Reversible sensori-neural hearing loss associated with sickle cell crisis.

Abstract

AbstractA 20‐year‐old black male, semicomatose in a sickle cell anemia crisis, regained consciousness with no evidence of useable clinical hearing. The patient had had no previous history of abnormal hearing or ear pathology prior to this crisis. Audiometric evaluation revealed a severe binaural sensori‐neural hearing loss. Within 11 days after regaining consciousness, the air conduction threshold averages went from levels of 65 db ISO for the left and 81 db ISO for the right to 27 db ISO for the left and 50 db ISO on the right. Speech audiometry was initially unobtainable because of the severe losses. By day 11 the speech scores were as follows: Left Ear, SRT 26 db, 88 percent discrimination. Right Ear, SRT 50 db, 38 percent discrimination. Three months later the patient had improved to the point that the air conduction thresholds averages were 15 db for the left and 39 db for the right. Speech testing thresholds had improved to SRT 14 db. 96 percent discrimination on the left and SRT 38 db, 68 percent discrimination on the right. It is postulated that the mechanism responsible for this severe hearing loss is a reversible ischemia of the stria vascularis with secondary organ of Corti hypoxia as a direct result of the sickle cell crisis.