Auditory System | Healthcare Professional

Hearing loss is common in MPS I disease and correlates with the severity of somatic disease. Deficits may be conductive or neurosensory in nature, but generally are mixed. The most important contributing factors include scarring as a result of frequent middle ear infection (caused by storage within the oro-pharynx leading to eustachian tube dysfunction), dysostosis of the intrinsic auditory bones and damage to the eighth cranial (auditory) nerve.^[1],[2]

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Did You Know?

The MPS I Registry program, sponsored and administered by Sanofi Genzyme, is a worldwide database that tracks health-related information for people with MPS I, allowing doctors and health care professionals to understand the disorder.

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References

Clarke, L.A. (1997) Clinical diagnosis of lysosomal storage diseases. In: Organelle Diseases. Clinical Features, Diagnosis, Pathogenesis and Management. Applegarth, D.A., Dimmick, J.E., and Hall, J.G. (eds.). Chapman and Hall Medical, London, p. 37.
Neufeld EF, Muenzer J. The Mucopolysaccharidoses. In: Valle D, Beaudet AL, Vogelstein B, Kinzler KW, Antonarakis SE, Ballabio A, Gibson K, Mitchell G. eds. The Online Metabolic and Molecular Bases of Inherited Disease New York, NY: McGraw-Hill; 2014. https://ommbid.mhmedical.com/content.aspx?bookid=971&sectionid=62642135. Accessed February 2019.