Hyperlipidemia has been implicated as a cause of sensorineural hearing loss. Forty‐five subjects (25 male, 20 female), age 6 to 57, from families with familial hypercholestcrolemia were prospectively evaluated with pure‐tone audiometrics (from 0.25 to 8 kHz) and serum lipoprotein quantification. Twenty had no lipid abnormality (N); 25 had familial hypercholesterolemia (FHC). All had clinically normal hearing and tympanometry. In 13 FHC and 11 N under 18, hearing at 6 and 8 kHz was reduced compared to age/sex adjusted values (NL) (p<0.001). FHC compared to N were not significantly different. In 12 FHC and nine N over 18, there was reduced hearing compared to NL at selected frequencies. FHC vs N revealed reduced hearing in higher frequencies for males (3–8 kHz) (p<0.01) and in lower frequencies for females (0.25–1 kHz) (p<0.01). FHC and N from FHC families compared to NL had decreased hearing at selected frequencies at all ages. The difference between FHC and N was more pronounced in subjects over 18.