Skip to content
American Academy of Audiology
  • PRACTICE RESOURCES
    • Practice Guidelines and Standards
    • Coding
    • Medicare/Medicaid
    • Compliance
  • EDUCATION & EVENTS
    • Event Calendar
    • Continuing Education
    • Online Learning and Certificate Programs
    • Research Grants and Scholarships
    • Faculty and Preceptor Resources
  • AAA ANNUAL CONFERENCE
    • Attend
    • Program and Events
    • Exhibit Hall
    • Exhibit and Sponsorship Opportunities
    • About
  • NEWS & PUBLICATIONS
    • News
    • Audiology Today
    • Journal of the American Academy of Audiology
    • Advertising
    • Permissions and Reprints
    • Press and Media Information
  • CAREERS
    • Career Center
    • Certification
    • Become an Audiologist
    • Doctoral Programs in Audiology
    • Recruiting
    • New Professional Toolkit
  • ADVOCACY
    • Legislative and Regulatory Activities
      • State Affairs
      • Federal Affairs
    • Legislative Action Center
    • Government Relations News
  • CONSUMERS
    • What Is an Audiologist
    • Hearing and Balance Symptoms and Conditions
    • Managing Hearing Loss
    • Seniors and Hearing Loss
    • Children and Hearing Loss
    • OTC Hearing Aid
  • ABOUT THE ACADEMY
    • Leadership
    • Committees and Task Forces
    • Honors and Awards
    • Get Involved
    • Sponsorships and Corporate Partners
    • Contact Us
  • MEMBERSHIP
    • Benefits
    • Ethics
    • Member Directory
    • Membership Renewals
FacebookTwitterLinkedinYoutubeInstagram
  • Students
  • Get Involved
  • Donate
  • Renew
American Academy of Audiology
Member Login
  • PRACTICE RESOURCES
    • Practice Guidelines and Standards
    • Coding
    • Medicare/Medicaid
    • Compliance
  • EDUCATION & EVENTS
    • Event Calendar
    • Continuing Education
    • Online Learning and Certificate Programs
    • Research Grants and Scholarships
    • Faculty and Preceptor Resources
  • AAA ANNUAL CONFERENCE
    • Attend
    • Program and Events
    • Exhibit Hall
    • Exhibit and Sponsorship Opportunities
    • About
  • NEWS & PUBLICATIONS
    • News
    • Audiology Today
    • Journal of the American Academy of Audiology
    • Advertising
    • Permissions and Reprints
    • Press and Media Information
  • CAREERS
    • Career Center
    • Certification
    • Become an Audiologist
    • Doctoral Programs in Audiology
    • Recruiting
    • New Professional Toolkit
  • ADVOCACY
    • Legislative and Regulatory Activities
      • State Affairs
      • Federal Affairs
    • Legislative Action Center
    • Government Relations News
  • CONSUMERS
    • What Is an Audiologist
    • Hearing and Balance Symptoms and Conditions
    • Managing Hearing Loss
    • Seniors and Hearing Loss
    • Children and Hearing Loss
    • OTC Hearing Aid
  • ABOUT THE ACADEMY
    • Leadership
    • Committees and Task Forces
    • Honors and Awards
    • Get Involved
    • Sponsorships and Corporate Partners
    • Contact Us
  • MEMBERSHIP
    • Benefits
    • Ethics
    • Member Directory
    • Membership Renewals
September 8, 2021

Cochlear Implantation in Patients with Pendred Syndrome

  • Audiology in the News

Should we provide cochlear implants earlier for children with large vestibular aqueduct syndrome?


Patterson and colleagues (2021) examined outcomes of nine pediatric patients with Pendred syndrome who received cochlear implants between 2003–2017.

Pendred syndrome is an autosomal recessive disorder defined by the combination of sensorineural hearing loss, goiter and an enlarged vestibular aqueduct (EVA). All patients had bilateral mild to profound sensorineural hearing loss, used hearing aids prior to cochlear implantation, and had enlarged vestibular aqueducts. Eight of the nine participants had cochlear dysplasia. The age at implantation was between nine months and seven years.

All electrodes were successfully implanted into the scala tympani, and all patients had good postoperative audiological outcomes. Postoperatively, the average AzBio sentence score was 97 percent. The patients were enrolled in a mainstream educational setting or early intervention school setting and were using remote microphone technology at school.

The authors concluded that cochlear implantation is an effective and successful treatment for children with Pendred syndrome for whom traditional amplification provide limited benefit. Outcomes have been proven highly successful overall with a low rate of complications despite the abnormal inner ear anatomy (Hall et al 2019).

References

Hall AC, Kenway B, Sanlit S, et al. (2019) Cochlear implant outcomes in large vestibular aqueduct syndrome—should we provide cochlear implants earlier? Otol Neurotol 40(e769–e773).

Patterson TE, Gonzalez VB, Carron JD. (2021) Cochlear implantation in patients with Pendred Syndrome. Am J Otolaryngol. 42(6).

Share this

Related Posts

A Virtual Reality System for Delivery of Military-Specific Vestibular Rehabilitation After Mild Traumatic Brain Injury: The Praxis Study Protocol

Panic Disorders in Patients with Vestibular Dysfunction: A Systematic Review and Meta-Analysis

Ectoderm Barcoding Reveals Neural and Cochlear Compartmentalization

Recent Posts

Audiology in the News

A Virtual Reality System for Delivery of Military-Specific Vestibular Rehabilitation After Mild Traumatic Brain Injury: The Praxis Study Protocol

In an article by Alroumi et al. (2025), treatment of mild traumatic brain injury (mTBI) through the use of virtual reality (VR) system was investigated….

Read More
Government Relations News

From Capitol Hill to Your Clinic: SPAN July Meeting on Medicaid Cuts

The State Policy Advocacy Network (SPAN) will convene again on July 29 for a critical meeting about Medicaid funding. SPAN brings together nationwide audiologists and…

Read More
The White House from the sky
Government Relations News

EHDI Program at Risk

On April 1, President Trump’s administration eliminated the entire branch of the Early Hearing Detection and Intervention (EHDI) program that works with states to analyze…

Read More
wave-mark

Join the Academy

Academy members receive many benefits for professional development, practice management, and community development.

Become a Member
COMM24-Academy_Website_Graphics-revised_logo(W)

American Academy of Audiology
11480 Commerce Park Drive
Suite 220
Reston, VA 20191

Tel: 703-790-8466
Fax: 703-790-8631

  • Practice Resources
  • Education and Events
  • AAA Annual Conference
  • News and Publications
  • Careers
  • Advocacy
  • About the Academy
  • Consumers and Patients
  • Students
  • Donate
  • Get Involved
  • Contact Us
  • Privacy Policy
  • Antitrust Policy and Guidelines
  • Terms of Use Policy
  • Accessibility Statement
  • Sitemap

© Copyright 2025 American Academy of Audiology

Website by Yoko Co

This content is an exclusive benefit for American Academy of Audiology members.

If you’re a member, log in and you’ll get immediate access.

 

Member Login

 

If you’re not yet a member, you’ll be interested to know that joining not only gives you access to top-notch resources like this one, but also invitations to member-only events, inclusion in the member directory, participation in professional forums, and access to patient resources, tools, and continuing education. Join today!

Scroll To Top
We use cookies to improve your experience on our site. By continuing to use our site, you accept our use of cookies. To learn more, please see our Privacy Policy.Ok