Interestingly, mitochondrial dysfunction is indicated to underlie some other deafness-dystonia syndromes e.g. Mohr–Tranebjaerg syndrome with defects in TIMM8A. TIMM8A is located in the mitochondrial intermembrane space and functions in mitochondrial morphology (Engl et al., 2012). Here, TIMM8A is linked to Mohr-Tranebjaerg syndrome.