ENT characteristics and therapeutic results in multisystemic disorders of mitochondrial encephalomyopathy

Table 2 PTA degrees of MEM subtypes

MEM subtypes	Normal hearing ≤ 25 dB HL	PTA degrees(mean frequencies of 0.5, 1, 2,4 kHz) ^[1]				SNHL/subtypes (N%)
MEM subtypes	Normal hearing ≤ 25 dB HL	Mild 26–40 dB HL	Moderate 41–60 dB HL	Severe 61–80 dB HL	Profound ≥ 81 dB HL	SNHL/subtypes (N%)
CPEO	7(22.6)	2(6.5)	2(6.5)	2(6.5)	2(6.5)	8/15(53.3)
MELAS	2(6.5)	0(0)	1(3.2)	1(3.2)	1(3.2)	3/5(60)
KSS	1(3.2)	1(3.2)	1(3.2)	2(6.5)	0(0)	4/5(80)
MIDD	0(0)	2(6.5)	1(3.2)	0(0)	1(3.2)	4/4(100)
LHON	3(9.7)	0(0)	0(0)	0(0)	0(0)	0/3(0)
Total	13(41.9)	5(16.1)	5(16.1)	5(16.1)	3(9.7)	19/32(59.4)

CPEO chronic progressive external ophthalmoplegia, MELAS mitochondrialencephalomyopathy lactic acidosis stroke-likeepisodes, KSS Kearns-Sayre syndrome, MIDD maternally inherited diabetes and deafness, LHON Leber hereditary optic neuropathy

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