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CHAPTER 1: GENERAL INFORMATION

 

On this page:

Section 30: Acoustic neuroma

Section 31: Otosclerosis

Section 32: Usher Syndrome

Click on the links in the left hand column to see the other sections

Section 30: Acoustic neuroma

 

An acoustic neuroma is a benign tumour that grows on the auditory (hearing) nerve. They generally grow very slowly and do not spread from the original site.

An acoustic neuroma can sometimes be called a vestibular schwannoma.

As the tumour grows it may begin to press on the auditory nerve and can affect hearing.

There are various treatments available ranging from observation of the acoustic neuroma to keep track of it, to surgery to remove it. These treatments are discussed in more detail on the website of the British Acoustic Neuroma Association.

More information about acoustic neuromas can be found from the British Acoustic Neuroma Association website.

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Section 31: Otosclerosis

 

This is a progressive disorder in which there is abnormal bone growth around the stapes (or stirrup), one of the bones in the middle ear.

The stapes may eventually become fused to the cochlea. This stops the bones from vibrating correctly. If the bones do not vibrate sound cannot be transmitted to the cochlea, resulting in a hearing loss.

Otosclerosis can be genetic and often runs in families, but some people can develop it without any past incidences in their family.

Otosclerosis can be speeded up by pregnancy as it can be affected by female hormones.

What might help?

People with otosclerosis can find wearing a hearing aid very helpful.

Sometimes an operation is performed, either a stapedectomy (removal and replacement of the stapes) or a stapedotomy (a hole is made in the stapes and a prosthetic is inserted), which usually restores some or most hearing.

More information:

Further information can be found in:

Deafness Research’s Otosclerosis factsheet
British Tinnitus Association’s website - page on otosclerosis

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Section 32: Usher Syndrome

Usher syndrome is a genetic disorder characterised by a hearing loss and gradual loss of vision due to retinitis pigmentosa (a deterioration of the cells in the retina). Some people with Usher’s may experience problems with their balance.

There are three different types of Usher Syndrome which develop in different ways:

Type 1: The person is born profoundly deaf and is likely to develop vision problems in their first 10 years

Type 2: The person is born with a hearing loss and may develop vision problems within their first 20 years

Types 1 and 2 are the most common.

Type 3: The person is born with normal hearing and sight but is likely to develop hearing and sight problems in early to mid adulthood.

Type 3 is quite rare.

The following organisations have lots of information about deafblindness:

Sense
Deafblind UK
Deafblind Scotland

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