Relationships between neurologic disorders and hereditary hearing loss.
Hearing loss is a common disorder that often has a neurologic etiology. Recently, there has been significant progress in the discovery of the genes that cause sensorineural hearing loss, and this has led to increased understanding of the pathophysiology of both syndromic and nonsyndromic hearing problems. These genes cover the range of processes involved in neurologic development and function, including structural genes, transcription factors, and tumor suppressors; genes involved in signal transduction processes, such as ion homeostasis and intracellular transport; and mitochondrial genes responsible for oxidative phosphorylation and energy production. Interactions between genes as well as between genes and environmental factors have also been documented. Understanding of these processes should lead to earlier and more accurate diagnosis and more effective treatment for neurologic disorders and hearing loss.