Table 3 Hearing outcomes among IOPD studies
From: Hearing characteristics of infantile-onset Pompe disease after early enzyme-replacement therapy
Authors | Year | Country | Patient no. | Gender | Age of ERT (day) | Hearing exam tool | Hearing outcome | Hearing impairment no. (ratio) | |
|---|---|---|---|---|---|---|---|---|---|
Male | Female | ||||||||
Kamphoven [12] | 2004 | Netherlands | 4 | NA | NA | NA | Tym, OAE, ABR, PTA | 4 hearing deficits (30–70 dB) | 4 (4/4) |
Kishnani [13] | 2006 | USA | 8 | 4 | 4 | 184.13 | OAE, ABR | 5 hearing deficits | 5 (5/8) |
Rohrbach [21] | 2010 | Switzerland | 1 | 0 | 1 | 56.00 | Tym, OAE, ABR, CPA | Mixed | 1 (1/1) |
van Capelle [20] | 2010 | Netherlands | 11 | 5 | 6 | 87.55 | Tym, OAE, ABR | 10 hearing deficits (30–90 dB) | 10 (10/11) |
Ebbink [20] | 2012 | Netherlands | 10 | 5 | 5 | 91.80 | NA | 9 hearing deficits (30–90 dB) | 9 (9/10) |
Hahn [23] | 2015 | Germany | 23 | 13 | 10 | 103.83 | NA | 43% deceased 3 hearing impairment | 3(NAa) |
Chien15 | 2015 | Taiwan | 10 | NA | NA | 21.60 | NA | 4 conductive, 2 mixed | 6 (6/10) |
Hsueh | 2021 | Taiwan | 19 | 6 | 13 | 11.05 | Tym, OAE, ABR, PTA, CPA | 2 conductive, 1 mixed, 4 sensorineural | 6 (6/19) |