HOME >> BIOLOGY >> NEWS
Study examines cause of hearing loss for patients with certain genetic disease

Patients with the genetic disorder von Hippel-Lindau disease may suddenly experience hearing loss because of a tumor-associated hemorrhage in the inner ear, according to a study in the July 4 issue of JAMA.

Endolymphatic sac tumors (ELSTs; tumors of the inner ear) occur sporadically but may be associated with von Hippel-Lindau disease (VHL; a genetic disease characterized by the development of blood vessel tumors in the retina of the eye and in the brain; lesions and cysts can also develop in other parts of the body). ELSTs are associated with significant dysfunction of hearing and balance, including sudden irreversible hearing loss. The mechanisms and appropriate treatments for this disorder are not well understood.

John A. Butman, M.D., Ph.D., of the National Institutes Health, Bethesda, Md., and colleagues conducted a study, between May 1990 and December 2006, to determine the mechanisms underlying hearing loss in patients with ELSTs. Clinical findings and audiologic data were correlated with magnetic resonance imaging and computed tomography imaging studies. Thirty-five patients with von Hippel-Lindau disease and ELSTs in 38 ears were identified.

the most common ELSTassociated clinical finding in this series was hearing loss. Sensorineural hearing loss (SNHL) occurred in 31 patients (89 percent) or 34 of the affected ears (89 percent) and was frequently moderate or profound. Other less frequent ELSTassociated findings included aural [pertaining to the ear or hearing] fullness, aural pain, facial nerve weakness, and aural pain and facial nerve weakness combined, the authors write.

Intralabyrinthine (inner ear) hemorrhage was found in 11 of 14 ears with sudden SNHL but occurred in none of the 17 ears with gradual SNHL or normal hearing. Tumor size was not related to SNHL.

Based on the relationship between the imaging and clinical findings in these patients, 3 distinct mechanisms (either alone or in combinati
'"/>

Contact: Shana Potash
301-594-5788
JAMA and Archives Journals
3-Jul-2007


Page: 1 2

Related biology news :

1. Study begins to reveal clues to the cause and progression of sepsis
2. Study finds gender differences in renal and other genes contributing to blood pressure
3. Study suggests estrogen deficiency can lead to obesity-induced high blood pressure after menopause
4. Study: Sticking to the sand might not be such good, clean fun for beachgoers
5. Study points to new way to predict death risk from torn aorta
6. Study identifies new gene therapy tools for inherited blindness
7. Study finds contaminated water reaching Floridas offshore keys
8. Study sheds light on why humans walk on two legs
9. Study explains how pathogens evolve to escape detection
10. Study finds hereditary link to premenstrual depression
11. Study identifies energy efficiency as reason for evolution of upright walking

Post Your Comments:
(Date:5/21/2013)... at Chicago College of Medicine will study gender differences in ... energy source -- and how changes in fat metabolism play ... 4-year grant from the National Heart, Lung and Blood Institute. ... for energy. These changes may play a major role in ... and women, says E. Douglas Lewandowski, director of the UIC ...
(Date:5/21/2013)... charted the path of insulin action in cells in ... blueprint for understanding what goes wrong in diabetes. ... Professor David James from Sydney,s Garvan Institute of Medical ... of the prestigious journal Cell Metabolism . , ... very important role in the body because it helps ...
(Date:5/20/2013)... is known about the effect of physical education (PE) ... University finds that increasing the amount of time that ... of obesity. , The study represents some of the ... youth obesity, and is forthcoming in the Journal ... of the study can be viewed at: http://www.sciencedirect.com/science/article/pii/S0167629613000556 ...
Breaking Biology News(10 mins):Do men's and women's hearts burn fuel differently? 2Insight into the dazzling impact of insulin in cells 2Insight into the dazzling impact of insulin in cells 3Gym class reduces probability of obesity, study finds for first time 2
(Date:5/21/2013)... May 21, 2013 Alpha Source, ... , will be exhibiting for the 13th consecutive ... Medical Instrumentation (AAMI) 2013 Conference and Expo. The ... distribute, and manufacture healthcare technologies. This year’s event ... Beach, California. , The three-day event draws ...
(Date:5/21/2013)... Raleigh, NC (PRWEB) May 21, 2013 ... and financial services company for clinical trials, announced ... Aggregate Spend Solutions, LLC. Clinverse’s ClinPay® FLS ... automated investigator payment process. SpendMD ™, ... only complete solution for tracking and reporting transfer ...
(Date:5/21/2013)... (PRWEB) May 21, 2013 As ... a strong push within the industry to deliver ... Manufacturing Organizations and pharma companies often means turning ... , Microtablets, developed in collaboration with Sensidose AB ... outsourcing can bring products to market sooner. The ...
(Date:5/21/2013)... , May 21, 2013  MacroGenics, Inc. ... study of margetuximab (MGAH22), an Fc-modified chimeric monoclonal ... expressing the HER2 oncoprotein," will be presented at ... of Clinical Oncology during the Developmental Therapeutics - ... at 4:15 – 4:30 PM.  The presentation will ...
Breaking Biology Technology:Alpha Source, Inc. to Exhibit at the AAMI 2013 Conference and Expo 2Clinverse, Inc. Announces Strategic Alliance with Aggregate Spend Solutions, LLC 2Clinverse, Inc. Announces Strategic Alliance with Aggregate Spend Solutions, LLC 3The Development of an Innovative Microtablet Dosage Form, a Success Story: Live Webinar Hosted by Xtalks 2MacroGenics Announces Margetuximab (MGAH22) Phase 1 Data Presentation at ASCO; Initiates Phase 2 Clinical Study to Evaluate Activity of Margetuximab in Patients with Metastatic Breast Cancer 2MacroGenics Announces Margetuximab (MGAH22) Phase 1 Data Presentation at ASCO; Initiates Phase 2 Clinical Study to Evaluate Activity of Margetuximab in Patients with Metastatic Breast Cancer 3
Cached News: