Otological and audiological aspects in turner syndrome

The ear and auditory function were studied in 62 subjects with Turner syndrome. The most frequent abnormality of the external ear was low setting of the auricles. Thirty patients were affected by chronic or recurrent middle-ear infection or by the sequelae of middle-ear disease, with a consequent conductive hearing loss (mean PTA 24.4 dBHL) in 16 cases. In two subjects a conductive hearing loss (mean PTA 30 dBHL) was attributed to a tympano-ossicular malformation. Bilaterally symmetrical sensorineural heating loss (SNHL) (mean PTA 15.3 dBHL) was found in 13 cases, nine of whom showed a progressive hearing loss towards the high frequencies, and four a dip in the middle frequency range. The hearing impairment was of cochlear type in eight cases, and of retrocochlear in five. The occurrence of SNHL was correlated with the 45,X karyotype. Turner girls and their parents must be adequately informed about the importance of an early otological and audiological evaluation and follow up.