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Damaged sensory hairs (stereocilia) of the hair cells; damaged hair cells degenerate and die. In humans and other mammals, dead hair-cells are never replaced; the resulting hearing loss is permanent. Inflammation of the exposed areas. This inflammation causes poor blood flow in the exposed blood vessels (vascular stasis), and poor oxygen supply for the liquid inside the cochlea (endolymphatic hypoxia) Those noxious conditions worsen the damaged hair cell degeneration. Synaptic damages, by excitotoxicity. Noise overstimulation causes an excessive release of glutamate, causing the postsynaptic bouton to swell and burst. However the neuron connection can be repaired, and the hearing loss only caused by the "wiring" (i.e. excitotoxicity) can thus be recovered within 2–3 days. Hair cell damage or death
When the ear is exposed to excessive sound levels or loud sounds over time, the overstimulation of the hair cells leads to heavy production of reactive oxygen species, leading to oxidative cell death. In animal experiments, antioxidant vitamins have been found to reduce hearing loss even when administered the day after noise exposure. They were not able to fully prevent it. Antioxidants however do not seem to be effective in protecting the human ear. Damage ranges from exhaustion of the hair (hearing) cells in the ear to loss of those cells. NIHL is, therefore, the consequence of overstimulation of the hair cells and supporting structures.
It is unclear how a reduced amount of this protein during development leads to learning disabilities, overgrowth, and the other features of Sotos syndrome.About 95 percent of Sotos syndrome cases occur by spontaneous mutation. Most of these cases result from new mutations involving the NSD1 gene. A few families have been described with more than one affected family member. These inherited cases enabled researchers to determine that Sotos syndrome has an autosomal dominant pattern of inheritance. Diagnosis
Diagnosis is based on physical examination, looking for excessive growth among other symptoms. There are no biochemical markers for the disease. Treatment
Treatment is symptomatic. There is no standard course of treatment for Sotos syndrome. Prognosis
Sotos syndrome is not a life-threatening disorder and patients may have a normal life expectancy. Developmental delays may improve in the school-age years; however, coordination problems may persist into adulthood, along with any learning disabilities and/or other physical or mental issues. Epidemiology
Incidence is approximately 1 in 14,000 births. See also
Beckwith-Wiedemann syndrome
Perlman syndrome
Proteus syndrome

References
External links
 Media related to Sotos syndrome at Wikimedia Commons
sotos at NIH/UW GeneTests