PMID: 2115509Jun 1, 1990Paper

Degeneration of speech, language, and hearing in a patient with mucopolysaccharidosis VII

International Journal of Pediatric Otorhinolaryngology
S P WallaceS E Gerber

Abstract

Mucopolysaccharidosis VII (MPS-VII) is probably the rarest of the mucopolysaccharidoses; literature reveals only 20 cases. We have had the opportunity to study and treat such a child in our clinic, and this paper documents his speech, language, and hearing. Results demonstrated a delay with respect to his chronological age in all cognitive, linguistic, and social domains. He had a mixed hearing loss which could have contributed to his diminishing speech and language abilities; he had chronic otitis media. After 59 h of speech and language intervention (over a period of 19 months), primarily for language treatment, standardized tests revealed that his scores had decreased over time. During this period, both his speech production and his hearing got poorer. At about the time of his 8th birthday, he underwent a permanent tracheostomy, altering further therapy. Although MPS-VII is a very rare disorder, what has been learned here may apply to other MPSs and even to other multiply handicapped patients. We hope that the presentation of our findings may assist others when confronted with complex, degenerative disorders.

References

Oct 12, 1977·European Journal of Pediatrics·R A PfeifferE Sattinger
Jan 1, 1981·The American Journal of Medicine·C Fisch

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Citations

Feb 5, 1998·Otolaryngology--head and Neck Surgery : Official Journal of American Academy of Otolaryngology-Head and Neck Surgery·B C PapsinS E Leighton
Jul 18, 2002·Hearing Research·Kevin K OhlemillerMark S Sands
Jan 24, 2008·Audiology & Neuro-otology·Yang-Sun ChoDong-Kyu Jin
May 12, 2011·Proceedings of the Japan Academy. Series B, Physical and Biological Sciences·Jin-ichi Inokuchi
Jan 11, 2011·Journal of Neurochemistry·Shinji GoJin-ichi Inokuchi
Jul 5, 2015·Cardiovascular Pathology : the Official Journal of the Society for Cardiovascular Pathology·Thomas J GniadekMarc K Halushka
Feb 26, 2016·Journal of Medical Genetics·Adriana M MontañoWilliam S Sly
Apr 8, 2006·Fetal Diagnosis and Therapy·Narayanaswamy Venkat-RamanKarl W Murphy
Aug 1, 1992·Journal of Speech and Hearing Research·J E ShanksL Schulte
Nov 18, 2018·Italian Journal of Pediatrics·Rita BaroneSerena Gasperini
Jul 13, 2021·Molecular Genetics and Metabolism·Julie B EisengartCara O'Neill

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