Otolaryngologic sequelae of Ehlers Danlos Syndrome in pediatric patients.

OBJECTIVE: As outlined by the NIH, Ehlers Danlos Syndrome (EDS) is a group of hereditary connective tissue disorders characterized by skin hyperelasticity, joint hypermobility, atrophic scarring, and blood vessel fragility, with no otolaryngological criteria for diagnosis. We aimed to compare otolaryngological disorders between children with EDS and those not affected by EDS. METHODS: A retrospective chart review was conducted using the US collaborative network within TriNetX. The EDS group was defined by ICD-10 code G47.33, while the non-EDS group excluded any patients with an EDS diagnosis. Cohorts were matched by age, sex, and race using propensity score matching. Pathologies analyzed included hearing loss (ICD-10H90, H91), otitis media (ICD-10H66, H65), allergic rhinitis, acute tonsillitis (ICD-10 J03), sinusitis (ICD-10 J32, J01), and obstructive sleep apnea (OSA) (ICD-10 G47.33). Chi-square and relative risk within a 95 % confidence interval were calculated. RESULTS: Propensity score matching yielded 6440 patients (male: N = 2,523, 39.2 %; female: N = 3,893, 60.5 %; unknown: N = 24, 0.37 %) with a mean age of 9.28 years (SD = 4.38). Children with EDS were 2.04 times more likely to be diagnosed with hearing loss, occurring in 286 (4.4 %) EDS children versus 140 (2.1 %) controls (P < 0.001). Children with EDS were 1.6 times more likely to be diagnosed with allergic rhinitis, occurring in 436 (6.8 %) EDS children versus 274 (4.2 %) controls (P < 0.001). Children with EDS were also 1.52 times (EDS: N = 350, 5.4 %; control: N = 231, 3.6 %) and 4.24 times (EDS: N = 335, 5.2 %; control: N = 79, 1.2 %) more likely to develop sinusitis and be diagnosed with OSA, respectively, compared to children without EDS (P < 0.001). However, children with EDS were only 0.71 times as likely to develop acute tonsillitis, with 101 (1.6 %) of EDS children compared to 142 (2.2 %) of control children being diagnosed (P = 0.009). No statistical difference was found in risk of developing otitis media. CONCLUSIONS: Children with EDS are at higher risk of developing hearing loss, allergic rhinitis, acute sinusitis, and OSA, possibly due to underlying immune dysfunction. Pediatric otolaryngologists should be vigilant about these otolaryngologic sequela in EDS patients.

Prognosis of Bilateral Sudden Sensorineural Hearing Loss: A Systematic Review and Meta-Analysis.

OBJECTIVES: To comprehensively examine the characteristics and prognosis of bilateral sudden sensorineural hearing loss (BSSHL) and its subtypes compared to unilateral sudden sensorineural hearing loss (USSHL). DATA SOURCES: PubMed, Scopus, and CINAHL. REVIEW METHODS: Databases were searched from inception to December 5, 2023, for studies reporting patient characteristics and audiometric outcomes for BSSHL and its simultaneous (Si-BSSHL) and sequential (Se-BSSHL) subtypes. Meta-analysis of continuous measures, proportions (%), mean differences (Δ), and odds ratio (OR) were performed. RESULTS: Eleven studies were included, consisting of 368 patients with BSSHL and 2,705 patients with USSHL. The pooled prevalence among all SSHL cases was 88.1% (95% CI: 81.2%-93.6%) for USSHL and 11.9% (95% CI: 6.4% to 18.8%) for BSSHL. PTA improvement following treatment with steroids was significantly worse in patients with BSSHL (Δ15.3 dB; 95% CI: 14.6 to 15.9; p < 0.0001) compared to patients with USSHL. There was no significant difference in post-treatment PTA improvement between the BSSHL subtypes. Patients with Si-BSSHL were significantly less likely to have an idiopathic etiology (OR: 0.4; 95% CI: 0.2 to 0.8; p = 0.01) and significantly more likely to have an autoimmune disease etiology (OR: 27.4; 95% CI: 2.2 to 336.1; p = 0.01), comorbid cardiovascular disease (OR: 2.3; 95% CI: 1.1 to 5.1; p = 0.03), and comorbid hypertension (OR: 2.5; 95% CI: 1.6 to 3.8; p < 0.0001) compared to patients with USSHL. CONCLUSIONS: BSSHL is a considerably rarer form of SSHL with worse prognosis compared to USSHL. BSSHL, and Si-BSSHL in particular, has significantly greater associations with systemic pathologies compared to USSHL. Laryngoscope, 2024.

The Importance of Research Experience With a Scoreless Step 1: A Student Survey at a Community-Based Medical School.

PURPOSE: As of January 26, 2022, the United States Medical Licensing Examination (USLME) step 1 exam went from a scored test to pass-fail step 1 (PFS1). The authors were interested in surveying medical students at a community-based medical school to observe their perceptions of the importance of student research given this recent change. METHOD: A Qualtrics survey was disseminated to medical students (years 1-4) via school emails. Data were analyzed using the Mann-Whitney test to assess Likert scale scores, and narrative comments were grouped as qualitative feedback. Survey dissemination and analysis of data were both conducted at a large community-based medical school. RESULTS: The survey sampled 104 students categorized into pre-clerkship (PC) and clerkship (CL) years, with a response rate of 33%. A contradiction was found, as indicated by the higher number (p = 0.047) of clerkship students interested in Primary Care/Family medicine residency compared to pre-clerkship students at 41% and 59%, respectively. Whereas participants who indicated they are interested in pursuing a competitive specialty for residency were 51% of pre-clerkship students over 41% of clerkship students (p = 0.047). Additionally, given the assessment change to pass/fail, students did in fact believe that residencies would now view research as a higher assessed component than before (79% pre-clerkship and 72% clerkship). However, a minority of students said that they increased their research efforts (41% and 47%). Most students supported the research opportunity improvements proposed in our survey. CONCLUSIONS: Efforts to make the step 1 exam pass/fail may have alleviated some stress related to performance but may have increased the perception of the importance of other components in a student's residency application. Our survey highlights how medical students at a community-based medical school perceive this change and how it has affected their research efforts.

Palatal Supernumerary Tooth in a Pediatric Patient.

This report describes the case of a child with a congenital palatal lesion that grew rapidly in the first year of life and was found to be a supernumerary tooth. A 14-month-old male presented with a congenital midline palatal lesion visible behind his newly erupted maxillary central incisors. The lesion had been present since birth and was round, raised, firm, and covered with normal-appearing mucosa. The results from CT imaging indicated the lesion was a rudimentary tooth crown. It was excised and confirmed to be a supernumerary tooth. The patient healed without complications. Congenital palatal lesions with this appearance are most commonly hamartomas, cysts, epulides, and teratomas. Congenital midline palatal lesions are uncommon, and supernumerary teeth are not typically in the differential diagnosis. Imaging is helpful for the management of congenital palatal lesions.

South Carolina's Medicaid Expansion: An Unsolved Problem for Vulnerable Populations.

South Carolina is home to millions of residents and is renowned for its subtropical climate and beautiful beaches. Nevertheless, the state's healthcare system faces significant challenges, with a ranking in the bottom 50% of all states, a statistic that warrants serious attention. One of the most pressing healthcare issues in South Carolina is accessibility. The state currently has a higher percentage of its population that is medically uninsured compared to the national average in the United States (18% vs 14%, respectively). Consequently, a lower proportion of South Carolinians enjoy access to healthcare services when compared to residents of other states. Unfortunately, recent efforts to expand coverage for more of its residents via the Medicaid expansion by the Affordable Care Act (ACA) have been opposed by legislation. Lack of accessible healthcare is a significant issue in this state and the state legislature should increase access, especially to low-income vulnerable populations.