Objective: A spectrum of auditory changes with mullerian defects has been reported. In a prospective controlled study, we aimed to assess hearing function of women with mullerian abnormalities.Methods: Thirty women with mullerian defects and 50 women with normal genital tract as a control group were evaluated. Audiologic evaluation was performed to each subject which included pure tone audiometry 250-8000 Hz for air conduction, 500-4000 Hz for bone conduction measurement.Results: Twenty three of 30 patients manifested a notch at 4 or 6 kHz with a magnitude between 10 to 30 dB. In one patient there was a slight sensorineural hearing loss at 500-1000 Hz. In 15 of 50 subjects in the control group a notch of 5-25 db at 4 or 6 kHz was demonstrated. The incidence of auditory defects was significantly higher in mullerian abnormalities group (80% vs 30%) (p<0.01).Conclusion: Auditory changes in high frequencies may exist in association with mullerian abnormalities.