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]
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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