Williams syndrome (WS) is a rather rare congenital disorder characterised by a series of cardiovascular, maxillo-facial and skeletal abnormalities. It sometimes displays otorhinolaryngological symptoms because of the relatively high incidence of secretory otitis media and hyperacusis, which may be present in up to 95% of patients. The present paper describes a case of WS associated with bilateral conductive hearing loss which was not related to secretory otitis media. Hyperacusis was, moreover, present in spite of the conductive deafness. Surgical or prosthetic treatment of hearing loss was delayed because of hyperacusis. Treatment of the hyperacusis by acoustic training, instead, yielded excellent, long-lasting remission of the symptoms.
A glomus tympanicum tumor that is associated with a visible retrotympanic mass is the most frequent cause of pulsatile tinnitus. The preoperative diagnostic approach to this lesion includes a meticulous physical examination as well as high-resolution CT, magnetic resonance angiography, and digital angiography, which can also be used for preoperative embolization. We report the use of color transcranial Doppler sonography in the evaluation of glomus tympanicum tumor in a 67-year-old woman with a 3-year history of left tinnitus. An otoscopic examination revealed a reddish pulsatile mass behind an intact tympanic membrane. No lesions were visualized on gray-scale sonography. Contrast-enhanced color transcranial Doppler sonography showed a vascular ovoid mass that measured 2 x 1 x 1 cm; spectral analysis of the lesion revealed arterial flow with a low resistance index. Color transcranial Doppler sonography helped define the dimensions and vascular characteristics of the lesion.
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