Featured Research

from universities, journals, and other organizations

Study Examines Cause Of Hearing Loss For Patients With Certain Genetic Disease

Date:
July 7, 2007
Source:
JAMA and Archives Journals
Summary:
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.

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 ELST--associated 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 ELST--associated 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 combination) may account for the audiovestibular [hearing and balance] morbidity associated with ELSTs. These include direct invasion of the otic capsule [embryonic cartilage capsule that surrounds the inner ear mechanism and develops into bony tissue] by tumor, intralabyrinthine hemorrhage, and endolymphatic hydrops [recurrent vertigo accompanied by ringing in the ears and deafness]."

"Since significant audiovestibular dysfunction, including deafness, can occur suddenly in a manner that is not related to tumor size, early surgical intervention may be warranted. While this study does not address the effectiveness of surgical resection, several small operative series indicate that complete resection of ELSTs can be curative, can alleviate vestibular symptomatology, and can be performed with hearing preservation and minimal morbidity."

"Therefore, in patients with hearing and imaging evidence of an ELST, surgery may be considered after weighing its potential risks to prevent neurologic worsening or amelioration of symptoms. To intervene early, prompt diagnosis based on clinical and imaging findings is necessary. Thus, serial clinical evaluations and high-resolution MRI and CT imaging to detect small ELSTs or intralabyrinthine hemorrhage are warranted in patients with VHL," the authors conclude.


Story Source:

The above story is based on materials provided by JAMA and Archives Journals. Note: Materials may be edited for content and length.


Cite This Page:

JAMA and Archives Journals. "Study Examines Cause Of Hearing Loss For Patients With Certain Genetic Disease." ScienceDaily. ScienceDaily, 7 July 2007. <www.sciencedaily.com/releases/2007/07/070703172421.htm>.
JAMA and Archives Journals. (2007, July 7). Study Examines Cause Of Hearing Loss For Patients With Certain Genetic Disease. ScienceDaily. Retrieved July 25, 2014 from www.sciencedaily.com/releases/2007/07/070703172421.htm
JAMA and Archives Journals. "Study Examines Cause Of Hearing Loss For Patients With Certain Genetic Disease." ScienceDaily. www.sciencedaily.com/releases/2007/07/070703172421.htm (accessed July 25, 2014).

Share This




More Health & Medicine News

Friday, July 25, 2014

Featured Research

from universities, journals, and other organizations


Featured Videos

from AP, Reuters, AFP, and other news services

New Painkiller Designed To Discourage Abuse: Will It Work?

New Painkiller Designed To Discourage Abuse: Will It Work?

Newsy (July 24, 2014) The FDA approved Targiniq ER on Wednesday, a painkiller designed to keep users from abusing it. Like any new medication, however, it has doubters. Video provided by Newsy
Powered by NewsLook.com
Doctor At Forefront Of Fighting Ebola Outbreak Gets Ebola

Doctor At Forefront Of Fighting Ebola Outbreak Gets Ebola

Newsy (July 24, 2014) Sheik Umar Khan has treated many of the people infected in the Ebola outbreak, and now he's become one of them. Video provided by Newsy
Powered by NewsLook.com
Condemned Man's US Execution Takes Nearly Two Hours

Condemned Man's US Execution Takes Nearly Two Hours

AFP (July 24, 2014) America's death penalty debate raged Thursday after it took nearly two hours for Arizona to execute a prisoner who lost a Supreme Court battle challenging the experimental lethal drug cocktail. Duration: 00:55 Video provided by AFP
Powered by NewsLook.com
Can Watching TV Make You Feel Like A Failure?

Can Watching TV Make You Feel Like A Failure?

Newsy (July 24, 2014) A study by German researchers claims watching TV while you're stressed out can make you feel guilty and like a failure. Video provided by Newsy
Powered by NewsLook.com

Search ScienceDaily

Number of stories in archives: 140,361

Find with keyword(s):
Enter a keyword or phrase to search ScienceDaily for related topics and research stories.

Save/Print:
Share:

Breaking News:
from the past week

In Other News

... from NewsDaily.com

Science News

    Health News

      Environment News

        Technology News



          Save/Print:
          Share:

          Free Subscriptions


          Get the latest science news with ScienceDaily's free email newsletters, updated daily and weekly. Or view hourly updated newsfeeds in your RSS reader:

          Get Social & Mobile


          Keep up to date with the latest news from ScienceDaily via social networks and mobile apps:

          Have Feedback?


          Tell us what you think of ScienceDaily -- we welcome both positive and negative comments. Have any problems using the site? Questions?
          Mobile: iPhone Android Web
          Follow: Facebook Twitter Google+
          Subscribe: RSS Feeds Email Newsletters
          Latest Headlines Health & Medicine Mind & Brain Space & Time Matter & Energy Computers & Math Plants & Animals Earth & Climate Fossils & Ruins