Radiologische und klinische Aspekte bei Patienten mit erweitertem Ductus und Saccus endolymphaticus und sensorineuraler Schwerhörigkeit
The large endolymphatic duct and sac (LEDS) is the most common radiological finding in patients with suspected inner ear malformation. Nevertheless LEDS is relatively unknown among general radiologists. The aims of this study were to evaluate the incidence of LEDS in the own patient population and to present our experiences regarding imaging findings, clinical presentation and follow up. Between 1994 and 2002 we retrospectively identified among 169 patients in whom an inner ear malformation was
... ar malformation was clinically suspected 17 patients with LEDS. In 10 patients no other abnormalities were detected, seven patients showed additional inner ear abnormalities. Audiometric data revealed sensorineural hearing loss in 22 ears, deafness in 5 ears and normal hearing in 1 case of 28 ears. In 67% the hearing loss was downward-fluctuating progressive. Twelve patients had partly repeated sudden hearing losses. A trigger for worsening of hearing was found in five patients. A correlation between the severity of morphological changes on imaging and the degree of hearing disturbances could not be detected. Our observations indicate that LEDS might be the cause of progressive hearing loss and repeated acute hearing losses in children and young adults. Even if there is no causal therapy an early diagnosis is needed to explain the patient the cause of his hearing loss, the prognosis and rules of behaviour which can delay possibly a progression.