Amelia Drake - Academia.edu (original) (raw)

Papers by Amelia Drake

The Cleft Palate Craniofacial Journal

Objective Craniofacial microsomia (CFM) is a broad clinical term used to describe a congenital co... more Objective Craniofacial microsomia (CFM) is a broad clinical term used to describe a congenital condition most commonly involving the underdevelopment of the external ear, mandible, soft tissues, and facial nerve. Despite medical advances, understanding of the psychological health and healthcare experiences of individuals with CFM and their caregivers remains limited. This article describes a research program designed to address these knowledge gaps, and identify opportunities for psychosocial intervention and improved healthcare provision. Design The Craniofacial microsomia: Accelerating Research and Education (CARE) research program aims to: 1) Conduct up to 160 narrative interviews with individuals and caregivers to validate a conceptual framework; 2) Administer an online international survey of up to 800 individuals with CFM and caregivers to identify predictors of psychological distress; 3) Perform up to 60 semi-structured interviews with healthcare providers and advocacy leader...

International Journal of Otorhinolaryngology, 2019

Background: Goldenhar Syndrome, also called Oculoauricular Vertebral Dysplasia, includes abnormal... more Background: Goldenhar Syndrome, also called Oculoauricular Vertebral Dysplasia, includes abnormalities of the first and second branchial arches. Ear abnormalities are present in approximately 92% of those with Goldenhar Syndrome [1]. There is a wide spectrum for ear anomalies; they can range from preauricular tags and pits, microtia, external auditory canal (EAC) atresia, ossicular malformations, and facial nerve hypoplasia, which can lead to an array of clinical findings [5]. Hearing loss is an unfortunate consequence of these clinical findings, and often results in subsequent speech and language delays [4]. Despite the widely reported auricular and audiologic manifestations of this disease, a review of the literature revealed no detailed description or analysis of congenital canal cholesteatoma specifically in this patient population. Objective: This study aims to characterize congenital external auditory canal cholesteatoma as a feature of Goldenhar Syndrome. Method: A retrospective case series was conducted with patients seen at the UNC Craniofacial Center in Chapel Hill, NC. Inclusion criteria included patients with a diagnosis of Goldenhar Syndrome also found to have congenital cholesteatoma necessitating surgery. A total of three patients were identified who met inclusion criteria. Results: Three patients with Goldenhar Syndrome were identified who also had congenital external auditory canal cholesteatoma. All three patients underwent surgical management of their disease. Conclusion: Congenital external auditory canal cholesteatoma appears to be a feature of Goldenhar Syndrome not widely described in current literature. Though rare, it is a relevant disease process with significant clinical implications for both hearing as well as speech and language development. Awareness of this condition can thus help guide practitioners in the care of these patients.

Women's Health Reports, 2021

Background: Insufficient support for balancing career and family responsibilities hinders retenti... more Background: Insufficient support for balancing career and family responsibilities hinders retention of physicianscientists. Programs to improve retention of this important group of faculty are crucial. Understanding the experiences of program implementers is key to refining and improving program offerings. Methods: We conducted an interpretive, descriptive, and qualitative study as part of an ongoing evaluation of the Doris Duke Charitable Foundation's Fund to Retain Clinical Scientists (FRCS) awards. We conducted telephone interviews with 12 program directors representing all 10 US medical schools who received the Doris Duke funding in 2016. Results: Of the 12 participants, 10 were women (83.3%). Participating program directors perceived the FRCS award as capable of producing paradigmatic changes regarding how responsibilities at home and work in academic medicine are viewed and integrated by early-career faculty members. The main qualitative themes that captured directors' experiences implementing the program were as follows: (1) championing a new paradigm of support, (2) lessons learned while implementing the new paradigm, (3) results of the new paradigm, and (4) sustaining the paradigm. Conclusions: These findings may help to inform development of similar programs to retain and support the career progress of physician-scientists with extraprofessional caregiving responsibilities. The interviews illuminate ways in which the Doris Duke FRCS award has driven institutional culture change by normalizing discussion and prompted reassessment of extraprofessional challenges and how best to aid early-career faculty members in overcoming these challenges.

Academic Medicine, 2021

As the nation seeks to recruit and retain physician-scientists, gaps remain in understanding and ... more As the nation seeks to recruit and retain physician-scientists, gaps remain in understanding and addressing mitigatable challenges to the success of faculty from underrepresented minority (URM) backgrounds. The Doris Duke Charitable Foundation Fund to Retain Clinical Scientists program, implemented in 2015 at 10 academic medical centers in the United States, seeks to retain physician-scientists at risk of leaving science because of periods of extraordinary family caregiving needs, hardships that URM faculty—especially those who identify as female—are more likely to experience. At the annual Fund to Retain Clinical Scientists program directors conference in 2018, program directors—21% of whom identify as URM individuals and 13% as male—addressed issues that affect URM physician-scientists in particular. Key issues that threaten the retention of URM physician-scientists were identified through focused literature reviews; institutional environmental scans; and structured small- and lar...

Academic Medicine, 2019

Purpose To enhance understanding of challenges related to work–life integration in academic medic... more Purpose To enhance understanding of challenges related to work–life integration in academic medicine and to inform the ongoing implementation of an existing program and the development of other interventions to promote success of physician–scientists. Method This study is part of a prospective analysis of the effects of the Fund to Retain Clinical Scientists (FRCS), a national program launched by the Doris Duke Charitable Foundation at 10 U.S. institutions, which provides financial support to physician–scientists facing caregiving challenges. In early 2018, 28 of 33 program awardees participated in semistructured interviews. Questions were about challenges faced by physician–scientists as caregivers and their early perceptions of the FRCS. Multiple analysts reviewed deidentified transcripts, iteratively revised the coding scheme, and interpreted the data using qualitative thematic analysis. Results Participants’ rich descriptions illuminated 5 interconnected themes: (1) Time is a cr...

Otolaryngologic Clinics of North America, 2019

Craniofacial interventions are common and the surgical options continue to grow. The issues encou... more Craniofacial interventions are common and the surgical options continue to grow. The issues encountered include micrognathia, macroglossia, midface hypoplasia, hearing loss, facial nerve palsy, hemifacial microsomia, and microtia. In addition, a unifying theme is complex upper airway obstruction. Throughout a child's life the focus of interventions may change from airway management to speech, hearing, and language optimization, and finally to decannulation and procedures aimed at social integration and self-esteem. Otolaryngologists play an important role is this arena and provide high-quality care while continuing to expand what can be done for our patients.

Current Opinion in Otolaryngology & Head and Neck Surgery, 1999

ABSTRACT Despite enormous advances in the field of pediatric airway, stridor continues to be the ... more ABSTRACT Despite enormous advances in the field of pediatric airway, stridor continues to be the hallmark sign of airway compromise. Recognition of the signs and symptoms of airway obstruction promotes early diagnosis. The diagnostic evaluation of stridor has progressed with newer techniques being studied. When appropriate, these are added to the time-honored techniques of direct laryngoscopy and bronchoscopy. For example, acoustic assessment of snoring intensity has been proposed as a study parameter during polysomnography. Acoustic rhinometry continues to evolve from the role, primarily, of research tool to a role of increasing clinical application. Even traditional techniques such as plain radiographs and fiberoptic laryngoscopy are being used to better quantify the degree of airway compromise.

International journal of pediatric otorhinolaryngology, 2015

To examine the characteristics of sleep in patients with Goldenhar Syndrome. Retrospective review... more To examine the characteristics of sleep in patients with Goldenhar Syndrome. Retrospective review of all polysomnography studies conducted at the University of North Carolina Hospitals between 2003 and 2013 on patients carrying the diagnosis of Goldenhar's Syndrome. A preponderance of patients demonstrated severe obstructive sleep apnea and hypercapnia. Patients with Goldenhar Syndrome should be screened for sleep apnea and hypercapnia.

The Laryngoscope, 1988

A case of alveolar soft part sarcoma of the nasal cavity is presented. Alveolar soft part sarcoma... more A case of alveolar soft part sarcoma of the nasal cavity is presented. Alveolar soft part sarcoma is a rare malignant neoplasm, which often affects females (ratio 2 to 1) in their second decade. It occurs most frequently in the extremities, with an unusual predilection to involve the right side of the body. Excluding the orbit, only a few cases have been reported in the head and neck area. We present the first reported case of alveolar soft part sarcoma limited to the nasal chamber.

Journal of Surgical Research, 2012

Archives of Otolaryngology–Head & Neck Surgery, 2003

Objective: To determine the effect of otitis media with effusion (OME) on perceptual masking (a p... more Objective: To determine the effect of otitis media with effusion (OME) on perceptual masking (a phenomenon in which spondee threshold for a 2-talker masker is poorer than for a speech-shaped noise masker). Design: Longitudinal testing over a 1-year period following insertion of tympanostomy tubes, using clinical and normal-hearing control groups. Subjects: Forty-seven children having a history of OME were tested. Possible testing intervals were just before the placement of tympanostomy tubes, and up to 3 separate occasions after the placement of the tubes. An agematched control group of 19 children was tested. Methods: A perceptual masking paradigm was used to measure the ability of the listener to recognize a spondee in either a speech-shaped noise or a 2-talker masker background. The masker was either continuous or gated on and off with the target spondee. Results: In gated masking conditions, children with a history of normal hearing showed only slight perceptual masking, but the children with a history of OME showed relatively great perceptual masking before surgery and up to 6 months following surgery. In continuous masking conditions, both groups of children showed relatively great perceptual masking and did not differ significantly from each other in this respect either before or after surgery. However, before surgery, the OME group showed higher thresholds in both the 2-talker and speechshaped noise maskers. Conclusions: In agreement with previous psychoacoustical findings, the relatively great perceptual masking in gated conditions shown by children with OME history may reflect a general deficit in complex auditory processing.

American Journal of Otolaryngology, 1987

Thyroglossal duct remnants are the most common congenital cystic lesions of the neck; however, a ... more Thyroglossal duct remnants are the most common congenital cystic lesions of the neck; however, a carcinoma arising in these structures is rare. Two new cases of a papillary adenocarcinoma arising in a thyroglossal duct cyst are presented. Preoperative evaluation, operative management, and postoperative care are discussed. Initial evaluation consisting of a thorough head and neck examination, palpation of the thy(oid gland, thyroid funotion tests, and selective use of thyroid imaging is recommended. Removal of the cyst and tract in the manner described by Sistrunk is advocated. If an adenocarcinoma is found in the cyst and if a carcinoma is found in the thyroid gland or a thyroid scan reveals a nodule, a total thyroidectomy is recommended. A modified neck dissection and total thyroidectomy is advocated for cervical metastases. Postoperatively, thyroid suppression and long-term follow-up are encouraged. A squamous cell carcinoma arising in a thyroglossal duct remnant appears more aggressive and requires complete excision and, for confirmed cervical metastases, radical neck dissection and postoperative radiation therapy.

Journal of Women's Health, 2020

Background: Research is needed to improve understanding of work-life integration issues in academ... more Background: Research is needed to improve understanding of work-life integration issues in academic medicine and to guide the implementation of the Doris Duke Charitable Foundation's Fund to Retain Clinical Scientists (FRCS), a national initiative offering financial support to physician-scientists facing caregiving challenges. Materials and Methods: In 2018, as part of a prospective program evaluation, the authors conducted a qualitative study to examine FRCS program participants' initial impressions, solicit descriptions of their career and caregiving experiences, and inquire how such factors might influence their professional advancement. The authors invited all 33 awardees who had been granted FRCS funding in the first year of the program to participate in the study, of whom 28 agreed to complete an interview. Analysts evaluated de-identified transcripts and explicated the data using a thematic analysis approach. Results: While participants described aspects of a culture that harbor stigma against caregivers and impede satisfactory work-life integration, they also perceived an optimistic cultural shift taking place as a result of programs like the FRCS. Their comments indicated that the FRCS has the potential to influence culture if institutional leadership simultaneously fosters a community that validates individuals both as caregivers and as scientists. Conclusions: Insights garnered from this qualitative study suggest that there is a pressing need for institutional leaders to implement programs that can foster awareness and normalization of caregiving challenges. In addition to providing funding and other tangible resources, interventions should strive to reinforce a broader culture that affirms the presence of work-life integration challenges and openly embraces solutions.

Supplemental Material, _Appendix_(supplemental_tables) for Provision and Utilization of Team- and... more Supplemental Material, _Appendix_(supplemental_tables) for Provision and Utilization of Team- and Community-Based Operative Care for Patients With Cleft Lip/Palate in North Carolina by Elliot Le, Peter Shrader, Hayden Bosworth, Jillian Hurst, Benjamin Goldstein, Amelia Drake, Jeyhan Wood, Lisa R. David, Christopher M. Runyan, Joao Ricardo Nickenig Vissoci, Matthew Harker and Alexander C. Allori in The Cleft Palate-Craniofacial Journal

Journal of Craniofacial Surgery, 2021

Children with craniofacial microsomia (CFM) are at increased risk for educational and social conc... more Children with craniofacial microsomia (CFM) are at increased risk for educational and social concerns. This study describes intervention services and frequency of teasing in a multinational population of children with CFM. Caregivers of children with CFM ages 3 to 18 years in the US and South America were administered a questionnaire. Additional information was gathered from medical charts and photographs. Participants (N = 169) had an average age of 10.1 ± 6.2 years, were primarily male (60%), and from the US (46%) or Colombia (32%). Most participants had microtia and mandibular hypoplasia (70%). They often had unilateral (71%) or bilateral (19%) hearing loss and 53% used a hearing aid. In the US, special education services were provided for 48% of participants enrolled in school; however, similar services were rare (4%) in South America and reflect differences in education systems. Access to any intervention service was higher in the US (80%) than in South America (48%). Caregiver...

The Cleft Palate-Craniofacial Journal, 2020

Objective: To examine neurodevelopment in preschool-aged children with craniofacial microsomia (C... more Objective: To examine neurodevelopment in preschool-aged children with craniofacial microsomia (CFM) relative to unaffected peers. Design: Multisite, longitudinal cohort study. Setting: Tertiary care centers in the United States. Participants: We included 92 children with CFM (“cases”) through craniofacial centers and clinics. Seventy-six children without CFM (controls) were included from pediatric practices and community advertisements. This study reports on outcomes assessed when participants were an average age of 38.4 months (SD = 1.9). Main outcome measures: We assessed cognitive and motor skills using the Bayley Scales of Infant and Toddler Development, third edition (Bayley-III), and language function using subtests from the Clinical Evaluation of Language Fundamentals—Preschool, second edition (CELF-P2). Results: Case–control differences were negligible for Bayley-III cognitive (effect sizes [ES] = −0.06, P = .72) and motor outcomes (ES = −0.19, P = .25). Cases scored lower ...

Nature Communications, 2021

Craniofacial microsomia (CFM) is the second most common congenital facial anomaly, yet its geneti... more Craniofacial microsomia (CFM) is the second most common congenital facial anomaly, yet its genetic etiology remains unknown. We perform whole-exome or genome sequencing of 146 kindreds with sporadic (n = 138) or familial (n = 8) CFM, identifying a highly significant burden of loss of function variants in SF3B2 (P = 3.8 × 10−10), a component of the U2 small nuclear ribonucleoprotein complex, in probands. We describe twenty individuals from seven kindreds harboring de novo or transmitted haploinsufficient variants in SF3B2. Probands display mandibular hypoplasia, microtia, facial and preauricular tags, epibulbar dermoids, lateral oral clefts in addition to skeletal and cardiac abnormalities. Targeted morpholino knockdown of SF3B2 in Xenopus results in disruption of cranial neural crest precursor formation and subsequent craniofacial cartilage defects, supporting a link between spliceosome mutations and impaired neural crest development in congenital craniofacial disease. The results e...

Carolina Digital Repository (University of North Carolina at Chapel Hill), 2016

Objective-Children with cleft lip and palate (CLP) often suffer from nasal obstruction which may ... more Objective-Children with cleft lip and palate (CLP) often suffer from nasal obstruction which may be related to effects on nasal volume. The objective of this study is to compare side:side volume ratios and nasal volume in patients with unilateral (UCLP) and bilateral (BCLP) clefts with age-matched controls. Study Design-Retrospective case-control study using three-dimensional nasal airway reconstructions Methods-We analyzed 20 subjects (age range: 7-12 years) with UCLP and BCLP from a regional craniofacial center who underwent cone beam CT (CBCT) prior to alveolar grafting. Ten multi-slice CT images from age-matched controls were also analyzed. Mimics ™ software (Materialise, Inc.) was used to create 3-dimensional reconstructions of the main nasal cavity and compute total and side-specific nasal volumes. Subjects imaged during active nasal cycling phases were excluded. Results-There was no statistically significant difference in affected:unaffected side volume ratios in UCLP (p=0.48) or left:right ratios in BCLP (p=0.25) when compared to left:right ratios in

Laryngoscope, Aug 12, 2015