title: Audiologic and vestibular assessment in patients with β-thalassemia major receiving long-term transfusion therapy.
abstract: BACKGROUND
  Life-long transfusion therapy with chelators is a treatment choice for patients with β-thalassemia major. Some investigators have proposed auditory impairment related to the use of deferoxamine, but the mechanisms remain unclear and whether or not deferiprone has similar side effects needs to be evaluated.
  
  
  METHODS
  Thirty-seven patients with β-thalassemia major who received regular transfusion in our hospital were enrolled. Chelation agents, including deferoxamine and deferiprone, were used. To assess audiologic function, otoscopy, pure tone audiometry (PTA), tympanometry, transient evoked oto-acoustic emission (TEOAE), and auditory brainstem response (ABR) were conducted. Bithermal caloric test was performed to evaluate vestibular function.
  
  
  RESULTS
  All of the 37 patients had normal findings on otoscopic evaluation and their tympanograms were type A. Thirteen patients (35.1%) had hearing impairment at one or more frequencies as detected by PTA. Compared to those without hearing impairment, patients with hearing impairment had lower serum ferritin levels (P = 0.01). Seven of 21 patients (33.3%) failed to pass the TEOAE, while 13 (61.9%) had abnormal ABR findings. Sixteen patients (80%) had canal paresis in the caloric test.
  
  
  CONCLUSIONS
  The incidence of auditory impairment and vestibular dysfunction was high in patients with β-thalassemia major receiving long-term transfusion therapy. Potential lesions of auditory impairment may exist anywhere along the auditory pathway, from the inner ear to the brainstem. Lower serum ferritin levels may be associated with hearing impairment. Therefore, regular check-ups of serum ferritin levels and periodic audiologic assessment are mandatory.
authors: Department of Pediatrics, Chung Shan Medical University Hospital, Taichung, Taiwan; Institute of Medicine, Chung Shan Medical University, Taichung, Taiwan; School of Medicine, Chung Shan Medical University, Taichung, Taiwan.
authors: Yu-Hua
authors: YH
authors: Chao
authors: Kang-Hsi
authors: KH
authors: Wu
authors: Ching-Yu
authors: CY
authors: Lin
authors: Ming-Hsui
authors: MH
authors: Tsai
authors: Ching-Tien
authors: CT
authors: Peng
authors: Han-Ping
authors: HP
authors: Wu
authors: Chia-Der
authors: CD
authors: Lin
mesh_terms: D000158:Acoustic Impedance Tests; D000293:Adolescent; D000328:Adult; D001301:Audiometry, Pure-Tone; D001803:Blood Transfusion; D002614:Chelating Agents / Q000009:adverse effects*; D002648:Child; D000077543:Deferiprone; D003676:Deferoxamine / Q000009:adverse effects; D016057:Evoked Potentials, Auditory, Brain Stem / Q000187:drug effects; D005260:Female; D034381:Hearing Loss / Q000139:chemically induced*; D006801:Humans; D008297:Male; D011728:Pyridones / Q000009:adverse effects*; D014722:Vestibule, Labyrinth / Q000187:drug effects*; D055815:Young Adult; D017086:beta-Thalassemia / Q000628:therapy*
chemical_list: D002614:Chelating Agents; D011728:Pyridones; D000077543:Deferiprone; D003676:Deferoxamine
keywords: chelation; ototoxicity; thalassemia; transfusion