Objectives: To describe the audiologic phenotype in osteogenesis imperfecta (OI).Study design: Observational study. Setting: Tertiary referral center.Patients: One hundred eighty-two patients with genetically confirmed OI, aged 3 to 89 years.Intervention: Diagnostic hearing evaluation through otoadmittance and acoustic stapedius reflex measurements, pure tone, and speech audiometry.Main outcome measure(s): Prevalence, type, severity, symmetry, and audiometric configuration of the hearing loss in OI. Progression of hearing thresholds was determined by constructing age-related typical audiograms.Results: Approximately 52.2% of all OI patients demonstrated hearing loss unilaterally (7.7%) or bilaterally (44.5%). Pure conductive, mixed and pure sensorineural hearing losses were observed in 8.5%, 37.8% and 11.6% of OI ears, respectively. Multiple linear regression revealed that thresholds progressed by 0.5 dB/year at 0.25 kHz to 0.8 dB/year at 0.8 kHz in the ears with conductive or mixed hearing loss. Pure sensorineural hearing loss progressed by less than 0.1 dB/year at 0.25 kHz to 1.2 dB/year at 8.0 kHz. Audiometric configuration was predominantly flat (70.5%) in the ears with conductive/mixed loss, and sloping (50.0%) in those with pure sensorineural loss. Conclusions:Patients with OI are at risk for hearing loss. The hearing loss in OI may initiate at a young age and is progressive. However, the rate of progression, as well as the hearing loss severity, onset and configuration depend on the type of hearing loss, which may be conductive/mixed or pure sensorineural. For both types, age-related threshold audiograms are constructed and may help the clinician to estimate the course of the hearing loss in patients with OI. In addition, they may be valuable to distinguish between hearing loss associated with OI and other similar forms of hearing loss, such as in otosclerosis.

In strength-trained, endurance-trained, and mixed-trained athletes, ventricular and vascular response assessed by 3DE, TDI, and STI underlies different adaptations of LV, RV, and aortic indexes.

An excessive alcohol intake has been reported as one of the possible causes or risk factors of 'alcoholic cardiomyopathy'. The possibility that this cardiomyopathy may improve or even reverse if the alcohol abuse has been terminated has been suggested, but unequivocal echocardiographic documentation of this improvement has never been described. This study reports the normalization of cardiac chamber dimensions and of variables of left ventricular function documented by M-mode and cross-sectional echocardiographic follow-up studies, after cessation of excessive consumption of alcohol, in three cases of alcoholic cardiomyopathy.

Tumor arising from the ceruminous glands of the external ear canal (EAC) are very rare and can present a diagnostic dilemma because of their varied clinical and histologic manifestations. To our knowledge, this is the first report to present a case of ceruminoma in pediatric age. We discuss the origin of these tumors and the importance of wide excision and of the immunohistochemistry for the diagnosis

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