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). This evidence concerns the gene TIMM8A and deafness dystonia syndrome.