Search

Hearing impairment has frequently been described in β-thalassemia patients with a significant impact on the patients' quality of life. Most studies provided evidence of deferoxamine (DFO) dose-related ototoxicity, however, the data is scarce regarding deferasirox (DFX) as a sole iron chelator. We aimed to assess the prevalence and risk factors of sensorineural hearing loss (SNHL) and vestibular dysfunction in regularly transfused β-thalassemia patients who had been treated with DFX film coated tablets. We conducted a case control study on 57 transfusion dependent β-thalassemia patients with a mean age of 15.3 years who received DFX FCT as monotherapy for at least one consecutive year, and 57 healthy age and sex-matching controls. Comprehensive audiological evaluations using pure tone audiometry (PTA) and transient evoked otoacoustic emission (TEOAE) as well as vestibular evaluation using Video-nystagmography (VNG) were done. SNHL was identified in 12 patients (21.1 %) using PTA and a statistically significant difference was detected between controls and patients at 6 KHz and 12 KHz frequencies. A higher incidence of SNHL was detected using TEOAE, 22 patients (43.1 %) failed to pass TEOAE, with a statistically significant decrease in the signal at frequencies 1, 4 KHz bilaterally and at frequencies 1.5, 2 KHz in the right ear compared to controls. Canal paresis was detected in 21 (36.8 %) of thalassemic children using bithermal caloric test with significantly more unilateral weakness than control children (P = 0.008). We found no significant correlation between audio-vestibular dysfunction and age, sex, serum ferritin, frequency of blood transfusion and dose of DFX FCT in thalassemic children. We conclude that the incidence of SNHL and vestibular dysfunction was high among transfusion dependent β-thalassemia patients. Therefore, we recommend performing pre-treatment baseline audio-vestibular assessment and yearly audio-vestibular monitoring to early detect high risk patients and initiate timely management to prevent permanent damage. [ABSTRACT FROM AUTHOR]

Vestibular Migraine in children can mimic other disorders, especially at presentation. Outcome is hard to predict and management may be challenging due to the fact that many of the patients are too young to describe their symptoms and these are not always accompanied by headache. To assess vestibulo-ocular reflex (VOR) in pediatric patients who meet criteria for defined Vestibular Migraine and to compare results to healthy controls. Twenty-one patients aged 11–16 years were included in this prospective multicentric study. VOR was assessed using the video Head Impulse Test by EyeSeeCam®(Interacoustics, Denmark). Patients with Vestibular Migraine (VM) have higher values of gain compared to asymptomatic patients. Video Head Impulse Test (vHIT) is a useful and relatively fast-to-perform examination in children compared to other vestibular tests. Patients with VM seem to have higher values of gain at vHIT. • Gain values in video Head Impulse Test are higher in pediatric patients with VestibularMigraine. [ABSTRACT FROM AUTHOR]

Acute vestibular syndrome is most often caused by vestibular neuritis or stroke, although demyelinating diseases may be responsible for 4% of all AVS episodes. The authors present the case of a previously healthy 17-year-old female patient complaining of spontaneous vertigo and right-sided hearing loss. Otoneurological examination suggested a peripheral vestibular cause and video head impulse test revealed a reduced vestibulo-ocular reflex gain. The presence of sensorineural hearing loss raised the suspicion of a central cause and prompted imaging evaluation. A brain MRI evidenced demyelinating lesions in the right middle cerebellar peduncle and the patient was ultimately diagnosed with Multiple Sclerosis. [ABSTRACT FROM AUTHOR]