Three patients from two families had an unusual phenotypical variant of late-onset hexosaminidase-A deficiency. The clinical picture was dominated by spinal motor neuron involvement mimicking juvenile-onset spinal muscular atrophy. Atypical features included prominent muscle cramps, postural and action tremor, recurrent psychosis, incoordination,… (More)
In the Australian red-claw crayfish Cherax quadricarinatus (von Martens) (Decapoda, Parastacidae), a gonochoristic species, seven different combinations of intersex individuals (with both male and female genital openings) have been described. However, to date, the genetic basis for this phenomenon has not been investigated. This study was designed to test a… (More)
After experiencing gradual, progressive sensorineural hearing loss, a patient underwent cochlear implant (CI) surgery. Postoperatively, the patient experienced vestibular symptoms with no improvement in hearing. High-resolution temporal bone CT scanning demonstrated extracochlear positioning of the CI electrode in the superior semicircular canal.
A technique to quantify the tandem Romberg test (posturography) was used to study ten patients with otosclerosis before (series 1), 48 hours after (series 2), and between two to four months after (series 3) a stapedectomy. The results were analyzed by power spectrum analysis and then compared to findings from electronystagmographic testing performed with… (More)