Perspectives on Hearing and Hearing Disorders: Research and Diagnostics

Coordinator's Column

Moore, R. E. — 2013-05-15T14:00:26-07:00

Gene Therapy for Hereditary Hearing Loss

McDougald, D. S., Cunningham, L. L., Chien, W. W. — 2013-05-15T14:00:26-07:00

Mutations in over 70 genes have now been identified as causing hereditary hearing loss in humans. This expanding knowledge about the genetics of hearing loss opens the door for the development of gene therapies aimed at restoring hearing function by delivering corrective DNA to the inner ears of patients with hearing loss caused by these mutations. This review summarizes some of the recent advances toward the goal of developing gene therapy as a treatment for hereditary hearing loss in humans.

A Brief Review of Cochlear™ Baha(R) Osseointegrated Solutions for the Treatment of Hearing Loss

Cire, G. — 2013-05-15T14:00:26-07:00

This article serves as an overview of the topic of implantable bone-anchored devices for the treatment of conductive, mixed-conductive, and single-sided sensorineural deafness. The author describes the history and background of this treatment option, along with information on indications and candidacy, and discusses clinical outcomes.

Changes in Localization Accuracy With Use of Bilateral Open-Fit Behind-the-Ear Hearing Aids in Two Children

Johnstone, P. M., Yeager, K. R., Johnson, N. K., Pomeroy, M. — 2013-05-15T14:00:26-07:00

In the study described in the article, two children had their prescribed behind-the-ear (BTE) hearing aids retro-fit to become open-fit (OF) BTEs. One child, age 12y, had a bilateral, high-frequency, sensorineural hearing loss (BHFL), with normal hearing in the low frequencies. The other child, age 6y, had a bilateral, reverse-slope, sensorineural hearing loss (BRSL), with normal hearing in the high frequencies. Sound localization measurements were made at Day 1 unaided and with the OF-BTEs and 1 year post for both children. Both children spontaneously reported an immediate improvement in the quality of their own voice when first exposed to the OF-BTEs. Only the younger child showed immediate localization advantage over unaided when using the OF-BTEs. However, changes in localization accuracy over time were measured for both children that could be attributed to "relearning" to localize with a new acoustic coupling of the hearing aid to the ear. After using OF-BTE hearing aids for 1 year, both children, when aided, could localize as well as age-matched peers with normal hearing.

Auditory Training: Evidence for Neural Plasticity in Older Adults

Anderson, S., Kraus, N. — 2013-05-15T14:00:26-07:00

Improvements in digital amplification, cochlear implants, and other innovations have extended the potential for improving hearing function; yet, there remains a need for further hearing improvement in challenging listening situations, such as when trying to understand speech in noise or when listening to music. Here, we review evidence from animal and human models of plasticity in the brain's ability to process speech and other meaningful stimuli. We considered studies targeting populations of younger through older adults, emphasizing studies that have employed randomized controlled designs and have made connections between neural and behavioral changes. Overall results indicate that the brain remains malleable through older adulthood, provided that treatment algorithms have been modified to allow for changes in learning with age. Improvements in speech-in-noise perception and cognition function accompany neural changes in auditory processing. The training-related improvements noted across studies support the need to consider auditory training strategies in the management of individuals who express concerns about hearing in difficult listening situations. Given evidence from studies engaging the brain's reward centers, future research should consider how these centers can be naturally activated during training.