Table 1.
Literature overview of hearing impairment in beta-thalassemia.
| Author and Year | # | Age | Phenotype | ICT | HL | SNHL | CHL | MHL | Method | HL Definition |
|---|---|---|---|---|---|---|---|---|---|---|
| De Virgiliis et al., 1979 [18] | 75 | ped. | TDT | DFO | 73.3% | 57.3% | 16% | n.r. | PTA | >20 dB in any frequency |
| Marsh et al., 1981 [46] | 1 | adult | NTDT | DFO | 0 | 0 | 0 | 0 | clinical interview | Not Applicable |
| Orton et al., 1985 [47] | 2 | n.r. | TDT | DFO | 100% | 100% | 0 | 0 | n.r. | n.r. |
| Barrat et al., 1987 [19] | 25 | mixed | TDT | DFO | 36% | 36% | 0 | 0 | PTA | >20 dB in any frequency |
| Albera et al., 1988 [20] | 153 | ped. | TDT | DFO | 71% | 71% | 15% | n.r. | PTA | >20 dB in any frequency |
| Masala et al., 1988 [16] | 100 | mixed | TDT | DFO | 24% | 12% | 12% | n.r. | PTA | >10 dB in any frequency |
| Cohen et al., 1990 [21] | 27 | mixed | TDT | DFO | 0 | 0 | 0 | 0 | PTA | >20 dB in any frequency |
| Porter et al., 1989 [4] *^ | 30 | mixed | TDT | DFO | 23.3% | 23.3% | 6.7% | n.r. | PTA | >20 dB in any frequency |
| Porter et al., 1989 [4] *^ | 17 | mixed | NTDT | DFO, none | 23.5% | 11.8% | 17.6% | n.r. | PTA | >20 dB in any frequency |
| Wonke et al., 1989 [34] | 50 | mixed | TDT | DFO, Ca-DTPA | 26% | 26% | n.r. | n.r. | PTA | >30 dB in any frequency |
| Triantafyllou et al., 1990 [48] | 120 | mixed | TDT | DFO | 36.6% | 10% | 15% | 12.2% | n.r. | n.r. |
| Argiolu et al., 1991 [12] | 309 | ped. | TDT | DFO | 15.5% | 15.5% | n.r. | n.r. | PTA | >20 dB in any frequency above 2000 Hz |
| Cuda et al., 1991 [35] | 50 | mixed | TDT | DFO | 26% | n.r. | n.r. | n.r. | PTA | >30 dB in any frequency or >25 dB in at least 2 frequencies |
| Sheikha et al., 1992 [39] | 3 | mixed | NTDT | n.r. | 100% | 66.6% | 33.3% | 0 | n.r. | n.r. |
| Wong et al., 1993 [49] | 34 | mixed | TDT | DFO | 11.8% | n.r. | n.r. | n.r. | BAEP | n.r. |
| Onerci et al., 1994 [22] * | 27 | mixed | TDT | DFO | 92.6% | 14.8% | 63% | 14.8% | PTA | >20 dB in any frequency |
| Onerci et al., 1994 [22] * | 7 | mixed | NTDT | none | 71.4% | 0 | 57.1% | 14.2% | PTA | >20 dB in any frequency |
| Sacco et al., 1994 [36] | 36 | mixed | TDT | DFO | 19.4 | 19.4% | n.r. | n.r. | PTA | >30 dB in any frequency |
| Kontzoglou et al., 1996 [23] | 88 | mixed | TDT | DFO | 27.3% | 27.3% | 0 | 0 | PTA | >20 dB in any frequency |
| Levine et al., 1997 [50] | 2 | adult | TDT | DFO | 50% | 50% | 0 | 0 | n.r. | n.r. |
| Meara et al., 1998 [41] | 1 | adult | NTDT | n.r. | 100% | 0 | 100% | 0 | n.r. | n.r. |
| Ambrosetti et al., 2000 [29] | 57 | adult | TDT | DFO | 33.3% | 26.3% | 7% | 0 | PTA | >25 dB in any frequency |
| Passat et al., 2001 [37] | 65 | ped. | TDT | nr | 29.2% | 27.7% | 1.5% | 0 | BAEP | >30 dB |
| Karimi et al., 2002 [24] | 128 | n.r. | TDT | DFO | 56% | 11.7 | n.r. | n.r. | PTA | >20 dB in any frequency |
| Chen et al., 2005 [30] | 25 | mixed | TDT | DFO | 20% | 20% | n.r. | n.r. | PTA | >25 dB in any frequency |
| Sonbolestan et al., 2005 [14] | 160 | n.r. | TDT | DFO | 48.7% | n.r. | n.r. | n.r. | PTA | >25 dB in any frequency or >10 dB in two sequential frequencies |
| Berjis et al., 2007 [15] | 160 | mixed | TDT | DFO | 50% | 50% | n.r. | n.r. | PTA | >20 dB in two consecutive frequencies, or > 40 dB in one frequency |
| Budak et al., 2008 [31] | 9 | adult | TDT | DFO | 16.6% | n.r. | 5.5% | 11.1% | PTA | >25 dB in any frequency |
| Delehaye et al., 2008 [25] | 60 | mixed | TDT | DFO | 50% | 50% | 0 | 0 | PTA, DPOAE | >20 dB at PTAv or increase of at least 20 dB in any frequency at follow up |
| Shamsian et al., 2008 [17] | 67 | mixed | TDT | DFO | 7.4% | 7.4% | 0 | 0 | PTA | >15 dB in any frequency |
| Thio et al., 2008 [42] | 1 | ped. | TDT | DFO | 100% | 0 | 100% | 0 | n.r. | n.r. |
| Faramarzi et al., 2010 [13] | 293 | mixed | TDT | DFO | 3.5% | 3.5% | 0 | 0 | PTA | >20 dB in any frequency |
| Vir et al., 2010 [51] | 26 | mixed | TDT | DFO, DFP | n.r. | n.r. | n.r. | n.r. | PTA | >= 26 dB in any frequency |
| Chao et al., 2013 [26] | 37 | mixed | TDT | DFO, DFP | 35.1% | n.r. | n.r. | n.r. | PTA | >20 dB in any frequency |
| Uygun et al., 2013 [52] | 169 | mixed | TDT | mixed | 14% | n.r. | n.r. | n.r. | n.r. | n.r. |
| Pereira da Silva et al., 2015 [53] | 2 | ped. | mixed | none | 100% | 0 | 100% | 0 | PTA | n.r. |
| Osma et al., 2015 [27] | 159 | mixed | mixed | mixed | 39% | 39% | n.r. | n.r. | PTA | >20 dB in any frequency |
| Bhardwaj et al., 2016 [33] | 30 | n.r. | TDT | mixed | 23.3% | n.r. | n.r. | n.r. | PTA, DPOAE | Average of 2, 4 and 8 kHz >25 dB |
| Badfar et al., 2017 [6] | 1422 | n.r. | TDT | DFO | 27.3% | 10.6% | 14.6% | 9.1% | n.r. | n.r. |
| Lanigan et al., 2017 [40] | 1 | ped. | TDT | DFX + DFO | 100% | 0 | 100% | 0 | PTA, OAE | n.r. |
| Hasan et al., 2018 [54] | 23 | mixed | TDT | DFO, none | 26.1% | 4.3% | 21,7% | n.r. | n.r. | n.r. |
| Sirisena et al., 2018 [43] | 1 | ped. | TDT | DFX | 100% | 0 | 100% | 0 | n.r. | n.r. |
| Alzaree et al., 2019 [28] | 48 | ped. | TDT | DFO | 23/29%§ | 23/29%§ | 0 | 0 | PTA, DPOAE | >20 dB in any frequency |
| Khan et al., 2019 [55] | 198 | mixed | TDT | DFX | 45.5% | 45.5% | n.r. | n.r. | PTA | n.r. |
| Khalaf et al., 2020 [56] | 100 | mixed | TDT | nr | 8% | 8% | n.r. | n.r. | n.r. | n.r. |
| Manara et al., 2021 [32] * | 51 | adult | TDT | mixed | 58.8% | 46% | 5.9% | 7.8% | PTA | >25 dB in any frequency |
| Manara et al., 2021 [32] * | 20 | adult | NTDT | mixed, none | 35% | 25% | 5% | 5% | PTA | >25 dB in any frequency |
Abbreviations: TDT: Transfusion dependent Thalassemia; NTDT: Non Transfusion dependent Thalassemia; HL: hearing loss; SNHL: Sensorineural Hearing Loss; CHL: Conductive Hearing Loss; MHL: Mixed hearing Loss; PTA: pure-tone audiometry; PTAv: pure-tone average; ICT: iron chelation therapy; DFO: deferoxamine; DFX: deferasirox; DFP: deferiprone; Ca-DTPA: calcium-diethylene triamine penta-acetic acid; BAEP: Brainstem Auditory Evoked Potentials; DPOAE: Distortion Product Otoacustic Emissions; OAE: Otoacustic Emissions. * Disaggregate data according to beta-thalassemia phenotype are presented for Porter, Onerci, and Manara. ^ one patient on deferoxamine with conductive hearing loss due to otosclerosis is mentioned, without reporting whether TDT or NTDT. This patient was not counted in the prevalence rate. § data refer to right and left ear; data on patients were not available.