Faculty Bibliography

Congenital cholesteatomas are defined as a collection of epithelium in the absence of prior surgery or pathologic retraction. They are most commonly found in the middle ear and are thought to arise from a residual epithelial rest present since birth; however, a small number of cases present with disease isolated to the mastoid bone. One such case and a review of prior reports are presented. A 29-year-old male with a 2-year history of headaches and no otologic surgery was found to have a destructive mass centered in the right mastoid bone, invading the jugular foramen and posterior fossa without middle ear disease. He underwent a mastoidectomy with complete resection of the cholesteatoma. A literature review identified 21 prior cases of isolated mastoid congenital cholesteatoma. Congenital cholesteatoma should be considered in the differential diagnosis when patients present with postauricular pain or headaches. Mastoidectomy is considered the treatment of choice.

OBJECTIVE:Post-tonsillectomy hemorrhage is a highly studied outcome of tonsillectomy with serious consequences. The off-label use of tranexamic acid (TXA) is of growing interest to control post-tonsillectomy hemorrhage but has not been incorporated in management guidelines. STUDY DESIGN/METHODS:Scoping review on the use of tranexamic acid for post-tonsillectomy hemorrhage. SETTING/METHODS:N/A. METHODS:A comprehensive literature search was performed across the following research databases: PubMed, Embase, CINAHL, and Web of Science. The search was limited to English-language studies and patients without prior diagnosis of bleeding disorders. The articles were screened for relevance based on inclusion and exclusion criteria. Our initial search generated 131 articles. RESULTS:A total of 24 articles were identified, published in mostly otolaryngology journals. Over 96 000 tonsillectomy cases were included. There was variability in administration routes: intravenous, nebulized, oral, and topical. Intravenous was most used, particularly as prophylaxis for post-tonsillectomy hemorrhage, and nebulized administration was more common in therapeutic settings. Dosing regimens ranged between 5 and 15 mg/kg. We found mixed results across studies regarding peri-operative and post-operative bleeding outcomes, though multiple studies demonstrated decreased intraoperative bleeding. Many studies concurred that TXA was safe to use for post-tonsillectomy hemorrhage. CONCLUSION/CONCLUSIONS:The existing literature indicates TXA shows promising results in safety and reducing intraoperative blood volume loss. Further prospective and randomized controlled trials are needed to ensure the clinical benefits of TXA in tonsillectomy surgery prior to the inclusion in clinical practice guidelines.

INTRODUCTION/BACKGROUND:This study investigates factors that relate to long-term educational and occupational outcomes of adults who received cochlear implants (CIs) during childhood. METHODS:A retrospective chart review was conducted on 109 adults who received a CI before the age of 15 between 2000 and 2012 at a US tertiary medical center. Demographic variables, speech perception scores, and educational and vocational achievements were analyzed. Current US Census and Bureau of Labor Statistics data were used for comparison. RESULTS:The median age at implantation was 2.81 years, and the median age at data collection was 27.30 years. Most subjects were unilaterally implanted (63.3%) and used an oral communication approach (89.0%). Educational outcomes showed that 17% completed a high school diploma or less, and 9% completed an associates or technical degree. Seventy-two percent of the subjects achieved a bachelor's degree or higher, significantly higher than the general US population (37.9%). Occupational outcomes indicated that subjects were employed across various job categories, with a higher proportion in jobs requiring considerable preparation (job zone 4) compared to the general population. There was a significant negative correlation between age at implantation and speech perception scores. Better word recognition scores were also associated with better educational and occupational outcomes. CONCLUSIONS:Adults who received CIs as children demonstrate excellent educational and occupational outcomes, surpassing those of the general US population. Early implantation and the absence of additional disabilities positively influence these outcomes. Continued investigation of nonspeech outcomes and the factors that influence them is essential to provide better support services for future cohorts.

BACKGROUND:To address financial barriers that limit access to genomic profiling and precision medicine, philanthropy supported clinical genomic testing was offered worldwide at no cost to patients with select rare cancers via the Make-an-IMPACT program. Herein, we report our findings in pediatric patients with solid or central nervous system (CNS) tumors. METHODS:Tumor DNA or CSF-derived circulating tumor DNA (CSF ctDNA) was analyzed using the MSK-IMPACT assay, supplemented by targeted RNA panel sequencing in select cases. Results were returned to the patients/families and treating oncologists. RESULTS:63 patients from 11 countries had successful MSK-IMPACT testing. The results provided clinically relevant new diagnostic or prognostic information in 41% and 38% of solid and CNS tumor patients, respectively. Potentially therapeutically actionable alterations were identified in 44% of pediatric solid tumor and 21% of pediatric CSF ctDNA samples, respectively. Four patients subsequently received molecularly guided therapy, resulting in partial responses in two and prolonged stable disease in one. Serial tumor and CSF sampling identified resistance mutations in two patients, informing additional molecular targeted therapy recommendations. CONCLUSIONS:The Make-an-IMPACT program provided global access to state-of-the-art tumor and CSF genomic profiling across a diverse cohort of pediatric cancer patients, providing clinically relevant and actionable diagnostic, prognostic and therapeutic information reported in real time to patients and local physicians.

OBJECTIVES/HYPOTHESIS/OBJECTIVE:To identify vocal tract configuration patterns in vocally healthy contemporary commercial music (CCM) singers during the production of five industry-typical vocal qualities, including various belting qualities and traditional/legit musical theater singing. STUDY DESIGN/METHODS:Prospective, observational study. METHODS:Seven professional musical theater singers (four females, three males) performed arpeggiated patterns using five different vocal qualities: traditional/legit, neutral belt, brassy belt, warm belt, and rock belt. Real-time magnetic resonance imaging captured midsagittal vocal tract configurations. Eight morphological measures were analyzed: lip opening, jaw opening, jaw protrusion, tongue dorsum height, uvula elevation, oropharyngeal opening, laryngeal height, and laryngeal tilt. Linear mixed-effects modeling explored relationships between vocal qualities and anatomical measurements. RESULTS:Lip opening, jaw opening, oropharyngeal opening, and laryngeal height showed significant differences across vocal qualities. The traditional/legit voice quality demonstrated the smallest lip, jaw, and oropharyngeal openings with the lowest laryngeal position. Rock belt showed the largest lip and jaw openings. Brassy belt and rock belt featured larger oropharyngeal space and higher tongue positions than other qualities, creating a "tube-like" shape rather than the previously suggested megaphone shape. Laryngeal positioning was elevated in all belting styles, but not in traditional/legit. No significant differences were found between male and female participants. Lower pitch tasks were characterized by smaller lip, jaw, and oropharyngeal openings compared with higher pitch tasks. CONCLUSION/CONCLUSIONS:This pilot study revealed distinct vocal tract configurations for different vocal qualities, particularly between traditional/legit and belting styles. The findings challenge previous assumptions about megaphone-shaped vocal tracts in belting, demonstrating more complex configurations. These results provide a foundation for identifying typical versus atypical vocal tract adjustments in CCM singing, with implications for voice pedagogy and clinical practice.

OBJECTIVE:Music, like all forms of art, seeks to communicate emotional content to its audience. The signal provided by cochlear implants (CI) does not faithfully represent the psychophysical relationships inherent in music; however, it is unknown whether targeted musical emotions are conveyed through electric only stimulation with a cochlear implant. METHODS:Twenty musical excerpts for which there was concordance among normal hearing (NH) listeners as to the emotion conveyed: (1) happy, (2) sad, (3) scary, or (4) peaceful were presented to cochlear implant (CI) subjects (n = 20) and age-matched NH controls (n = 8) through a specifically designed iPad application. The musical clips were original recordings of western music from various musical genres but not widely familiar to participants. Subjects also completed a music background questionnaire. RESULTS:CI users identified the target emotion in only 57% of excerpts compared with NH listeners who correctly identified the target emotion in 76.9% of excerpts. Musical excerpts with the target emotion of happy were recognized in 84% of cases by CI users compared with 75% among NH controls while the remaining target emotions were much more difficult for CI users compared with NH raters. Length of CI experience, speech perception scores, and musical training or listening habits did not correlate with performance on this task. CONCLUSIONS:Current CI technology and processing strategies do not convey the range of emotions conveyed in music as recognized by normal hearing subjects. This difficulty may explain the reported lack of interest many patients have in music after CI despite a passion for music before onset of hearing loss. LEVEL OF EVIDENCE/METHODS:3 Laryngoscope, 135:2112-2119, 2025.

Gliomas are a major cause of cancer-related deaths in adolescents and young adults (AYAs; ages 15-39 years). Different molecular alterations drive gliomas in children and adults, leading to distinct biology and clinical consequences, but the implications of pediatric- versus adult-type alterations in AYAs are unknown. Our population-based analysis of 1,456 clinically and molecularly characterized gliomas in patients aged 0-39 years addresses this gap. Pediatric-type alterations were found in 31% of AYA gliomas and conferred superior outcomes compared to adult-type alterations. AYA low-grade gliomas with specific RAS-MAPK alterations exhibited senescence, tended to arise in different locations and were associated with superior outcomes compared to gliomas in children, suggesting different cellular origins. Hemispheric IDH-mutant, BRAF p.V600E and FGFR-altered gliomas were associated with the risk of malignant transformation, having worse outcomes with increased age. These insights into gliomagenesis may provide a rationale for earlier intervention for certain tumors to disrupt the typical behavior, leading to improved outcomes.

Alston's singing mice (Scotinomys teguina) are highly vocal Central American rodents that produce structured "songs" (duration: 5-10 s),¹