Tinnitus in a patient with beta-thalassaemia intermedia on long-term treatment with desferrioxamine

Marsh, Michael N.; Holbrook, Ian; Clark, Christine; Shaffer, Jonathan

doi:10.1136/pgmj.57.671.582

Cited by 15 publications

(3 citation statements)

References 8 publications

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“…Despite this, De Virgiliis suggested that iron overload per se was the likely cause of hearing loss, together with the bone marrow expansion. Thereafter, some cases of suspected DFO-related ototoxicity were described [ 46 , 47 ], raising the interest in monitoring hearing function in beta-thalassemia patients undergoing chelation.…”

Section: Iron Chelation and Hearing Lossmentioning

confidence: 99%

“…In addition, no study included a healthy control group or mentioned the prevalence of tinnitus in the general population. In beta-thalassemia, tinnitus prevalence rate ranged widely from 3.3% to 38% [ 4 , 15 , 30 , 35 , 44 , 46 , 57 , 62 , 65 ], so that tinnitus was defined as either rare or common, up to being the most common hearing symptom in thalassemic patients, both in subjects with normal audiogram and in those with hearing loss.…”

Section: Tinnitusmentioning

confidence: 99%

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Hearing Loss in Beta-Thalassemia: Systematic Review

Tartaglione

Carfora

Brotto

et al. 2021

JCM

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In the last half century, the life expectancy of beta-thalassemia patients has strikingly increased mostly due to regular blood transfusions and chelation treatments. The improved survival, however, has allowed for the emergence of comorbidities, such as hearing loss, with a non-negligible impact on the patients’ quality of life. This thorough review analyzes the acquired knowledge regarding hearing impairment in this hereditary hemoglobinopathy, aiming at defining its prevalence, features, course, and possible disease- or treatment-related pathogenic factors. Following PRISMA criteria, we retrieved 60 studies published between 1979 and 2021. Diagnostic tools and criteria, forms of hearing impairment, correlations with beta-thalassemia phenotypes, age and sex, chelation treatment and laboratory findings including iron overload, were carefully searched, analyzed and summarized. In spite of the relatively high number of studies in the last 40 years, our knowledge is rather limited, and large prospective studies with homogeneous diagnostic tools and criteria are required to define all the aforementioned issues. According to the literature, the overall prevalence rate of hearing impairment is 32.3%; age, sex, and laboratory findings do not seem to correlate with hearing deficits, while the weak relationship with clinical phenotype and chelation treatment seems to highlight the presence of further yet to be identified pathogenic factors.

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Section: Iron Chelation and Hearing Lossmentioning

confidence: 99%

Section: Tinnitusmentioning

confidence: 99%

Hearing Loss in Beta-Thalassemia: Systematic Review

Tartaglione

Carfora

Brotto

et al. 2021

JCM

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“…In 1979, a high rate of hearing loss (73.3%) was reported in children treated with high doses of deferoxamine, even though iron overload and bone marrow expansion were considered responsible for hearing deficit. Subsequent studies on high‐dose chelator‐related ototoxicity 17,18 eventually drove to a close monitoring of hearing function and to a resizing of chelation dosage (no more than 40 mg/kg/day) 1,19 . Since then, scarce audiological data have become available regarding low doses of deferoxamine or other chelators especially in comparison with healthy controls.…”

Section: Discussionmentioning

confidence: 99%

Hearing loss in beta‐thalassaemia: An Italian multicentre case–control study

Manara,

Brotto,

Barillari

et al. 2024

Br J Haematol

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SummaryBackgroundDespite numerous studies, the true scenario of hearing loss in beta‐thalassaemia remains rather nebulous.Materials and methodsPure tone audiometry, chelation therapy, demographics and laboratory data of 376 patients (mean age 38.5 ± 16.6 years, 204 females, 66 non‐transfusion‐dependent) and 139 healthy controls (mean age 37.6 ± 17.7 years, 81 females) were collected.ResultsPatient and control groups did not differ for age (p = 0.59) or sex (p = 0.44). Hypoacusis rate was higher in patients (26.6% vs. 7.2%; p < 0.00001), correlated with male sex (32.6% in males vs. 21.8% in females; p = 0.01) and it was sensorineural in 79/100. Hypoacusis rate correlated with increasing age (p = 0.0006) but not with phenotype (13/66 non‐transfusion‐dependent vs. 87/310 transfusion‐dependent patients; p = 0.16). Sensorineural‐notch prevalence rate did not differ between patients (11.4%) and controls (12.2%); it correlated with age (p = 0.01) but not with patients' sex or phenotype. Among adult patients without chelation therapy, the sensorineural hypoacusis rate was non‐significantly lower compared to chelation‐treated patients while it was significantly higher compared to controls (p = 0.003).ConclusionsSensorineural hypoacusis rate is high in beta‐thalassaemia (about 21%) and it increases with age and in males while disease severity or chelation treatment seems to be less relevant. The meaning of sensorineural‐notch in beta‐thalassaemia appears questionable.

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2001

The Thalassaemia Syndromes

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Tinnitus in a patient with beta-thalassaemia intermedia on long-term treatment with desferrioxamine

Cited by 15 publications

References 8 publications

Hearing Loss in Beta-Thalassemia: Systematic Review

Hearing Loss in Beta-Thalassemia: Systematic Review

Hearing loss in beta‐thalassaemia: An Italian multicentre case–control study

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