VALERIE A. STREET, PHD

Research Associate Professor

Department
Otolaryngology - Head and Neck Surgery

Research Affiliations
Virginia Merrill Bloedel Hearing Research Center

Specialties
Molecular Genetics

Sites of Practice, Maps, and Directions
Virginia Merrill Bloedel Hearing Research Center (VMBHRC)
University of Washington Medical Center (UWMC)

Areas of Expertise
Genetics of Hearing Loss
Charcot-Marie-Tooth (CMT) Disease

Graduate Education
Ph.D. Molecular Pharmacology, University of Washington, Seattle, WA 1995

Postgraduate Education
Charcot-Marie-Tooth Fellow, Medical Genetics and Pediatrics, University of Washington, 2000
Senior Postdoctoral Fellow, Otolaryngology Department, University of Washington, 1998-1999
Postdoctoral Fellow, Geriatric Research Education and Clinical Center, Veterans Affairs Puget Sound Health Care System, Seattle, WA, 1996-1997

Local and National Leadership Positions
Director, The HEAR Group, The Western Hereditary Equilibrium and Auditory Research Group

Primary Research Support
Principal Investigator, NIH, NIDCD, DC006901, Genetics of Progressive Sensorineural Hearing Loss

Most Significant Recent Articles

• A novel DFNA9 mutation in the vWFA2 domain of COCH alters a conserved cysteine residue and intrachain disulfide bond formation resulting in progressive hearing loss and site-specific vestibular and central oculomotor dysfunction
• Modifier controls severity of a novel dominant low-frequency Myosin VIIA (MYO7A) auditory mutation
• Mutation of a putative protein degradation gene LITAF/SIMPLE in Charcot-Marie-Tooth disease 1C
• Mutations in a plasma membrane Ca2+-ATPase gene cause deafness in deafwaddler mice
• The type 1 inositol 1,4,5-trisphosphate receptor gene is altered in the opisthotonos mouse

Research Activities
Our research begins by collaborating with a large family that has inherited hearing loss and/or balance problems. We provide these families with clinical hearing and vestibular evaluations. We ask each member of the family to donate a small blood sample from which we isolate genetic material DNA. Our goal is to find the genetic mutation underlying the sensory challenge experienced by the family. Once we have identified the genetic mutation, we notify the families in writing and also try to present our findings in person at a family reunion. The next step is to study the gene and protein altered in the family to learn more about how the protein functions in the ear. This will hopefully allow us to gain new insights into the ear and potentially help a number of people with different types of hearing and vestibular deficits.

Invited Lectures

• Genetic Analysis in Large Families with Inherited Hearing Loss. Oto-HNS Alumni Day 2005
• Genetics of Hearing Loss: Past, Present, and Future. Ears, Hearing and Beyond, Seattle, WA, 2006
• Identification of Hearing Impaired Families with Mutations in MYO7A and COCH. Montana Audiology Conference, Chico, Montana, 2007

Practice Priorities
On Research: "It has been a delightful experience to collaborate with the families in our research program. I enjoy discussing project ideas with other researchers - I am always learning something new."

On Teaching: "Watching a student grasp a concept for the first time and then become excited about their new insight makes teaching very rewarding for me. It also reminds me how lucky I am to conduct research at the University of Washington."

Personal Information
I enjoy golfing, gardening, boating, traveling, reading, and spending time with my husband and son.

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