title: Bilateral sensorineural deafness in adenosine deaminase-deficient severe combined immunodeficiency.
abstract: Adenosine deaminase deficiency presents with severe combined immunodeficiency and is treatable by bone marrow transplantation. With improved survival, the nonimmunologic manifestations of this condition are becoming apparent. We report a high incidence of bilateral sensorineural deafness in transplanted patients, which highlights the systemic nature of the disease.
authors.affiliation: Department of Audiology, Great Ormond Street Hospital for Children NHS Trust, London, United Kingdom.
authors.forename: Wendy
authors.initials: W
authors.lastname: Albuquerque
authors.forename: Hubert B
authors.initials: HB
authors.lastname: Gaspar
mesh_terms: D000243:Adenosine Deaminase / Q000172:deficiency*; D000293:Adolescent; D000328:Adult; D000367:Age Factors; D001301:Audiometry, Pure-Tone; D016026:Bone Marrow Transplantation; D016022:Case-Control Studies; D002648:Child; D002675:Child, Preschool; D015331:Cohort Studies; D005260:Female; D006319:Hearing Loss, Sensorineural / Q000150:complications*; D006801:Humans; D007223:Infant; D008297:Male; D016511:Severe Combined Immunodeficiency / Q000150:complications* / Q000601:surgery
chemical_list: D000243:Adenosine Deaminase