Allyship to Advance Diversity, Equity, and Inclusion in Otolaryngology: What We Can All Do.

Purpose of review: To summarize the current literature on allyship, providing a historical perspective, concept analysis, and practical steps to advance equity, diversity, and inclusion. This review also provides evidence-based tools to foster allyship and identifies potential pitfalls. Recent findings: Allies in healthcare advocate for inclusive and equitable practices that benefit patients, coworkers, and learners. Allyship requires working in solidarity with individuals from underrepresented or historically marginalized groups to promote a sense of belonging and opportunity. New technologies present possibilities and perils in paving the pathway to diversity. Summary: Unlocking the power of allyship requires that allies confront unconscious biases, engage in self-reflection, and act as effective partners. Using an allyship toolbox, allies can foster psychological safety in personal and professional spaces while avoiding missteps. Allyship incorporates goals, metrics, and transparent data reporting to promote accountability and to sustain improvements. Implementing these allyship strategies in solidarity holds promise for increasing diversity and inclusion in the specialty.

Factors associated with delayed referral and hearing rehabilitation for congenital sensorineural hearing loss.

OBJECTIVES: To identify the impact of social determinants of health and clinical status on referral and intervention for congenital sensorineural hearing loss. STUDY DESIGN: Retrospective chart review of children with confirmed sensorineural or mixed hearing loss between 2013 and 2021 at a single academic medical institution. METHODS: Referral rates and timing for hearing rehabilitation, rates and timing of completed evaluation, and rate and timing of amplification were recorded. Patient demographics included gestational age, race, ethnicity, sex, hearing loss severity, and CMV status. RESULTS: There were 216 children with confirmed sensorineural or mixed hearing loss, of which 77 had a unilateral hearing loss and 89 a severe or profound hearing loss. Delayed referral for hearing aid evaluation was noted in premature patients (median 375 days premature, median 147 term; p < 0.01) and publicly insured patients (median 215 days, median 123 private; p = 0.04). Delayed time to hearing aid fitting was noted for non-white patients (median 325 days, median 203 white patients; p < 0.01), publicly insured patients (median 309 days, median 212 private insurance; p < 0.02), and premature patients (median 462 days, median 224 term; p = 0.03). White patients were more likely to be referred for cochlear implant (p = 0.03).Privately insured patients and patients with a positive CMV test were more likely to be referred for cochlear implant evaluation, be seen in the cochlear implant clinic, and undergo implantation (p < 0.05). Non-white patients had a delay in cochlear implantation referral (median 928 days, median 398 days white patients; p = 0.05). Prolonged interval between evaluation in cochlear implant clinic to implantation was noted for privately insured patients (median 125 days; median 78 days publicly insured; p = 0.05). CONCLUSIONS: Sociodemographic factors were significantly associated with hearing amplification referral rates and time until amplification for children with identified congenital sensorineural hearing loss. For cochlear implantation, insurance type, CMV status were significantly associated with rate and timing of cochlear implant pathway.

Leveraging the virtual landscape to promote diversity, equity, and inclusion in Otolaryngology-Head & Neck Surgery.

The pandemic era has wrought disruptive changes across all aspects of academic medicine, transforming clinical care systems, research enterprises, and educational practices. Among these sweeping changes, some of the most significant for Otolaryngology-Head & Neck Surgery (OHNS) relate to innovative use of virtual communication. The virtual landscape has not only redefined the delivery of patient care but also expanded educational interactions across the career continuum. As the architecture of relationships has been reimagined, the traditional Halstedian teaching paradigm has evolved, now integrating in-person, virtual, and hybrid learning. Once formidable barriers to distance learning have diminished, giving way to social networks that support mentorship, coaching, and sponsorship. Creative use of technology supports collaboration, feedback, spaced learning, scaffolding, and interleaving. These advances have immediate significance for OHNS, a highly collaborative specialty that leads in technological innovation and aspires to improve diversity. Whereas traditional efforts to grow the ranks of underrepresented in medicine (URiM) individuals in OHNS yielded only incremental progress, the virtual landscape is unveiling new strategies for reengineering narrow or leaky pipelines. Strategies that can help attract URiM applicants include inclusive departmental web presence, interactive online clinical experiences, virtual interviews, and remote research opportunities. As students, surgeons, and scientists collectively embrace technology, accelerated progress toward diversity, equity, and inclusion (DEI) becomes possible. Maximizing these opportunities requires aligning national and institutional imperatives for diversity with departmental priorities and mission. Finally, intentional outreach and holistic assessment support growth of structured virtual communities that foster equitable access for those who need it most.

Tackling the Minority Tax: A Roadmap to Redistributing Engagement in Diversity, Equity, and Inclusion Initiatives.

Academic medical centers striving to implement diversity, equity, and inclusion (DEI) and antiracism initiatives often ask faculty to volunteer substantial time to committee work, recruitment, mentoring, community, and administrative responsibilities. These requests are not in lieu of current workload and seldom count toward scholarship; the service may go unrecognized, unrewarded, and uncompensated. URiM faculty (underrepresented in medicine) providing such service thus pay a minority tax when precious time is syphoned away from career-advancing activities and personal growth. The resulting strain on available resources has social, psychological, and monetary ramifications that can undermine the long-term objectives of DEI initiatives. We examine the facets of the minority tax, consider the current state of diversity, and present a roadmap to redistribute, reform, and reduce URiM taxation through shared engagement in DEI initiatives. Key interventions include ascribing value to DEI efforts, implementing evidence-based policies to reduce bias, and promoting mentorship, sponsorship, and allyship.

Perceptions of Otolaryngology Residency Among Students Underrepresented in Medicine.

OBJECTIVE: Otolaryngology-Head and Neck Surgery (OHNS) has historically been one of the least diverse surgical subspecialties. The objective of this study was to better understand perceptions of OHNS from underrepresented students in medicine (URiM) and identify factors affecting URiM application to the specialty. STUDY DESIGN: Survey via electronic questionnaire. METHODS: An anonymous, 22-question electronic survey was administered nationally to URiM medical students (N = 388) regarding factors that play a role in developing an interest in applying to OHNS. Responses to questions were compared between URiMs applying to OHNS and those applying to other fields. RESULTS: Thirty-six percent of respondents identified as African American and 26% as Latino. Students completed the survey in all years of medical school. Research opportunities (H(2) = 18.58, P < .001) and having a race-concordant role model were the most important factors for those pursing OHNS residency. Personality fit and interactions with OHNS faculty had the greatest influence on their decision to pursue OHNS residency. Board scores (e.g., USMLE Step 1/2CK Scores), competitiveness, lifestyle during residency, the influence of application costs, and racial/ethnic and gender distributions did not reach statistical significance or were noninfluential. CONCLUSION: URiMs applying to OHNS value race-concordant mentorship, are involved in research, and have meaningful relationships with their respective OHNS department. We found that establishing meaningful connections with URiM mentors significantly impacts URiM students considering OHNS. While this cohort was not influenced by overemphasis of board scores within the OHNS match process, the COVID-19 pandemic negatively impacted this aspect of their application, along with away rotations. LEVEL OF EVIDENCE: NA Laryngoscope, 132:2335-2343, 2022.

The impact of social determinants of health and clinical comorbidities on post-tympanotomy tube otorrhea.

OBJECTIVES: To measure the impact of social determinants of health and clinical comorbidities on the incidence of post-tympanotomy tube otorrhea (PTTO). METHODS: Retrospective observational cohort study. All children between the ages of 0 and 17 having tympanotomy tube placement between 2009 and 2019. Between group comparisons entailed the calculation of odds ratios (OR) with 95% confidence intervals and associated p-values. RESULTS: Among 12,757 patients who underwent myringotomy and tube placement, 2217 (17.4%) presented with PTTO within 1 year. Race and sex did not correlate with the development of PTTO. Non-Hispanic ethnicity had a negative association with PTTO (OR: 0.80 (0.70-0.91), p < .0001). Insurance status correlated with incidence of PTTO with a higher rate noted among those with public insurance (OR: 1.12 (1.02-1.23), p = .02) and a lower rate among those with private insurance (OR: 0.84 (0.77-0.92), p < .0001). Craniofacial abnormalities had the strongest positive correlation with PTTO, particularly, cleft lip and/or cleft palate (OR>2.24, p < .0001). Immunodeficiency had similar impact on PTTO (OR: 2.38 (1.46-3.91), p < .0001). Asthma and prematurity did not significantly correlate with occurrence of PTTO. CONCLUSION: Higher rates of PTTO correlated strongest with clinical factors; particularly craniofacial abnormalities and immunodeficiency. Social determinants, including private insurance and non-Hispanic ethnicity, were associated with lower rates of PTTO. Race and sex did not show significant correlations.

African American Otolaryngologists: Current Trends and Factors Influencing Career Choice.

OBJECTIVES: 1) Identify factors that influence African American physicians to choose a career in otolaryngology; 2) determine the predominant practice setting for African American otolaryngologists who can be role models; and 3) determine if the presence of an African American otolaryngologist in academic setting influences career choice. METHODS: Survey methodology included a 15-item survey to determine trends in practice and factors that influenced choice of specialty. RESULTS: The results were reviewed for trends influencing career choice and practice location and stratified by age group. Most African American otolaryngologists are in academic practice and have subspecialty fellowship training. Enjoying medical student clerkship was the most frequently cited reason why African Americans chose otolaryngology as a career regardless of age. Early exposure was a driving factor in those 30 to 40 years old. Receiving mentorship was less influential in career choice for all age groups, but there was a positive association between those who were mentored in training and those who mentor faculty. CONCLUSION: The findings suggest the continued need for initiatives to increase African Americans in our specialty. Encouraging early exposure, intentional mentoring of students, and development of African American role models who can be mentors may help increase the number of African American otolaryngologist faculty. This can help our specialty achieve racial parity in a percentage that matches the number of African Americans in the United States workforce. LEVEL OF EVIDENCE: 5 Laryngoscope, 130:2336-2342, 2020.

The effect of losing federal coverage through the Affordable Care Act on ear tube placements at an urban children's hospital.

PURPOSE: 15-31% of the population in a large Mid-western city is between 100 and 400% of the Federal Poverty Level, thus qualifying for health care coverage under the Affordable Care Act (ACA). Coverage for their children would potentially be available under Children's Health Insurance Program (CHIP) or Medicaid programs. Loss of funding for these programs could be devastating for this community. METHODS: We retrospectively reviewed 1162 charts of pediatric patients with tympanostomy tube (TT) placement pre-ACA from November 2012 to December 2013 and 1606 charts post-ACA from January 2014 to July 2015. We filtered demographics by health insurance (Medicaid/CHIP/Other), residential zip codes, identified race/ethnicity within those zip codes as well as gender and age of patients getting TT during these periods. RESULTS: Bivariate analysis of these demographics between the two periods showed statistical significance (p = 0.0098) between White Hispanic/Latino children receiving ear tubes (pre-ACA = 3.8%, post-ACA = 6.4%). However, there was no statistical significance for insurance enrollment (Medicaid or non-Medicaid) and other races (White-not Hispanic/Latino (nHL), African American, Other/Unknown/Refused) with respect to TT placement. Using pre-ACA period and White nHL females as arbitrary reference, a multivariate logistic regression showed that patients requiring TT surgery were equally likely to be covered on Medicaid either before or after ACA. CONCLUSION: We demonstrated that the pre and post ACA Medicaid coverage for TT surgery did not change. Underserved children did not obtain other forms of insurance during this time. This demonstrates a potentially catastrophic loss of coverage for children should Medicaid/CHIP benefits be lost to sole coverage under the ACA.

Diversity in Otolaryngology Residency Programs: A Survey of Otolaryngology Program Directors.

Objective As the population of the United States becomes increasingly racially and ethnically diverse, it is important that the medical profession reflect these changes. Otolaryngology has previously been identified as one of the surgical subspecialties with the smallest presence of those underrepresented in medicine. In the context of this study, the term underrepresented in medicine is defined as blacks, Latinos, Native American, and Native Hawaiians. The purpose of this study was to describe the current state of otolaryngology residency programs in terms of diversity of resident and faculty cohort, explore general interviewing practices, and investigate recruitment of underrepresented in medicine applicants. Study Design Survey via electronic questionnaire. Setting Academic otolaryngology residency programs. Subjects and Methods A 14-item survey was distributed to 105 program directors asking them to consider their program's past 15 years of existence. Results With a response rate of roughly 30%, we found that over one-third of responding programs had matriculated 1 or fewer underrepresented in medicine residents. There was a statistically significant association between the number of underrepresented in medicine faculty and the number of underrepresented in medicine residents matriculated ( P = .02). Conclusion The authors stress the importance of underrepresented in medicine faculty mentorship. Although not statistically significant in this study, increasing the number of underrepresented in medicine applicants interviewed, as well as recommending outreach programs, may help to improve underrepresented minority matriculation into residency programs as demonstrated in the literature.

The Otolaryngologist's Role in Providing Gender-Affirming Care: An Opportunity for Improved Education and Training.

Currently, there are limited resources and training available for otolaryngologists and otolaryngology practice personnel to provide gender-affirming care for transgender or gender nonconforming patients. This unique patient population may present to our offices for gender-specific care or with complaints of the ear, nose, and throat unrelated to gender identity. Our current practice has unintentional but direct consequences on our patients care, as transgender patients often report negative experiences in the healthcare setting related to their gender identity. The absence of resources and training is also seen in other specialties. Physicians who create an environment where patients of all gender identities feel welcome can better meet their patients' health care needs. In addition, otolaryngologists can play a role in easing the gender dysphoria experienced by transgender patients. We suggest educational content should be created for and made available to otolaryngologists and office staff to provide gender-affirming care.

Do you need to operate following recovery from complications of pediatric acute sinusitis?

OBJECTIVES: There are many studies that evaluate the role of surgery in the treatment of complications of pediatric acute sinusitis; however there are few studies, if any that report the incidence of surgery following recovery from acute complicated sinusitis. The goal of this study was to report the incidence and indications for surgical intervention after recovery from complications of pediatric acute sinusitis. METHODS: We reviewed the records of all children admitted to a tertiary care children's hospital between January 2005 and September 2010 with a diagnosis of sinusitis and an orbital or intracranial complication. Eighty-six patients met inclusion criteria. Charts were reviewed for type of complication, initial treatment (medical or surgical), type of procedure, secondary procedures, age, and comorbidities. Statistical analysis was completed using independent samples student t-tests and Mann-Whitney tests. RESULTS: A total of 86 patients with a mean age of 6.38 years (2 months to 18 years) were identified. Eighty patients had orbital complications while six presented with intracranial complications. Twenty-seven patients (31%) underwent sinus surgery during the acute phase of their illness whereas 59 patients (69%) were treated medically. After hospitalization and recovery for acute complicated sinusitis, surgery was performed on nine patients (mean age 4.86 years) within 1 month to 2 years post hospitalization. Of the nine patients who required secondary surgery following resolution of the initial complicated sinusitis, four patients were following initial surgical intervention and five patients had initially resolved their complication with medical therapy alone. Indications for subsequent surgery included failure of medical therapy for persistent rhinosinusitis (8 patients) and second complication (1 patient). CONCLUSIONS: This study suggests that following resolution of complicated pediatric rhinosinusitis, very few patients may need further surgical intervention. Subsequent intervention is best guided by clinical judgment, symptoms during outpatient clinic visits, and failure of medical therapy.

Heterotopic salivary tissue.

Salivary tissue can be present in the head and neck outside the usual locations of the major and minor salivary glands. This can be in the form of accessory salivary glands, in association with branchial cleft anomalies, or, less commonly, as heterotopic salivary gland tissue (HSGT). Heterotopic salivary gland tissue is defined as salivary tissue outside of the expected locations of major, minor, and accessory salivary glands with absence of clinical or histologic features of branchial cleft anomalies. Here we present the case of a 13-year-old girl who presented with a draining sinus of the lower neck, which was excised and, on histologic analysis, was consistent with HSGT. We include photographs and histologic images. A review of the literature on heterotopic salivary tissue in the neck is then presented including discussion of the presentation, clinical features, important considerations, and recommendations for management.

Ofloxacin otic drops vs neomycin-polymyxin B otic drops as prophylaxis against early postoperative tympanostomy tube otorrhea.

OBJECTIVES: To evaluate the incidence of tympanostomy tube (TT) sequelae, tube otorrhea, and tube obstruction immediately postoperatively in patients receiving TT for otitis media and to compare patients receiving postoperative otic drops with controls. DESIGN: Blinded randomized control trial. SETTING: A tertiary pediatric otolaryngology practice. SUBJECTS: The study population comprised 306 patients undergoing TT placement. INTERVENTIONS: The 306 patients were enrolled into the following 3 groups: (1) those receiving no postoperative otic drop prophylaxis (control group), (2) those receiving ofloxacin otic drops (FLOX group), and (3) those receiving neomycin sulfate-polymyxin B sulfate-hydrocortisone otic drops (COS group). RESULTS: Overall otorrhea rates postoperatively were 14.9% for the control group, 8.1% for the FLOX group, and 5.5% for the COS group. When controlling for disease severity, the rate of otorrhea was significantly higher for the control group than for both the FLOX (P = .04) and COS (P = .01) groups. Nonpatent, plugged, tube rates were added to otorrhea rates for a TT failure analysis postoperatively. The control group demonstrated a significantly greater failure rate (29.9%) than both the FLOX (12.1%) and COS (7.7%) groups. The only differences between the patients in the 2 groups receiving drops were that ofloxacin was more well liked by patients (P = .04) and caused less pain (P = .004). CONCLUSIONS: Nonpatency and otorrhea are the most frequent sequelae immediately following TT placement. Few studies have compared different treatment regimens in a randomized controlled trial. These results demonstrate that otic drops clearly provide benefit postoperatively in preventing TT plugging and otorrhea but primarily in patients who have middle ear fluid at the time of TT placement. In addition, consideration of drop choice should be based on patient tolerance and medication safety profiles.

Bilateral epistaxis in children: efficacy of bilateral septal cauterization with silver nitrate.

OBJECTIVE: To evaluate the rate of control of epistaxis in patients with bilateral complaints using silver nitrate and to assess short-term complications from this therapy. STUDY DESIGN AND SETTING: Retrospective chart review with prospective incident case identification in a tertiary referral pediatric otolaryngology practice. SUBJECTS AND INTERVENTIONS: Forty-six consecutive patients presenting to a single pediatric otolaryngologist with complaints of bilateral epistaxis received bilateral silver nitrate cauterization to the anterior septum and were seen in follow-up 2 months later to assess for control of epistaxis and complications from this therapy. RESULTS: Ninety-three percent (43/46) of patients followed-up at 2 months after therapy. About 93% (40/43) had total or near-total resolution of their epistaxis. Five percent (2/43) of patients returned to the clinic after initial successful control for a second cauterization during the period of study. No significant complications of infection, perforation, long-term crusting, tattooing or mucocutaneous or allergic reactions were identified. CONCLUSIONS: Bilateral epistaxis can be treated at a single visit with bilateral silver nitrate therapy in children. This treatment is highly effective, well-tolerated, and appears to have a low rate of complications.

Perioperative management of sickle cell disease children undergoing adenotonsillectomy.

OBJECTIVE: To determine if outpatient tailored perioperative management has the same complication rate as that previously published for sickle cell disease (SCD) children who undergo adenotonsillectomy. STUDY DESIGN AND SETTING: Retrospective chart review of SCD children who underwent tonsillectomy with outcome measures of perioperative management and postsurgical complications. RESULTS: Of 41 patients, 61% were monitored for <24 hours with the average length of stay being 2 days (range, 1 to 10). Postoperative complications were noted in 9 (22%) patients, including 8 (20%) with hypoxia, 4 (10%) fever, 3 (8%) acute chest syndrome, and 1 (2.4%) airway fire, which are similar to the published literature. CONCLUSIONS: Current literature supports extensive perioperative management with hospital stays averaging 3 to 5 days. These data demonstrate that the majority of these patients can be managed with <24-hour hospitalization stays with similar complication rates as previously described in the literature. SIGNIFICANCE: This study establishes a new paradigm for perioperative management of SCD children who undergo adenotonsillectomy. EBM RATING: C-4.

Fungal causes of otitis externa and tympanostomy tube otorrhea.

OBJECTIVE: To describe the occurrence of fungal organisms in the setting of otitis externa and tympanostomy tube otorrhea, review the treatment course, timing of diagnosis, organism identified and time to resolution with fungal infections. DESIGN: Retrospective review. SETTING: Pediatric otolaryngology clinic within a tertiary care hospital. PATIENTS: One hundred and sixty-six patients (ages 16 days to 18 years) with fungal organisms on ear culture. OUTCOME MEASURES: Number of prior therapies, number of office visits, time to resolution and anti-fungal therapy. RESULTS: Ear cultures positive for fungal organisms were found in 166 patients seen between 1 January 1996 and 30 September 2003 from a total of 1242 patients undergoing ear culture. Comparing the 3-year period (1996-1998) prior to the availability of fluoroquinolone ototopical drops to the 3-year period after (1999-2001), there is a statistically significant increase in the incidence of positive fungal culture (p<0.001). Otitis media was diagnosed in 72% of these children, with otitis externa comprising 25%. Approximately 3% carried a diagnosis of both otitis externa and otitis media. Candida albicans was identified in 43% of fungal organism-positive cultures. Candida parapsilosis was found in 24% of and Aspergillus fumigatus in 13%. The remainder of the cultures yielded three other Candida and three other Aspergillus species, each at less than 5%. Time to resolution ranged from 1 week to 9 months, with a median of 3.8 weeks for symptom resolution. Patients were treated with an average of 1.7 oral antibiotics and 1.1 ototopical agent before a culture was taken. CONCLUSIONS: Otorrhea due to fungal organisms occurs in the setting of refractory infection and is often discovered after multiple oral and ototopical antibacterial medications. Due to the extended treatment period required to clear fungal organism, timely diagnosis with culture for bacteria and fungus is required in patients with persistent otorrhea. An increase in incidence of fungal infections of the ear was found in the period after widespread use of ofloxacin began.

The effect of polysomnography on pediatric adenotonsillectomy postoperative management.

OBJECTIVE: We retrospectively investigated the effect and predictability of preoperative polysomnography (PSG) on the postoperative course of younger pediatric patients undergoing adenotonsillectomy. STUDY DESIGN AND SETTING: A retrospective chart review was performed for patients 3 years of age and younger who had undergone adenotonsillectomy between July 1997 and July 2002 at the Children's Hospital of Wisconsin. RESULTS: Two hundred eighty-two patients were identified. Forty-three patients had preoperative PSG. No correlation between the severity of PSG results and postoperative course was identified. CONCLUSIONS: The role of PSG in upper airway obstruction and OSA remains controversial. This study suggests that although the complication rate may be higher in this younger population, these complications do not appear to have a large impact on their length of stay. SIGNIFICANCE: This study suggests that the 3-years-and-younger group, in the absence of other comorbidities, can safely undergo adenotonsillectomy without undergoing preoperative PSG. EBM raing: C.

Long-term results of Armstrong beveled grommet tympanostomy tubes in children.

OBJECTIVE: Many different tympanostomy tubes have been developed with different sizes, shapes, compositions, and coatings. Despite the frequency of ventilation tube placement, very few large studies have examined the outcomes of patients receiving this procedure. An ideal tube would be easy to insert and would extrude at a predictable interval without complications. This study was performed to assess outcome measures and complication rates of the Armstrong beveled grommet tube. DESIGN: A retrospective case series of patients who had Armstrong beveled grommet tympanostomy tubes placed over a 3 year period by two Children's Hospital of Wisconsin pediatric Otolaryngology staff. MAIN OUTCOME MEASURES: Patient age, diagnosis, operative findings, and time to tube extrusion were reviewed. Otorrhea, perforation, and cholesteatoma rates were also assessed. RESULTS: Five hundred seven consecutive patients who had Armstrong tubes placed were reviewed. One thousand ninety-six Armstrong tubes were placed in these patients. Follow-up to extrusion rates were available for 756 tubes. The mean patient age at tube placement was 33.3 months, and the median age was 23 months. Mean and median times to extrusion were 16.5 and 15.5 months. One hundred sixty episodes of otorrhea were noted in 148 patients. Four patients had histories of cholesteatoma, none of which developed in conjunction with Armstrong tubes. Ten (1.32%) perforations that have not resolved over time were noted after Armstrong tube placement. CONCLUSIONS: Armstrong beveled grommet tympanostomy tubes have complication rates comparable with those reported for Armstrong or other short-acting tubes in smaller series.

Connexin 26 and connexin 30 mutations in children with nonsyndromic hearing loss.

OBJECTIVES/HYPOTHESIS: Mutations in the connexin 26 (Cx26) or gap junction beta 2 gene are the leading cause of hereditary nonsyndromic sensorineural hearing loss in Caucasians. The Cx26 coding region of 68 children with nonsyndromic sensorineural hearing loss was sequenced to determine the frequency and type of Cx26 mutations in this population. Screening was also performed for a common connexin 30 (Cx30) or gap junction beta 6 mutation (del [GJB6-D13S1830]). Children also underwent audiological testing to determine whether any correlation exists between Cx26 mutations and severity of hearing loss. STUDY DESIGN: In all, 68 children with nonsyndromic sensorineural hearing loss were screened for Cx26 and Cx30 mutations by polymerase chain reaction and direct sequencing. METHODS: Genomic DNA was amplified by polymerase chain reaction using primers that flank the entire Cx26 coding region. Screening for the 342-kb Cx30 deletion was performed using primers that amplified the breakpoint junction of the deletion. The amplicons were then sequenced in both directions and analyzed for mutations. Audiometric testing, including pure-tone audiometry and auditory evoked brainstem response, was also performed to determine the degree of hearing loss. RESULTS: Twenty-seven of 68 children tested had mutations in Cx26 with 35delG being the most prevalent. Ten additional Cx26 mutations were detected including a novel compound heterozygote. Two children were heterozygous for the Cx30 del (GJB6-D13S1830) mutation. CONCLUSION: Cx26 and Cx30 mutations were present in 41.2% of children tested in the study population. Audiometric data supported previous studies demonstrating a greater degree of hearing loss in subjects who are homozygous for the 35delG mutation.