PURPOSE: To identify the main hearing disorders in individuals with Steinert's myotonic dystrophy. METHODS: Five individuals were submitted to tonal and vocal audiometry, acoustic impedance tests, examination of Eustaquian tube function, acoustic reflex investigation, and acoustic reflex decay. They also answered two questionnaires: a) Performance of auditory function; b) Psycho-acoustic-social investigation. RESULTS: The study showed that 60% of the sample presented normal hearing thresholds, 10% had conductive hearing loss and 30% had hearing loss in high frequencies. Concerning acoustic reflexes, it was observed that 30% of the subjects presented deficits at 0.5 and 1 kHz and 40% at 2 and 4 kHz. Acoustic reflex decay was observed in 10% of the sample at 0,5 and 1 kHz. CONCLUSION: The study showed that the typical auditory profile of high-frequencies hearing loss was observed in 30% of the sample.
Myotonic dystrophy; Myotonic dystrophy; Hearing disorders; Hearing; Acoustic impedance tests; Stapedius; Eustachian tube; Reflex
