A study in the July 4 issue of JAMA has found that patients with the genetic disorder von Hippel-Lindau disease may suddenly experience hearing loss.
A tumor associated hemorrhage in the inner ear—endolymphatic sac tumors (ELSTs)— occur sporadically in von Hippel-Lindau disease patients, but may be associated with the disease. ELSTs may cause significant dysfunction of hearing and balance, including sudden irreversible hearing loss.
The study followed 35 patients with von Hippel-Lindau disease and ELSTs in 38 ears were identified. Sensorineural hearing loss (SNHL) occurred in 31 patients (89%) or 34 of the affected ears (89%) and was frequently moderate or profound.