Auditory System

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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  1. 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.
  2. 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.§ionid=62642135. Accessed April 11, 2017.