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Karger AG","issue":"6","license":[{"start":{"date-parts":[[2011,1,1]],"date-time":"2011-01-01T00:00:00Z","timestamp":1293840000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/www.karger.com\/Services\/SiteLicenses"},{"start":{"date-parts":[[2011,1,1]],"date-time":"2011-01-01T00:00:00Z","timestamp":1293840000000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.karger.com\/Services\/SiteLicenses"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Audiol Neurotol"],"published-print":{"date-parts":[[2011]]},"abstract":"<jats:p>\u2018Auditory neuropathy\u2019 (AN), the term used to codify a primary degeneration of the auditory nerve, can be linked &lt;i&gt;directly&lt;\/i&gt; or &lt;i&gt;indirectly&lt;\/i&gt; to mitochondrial dysfunction. These observations are based on the expression of AN in known mitochondrial-based neurological diseases (Friedreich\u2019s ataxia, Mohr-Tranebj\u00e6rg syndrome), in conditions where defects in axonal transport, protein trafficking, and fusion processes perturb and\/or disrupt mitochondrial dynamics (Charcot-Marie-Tooth disease, autosomal dominant optic atrophy), in a common neonatal condition known to be toxic to mitochondria (hyperbilirubinemia), and where respiratory chain deficiencies produce reductions in oxidative phosphorylation that adversely affect peripheral auditory mechanisms. 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