellent quality.

Standard culture medium contains bovine serum. If standard culture methodology is used for future human tissue-engineering, unknown risks of infection from bovine disease or immune reaction to foreign proteins theoretically might occur. In this study we wished to evaluate the potential of chondrocyte expansion using autologous and serum free media.Autologous auricular cartilage was harvested in a swine model. An initial concentration of 100x10(3) cells per group were expanded in three groups. Group A, F-12 with 10% fetal calf serum; Group B, F-12 supplemented with 10% autologous serum; Group C, F-12 supplemented with growth factors. Cell numbers were counted at days 3, 6, 9 and 12.The cells in all the three groups exhibited normal chondrocyte morphology. At early time points there was a statistically significant difference in the number of cells between Group A and the two other groups (p0.05). By day 12, both Groups A and C demonstrated greater cell number as compared to Group B (p0.05).The results suggest that both autologous serum as well as serum free media might be substituted for the expansion of the number of chondrocytes, thus avoiding the potential need for a bovine serum supplement.

Background Hearing loss is highly prevalent among adults in the United States. Hypertension also is common and often treated with diuretics. Hypertension may increase the risk of hearing loss by decreasing vascular supply to the stria vascularis. Use of thiazides has been anecdotally associated with hearing loss. In small studies, furosemide use has been associated with hearing loss that is usually reversible, but can be permanent. We investigated the relation among hypertension, diuretic use, and hearing loss in a prospective cohort of 54,721 women in the Nurses' Health Study I, 1994 to 2012. Methods Eligible participants included 54,721 female nurses aged 48 to 73 years in 1994 who provided information on thiazide diuretic and furosemide use in 1994, answered the question on hearing loss over their lifetime in 2012, and did not report hearing loss with date of onset before date of onset of hypertension diagnosis or medication use. The outcome was self-reported hearing loss. Cox proportional hazards regression was used to adjust for potential confounders. Results During 774,096 person-years of follow-up, 19,296 cases of hearing loss were reported (incidence rate, 25 cases per 1000 person-years). At baseline in 1994, the mean age was 57.9 years and mean body mass index was 26.3 kg/m 2 . Some 30.8% of participants had a history of hypertension. History of hypertension was independently associated with a modestly higher risk of hearing loss (multivariable adjusted relative risk, 1.04 [1.01-1.07]). Among women with a history of hypertension, neither thiazide diuretic (multivariable adjusted relative risk, 1.07 [0.99-1.16]) nor furosemide use (multivariable adjusted relative risk, 0.91 [0.75-1.09]) was significantly associated with risk of hearing loss when compared with women not taking antihypertensive medications. There was no significant effect modification by age. Conclusions History of hypertension was associated with a small increased risk of hearing loss. Thiazide diuretic use and furosemide use were not associated with risk of hearing loss among women with a history of hypertension.

Objective: Butterfly graft inlay tympanoplasty is a well-established technique for the repair of small perforations. However, the efficacy of the technique for medium and large tympanic membrane perforations remains unknown. Study Design: Retrospective case series. Methods: Postauricular tympanoplasty and tympanomastoidectomy using a large butterfly cartilage inlay graft (>4 mm diameter to total drum replacement) were analyzed in 90 pediatric patients (99 ears). Results: Patient ages ranged from 2 to 20 years; mean follow-up duration was 27.6 months. Successful closure occurred in 92% of the ears. No graft lateralized nor displaced into the middle ear. No retraction pocket occurred during the follow-up period. In 62 cases, intact canal wall or canal wall window tympanomastoidectomy was performed; Fifty-one (82.2%) of the patients having mastoidectomy procedures had chronic otitis media with cholesteatoma. The mean preoperative to postoperative four-tone air-bone gap improved from 23 to 21 dB; the number of patients with 0 to 10 dB hearing results increased from 16 ears preoperatively to 32 ears postoperatively. Postoperative suboptimal results included eight patients with postoperative perforations in the residual tympanic membrane adjacent to an intact cartilage graft; two of these patients were the only individuals who exhibited otorrhea. Conclusions: Cartilage butterfly graft inlay tympanoplasty is effective in the vast majority of patients with moderate to large perforations. The closure rate exceeded 90% with no graft displacement, postoperative adverse events were respectably low, and hearing results improved or remained stable despite the need for concurrent mastoidectomy in the majority of patients.

Objective. Many adolescents and young adults consciously expose themselves to loud music for entertainment. We hypothesized that these individuals might not be aware that exposure to loud music could result in hearing loss. Furthermore, we wished to assess the feasibility of a web-based survey to collect health information from this group.Methods. A 28-question survey was designed to target adolescents and young adults. The survey contained questions about views toward general health issues, including hearing loss, and was presented to random visitors at the MTV web site.Results. In 3 days, 9693 web surveys were completed. Hearing loss was defined on a Likert scale as “a very big problem” by 8% of respondents compared with other health issues: sexually transmitted diseases, 50%; alcohol/drug use, 47%; depression, 44%; smoking, 45%; nutrition and weight issues, 31%; and acne, 18%. Notably, most respondents had experienced tinnitus or hearing impairment attending concerts (61%) and clubs (43%). Only 14% of respondents had used earplugs; however, many could be motivated to try ear protection if they were aware of the potential for permanent hearing loss (66%) or were advised by a medical professional (59%).Conclusions. A majority of young adults have experienced tinnitus and hearing impairment after exposure to loud music. Fortunately, many of these individuals could be motivated to wear ear protection. This novel web-based survey technique rapidly generated a large database and is a feasible method to obtain health data from this group.

Significant challenges remain in generating tissue-engineered cartilage in immunocompetent animals. Scaffold materials such as polyglycolic acid lead to significant inflammatory reactions, inhibiting homogeneous matrix synthesis. This study examined the generation of tissue-engineered cartilage, using a polyglycolic acid-polylactic acid copolymer (Ethisorb; Ethicon, Norderstedt, Germany) in an autologous immunocompetent pig model. The goals of this study were to determine the role of interleukin 1alpha (IL-1alpha) in this system and to assess the effect of serum treatment on tissue generation. Porcine auricular chondrocytes were seeded onto Ethisorb disks cultured for 1 week in medium supplemented with either fetal bovine serum or serum-free insulin-transferrin-selenium supplement. Specimens were implanted autogenously in pigs with unseeded scaffolds as controls. After 1, 4, or 8 weeks, six specimens from each group were explanted and analyzed histologically (hematoxylin and eosin, safranin O, trichrome, and Verhoeff's staining) and biochemically (glycosaminoglycan content). The presence and distribution of IL-1alpha were assessed by immunohistochemistry. Histology revealed acute inflammation surrounding degrading scaffold. Cartilage formation was observed as early as 1 week after implantation and continued to increase with time; however, homogeneous matrix synthesis was not present in any of the specimens. Strong IL-1alpha expression was detected in chondrocytes at the implant periphery and in cells in the vicinity of degrading polymer. Histologically there was no significant difference between the experimental groups with respect to the amount of matrix synthesis or inflammatory infiltration. The glycosaminoglycan content was significantly higher in the serum-free group. These results suggest that inflammatory reactions against scaffold materials and serum components lead to the production of cytokines such as IL-1alpha that may inhibit cartilage tissue formation in autologous transplant models.

Objectives/Hypothesis: Current surgical techniques for the correction of microtia are challenging. Research in the field of tissue engineering is providing insight into chondrocyte behavior for a possible future treatment of microtia. The authors wished to evaluate the biological potential of chondrocytes isolated from microtia cartilage as compared with normal auricular cartilage as a source of tissue-engineered cartilage. Study Design: A comparative research design to study the potential of microtia cartilage chondrocytes with normal auricular chondrocytes as a source of tissue-engineered cartilage. Methods: Cartilage specimens from 12 pediatric patients (six normal auricular specimens and six auricular specimens with microtia) were obtained. The chondrocytes were isolated and cultured in vitro; chondrocyte number was increased by passaging. Each type of cell was implanted in nude mice to generate tissue-engineered cartilage. Eight weeks after implantation the specimens were dissected and removed. Results were compared between the normal auricular and microtia specimens in regard to cell number expansion in vitro and generation of tissue-engineered cartilage in vivo. Results: An initial mean cell number of 150,000 cells in each group (normal and microtia) increased to an average cell number of 120 million cells/mL in the normal and 130 million cells in the microtia subgroups, respectively, at the end of the second passage. Histologically, both types of chondrocytes generated normal elastic cartilage. Conclusion: The study demonstrated the potential of cells isolated from microtia cartilage to generate tissue-engineered cartilage. Microtia cartilage represents an important additional donor source for the possible generation of a human tissue-engineered auricle.

Objective: To undertake a comprehensive investigation into the very high incidence of congenital deafness on the Macano peninsula of Margarita Island, Venezuela. Methods: Numerous visits were made to the isolated island community over a 4-year-period. During these visits, it became apparent that a significant number of individuals complained of problems with hearing and vision. Socioeconomic assessments, family pedigrees and clinical histories were recorded on standard questionnaires. All individuals underwent thorough otolaryngologic and ophthalmologic examinations. Twenty milliliters of peripheral venous blood was obtained from each participant. A Genome-wide linkage analysis study was performed. Polymorphic microsatellite markers were amplified by polymerase chain reaction and separated on polyacrylamide gels. An ABI 377XL sequencer was used to separate fragments and LOD scores were calculated by using published software. Results: Twenty-four families were identified, comprising 329 individuals, age range 1–80 years, including 184 children. All families were categorized in the lower two (least affluent) socioeconomic categories. A high incidence of consanguinity was detected. Fifteen individuals (11 adults, 4 children) had profound congenital sensorineural hearing loss, vestibular areflexia and retinitis pigmentosa. A maximum LOD score of 6.76 (Linkage >3.0), between markers D11s4186 and D11s911, confirmed linkage to chromosome 11q13.5. The gene myosin VIIA ( MYO7A ) was confirmed in the interval. Clinical and genetic findings are consistent with a diagnosis of Usher syndrome 1B for those with hearing and vision problems. Conclusions: We report 15 Usher syndrome 1B individuals from a newly detected Latin American socio-demographic origin, with a very high prevalence of 76 per 100,000 population.

To grow a complete human size auricle by utilizing the principles of tissue engineering, a large number of chondrocytes is required for initial implantation. The number of chondrocytes can be increased by repeated passaging or by incubation with different growth factors, both of which can promote dedifferentiation. New methods of chondrocyte expansion over a relatively brief time period for potential practical application are required. In this study auricular chondrocytes were obtained from patients and cultured in vitro. Two groups of cells were created. Group A chondrocyte number was increased by repeated passaging. Group B cells were grown from floating culture medium and their number was increased both by passaging and by repeated recycling of the culture medium. Chondrocytes from both groups were implanted in nude mice for 8 weeks to generate tissue-engineered cartilage. Flow cytometry studies performed on both groups confirmed the presence of two distinct populations of structures as the source of chondrocytes from the recycled medium. Repeated recycling of the culture medium demonstrates a promising method to increase the number of chondrocytes in vitro for clinical application.

Cytokinetic abnormalities in DNA content, such as aneuploidy, haploidy, and tetraploidy, have been found to occur in human cartilaginous tumors. The high number of chondrocytes needed for tissue-engineered cartilaginous implants requires the cells to be passaged repeatedly. The theoretical risk of changes in the normal diploid state of these cells during their growth in vitro and after generation of tissue-engineered cartilage in vivo is not known. Materials and methods Auricular chondrocytes were obtained from 6 patients and cultured in vitro. Chondrocyte number was increased by repeated passaging. The passaged cells were implanted in nude mice for 8 weeks to generate tissue-engineered cartilage. Fresh control chondrocytes along with the passaged cells and cells obtained from the tissue-engineered constructs were collected and compared for DNA content by flow cytometry.Flow cytometry demonstrated 100% diploidy with no evidence of aneuploidy, haploidy, or tetraploidy in all groups of cells. Histology of the tissue-engineered cartilage also showed no evidence of cellular atypia.The number of human auricular chondrocytes can be increased by repeated passaging and passaged chondrocytes can be safely used for implantation to generate tissue-engineered constructs without a change in the normal diploid state of the cells. Histology of the cartilage generated showed normal features without atypia.

Background Cytokinetic abnormalities in DNA content, such as aneuploidy, haploidy, and tetraploidy, have been found to occur in human cartilaginous tumors. The high number of chondrocytes needed for tissue-engineered cartilaginous implants requires the cells to be passaged repeatedly. The theoretical risk of changes in the normal diploid state of these cells during their growth in vitro and after generation of tissue-engineered cartilage in vivo is not known. Materials and methods Auricular chondrocytes were obtained from 6 patients and cultured in vitro. Chondrocyte number was increased by repeated passaging. The passaged cells were implanted in nude mice for 8 weeks to generate tissue-engineered cartilage. Fresh control chondrocytes along with the passaged cells and cells obtained from the tissue-engineered constructs were collected and compared for DNA content by flow cytometry. Results Flow cytometry demonstrated 100% diploidy with no evidence of aneuploidy, haploidy, or tetraploidy in all groups of cells. Histology of the tissue-engineered cartilage also showed no evidence of cellular atypia. Conclusion The number of human auricular chondrocytes can be increased by repeated passaging and passaged chondrocytes can be safely used for implantation to generate tissue-engineered constructs without a change in the normal diploid state of the cells. Histology of the cartilage generated showed normal features without atypia.

Objective: To assess Internet use among a group of Brazilian pediatricians and otolaryngologists and to inquire about possible Web based medical resources. Methods: Questionnaires were distributed among attendees of a 2001 Pediatric Otolaryngology meeting in Brazil. Multiple choice or yes/no questions related to the use of the Internet among physicians were presented. Results: All of the 99 respondents of an estimated 900 attendees, had Internet access (high-speed 18%, conventional 77%); 52% at home, 42% in the office and 6% at both sites. Eighty-one percent of those responding regularly used the Internet for medical updating. Sharing a pediatric otolayngology medical association Web site with patients was desired by 73%; an interest in personal Web based medical information and updates was demonstrated by 97%. Five percent of respondents already had established personal practice Web sites and 54% agreed with potential benefits for improving medical practice. Conclusion: The potential role of electronic communication and a desire to establish a pediatric otolaryngology Web site that would maximize inclusiveness is appreciated by this group of Brazilian otolaryngologists and pediatricians.

OBJECTIVE: We present the application and initial results of a CO2 laser technique for the treatment of medically refractory chronic granular myringitis (CGM).STUDY DESIGN AND SETTING: Retrospective case series of 15 treated ears in 13 consecutive patients between the ages of 6 and 14 years (median age, 9.0 years) cared for in a tertiary care specialty hospital.RESULTS: Eleven of 15 treated ears had total resolution of CGM and associated symptoms; median follow-up time was 10 months. Three ears were improved, and 1 ear remained unchanged. Three of 5 preoperative tympanic membrane perforations healed after laser treatment; 1 patient developed a postoperative, dry perforation. Hearing was not impaired in any patient tested.CONCLUSION: Preliminary results suggest that CGM, when refractory to medical treatment, can often be treated effectively by a single laser treatment.

To the Editor: The pathogenesis of microtia, a rare congenital malformation of the external ear, remains elusive. Phenotypes range from minor deformities, such as preauricular tags, to anotia, the ...

Objective: Auricular reconstruction for repair of severe congenital microtia can provide the patient with a realistic-looking pinna. However, an unseen functional cephaloauricular sulcus is necessary, especially for patients with eyeglasses. Technique: (1) A skin graft is harvested. (2) The ear framework is separated substantially from the side of the head. (3) A crescent of hair-bearing scalp skin is excised. (4) The donor skin graft is divided. One part of the graft surfaces the superolateral and medial portions of the auricular framework into the sulcus depth; the remainder covers the side of the head. Results: This simple technique was effective. Minor transient sequelae have included granulation tissue (2 patients), localized infection (2 patients), and adhesion (1 patient). Patients requiring glasses have worn frames soon after surgery, and no sulcus problems have resulted. Conclusion: Creation of this postauricular sulcus during microtia reconstruction is easily performed and offers the patient a durable and functional space, especially for eyeglass wear. (Otolaryngol Head Neck Surg 1999;120:789-93.)

DFNA9 is an autosomal dominant, nonsyndromic, progressive sensorineural hearing loss with vestibular pathology. Here we report three missense mutations in human COCH (previously described as Coch5b2), a novel cochlear gene, in three unrelated kindreds with DFNA9. All three residues mutated in DFNA9 are conserved in mouse and chicken Coch, and are found in a region containing four conserved cysteines with homology to a domain in factor C, a lipopolysaccharide-binding coagulation factor in Limulus polyphemus. COCH message, found at high levels in human cochlear and vestibular organs, occurs in the chicken inner ear in the regions of the auditory and vestibular nerve fibres, the neural and abneural limbs adjacent to the cochlear sensory epithelium and the stroma of the crista ampullaris of the vestibular labyrinth. These areas correspond to human inner ear structures which show histopathological findings of acidophilic ground substance in DFNA9 patients.

SummaryWe report that the Bjornstad syndrome gene maps to chromosome 2q34-36. The clinical association of sensorineural hearing loss with pili torti (broken, twisted hairs) was described >30 years ago by Bjornstad; subsequently, several small families have been studied. We evaluated a large kindred with Bjornstad syndrome in which eight members inherited pili torti and prelingual sensorineural hearing loss as autosomal recessive traits. A genomewide search using polymorphic loci demonstrated linkage between the disease gene segregating in this kindred and D2S434 (maximum two-point LOD score = 4.98 at θ = 0). Haplotype analysis of recombination events located the disease gene in a 3-cM region between loci D2S1371 and D2S163. We speculate that intermediate filament and intermediate filament-associated proteins are good candidate genes for causing Bjornstad syndrome.

Auricular reconstruction of microtia is available and is extremely satisfying for the patient, family, and care givers. The patient can look forward to a realistic pinna. With current research activity, auricular reconstruction could evolve even further. The pediatrician is an extremely important member of this effort in providing valuable reassurance, direction, and surveillance for patients with these unusual auricular malformations.

Auricular construction for a child born with microtia is a rewarding endeavor that can provide the patient with a realistic pinna. However, not all reconstructive problems have been solved. For example, the donor sites for the rib harvest and for subsequent skin grafting are not without morbidity and scar formation. Furthermore, in some children, an unexpected shortage of rib can be encountered. Surgical techniques that address these problems are described. (Arch Otolaryngol Head Neck Surg. 1996;122:617-620)

The initial optimal assessment of the neonate born with unilateral facial paralysis should be performed as soon after birth as possible by a multidisciplinary team with the goal being to distinguish between a traumatic or developmental etiology. Electrophysiologic studies, particularly ENOG testing, are essential for this differentiation; radiologic and audiologic studies are of potential diagnostic benefit. In children determined to have a unilateral facial paralysis of developmental origin, subsequent clinical exams complemented by electrophysiologic testing facilitate prognosis. Unfortunately, in the majority of developmental etiology cases, the likelihood of spontaneous improvement is negligible. In these children, reanimation procedures will need to be considered with limited information in the current literature to dictate the optimal age of such surgical intervention.

Objective: To study congenital auricular malformation, an uncommon but serious condition. The literature about auricular malformation separates the medical, otologic, and auricular reconstructive elements; this study addresses all elements in a single report. Design: A retrospective chart analysis of patients with microtia and severe auricular malformation from initial presentation through surgical reconstruction for those of appropriate age. Setting: A single author's experience in a practice at a referral institution. Patients: Ninety-two patients (108 ears). Results: Early presentation at less than 1 year of age was noted for 40 patients. Coexisting medical conditions were noted in 16 patients. Seven patients demonstrated facial paralysis. Unexpected sensorineural hearing loss was discovered in six patients. Twenty-four patients had undergone auricular reconstruction at the time of this report. Additional patients had undergone skin tag removal, ventilation tube placement, and atresia repair. Conclusions: Children with microtia and significant auricular malformation require global attention to early family guidance, to expected and unexpected hearing loss, to language development, to associated medical conditions, and to both auricular and otologic reconstruction issues. (Arch Otolaryngol Head Neck Surg. 1995;121:57-62)

Objective: Music-induced hearing loss is a growing public health concern. Potential contributors include intentional exposure to loud music volume and lack of awareness about potential hearing damage. We wanted to evaluate current trends in music listening behaviors, perception of personal risk, and hearing loss education in 2 musically inclined demographics.Method: An anonymous online 89-question survey instrument was made available to a convenience sample of visitors on both VH1.com and CMT.com Web sites. Respondents were queried about attitudes and behaviors related to hearing protection and noise exposure. A P < .05 value was used for independent t and z tests.Results: Total respondents: 1601. The minority of respondents felt hearing loss created a personal problem (19%), yet 43% had experienced hearing related issues. Respondents under 21 displayed potentially more harmful listening behaviors and symptoms of hearing loss, particularly with MP3 player use (P < .05). Popular media was the most informat...