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

NCT ID: NCT00789061

Last Updated: 2008-11-11

Basic Information

• Recruitment Status: UNKNOWN
• Clinical Phase: PHASE2/PHASE3
• Total Enrollment: 120 participants
• Study Classification: INTERVENTIONAL
• Study Start Date: 2006-08-31
• Study Completion Date: 2009-07-31

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 Description

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 fluctuating hearing loss which was refractory to traditional treatment. In some cases, acute hearing loss recurred after the medication was discontinued. Therefore, we launch the current clinical trial to investigate the efficacy of proton pump inhibitor in preventing and treating acute hearing loss in patients with SLC26A4 mutations.

Conditions

Hearing Loss

Keywords

Acute hearing loss in patients with SLC26A4 mutations

Study Design

• Allocation Method: RANDOMIZED
• Intervention Model: PARALLEL
• Primary Study Purpose: PREVENTION
• Blinding Strategy: TRIPLE

Interventions

Proton pump inhibitor

One dosage/day/year

Intervention Type: DRUG

Other Intervention Names

Taquidine,Lansoprazole

Eligibility Criteria

Inclusion Criteria

• Patients with homozygous or heterozygous SLC26A4 mutations
• Patients with acute hearing loss

Exclusion Criteria

• Patients who do not fulfill both of the above criteria

• Eligible Sex: ALL
• Accepts Healthy Volunteers: No

Sponsors

National Taiwan University Hospital

OTHER

Sponsor Role: lead

Principal Investigators

Chuan-Jen Hsu, MD

Role: STUDY_DIRECTOR

Department of Otolaryngology, National Taiwan University Hospital

Chen-Chi Wu, MD

Role: PRINCIPAL_INVESTIGATOR

Department of Otolaryngology, National Taiwan University Hospital

Locations

National Taiwan University Hospital

Taipei, , Taiwan

Site Status: RECRUITING

Countries

Taiwan

Central Contacts

Chen-Chi Wu, MD

Role: CONTACT

Phone: +886-2-23123456

Email: chenchiwu@hotmail.com

Other Identifiers

950805

Identifier Type: -

Identifier Source: org_study_id