I. Pediatric Otology/Audiology

Outcomes and Disparities in Childhood Hearing Loss

Investigator: Dylan Chan, MD

Collaborators: Abel David, MD, Kara Brodie, MD, Madeline Strohl, MD, Katie Gutierrez, MS2, Ana Marija Sola, MS3

Project Description: The majority of existing research in audiology and otolaryngology for children who are deaf or hard-of-hearing focus narrowly on quantitative audiologic outcomes, or sometimes formal speech and language testing, in controlled populations of children. The nature and impact of demographic disparities on these family- and patient-centered outcomes of interest is understudied, and is the focus of the clinical research program of the Children's Communication Center. Specific projects include:

1. Development of an Access Challenge Index to risk-stratify children who are deaf or hard-of-hearing

2. Evaluation of impact of different interventions for congenital aural atresia on functional disability and quality of life

3. Use of Language ENvironment Analysis technology in speech and language tracking in deaf and hard-of-hearing children

4. Development of a parent-child dual quality of life inventory for infants and toddlers who are deaf or hard-of-hearing

5. Early ophthalmology findings in children with hearing loss found to have genetic variants consistent with Usher syndrome

6. Outcomes of a community-based preschool hearing screening program

7. Disparities in the United State newborn hearing screening program

8. Valganciclovir for the treatment of congenital CMV-associated hearing loss.

9. Utility of functional disability surveys in determining management for otitis media

The Unfolded Protein Response in genetic and acquired hearing loss

Investigator: Dylan Chan, MD, PhD

Collaborator: Elliott Sherr, MD, PhD

Project Description: Our basic-science laboratory has implicated the Unfolded Protein Response (UPR), a ubiquitous pathway involved in coordinating the cell's response to endoplasmic reticulum stress, in hearing loss relating to noise exposure or cisplatin toxicity. We have found that modulating the UPR can protect against hearing loss in mouse models, and have identified a novel deafness mutant, TMTC4. We use a combination of live calcium imaging and in vivo auditory physiology, together with expression analysis and immunohistochemistry, to explore the pathophysiology of UPR-mediated hearing loss.

II. Airway/Sleep/Swallowing

Impact of Monthly Pediatric Multidisciplinary Airway Conference on Readiness for Interprofessional Learning

Investigator: Jacqueline Weinstein, MD

Collaborators: Lia Jacobson, MD, Abel David, MD, Kristina Rosbe, MD

Project Description: A monthly multidisciplinary pediatric airway conference was established at our multi-facility, tertiary care academic pediatric medical center with the goals of improving collaboration, expanding knowledge-sharing and developing safety initiatives in the comprehensive management of pediatric patients with complex airway considerations. The purpose of our study is to assess the impact of this conference on faculty and trainee's attitudes toward interprofessional learning using a validated measure (Readiness for Inter-professional Learning Scale).

Techniques/Methods: Prospective survey collection

Management Guidelines for Pediatric Obesity and OSA

Investigator: Garani Nadaraja, MD

III. Other topics in Pediatric Otolaryngology

Efficacy of FESS vs Combined FESS and Neurosurgical Intervention in the Treatment of Subdural Empyema

Investigator: Jacqueline Weinstein, MD

Collaborator: Yi Cai, MD, Andrea Embrechts RN, Jordan Virbalas, MD, Josephine Czechowicz, MD

Contact: [email protected]

Project Description: Subdural empyema is rare but serious complication of acute sinus infection. Treatment efforts focus on IV antibiotics with surgical source control, drainage of abscess. The purpose of our study is to determine whether functional endoscopic sinus surgery alone vs combined neurosurgical intervention with FESS (both with long-term IV antibiotics) resulted in superior outcomes with regard to length of stay, postoperative morbidity, need for additional surgery, etc.

Techniques/Methods: Retrospective chart review

Genetic Profiling in Pediatric Head and Neck Cancer

Investigator: Dylan Chan, MD, PhD

Collaborator: Madeleine Strohl, MD

Project Description: Neoplasms of the head and neck in children can be challenging to diagnose definitively. Use of genetic profiling of tumors can be valuable to narrow diagnosis, as well as to direct targeted treatment. In this study, we evaluate the utility of the UCSF500 panel to guide management of pediatric head and neck tumors.

Role of Balloon Sinuplasty in Pediatric Sinus Disease

Investigator: Garani Nadaraja, MD

Global Health in Pediatric Otolaryngology

Investigator: JoAnn Czechowicz, MD

Project Description: We are studying hearing and neurocognitive outcomes in children with HIV in Nairobi, Kenya; working with the UCSF Center for Global Surgical Studies to assess surgical need and capacity and develop national surgical plans in Uganda and Cameroon; and study capacity building and surgical education.

Clinical Outcomes in Children with Vascular Anomalies

Investigators: JoAnn Czechowicz, MD, and Kristina Rosbe, MD

Project Description: At part of the Birthmark and Vascular Anomalies Center (BVAC), we have a diverse set of studies related to children with vascular anomalies, including:

1. Outcomes of patients diagnosed prenatally with lymphatic malformation

2. Airway hemangioma incidence and outcomes in PHACE patients (multi-institutional study)

3. QOL assessment of pediatric patients with venous malformations of the head and neck including sleep and swallowing symptoms both prior to and after treatment

IV. Selected Publications from UCSF Pediatric Otolaryngology

Xu MJ, Stanford-Moore G, Czechowicz JA (2019). Association of Ebola Virus Infection with Hearing Loss in Regions Where Ebola Virus Infection Is Endemic: A Systematic Review. JAMA Otolaryngol Head Neck Surg 145(7):669-675. PMID:31095264

Cedars E, Kriss H, Lazar AA, Chan C, Chan DK (2018). Use of otoacoustic emissions to improve outcomes and reduce disparities in a community preschool hearing screening program. PLoS One 13(12):e0208050. PMID:30532159

Mamlouk MD, Zimmerman B, Mathes EF, Rosbe KW (2018). Hearing loss in PHACE syndrome: clinical and radiologic findings. Child Nerv Syst 34(9):1717-1724. PMID:29748705

Li J, Akil O, Rouse SL, McLaughlin CW, Matthews IR, Lustig LR, Chan DK, Sherr EH (2018). Deletion of Tmtc4 activates the unfolded protein response and causes postnatal hearing loss. J Clin Invest 128(11):5150-5162. PMID:30188326

Czechowicz JA, Long-Boyle JR, Rosbe KW, Mathes EF, Frieden IJ, Shimano KA (2018). Sirolimus for management of complex vascular anomalies - A proposed dosing regimen for very young infants. Int J Pediatr Otorhinolaryngol 105:48-51. PMID: 29447818

Montague GL, Bly RA, Nadaraja GS, Conrad DE, Parikh SR, Chan DK (2018). Endoscopic percutaneous suture lateralization for neonatal bilateral vocal fold immobility. Int J Pediatr Otorhinolaryngol 108:120-124. PMID: 29605340

Durr ML, Meyer AK, Kezirian EJ, Mamlouk MD, Frieden IJ, Rosbe KW (2017). Sleep-disordered breathing in pediatric head and neck vascular malformations. Laryngoscope 127(9):2159-2164. PMID: 28092114

Su BM, Park JS, Chan DK (2017). Impact of Primary Language and Insurance on Pediatric Hearing Health Care in a Multidisciplinary Clinic. Otolaryngol Head Neck Surg 157(4):722-730. PMID: 28895463

Su BM, Chan DK (2017). Prevalence of Hearing Loss in US Children and Adolescents: Findings From NHANES 1988-2010. JAMA Otolaryngol Head Neck Surg 143(9):920-927. PMID: 28750123.

Park JS, Chan DK, Parikh SR, Meyer AK, Rosbe KW (2016). Surgical outcomes and sleep endoscopy for children with sleep-disordered breathing and hypotonia. Int J Pediatr Otorhinolaryngol 90:99-106. PMID: 27729162.

Chan DK, Rouse SL (2016). Sound-induced intracellular Ca2+ dynamics in the adult hearing cochlea. PLoS One 11(12):e0167850. PMID: 27959894.

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