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

Table 3.

Characteristics of 13 children with Pompe disease, and the amount of hearing loss

Patient	Age at hearing test (years)	Gender (M/F)	Motor status^a	Respiratory function^a	Hearing loss (dB)^a
Patient	Age at hearing test (years)	Gender (M/F)	Motor status^a	Respiratory function^a	(right ear)	(left ear)
1	13.1	M	Ambulant, prox weak	Normal	nh	nh
2	6.0	M	Ambulant, prox weak	Normal	33.3 cond	nh
3	12.7	F	Ambulant, prox weak	Diminished	nh	nh
4	11.9	M	Partially wheelchair dep.	Normal	nh	nh
5	12.7	F	Tetraplegic	Invasive ventilation	35 cond	35 cond
6	5.2	M	Ambulant	Normal	nh	nh
7	3	M	Ambulant, prox weak	Normal	nh	nh
8	16	M	Ambulant	Diminished	nh	nh
9	13	M	Ambulant, prox weak	Diminished	nh	nh
10	9	F	Ambulant, prox weak	Normal	nh	nh
11	15.2	M	Ambulant, prox weak	Diminished, BIPAP	nh	nh
12	16.4	F	Paresis legs, scoliosis	Diminished, BIPAP	nh	nh
13	9.2	F	Partially wheelchair dep.	Diminished, BIPAP	nh	nh

dB Decibels, nh normal hearing, cond conductive hearing loss, prox weak proximal weakness, BIPAP bi-level positive airway pressure

^aBefore start of enzyme replacement therapy