Document Type

Capstone Project

Publication Date

Spring 3-13-2023

First Advisor

Antony Joseph, MA., Au.D., Ph.D., ABAC, CCC-A, CPS/A, F-NAP

Keywords

Charcot-Marie-Tooth disease, CMT, Cochlear implantation, amplification, sensorineural, hearing loss, Single-sided deafness, SSD

Disciplines

Communication Sciences and Disorders | Speech and Hearing Science | Speech Pathology and Audiology

Abstract

Abstract 1- Introduction: Charcot-Marie-Tooth (CMT) disease is a rare and chronic hereditary disorder that causes motor and sensory damage to the peripheral nervous system. As the disease progresses, hearing sensitivity may worsen. Case Presentation: A young adult-aged patient presented to the clinic with congenital hearing loss and difficulty with conversational speech in quiet and noisy conditions. Discussion: When encountering a patient with CMT, clinicians should be cognoscente of amplification options, especially if nerve pathways begin to deteriorate. When audiologic testing reveals a progression of hearing loss, alternative amplification may be indicated because conventional hearing aids may not provide enough benefit for CMT cases. Conclusion: Cochlear implantation is a valid option for individuals with CMT if they meet the candidacy requirements and no longer perceive a benefit from conventional hearing aids.

Abstract 2- Introduction: Congenital or acquired, single-sided deafness (SSD) may be defined as the inability to hear in one ear, resulting in unilateral hearing loss. Research data supports cochlear implantation for the management of SSD to preserve residual hearing in the affected ear. Case Presentation: A young pediatric-aged patient presented with a congenital SSD. Her father reported minimal benefit from the prescribed hearing aid. Discussion: Amplification alternatives were recommended based on current data because the use of a single conventional hearing aid might not provide enough benefit for patients with SSD. Conclusion: Cochlear implantation is a viable option for individuals with SSD, as long as they meet the new candidacy requirements.

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