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. 2010 Jul 2;33(5):597–602. doi: 10.1007/s10545-010-9144-0

Table 2.

Summary of audiometric results of the 11 classic infantile patients at baseline and after a maximum period of 6 years of enzyme replacement therapy with recombinant human α-glucosidase

Patient Age Right ear Left ear
EHT Tymp OAE I–V III–V EHT Tymp OAE I–V III–V
Baseline
 1 3 m 30 Normal Absent Normal N.A. 60 Normal Absent Normal N.A.
 2 7 m 70 Normal Absent Normal N.A. 90 Abnormal Absent Normal N.A.
 3 8 m 40 Normal Absent Normal N.A. 40 Normal Absent Normal N.A.
 4 2.5 m 40 Abnormal Absent Normal N.A. 60 Abnormal Absent Normal N.A.
 5 2 m 80 Normal Absent Normal N.A. 60 Normal Absent Normal N.A.
 6 1 m 20 Normal Present Prolonged 2.2 20 Normal Present Prolonged 2.7
 7 0.5 m 50 Normal Absent Normal N.A. 40 Normal Absent Normal N.A.
 8 0.1 m 10 Normal Present Prolonged 3.6 10 Normal Present Prolonged 2.5
 9 2 m 60 ? ? Prolonged 3.3 60 Normal Absent Normal N.A.
 10 2.5 m 40 Normal Absent Normal N.A. 40 Normal Absent Normal N.A.
 11 3.5 m 70 Abnormal Absent Normal N.A. 70 Abnormal Absent Normal N.A.
After maximum 6 years of enzyme replacement therapy
 1 6 y 80 Abnormal Absent Normal N.A. 80 Abnormal Absent Normal N.A.
 2 6 y 70 Normal Absent Normal N.A. 70 Normal Absent Normal N.A.
 3 2 y 60 Abnormal Absent Prolonged 2.3 80 Abnormal Absent Prolonged 3.3
 4 4 y 80 Abnormal Absent Normal N.A. 90 Abnormal Absent Normal N.A.
 5 4 y 80 Normal Absent Normal N.A. 90 Abnormal Absent Normal N.A.
 6 5 y 40 Abnormal Absent Prolonged 2.3 50 Abnormal Absent Prolonged 3.4
 7 5 y 70 Abnormal Absent Prolonged 2.2 50 Abnormal Absent Prolonged 1.0
 8 3 y 20 Normal Present Prolonged 2.3 20 Normal Present Prolonged 2.3
 9 3 y 80 Abnormal Absent Prolonged 2.3 80 Abnormal Absent Prolonged 2.9
 10 1 y 40 Abnormal Absent Normal N.A. 40 Abnormal Absent Normal N.A.
 11 died

m Months, y years, EHT estimated hearing threshold (dB HL, decibels hearing level), Tymp tympanogram result, OAE otoacoustic emission result, I–V auditory brainstem response inter-peak interval I to V, III–V auditory brainstem response inter-peak interval III to V (data are represented by z-scores, >1.96 is considered abnormal), N.A. not applicable