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Human deafness dystonia syndrome is a mitochondrial disease:

Koehler, Carla M. ; Leuenberger, Danielle ; Merchant, Sabeeha...
Proceedings of the National Academy of Sciences.  96 (1999)  5 - p. 2141-2146 , 1999
Link: https://doi.org/10.1073/pnas.96.5.2141