Abstract

The prevalence of Auditory Neuropathy Spectrum Disorder (ANSD) ranges from 0.5 – 11% (Vijayasarathy & Shetty, 2021, p. 1). ANSD may be present at birth, but later onset of ANSD is possible for patients ranging from 10 – 25 years of age (p. 1). Therefore, this study investigated the knowledge of pediatric audiologists’ regarding Auditory Neuropathy Spectrum Disorder (ANSD). This study also aimed to explore the level of familiarity of pediatric audiologists with regards to the treatment options for Auditory Neuropathy Spectrum Disorder, including advances in genetic testing and protein replacement developments. The instrument used to collect the data for the current study was an HSRC-approved quantitative investigation making use of survey research techniques. The survey was sent to various ASHA Special Interest Groups (SIG), as well as audiology organizations such American Cochlear Implant Association (ACIA), Children’s Hospitals, the Educational Audiology Association (EAA), and social media platforms. Several major conclusions were made from the current study including the following: cases seen within the last twelve months for Auditory Neuropathy Spectrum Disorder (ANSD) indicated that the low prevalence of this condition leads to smaller caseloads for audiologists. Audiologists’ familiarity with the range of management options for ANSD reveals a strong understanding of the available approaches for pediatric patients. Audiologists’ comfort levels in managing children with ANSD across various options (No Hearing Technology, Hearing Aids, and Cochlear Implants) showed no significant differences in comfort levels. Most audiologists indicated a preference for the term "ANSD," suggesting that this designation is widely accepted as the clearest and most appropriate within the profession. Analysis on genetics and gene therapy showed that audiologists are generally knowledgeable about the genetic causes of ANSD, with most referring pediatric patients for genetic testing. However, a small proportion of audiologists “rarely” or “never” refer patients for genetic testing. While most audiologists are aware of ongoing gene therapy and otoferlin replacement trials, fewer are familiar with the two U.S.-based companies approved for clinical trials specifically targeting gene therapy for ANSD.

Advisor

Goldberg, Donald

Department

Communication Sciences and Disorders

Disciplines

Communication

Keywords

Auditory Neuropathy Spectrum Disorder (ANSD), Hearing Aid(s) (HAs), Cochlear Implant(s) (CIs), Genetic Testing, Gene Therapy, Otoferlin Replacement Trials

Publication Date

2024

Degree Granted

Bachelor of Arts

Document Type

Senior Independent Study Thesis

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