Hearing Defects in Males with Sex Chromosome Anomalies

Abstract

Hearing was studied in 26 clinically and cytogenetically analysed men with different types of sex chromosome anomaly usually 47,XXY (Kline-felter's syndrome). The number of hearing threshold impairments of probably endogenous origin was unusually high (19%). In addition, several cases demonstrated small but distinot dips in the middle frequency range. This finding is of interest since this specific type of hearing threshold pattern is also recorded in nearly half of the cases with another sex chromosome anomaly, Turner's syndrome. Furthermore, a similar threshold configuration is found in presumptive carriers of autosomal recessive genes for deafness. In contrast to the patients with Turner's syndrome, pathologically high stapedius-reflex thresholds were recorded in 35% of the present material.