Hearing loss in Turner syndrome: results of a multicentric study.

UNLABELLED: The purpose of this article was to evaluate otological diseases in 173 patients (pts) with Turner syndrome (TS). STUDY DESIGN: 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.

Comparative evaluation of ABR abnormalities in patients with and without neurinoma of VIII cranial nerve.

Although the diagnostic reliability of auditory brainstem responses (ABR) in acoustic neuromas has been revised due to its poor sensitivity (demonstrated above all in smaller tumours), and its limited specificity, this method is still used as the initial otoneurological approach. To contribute to the clinical use of this method, in particular with the aim of reducing the number of false positives, a retrospective study was carried out in two groups of patients affected by unilateral sensorineural hearing loss with auditory brainstem response abnormalities: in the first group (50 cases: true positives) hearing loss was the expression of an acoustic neuroma shown by magnetic resonance imaging, in the second group (130: false positives) magnetic resonance imaging was negative. In both groups, auditory brainstem response recordings showed abnormalities suggesting retro-cochlear disorders such as: (1) complete absence of response not justified by the extent of the hearing loss, (2) presence of only wave I, (3) increase in wave V absolute latency with normal I-V interpeak latency, (4) increase in wave V absolute latency, the sole component, 5) increase in wave V absolute latency and I-V interpeak latency. A comparison between the two groups made it possible to show that the finding of "major" auditory brainstem response alterations (complete absence of response not justified by the extent of the hearing loss or presence of only wave I) is correlated with a high probability of the presence of a neuroma, while other abnormalities (wave V latency and I-V interpeak latency increase) have no particular predictive value since percentages are almost identical in the two groups. Wave V latency increase with normal I-V interpeak latency was observed in only one case of acoustic neuroma and this clinical finding is not easy to interpret. It would not appear possible, based on current knowledge, to further improve the reliability of this test, and, therefore, its use in oto-neurological diagnostics remains limited.

[Computerized stabilometry in the monitoring of antiepileptic therapy in children]. / La stabilometria computerizzata nel monitoraggio della terapia antiepilettica in età pediatrica.

Computerized stabilometry is an useful test to monitor postural effects of anticonvulsant therapy in adults. Our study was carried out on 65 epileptic children: 51 were treated with CBZ or PHT or VPA or PB in monotherapy, and 14 were not on therapy, in the aim to observe abnormalities of postural control in pediatric population. Computerized stabilometry has to be considered auxiliary monitoring to evaluate toxic effect of anticonvulsant therapy in children.