The effect of oral guaifenesin on pediatric chronic rhinitis: A pilot study.

OBJECTIVES: To investigate the effectiveness of guaifenesin in the relief of nasal symptoms in children with chronic rhinitis (CR). We hypothesized that guaifenesin use over a 14-day study period would improve subjective nasal complaints in pediatric patients with chronic rhinitis, as measured by the SinoNasal-5 (SN-5) survey. We also hypothesized improvement in nasal volume and cross-sectional area with guaifenesin. STUDY DESIGN: Randomized, placebo-controlled, parallel group, masked clinical trial. METHODS: The study consisted of a 14-day, randomized, placebo-controlled, parallel group, masked clinical trial of oral guaifenesin for CR in children aged 7-18 years. A 2:1 ratio of subjects on active medication to placebo was used. The study was approved by the Western Institutional Review Board. On initial enrollment and at the conclusion of therapy, the SN-5 was completed by parents, acoustic rhinometry measurements performed, and mucus sampling for rheology was obtained. RESULTS: 30 subjects were enrolled in the study, with 20 receiving guaifenesin and 10 placebo. Treatment with guaifenesin for 14 days produced a significant mean change towards clinical improvement in SN-5 scores compared with placebo (p = 0.013). There was no significant difference in quality of life assessment scores between the two groups or in any of the acoustic rhinometry parameters. Many of the study subjects had difficulty producing a mucus sample sufficient for analysis. CONCLUSIONS: Based upon our pilot data, it appears that guaifenesin treatment may produce objective improvements in pediatric patients with CR. Further research with larger samples sizes, inclusion of children younger than 6, and biophysical mucus analyses is warranted. LEVEL OF EVIDENCE: Level 2b.

Otolaryngology Resident Well-Being and Perceptions of the Clinical Learning Environment.

OBJECTIVE: To measure the current state of organizational and well-being factors in otolaryngology residency programs and associate these perceptions with demographics, pursuit of subspecialty fellowships, and performance on the Otolaryngology Training Examination (OTE). MATERIALS AND METHODS: Anonymous mail and online survey study of otolaryngology residents from the Southern, Mid-Atlantic, and East South-Central Regions of the United States. SUMMARY OF RESULTS: A total of 46 otolaryngology residents across 14 residency training programs (22% resident response rate) completed our survey. Residents who scored above the 80th percentile on the OTE perceived greater organizational support (median = 3.84) than residents who scored below the 40th percentile (median = 3.31), U = 48.00, P = .047, η2 = 0.14. Residents interested in fellowship reported less burnout (median = 2.44) compared to those who did not plan to pursue fellowship (median = 3.56), U = 105.00, P = .010, η2 = 0.05. Residents pursuing fellowship also reported less work-life strain (median = 2.56) than those forgoing fellowship (median = 2.89), U = 126.00, P = .044, η2 = 0.10. Residents with children reported greater work-life strain (median = 3.11) compared to those without (median = 2.56), U = 60.50, P = .008, η2 = 0.15. CONCLUSION: For otolaryngology residents in this survey sample, the perception of organizational support and well-being may influence resident performance (on OTE examinations) and ultimate career goals (fellowship applications). Program directors and coordinators can use this information to strengthen the perceptions of organizational support as well as improve the clinical learning environment to optimize training conditions for their residents. Residency program directors can also use the identified study measures to assess resident perceptions of the clinical learning environment and well-being for annual evaluation and improvement purposes.

An Analysis of Risk Factors in Unilateral Versus Bilateral Hearing Loss.

A retrospective review of children with confirmed hearing loss identified through universal newborn hearing screening (UNHS) in Virginia from 2010 to 2014 was conducted in order to compare the incidence of Joint Committee on Infant Hearing (JCIH) risk factors in children with unilateral hearing loss (UHL) to bilateral hearing loss (BHL). Over the 5-year study period, 1004 children (0.20% of all births) developed a confirmed hearing loss, with 544 (51%) children having at least one JCIH risk factor. Overall, 18% of children with confirmed hearing loss initially passed UNHS. Of all children with risk factors, 226 (42%) demonstrated UHL and 318 (58%) had BHL. The most common risk factors for UHL were neonatal indicators (69%), craniofacial anomalies (30%), stigmata of HL syndromes (14%), and family history (14%). The most common risk factors in BHL were neonatal indicators (49%), family history (27%), stigmata of HL syndromes (19%), and craniofacial anomalies (16%). Children with the risk factor for positive family history were more likely to have BHL, while those with craniofacial anomalies were more likely to have UHL (P < .001). Neonatal indicators were the most commonly identified risk factor in both UHL and BHL populations. Children with UHL were significantly more likely to have craniofacial anomalies, while children with BHL were more likely to have a family history of hearing loss. Further studies assessing the etiology underlying the hearing loss and risk factor associations are warranted.

Perioperative interdisciplinary approach for reduction of opioid use in pediatric tonsillectomy: Protocol using dexmedetomidine and bupivicaine as adjunct agents.

IMPORTANCE: Pediatric tonsillectomy is a common procedure now being performed most often for patients with obstructive sleep apnea, which has been associated with increased sensitivity to the respiratory side effects of opioid medications. This study investigates a strategy to decrease the use of opiate medications in a particularly vulnerable population. OBJECTIVE: Describe an interdisciplinary approach between Otolaryngologists and Anesthesiologists to decrease opiate use in tonsillectomy patients. Demonstrate safety of this protocol. Evaluate the effect of the protocol on intraoperative need for opiate medications and inhaled anesthetic use. Perform cost analysis of the protocol. DESIGN: Retrospective case-control study with cost analysis. SETTING: Tertiary Care Hospital. PARTICIPANTS: Pediatric patients undergoing tonsillectomy at a tertiary care hospital. INTERVENTIONS: Preoperative and intraoperative dexmedetomidine with local bupivacaine injection into the tonsillar fossa. MEASURES: Intraoperative need for sevoflurane, opiate, and propofol. Post-operative pain scores, and utilization of post-operative opiate, acetaminophen, and ibuprofen pain medications. Post-operative adverse events. Cost analysis of protocol. RESULTS: This protocol led to a decrease in intraoperative opiate use by 49.6%, a decrease in intraoperative sevofluorane use by 18%, and a lower reported maximum post-operative pain score without any increase in adverse events. The protocol added a small increase in medication cost of $4.07 to each procedure. CONCLUSION: The use of dexmedetomidine and local anesthetic in pediatric tonsillectomy is a safe and effective protocol that allows for the reduction of opiate use and improved post-operative pain control. KEY POINTS: Question: Can the combination of dexmedetomidine and infiltration of local anesthetic reduce overall opioid use for peediatric patients undergoing tonsillectomy? FINDINGS: In this case-control study, use of dexmedetomidine and local anesthetic injected into the tonsillar fossa led to a decrease in intraoperative opiate use by 49.6%, a decrease in intraoperative sevofluorane use by 18%, and a lower reported maximum pain score without an increase in adverse events. Meaning: Use of dexmedetomidine and local anesthetic as anesthetic adjuncts may help reduce need for intraoperative opiates and decrease the use of volatile anesthetic agents in pediatric tonsillectomy patients, which are undesirable medications in the pediatric population for their respective respiratory depression and potentially neurotoxic side effects.

Surgical Resident Burnout and Job Satisfaction: The Role of Workplace Climate and Perceived Support.

BACKGROUND: Surgical residents train under immense stress, often manifesting into poor well-being. While recent research identifies methods of coping with stress, few studies empirically investigate the role of the environment on surgical resident well-being. We aimed to assess surgical resident perceptions of workplace climate, organizational support, burnout, and job satisfaction to test a mediation model identifying antecedents to well-being. MATERIALS AND METHODS: A convenience sampling of program directors from general surgery within the Eastern region of the United States were emailed to request either agenda time to collection data via paper survey or to forward an electronic survey link to their residents between March 2016 and June 2016. The survey included scales demonstrating validity evidence on well-being, climate, and perceptions of support. RESULTS: Based on 160 general surgery residents (out of 557; 29% response rate) across 19 training programs, our mediation model found that job satisfaction was significantly predicted by workplace climate directly (direct effect = 0.37, 95% CI [0.19, 0.55]) and indirectly (specific indirect effect = 0.07, 95% CI [0.01, 0.13]) through perceived organizational support and burnout, while controlling for training year and gender, F(5,147) = 53.76, P < 0.001, Rsq. = 0.65. CONCLUSIONS: Medical education requires an additional focus on how the clinical learning environment affects surgical resident well-being. Health systems and training programs will need to collaborate on workplace innovations to improve workplace climate for trainees to address the concerns of well-being with a modern surgical workforce.

High-Efficiency Nose-to-Lung Aerosol Delivery in an Infant: Development of a Validated Computational Fluid Dynamics Method.

Background: Computational fluid dynamics (CFD) provides a powerful tool for developing new high-efficiency aerosol delivery strategies, such as nose-to-lung (N2L) aerosol administration to infants and children using correctly sized aerosols. The objective of this study was to establish numerically efficient CFD solution methods and guidelines for simulating N2L aerosol administration to an infant based on comparisons with concurrent in vitro experiments. Materials and Methods: N2L administration of a micrometer-sized aerosol (mass median aerodynamic diameter [MMAD] = 1.4 µm) was evaluated using concurrent CFD simulations and in vitro experiments. Aerosol transport and deposition was assessed in a new nasal airway geometry of a 6-month-old infant with a streamlined nasal cannula interface, which was constructed as a CFD mesh and three-dimensionally printed to form an identical physical prototype. CFD meshes explored were a conventional tetrahedral approach with near-wall (NW) prism elements and a new polyhedral mesh style with an equally refined NW layer. The presence of turbulence in the model was evaluated using a highly efficient low-Reynolds number (LRN) k-ω turbulence model, with previously established NW corrections that accounted for anisotropic wall-normal turbulence as well as improved NW velocity interpolations and hydrodynamic particle damping. Results: Use of the new polyhedral mesh was found to improve numerical efficiency by providing more rapid convergence and requiring fewer control volumes. Turbulent flow was found in the nasal geometry, generated by the inlet jets from the nasal cannula interface. However, due to the small particle size, turbulent dispersion was shown to have little effect on deposition. Good agreement was established between the CFD predictions using the numerically efficient LRN k-ω model with appropriate NW corrections and in vitro deposition data. Aerosol transmission efficiencies through the delivery tube, nasal cannula, and infant nasal model, based on experimental and CFD predictions, were 93.0% and 91.5%, respectively. Conclusions: A numerically efficient CFD approach was established to develop transnasal aerosol administration to infants and children. Small particle aerosols with aerodynamic diameters of ∼1.5 µm were confirmed to have low inertial depositional loss, and have low deposition from turbulent dispersion, making them ideal for high-efficiency lung delivery through an infant nasal cannula interface.

Analysis of risk factors associated with unilateral hearing loss in children who initially passed newborn hearing screening.

OBJECTIVE: To analyze 2007 Joint Committee on Infant Hearing (JCIH) risk factors in children with confirmed unilateral hearing loss (UHL) who initially passed newborn hearing screening. METHODS: Retrospective record review of 16,108 infants who passed newborn hearing screening but had one or more JCIH risk factors prompting subsequent follow-up through the universal newborn hearing screening (UNHS) program in Virginia from 2010 to 2012. The study was reviewed and qualified as exempt by the Virginia Commonwealth University Institutional Review Board (IRB) and the Virginia Department of Health. RESULTS: Over the 2-year study period, 14896 (4.9% of total births) children passed UNHS but had the presence of one or more JCIH risk factor. Ultimately, we identified 121 babies from this group with confirmed hearing loss (0.7%), with 48 babies (0.2%) showing UHL. The most common risk factors associated with the development of confirmed UHL after passing the initial screen were neonatal indicators, craniofacial anomalies, family history, and stigmata of syndrome associated with hearing loss. CONCLUSION: Neonatal indicators and craniofacial anomalies were the categories most often found in children with confirmed unilateral hearing loss who initially passed their newborn hearing screen. While neonatal indicators were also the most common associated risk factor in all hearing loss, craniofacial abnormalities are relatively more common in children with UHL who initially passed newborn hearing screening. Further studies assessing the etiology underlying the hearing loss and risk factor associations are warranted.

Improving resident well-being and clinical learning environment through academic initiatives.

BACKGROUND: Organizational effects on job satisfaction, burnout, work-life balance, and perceived support have not been studied in the context of the clinical learning environment. We evaluated the relationship between academic resources and resident well-being, the clinical learning environment, and in-service examination performance of surgical residents. MATERIALS AND METHODS: Residents of general surgery and surgical specialty programs were recruited from March 2016 through June 2016 across the Southeast, Mid-Atlantic, and Northeast regions. Program directors were asked to allow distribution of a paper survey or to forward an electronic survey link onto residents. Five dichotomous questions were asked regarding access to academic resources. Validated measures were obtained assessing resident well-being and perceived clinical learning environment. Data were analyzed through t-tests and chi-squared test of independence. RESULTS: We received 276 respondents across 50 programs. Residents perceiving adequate support to succeed had less burnout (P = 0.008), better resilience (P = 0.009), better job satisfaction (P < 0.001), less work/life strain (P = 0.001), better workplace climate (P < 0.001), better organizational support (P < 0.001), and were more likely to have high performance on the in-service examination (P = 0.001). Specific resources including educational stipends, review questions, in-service board prep, and support for poor performers correlated with improved well-being and perceived clinical learning environment. CONCLUSIONS: Provision of academic resources has implications beyond in-service examination performance, correlating with improved resident well-being and perceptions of the clinical learning environment.

The use of propranolol in the treatment of subglottic hemangiomas: A literature review and meta-analysis.

OBJECTIVES: 1) Describe the origins of the use of propranolol in the treatment of subglottic hemangiomas, 2) Perform meta-analysis of all case reports and series in which propranolol was used to treat subglottic hemangiomas. STUDY DESIGN: Literature review and meta-analysis. METHODS: A total of 61 cases were identified from 19 scholarly articles. Cases were assessed by parameters including age at diagnosis, presence of other hemangiomas, percent airway obstructed, dose of propranolol, treatment duration, age at therapy termination, use of steroids, and treatment failure. Treatment failure was defined as: 1) Need for surgery after initiation of propranolol, 2) Return of symptoms, or 3) Endoscopic worsening/recurrence of hemangioma. All data was subjected to comprehensive statistical analysis. RESULTS: Though not statistically significant, a trend was noted towards a decreased treatment failure rate with increasing doses of propranolol (p = 0.0563). The use of concurrent steroids was associated with a higher failure rate (p = 0.0487). Notably, no associations were observed between the presence of additional hemangiomas, prior surgery, or increased initial percent airway obstruction with treatment failure. CONCLUSION: Propranolol is rapidly becoming the standard of care in the treatment of subglottic hemangiomas. Despite widespread adoption, the rarity of this condition has limited previous studies to case reports and small series. No evidence-based guidelines exist for proper dosing of propranolol. The results of this meta-analysis suggest a benefit to higher doses of propranolol (3 mg/kg/day), though further investigation is needed.

Prophylactic antibiotic therapy for fractures of the maxillary sinus.

We conducted a study to examine the incidence of acute sinusitis following maxillary sinus fractures, as well as the impact of antibiotics in the postinjury period. Fifty patients who presented to our institution with a fracture of the maxillary sinus were prospectively randomized to receive either a nasal saline spray and a 3-day course of antibiotics (either amoxicillin/clavulanate or levofloxacin) or nasal saline alone; there were 25 patients in each group. After a minimum of 3 days, all patients were assessed for acute sinusitis. Follow-up data were available on 17 patients (a total of 21 fractured sinuses) in the antibiotic group (68%) and 14 patients (17 fractured sinuses) in the control group (56%). The groups were balanced in terms of demographics, location of fractures, mechanism of fracture, and time to follow-up. After 3 days, 95.23% of the fractured sinuses in the antibiotic group and 88.23% of the fractured sinuses in the control group exhibited signs or symptoms consistent with or suggestive of acute sinusitis. Very few sinuses in either group showed no evidence of sinusitis: only 1 in the antibiotic group (4.76%) and 2 in the control group (11.76%); this difference was not significant (p = 0.5768). We conclude that while the clinical rate of acute sinusitis after maxillary sinus fractures is high, a 3-day course of antibiotics is not effective in preventing its symptoms in the postinjury period.

Attainment of surgical competence in otolaryngology training.

OBJECTIVES/HYPOTHESIS: Our aim was to determine the postgraduate year (PGY) of residency at which residents achieve competence in key otolaryngologic procedures as perceived by residents and program directors (PDs), determine resident or programmatic factors affecting PGY at which residents perceive attainment of competence, and evaluate the relationship between resident and PD perceptions for attainment of competence in these procedures. STUDY DESIGN: Cross-sectional survey. METHODS: We surveyed residents and PDs in US otolaryngology residencies in 2011 using SurveyMonkey.com and assessed subjective attainment of competence by residents for 32 otolaryngologic procedures. PGY at which subjective competence achieved per resident perception was determined using a logistic regression model; PD perception was determined by mean calculation. RESULTS: Two hundred seventy-seven residents (19.45%) and 39 PDs (37.86%) completed surveys. Residents achieved subjective competence later than expected by PDs for 25 of 32 procedures, although differences were generally small. The largest disparities were observed for nonsurgical office-based procedures, for which <90% of residents reported competence by PGY-5, whereas PDs expected competence before PGY-3. The greatest number of disparities was present in the subspecialty area of facial plastic and reconstructive surgery. Male gender predicted earlier self-reported attainment of competence in 10 of 32 procedures (P < .05). Degrees held, fellowship plans, and the size of the program were not significant predictors of PGY at which residents reported competence for most procedures. CONCLUSIONS: We provide baseline data on PGY at which residents and PDs feel competence is achieved in key otolaryngologic procedures. These results may guide milestone development for resident training and assessment. LEVEL OF EVIDENCE: NA.

Variability in Nose-to-Lung Aerosol Delivery.

Nasal delivery of lung targeted pharmaceutical aerosols is ideal for drugs that need to be administered during high flow nasal cannula (HFNC) gas delivery, but based on previous studies losses and variability through both the delivery system and nasal cavity are expected to be high. The objective of this study was to assess the variability in aerosol delivery through the nose to the lungs with a nasal cannula interface for conventional and excipient enhanced growth (EEG) delivery techniques. A database of nasal cavity computed tomography (CT) scans was collected and analyzed, from which four models were selected to represent a wide range of adult anatomies, quantified based on the nasal surface area-to-volume ratio (SA/V). Computational fluid dynamics (CFD) methods were validated with existing in vitro data and used to predict aerosol delivery through a streamlined nasal cannula and the four nasal models at a steady state flow rate of 30 L/min. Aerosols considered were solid particles for EEG delivery (initial 0.9 µm and 1.5 µm aerodynamic diameters) and conventional droplets (5 µm) for a control case. Use of the EEG approach was found to reduce depositional losses in the nasal cavity by an order of magnitude and substantially reduce variability. Specifically, for aerosol deposition efficiency in the four geometries, the 95% confidence intervals (CI) for 0.9 and 5 µm aerosols were 2.3-3.1 and 15.5-66.3%, respectively. Simulations showed that the use of EEG as opposed to conventional methods improved delivered dose of aerosols through the nasopharynx, expressed as penetration fraction (PF), by approximately a factor of four. Variability of PF, expressed by the coefficient of variation (CV), was reduced by a factor of four with EEG delivery compared with the control case. Penetration fraction correlated well with SA/V for larger aerosols, but smaller aerosols showed some dependence on nasopharyngeal exit hydraulic diameter. In conclusion, results indicated that the EEG technique not only improved lung aerosol delivery, but largely eliminated variability in both nasal depositional loss and lung PF in a newly developed set of nasal airway models.

The clinical and audiologic features of hearing loss due to mitochondrial mutations.

OBJECTIVES: To characterize mitochondrial sequence variants present in a nationwide hereditary deafness DNA repository of samples from deaf subjects and to define the clinical presentation and audiometric characteristics of individuals with a mitochondrial sequence variant. STUDY DESIGN: Retrospective review of results for select mitochondrial mutations performed on DNA samples from subjects compiled from 1997 to 2009. SETTING: National hereditary deafness DNA repository. SUBJECTS AND METHODS: Available samples from subjects in the repository were screened to identify those with mitochondrial sequence variants. Clinical data on the nature of mutation, type and severity of the hearing loss, and sex, age at diagnosis, family history of hearing loss, and ethnicity were analyzed. RESULTS: Eighty-six patients were identified with mitochondrial mutations or 3.5% of the subjects studied. Among those with mitochondrial mutations, 21 (24.4%) had the m.7445A>G substitution, 18 (20.9%) had the m.1555A>G substitution, 18 (20.9%) had the m.961T>G substitution, and 29 (33.7%) had a m.961delT+C(n) complex deletion. The majority of patients had bilateral severe to profound hearing loss. Fifty-three (62%) patients were female, and a family history of hearing loss was documented in 66 (76.7%) patients. The deafness was recognized prior to 3 years of age in 26 patients. CONCLUSION: Mitochondrial deafness in this sample was associated with a variety of genetic mutations and a wide spectrum of clinical presentations. Because of increased aminoglycoside susceptibility associated with some forms of mitochondrial deafness, matrilineal relatives may be at risk in those cases, highlighting the importance of making an accurate diagnosis prior to exposure.

Risk factors associated with unilateral hearing loss.

OBJECTIVE: To analyze the presence of Joint Committee on Infant Hearing (JCIH) risk factors and co-occurring birth defects (CBDs) in children with unilateral hearing loss (UHL). DESIGN: Retrospective review. SETTING: Statewide registry of universal newborn hearing screen data for all children born in Virginia from 2002 through 2008. PATIENTS: The study population comprised 371 children with confirmed UHL. MAIN OUTCOME MEASURES: Universal newborn hearing screen status, presence or absence of JCIH risk factors, and CBDs RESULTS: Of the 371 children with confirmed unilateral hearing loss, 362 (97.5%) were identified through a failed universal newborn hearing screen. Of these 362 children, 252 (69.6%) had no JCIH risk factors and 110 (30.3%) had 1 or more risk factor reported. Nine children (2.5%) with 1 or more risk factors passed the universal newborn hearing screen but had later-onset UHL. Craniofacial anomaly was the most commonly reported JCIH risk factor in 48 children (43.6%). A family history of permanent childhood hearing loss was present in 24 children (21.8%). Twenty children (18.2%) had stigmata associated with a syndrome including hearing loss. Of the 110 children with UHL and a JCIH risk factor, additional CBDs were identified in 83 (75.5%). An ear-specific anomaly was most prevalent in 37 infants (44.6%), followed by cardiovascular anomalies in 34 infants (41.0%). CONCLUSIONS: Thirty percent of children with confirmed UHL had a JCIH risk factor, most commonly craniofacial anomalies, family history of hearing loss, and stigmata of syndromes associated with hearing loss. However, the absence of JCIH risk factors does not preclude the development of UHL. Further studies assessing the etiology of UHL and risk factor associations are warranted.

Middle ear fluid characteristics in pediatric otitis media with effusion.

OBJECTIVE: Persistent otitis media with effusion is caused by poor clearance of middle ear fluid usually following an episode of acute otitis media. This fluid is thought to be viscous and poorly transportable by cilia. Because a subset of children require multiple myringotomy and tube placements for recurrent disease, we hypothesized that children requiring repeated procedures would have effusion fluid that was more viscous and less transportable than those having their first procedure. DESIGN: Prospective clinical study. SETTING: Tertiary care center. PATIENTS AND INTERVENTIONS: Middle ear secretions were collected at the time of myringotomy and tube insertion in 36 children accrued sequentially. Twenty-six of these children were having their first procedure and 10 had previously undergone myringotomy and tube placement. MAIN OUTCOME MEASURES: The secretions were evaluated for in vitro mucociliary transportability, and dynamic rheology in a magnetic microrheometer. RESULTS: Children with the need for repeated procedures had effusions with lower mucociliary transportability, and overall higher mean measures of surface mechanical impedance/frictional adhesion, but these did not reach statistical significance. Mucopurulent effusions had significantly greater transportability than both the mucoid and serous effusions in both groups. CONCLUSIONS: Persistent or recurrent otitis media with effusion is associated with poorly transportable middle ear fluid, which may have higher frictional adhesion. The best mucociliary transportability was measured in mucopurulent effusions.

Etiology of unilateral hearing loss in a national hereditary deafness repository.

PURPOSE: The aim of this study was to characterize the genetic, audiologic, and epidemiologic characteristics of unilateral hearing loss (HL) in a national hereditary deafness repository. MATERIALS AND METHODS: This is a prospective clinical study involving 34 subjects identified in a national hereditary deafness repository. Clinical data and family history of HL were obtained on enrollment. Candidate deafness genes were screened by single-stranded conformation polymorphism, and mutations were confirmed with sequencing. RESULTS: Thirty-four subjects (19 males, 15 females) with unilateral HL were identified, ranging in age from 2 months to 36 years. The mean age at diagnosis was 7 years, and the left ear was affected in 62% of the cases. The racial distribution of our sample was 62% white, 23% African American, and 15% Hispanic. Imaging results were available in 47%, and most (69%) were considered normal. Nineteen percent had enlarged vestibular aqueducts, 2 had ipsilateral Mondini dysplasia, and 1 had a common cavity deformity. Twenty subjects (59%) had a family history of HL, with 26% specifically reporting familial unilateral HL. Mutational screening revealed sequence variants in the GJB2 (connexin 26), GJB3 (connexin 31), TECTA, and COCH genes. Two novel mutations were detected in COCH and TECTA. CONCLUSIONS: Sequence variants in known deafness genes were detected in more than one-third of our study population, suggesting that gene/gene or gene/environmental interactions may indeed play a role in the etiology of some cases of unilateral deafness. Further prospective studies including congenital cytomegalovirus screening at birth and molecular screening of deafness genes in children with congenital unilateral HL will be required to establish the etiology of unilateral deafness with certainty.