Table 1.

Audiological abnormalities in FRDA individuals

Study	Audiological tests	Hearing impairment	GAA correlation performed?	No of patients	Age range	GAA range
Jabbari et al., 1983 [5]	ABR, ART, Tymp	100% abnormal ABR	No	5		-
Durr et al., 1996 [1]	PTA, ABR	13% abnormal PTA, 61% abnormal ABR	Yes (no correlation was found)	140 (only 69 had ABR)	7–77	120–1700
Santoro et al., 2000 [14]	ABR	-	Yes (no correlation was found)	24	9–43	200–1093
Rance et al., 2008 [10]	PTA, ABR, ART, speech in noise test	30% abnormal ABR and ART, 90% abnormal speech in noise	Yes, (no correlation was found)	10	8–28	447–780
Rance et al., 2010 [4]	PTA, OAEs, ABR, temporal processing test	64% with temporal processing deficit, 50% abnormal ABR,	Yes, (significant correlation was found only between amplitude modulation detection and GAA1)	14	16–52	447–1099
Rance et al., 2012 [8]	PTA, ABR, LiSN-S	52% abnormal ABR, 22% abnormal PTA (4 freq. average)	Yes (no correlation was found between LiSN-S subscores and GAA1)	23	9–55	447–1298
Zeigelboim et al., 2018 [15]	PTA, ABR, Immittance	43% abnormal PTA, 57% abnormal ABR, 50% abnormal immittance	No	30	6–72	-
Giroudet et al., 2018 [16]	PTA, OAEs, standard ABR, split ABR, speech in noise	24% abnormal PTA, 75% abnormal speech in noise, 92% abnormal standard ABR, 38% abnormal split ABR	No	37	12–63	-
Koohi et al., (present study)	PTA, OAEs, ABR, SiQ, SiB, LiSN-S, GIN	45% abnormal PTA, 15% abnormal OAEs, 77% abnormal ABR, 77% abnormal SiQ, 100% abnormal SiB, 95% abnormal LiSN-S, 77% abnormal GIN	Yes	27	17–58	100–1050

ABR, auditory brainstem response; PTA, pure-tone audiometry; Tymp, tympanometry; ART, acoustic reflex threshold; OAEs, otoacoustic emissions; GIN, gaps in noise; LiSN-S, Listening in Spatialized; Noise Sentences Test SiB, speech in babble test; SiQ, speech in quiet