According to Van Den Bogaert et. al. “Among white adults, otosclerosis is the single most common cause of hearing impairment.”  Otosclerosis is a disease in which abnormal growth occurs on the bones of the middle ear resulting in a worsening ability for sounds to be conveyed to the cochlear (a conductive hearing loss). The mean age of onset is in the third decade and 90% of those affected are under 50 at the time of diagnosis (Van Den Bogaert et. al). It is not certain what causes it – possibly it is an autoimmune disease, but more probably a combination of genetic and environmental factors (Merchant et al.). When it can be caught there are possible interventions such as fluoride (no evidence that this is effective), hearing aids or surgery (which can be effective), but it cannot be ‘cured’.

People with, or thought to have had otosclerosis, include:

Actor Richard Thomas (famous on TV for The Waltons etc), Beethoven, Harriet Martineau, Frankie Valli,

Howard Hughes, and Queen Alexandra (see also here http://www.hearingreview.com/issues/articles/2004-10_02.asp)

http://www.healthyhearing.com/content/articles/Assistance/Awareness/22442-Actor-richard-thomas-john

Merchant, Saumil N. et al. Otosclerosis, Chapter 237e p.3453-85, inGleeson, Michael et al. eds. Scott-Brown’s otorhinolaryngology, head and neck surgery. 7th ed. London:  Hodder Arnold,   2008

Van Den Bogaert et al., Otosclerosis, Chapter 27 p.419-28 in Willems, Patrick J. (ed.) Genetic hearing loss. New York:  Marcel Dekker,   2004

http://www.patient.co.uk/health/Otosclerosis.htm

Deafness Research UK

Below are five recent articles on otosclerosis from PubMed –

Decision making in advanced otosclerosis: an evidence-based strategy.