Clinical Trial: Applying Proton Pump Inhibitor to Prevent and Treat Acute Fluctuating Hearing Loss in Patients With SLC26A4 Mutation

Study Status: Completed
Recruit Status: Unknown status
Study Type: Interventional

Official Title: Applying Proton Pump Inhibitor to Prevent and Treat Acute Fluctuating Hearing Loss in Patients With SLC26A4 Mutation

Brief Summary: Disequilibrium between acid and base in the inner ear was suggested to be an important factor leading to hearing impairment associated with SLC26A4 mutations. For acid-base homeostasis in the inner ear, gastric-type proton pumps might demonstrate antagonistic effects to pendrin, the protein encoded by SLC26A4. To investigate whether proton pump inhibitors might prevent or treat acute fluctuating hearing loss related to SLC26A4 mutations, we launch the current double-blind randomized clinical trial.

Detailed Summary:

Hereditary hearing loss is the most common inherited sensory defect, affecting about 1 per 1000 children. With the advances in molecular genetics, the nature of hereditary hearing loss has started to be unraveled. A plethora of deafness genes were discovered in the past years, and among them certain genetic mutations were noted to be extraordinarily popular in the hearing-impaired population. For example, mutations in the SLC26A4 gene have been documented with high prevalence in a variety of ethnic backgrounds, including Caucasians, Japanese and Han Chinese.

The two specific clinical features of patients with SLC26A4 mutations are inner ear malformations and fluctuating hearing loss. For decades, the latter has constituted a treatment difficulty for pediatric otologists, because traditional regimens, such as steroid or intracranial-pressure-lowering-medication, usually could not achieve satisfactory and predictable outcomes. Nevertheless, as basic researches in recent years began to shed light on the pathogenesis of hearing loss from SLC26A4 mutations, novel strategies could be developed based on some of these crucial findings. For instance, disequilibrium between acid and base in the inner ear was reported to be an important factor leading to deafness in SLC26A4 knock-out mice. And for acid-base homeostasis in the inner ear, proton pumps were found to demonstrate antagonistic effects to pendrin, the protein encoded by SLC26A4. Consequently, regimens which can modulate the function of proton pumps, like proton pump inhibitors, might be a good choice to prevent or treat acute or chronic hearing loss related to SLC26A4 mutations or degenerative dysfunction.

Corresponding to this postulation, clinically we experienced significant recovery of hearing loss in several patients with SLC26A4 mutations who suffered from acute fluct
Sponsor: National Taiwan University Hospital

Current Primary Outcome: Decrease in attack frequency or improvement of hearing loss [ Time Frame: 3 years ]

Original Primary Outcome: Same as current

Current Secondary Outcome:

Original Secondary Outcome:

Information By: National Taiwan University Hospital

Dates:
Date Received: July 8, 2007
Date Started: August 2006
Date Completion: July 2009
Last Updated: November 10, 2008
Last Verified: November 2008