Gene Therapy Holds Promise for Reversing Congenital Hearing Loss

ScienceDaily (July 25, 2012) — A new gene therapy approach can reverse hearing loss caused by a genetic defect in a mouse model of congenital deafness, according to a preclinical study published by Cell Press in the July 26 issue of the journal Neuron. The findings present a promising therapeutic avenue for potentially treating individuals who are born deaf.

"This is the first time that an inherited, genetic hearing loss has been successfully treated in laboratory mice, and as such represents an important milestone for treating genetic deafness in humans," says senior study author Lawrence Lustig of the University of California, San Francisco.

Hearing loss is one of the most common human sensory deficits, and it results from damage to hair cells in the inner ear. About half of the cases of congenital hearing loss are caused by genetic defects. However, the current treatment options -- hearing amplification devices and cochlear implants -- do not restore hearing to normal levels. Correcting the underlying genetic defects has the potential to fully restore hearing, but previous attempts to reverse hearing loss caused by genetic mutations have not been successful.

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Bone-Anchored Hearing Aids Appear Beneficial for Hearing-Impaired Children

ScienceDaily (Feb. 21, 2011) — Bone-anchored hearing aids appear helpful in improving hearing and quality of life in children with hearing loss in one or both ears, according to a report in the February issue of Archives of Otolaryngology -- Head & Neck Surgery, one of the JAMA/Archives journals.

"Since its introduction more than 30 years ago, the bone-anchored hearing aid (BAHA) has become an established treatment option for auditory rehabilitation in patients with chronic conductive or mixed hearing loss," the authors write as background information in the article. Although the BAHA was most commonly fitted in adults when it was first introduced, it has gradually become a popular option for children with bilateral conductive hearing loss who are too young to undergo alternative surgical options.

Maarten J. F. de Wolf, M.D., and colleagues at the Radboud University Nijmegen Medical Centre, Nijmegen, the Netherlands, obtained information about 31 children who were current BAHA users. Data were collected through questionnaires answered by the children and their parents. Eligible children were a minimum of 4 years old at the time of BAHA fitting, and had been using the device for one to four years. Patients with both bilateral hearing loss (16 children) and unilateral hearing loss (15 children) were evaluated.

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