Bilateral sensorineural deafness in adenosine deaminase-deficient severe combined immunodeficiency.
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.
Albuquerque
Wendy
W
Department of Audiology, Great Ormond Street Hospital for Children NHS Trust, London, United Kingdom.
Gaspar
Hubert B
HB
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
D000243:Adenosine Deaminase