Here we report a novel consanguineous family with arOA and peripheral neuropathy (in the proband only) but without apparent auditory neuropathy, in which we demonstrate linkage to the second reported locus on chromosome 11q14.1-q21, OPA7 (OMIM 612989), and identify the recurrent mutation p.Arg55X at homozygous state in TMEM126A in the three affected patients. The gene discussed is TMEM126A; the disease is auditory neuropathy.