Table 3.

Hearing aid outcomes

Publication	Cases	Outcomes
Chiu et al. (2010)	6	“All the 6 homozygotes had limited benefits from hearing aids”
Costa et al. (2012)	2	“Could not tell whether the hearing aid was working or not” “The child obtained no benefits from the device”
Loundon et al. (2005)	1	“The mean threshold in free field… was 75 dB with hearing aids”
Marlin et al. (2010)	3	“Pure-tone hearing levels improved by 10… and 15 dB… with hearing aid amplification, but did not dramatically improve vocal audiometry performance”
Rouillon et al. (2006)	2	“No improvement was seen with the bilateral powerful hearing aid”
Runge et al. (2013)	2	“Aided benefit was reportedly limited to inconsistent increase in sound awareness”
Santarelli et al. (2015)	8	“Aided thresholds showed a marked improvement in pure tone sensitivity… however, they proved to be above the intensity range of conversational speech”
Santarelli et al. (2021)	2	“Both adult subjects had tried hearing aids without benefit”
Tang et al. (2017)	1	“He had worn hearing aids in both ears since… birth, but his ability to communicate was not improved”
Varga et al. (2003)	3	“All the children reported receiving no benefit from hearing aids”
Zadro et al. (2010)	1	“The child showed poor perceptive skills, only detection of sounds and words using hearing aids”
Zhang et al. (2013)	1	“6-month trial of conventional amplification failed to show benefit”

The outcomes of hearing aids reported in 32 individuals across 12 publications are summarized here. Reports were typically subjective, and no individual received from hearing aids benefit that improved conversational speech perception