Hearing loss in Turner syndrome: Results of a multicentric study

Abstract

One hundred and seventy-three pts, mean chronological age (CA) 12+/-6.2 yr. Patients were submitted to different therapies: GH, estrogen therapy (EE), no therapy (no tx). Seventy-nine pts (CA 11 yr) had no otological diseases. Conductive hearing loss (CHL) occurred in 38.7% (CA 11 yr) and otoscopy was: persistent secretory otitis media in 55.2%, chronic otitis media in 10.4%, pars flaccida retraction pocket in 19.4%, mostly bilateral. Cholesteatoma was present in 15%. Sensorineurinal hearing loss (SNHL) occurred in 15.6% (CA 16 yr), 11 of whom were affected by high tone loss, and 15 by loss in midfrequencies (dip between 0.5-3 kHz), bilateral in 93%. Degree of hearing loss (HL) was mild [20-40 decibel hearing level (dBHL)] in 15%, moderate (45-60 dBHL) in 31%, severe (65-80 dBHL) in 8%, profound (dBHL>85) in 2%. We found a significant association between CHL and karyotype 45, X (p<0.025), congenital cranio-facial abnormalities, prevalently with low-set ears (p<0.04), narrow and/or high arched palate (p<0.018), and micrognathia (p<0.004). Our study confirms that the high prevalence of middle ear infections and CHL in TS are probably due to growth disturbances of the structures from the first and second branchial arches. We did not find any association between EE, GH, and HL. We recommend a regular audiological follow-up, especially during childhood, to prevent important middle ear anatomic sequele and to identify HL at an early stage, as the impact on social functioning may be significant.

TRANSITION IN ENDOCRINOLOGY: Treatment of Turner's syndrome during transition

“…In younger patients, CHL occurred in 38% and persistent secretory otitis media in 55%. SNHL frequency was similar (94).…”

“…(93,95). In younger patients, CHL was more common (94). Recent guidelines have recommend screening at least every 2-3 years in all asymptomatic patients, and more frequently in those with established hearing loss or new symptoms of hearing problems (2).…”

Otologic and audiologic features of ethnic Chinese patients with Turner syndrome in Taiwan

“…4,5,7 Some reports suggest that CHL is associated with impaired Eustachian tube function resulted from congenital craniofacial abnormalities. 14,18 As for the correlation between variable karyotype abnormality and CHL in TS, various authors 8,14,18 have reported that monosomy 45,X and 45X/46Xi(Xq) are strongly associated with CHL. However, our study did not reveal a significant correlation between CHL and monosomy 45,X or variable craniofacial anomalies.…”

“…17 The common reasons for conductive hearing loss (CHL) in TS patients are recurrent OM with effusion, chronic OM, cholesteatoma formation (likely as the result of failure of the Eustachian tube associated with lymphedema 4 ), and the actual anatomic shortening of the skull base or congenital craniofacial abnormalities. 18 Several case studies suggest that monosomy 45,X 8,14,18 or isochromosome 46,i(Xq) 8 is significantly associated with CHL in TS patients.…”

Effects of Noise Exposure on the Auditory Function of Ovariectomized Rats with Estrogen Deficiency

“…In addition, estrogen deficiency may affect the hearing function of women [16,17] . In the laboratory, bilateral ovariectomy in female rats may be an appropriate approach to simulate the effects of estrogen deficiency on hearing function [18,19] .…”