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Hearing management

Updated 04 December 2017

Gene therapy may restore hearing loss

Scientists using gene therapy have partially restored hearing and balance in profoundly deaf mice, according to a study published in the journal Nature Medicine.

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Scientists using gene therapy have partially restored hearing and balance in profoundly deaf mice, according to a study published in the journal Nature Medicine.

The research, still in its early stages and restricted to lab animals, may open up new avenues for tackling Usher syndrome, an inherited form of human deafness that usually goes hand in hand with blindness.

Researchers led by Michelle Hastings at the Rosalind Franklin University of Medicine and Science in Chicago, Illinois, aimed at a gene called USH1C which has been implicated in the "Type 1" form of Usher syndrome.

USH1C controls a protein called harmonin, which plays a vital role in hair cells - the cells in the cochlea of the inner ear that respond to sound waves and send an electrical signal to the brain.

Faulty version of gene 'switched off'

The team devised a tiny strand of genetic material called an antisense oligonucleotide to "switch off" a faulty version of the gene that produces truncated forms of the protein.

The therapy was injected in newborn mice that had been genetically engineered to have the mutation.

A single injection partially restored their hearing at very low frequencies, and also reduced head tossing, a behaviour caused by impaired balance.

"These effects were sustained for several months, providing evidence that congenital deafness can be effectively overcome by treatment early in development to correct gene expression," the study says.

Transforming cells

After the experiment, the mice were dissected, and their cochleas were found to have grown some hair cells.

The success of antisense oligonucleotides adds a further weapon in the quest to overcome deafness.

Last month, doctors at the Massachusetts Eye and Ear and Harvard Medical School reported on a gene drug that transformed cells in the cochlea into hair cells.

In 2012, investigators at the University of California, San Francisco targeted a fix for a faulty version of a gene called VGLUT3. The gene controls a protein that is vital for hair cells to send the signals they pick up.

(AFP, February 2013)

Read more: 

More success with gene therapy for blindness

Types of hearing loss

Gene therapy helps repair heart muscle

 

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Hearing Expert

Dr Kara Hoffman graduated from UCT in 2004, thereafter she completed her year of community service in Durban. In 2010 she completed her Masters Degree in Paediatric Aural Rehabilitation from UKZN. In 2016, she became a Doctor of Audiology through the University of Arizona (ATSU). Dr Hoffman and her partner Lauren Thompson opened a fully diagnostic audiology practice called Thompson & Hoffman Audiology Inc. In 2011 with world-class technology and equipment to be able to offer the broad public all hearing-related services including hearing testing for adults and babies, vestibular (balance) assessments and rehabilitation, industrial audiology, hearing devices, central auditory processing assessments for school-aged children, school screening, neonatal hearing screening programmes at Alberlito and Parklands Hospital, cochlear implants and other implantable devices, medicolegal assessments and advanced electroacoustic assessments of hearing. Thompson and Hoffman Audiology Inc. are based at Alberlito Hospital in Ballito, St Augustines Hospital in Durban and at 345 Essenwood Road, Musgrave. The practices are all wheelchair friendly. There are three audiologists that practice from Thompson & Hoffman – including Dr Kara Hoffman, Lauren Thompson & Minette Lister. The practice boasts professional, highly qualified, and extensive diagnostic services where all your hearing healthcare needs can be met. The additional licensing in vestibular assessment and rehabilitation, paediatric rehabilitation and cochlear implantation places this practice in one of the top specialist audiological positions in South Africa, with a wealth of experience in all clinical areas of audiology and is a very well respected and sought-after practice.

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