{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,8]],"date-time":"2026-05-08T00:57:02Z","timestamp":1778201822013,"version":"3.51.4"},"reference-count":13,"publisher":"Wiley","issue":"9","license":[{"start":{"date-parts":[[2006,8,9]],"date-time":"2006-08-09T00:00:00Z","timestamp":1155081600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Eur J Clin Investigation"],"published-print":{"date-parts":[[2006,9]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p><jats:bold>Background\u2002<\/jats:bold> Fabry disease is an X\u2010linked lysosomal storage disorder characterized by multi\u2010organ dysfunction, including hearing loss \u2013 mainly sensorineural. The recent introduction of enzyme replacement therapy (ERT) has resulted in improvements in renal and cardiac function, pain and quality of life. One study has also suggested small improvements in high\u2010frequency hearing. In this paper, we study the effect of ERT on hearing in patients in the Europe\u2010wide database \u2013 the Fabry Outcome Survey (FOS).<\/jats:p><jats:p><jats:bold>Materials and methods\u2002<\/jats:bold> Twenty\u2010six patients in FOS had pure\u2010tone audiometry performed up to 6\u00a0months before starting ERT with agalsidase alpha and after a median of 12\u00a0months of treatment. We assessed changes in hearing thresholds, expressed as deviations from the 50th centile of the normal population (International Organization for Standardization ISO 7029) to correct for age\u2010related non\u2010specific hearing deterioration.<\/jats:p><jats:p><jats:bold>Results\u2002<\/jats:bold> Hearing did not change significantly in ears with normal hearing (less than 10\u00a0dB deviation from the 50th centile of ISO 7029) or those with severe hearing loss (more than 40\u00a0dB deviation from the 50th centile of ISO 7029) at baseline. In ears with a mild or moderate hearing loss at baseline, hearing thresholds, expressed as deviations from the normal 50th centile, improved significantly by 4\u20137\u00a0dB at most frequencies (<jats:italic>P\u00a0&lt;<\/jats:italic>\u00a00\u00b705).<\/jats:p><jats:p><jats:bold>Conclusions\u2002<\/jats:bold> Agalsidase alpha stabilizes, and possibly improves, hearing in Fabry patients who have not already progressed to severe hearing loss. Further follow\u2010up of these patients will determine the longer\u2010term effects of ERT.<\/jats:p>","DOI":"10.1111\/j.1365-2362.2006.01701.x","type":"journal-article","created":{"date-parts":[[2006,8,9]],"date-time":"2006-08-09T08:45:24Z","timestamp":1155113124000},"page":"663-667","source":"Crossref","is-referenced-by-count":38,"title":["Agalsidase alpha and hearing in Fabry disease: data from the Fabry Outcome Survey"],"prefix":"10.1111","volume":"36","author":[{"given":"D.","family":"Hajioff","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"S.","family":"Hegemannn","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"G.","family":"Conti","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"M.","family":"Beck","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"G.","family":"Sunder\u2010Plassmann","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"U.","family":"Widmer","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"A.","family":"Mehta","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"A.","family":"Keilmann","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2006,8,9]]},"reference":[{"key":"e_1_2_6_2_2","doi-asserted-by":"publisher","DOI":"10.1001\/jama.281.3.249"},{"key":"e_1_2_6_3_2","doi-asserted-by":"publisher","DOI":"10.1006\/rwgn.2001.0443"},{"key":"e_1_2_6_4_2","doi-asserted-by":"publisher","DOI":"10.1517\/14712598.2.3.325"},{"key":"e_1_2_6_5_2","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2362.2004.01309.x"},{"key":"e_1_2_6_6_2","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2362.2004.01424.x"},{"key":"e_1_2_6_7_2","doi-asserted-by":"publisher","DOI":"10.1136\/jmg.38.11.769"},{"key":"e_1_2_6_8_2","doi-asserted-by":"publisher","DOI":"10.1136\/jmg.38.11.750"},{"key":"e_1_2_6_9_2","doi-asserted-by":"publisher","DOI":"10.1186\/1471-2350-3-10"},{"key":"e_1_2_6_10_2","doi-asserted-by":"crossref","first-page":"33","DOI":"10.1111\/j.1651-2227.2003.tb00219.x","article-title":"Auditory and vestibular findings in Fabry disease: a study of hemizygous males heterozygous females","volume":"443","author":"Conti G","year":"2003","journal-title":"Acta P\u00e6diatr Suppl"},{"key":"e_1_2_6_11_2","doi-asserted-by":"crossref","unstructured":"HegemannS HajioffD ContiG BeckM Sunder\u2010PlassmannG.Hearing loss in Fabry disease: data from FOS \u2013 the Fabry Outcome Survey.Eur J Clin Invest2006;36:654\u201362.","DOI":"10.1111\/j.1365-2362.2006.01702.x"},{"key":"e_1_2_6_12_2","doi-asserted-by":"publisher","DOI":"10.1023\/B:BOLI.0000009948.86528.72"},{"key":"e_1_2_6_13_2","unstructured":"International Organization for Standardization (2000). Acoustics \u2013 statistical distribution of hearing thresholds as a function of age ISO 7029:2000.http:\/\/www.iso.org."},{"key":"e_1_2_6_14_2","doi-asserted-by":"publisher","DOI":"10.1177\/000348948909800509"}],"container-title":["European Journal of Clinical Investigation"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1111%2Fj.1365-2362.2006.01701.x","content-type":"unspecified","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/j.1365-2362.2006.01701.x","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,10,13]],"date-time":"2023-10-13T06:54:53Z","timestamp":1697180093000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1111\/j.1365-2362.2006.01701.x"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2006,8,9]]},"references-count":13,"journal-issue":{"issue":"9","published-print":{"date-parts":[[2006,9]]}},"alternative-id":["10.1111\/j.1365-2362.2006.01701.x"],"URL":"https:\/\/doi.org\/10.1111\/j.1365-2362.2006.01701.x","archive":["Portico"],"relation":{},"ISSN":["0014-2972","1365-2362"],"issn-type":[{"value":"0014-2972","type":"print"},{"value":"1365-2362","type":"electronic"}],"subject":[],"published":{"date-parts":[[2006,8,9]]}}}