Congenital unilateral sensorineural hearing loss (uSNHL) is associated with speech-language delays and academic difficulties. Yet, controversy exists in the choice of diagnosis and intervention methods. A cross-sectional prospective design was used to study hearing loss cause in twenty infants with congenital uSNHL consecutively recruited from a universal neonatal hearing-screening program. All normal-hearing ears showed ≤20 dB nHL auditory brainstem response (ABR) thresholds (ABRthrs). The impaired ear median ABRthr was 55 dB nHL, where 40% had no recordable ABRthr. None of the subjects tested positive for congenital cytomegalovirus (CMV) infection. Fourteen subjects agreed to participate in magnetic resonance imaging (MRI). Malformations were common for all degrees of uSNHL and found in 64% of all scans. Half of the MRIs demonstrated cochlear nerve aplasia or severe hypoplasia and 29% showed inner ear malformations. Impaired ear and normal-hearing ear ABR input/output functions on a group level for subjects with ABRthrs < 90 dB nHL were parallel shifted. A significant difference in interaural acoustic reflex thresholds (ARTs) existed. In congenital uSNHL, MRI is powerful in finding a possible hearing loss cause, while congenital CMV infection may be relatively uncommon. ABRs and ARTs indicated an absence of loudness recruitment, with implications for further research on hearing devices.

• Automated estimation of tICV is in good agreement with manual tracing. • Consistent tICV estimations from repeated measurements demonstrate the robustness of the algorithm. • Automatically segmented volumes seem less variable than those from manual tracing. • Unbiased and automated tlCV estimation is possible from CT.

Objective To describe a case of asymptomatic superior semicircular canal dehiscence. Method Clinical case report. Results A 50-year-old man presenting with right-sided Ménière´s disease also showed an enhanced response on vestibular evoked myogenic potential testing for the left ear. Unilateral left-sided superior semicircular canal bone dehiscence was clearly visualised on a subsequent temporal bone computed tomography scan. These findings were consistent with superior canal dehiscence syndrome. However, the patient did not complain of any specific superior canal dehiscence syndrome symptoms. Given that vestibular evoked myogenic potential testing may detect asymptomatic forms of superior canal dehiscence, as noted in this case, such testing seems to exhibit reduced specificity for superior canal dehiscence syndrome. Conclusion An enhanced response on vestibular evoked myogenic potential testing in isolation appears to be a weaker indicator of superior canal dehiscence syndrome, and rather a marker of superior semicircular canal dehiscence.