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At least 649 records · Page 36Linked to original sources
PubMed2003

Hypotonia, congenital hearing loss, and hypoactive labyrinths.

The records of all patients attending a neurosensory genetics clinic over an 11-year period were reviewed. Of the 450 patients seen, 31 presented with sensorineural hearing loss, hypotonia, and delay in the acquisition of motor milestones. Of these, 4 children were found who did not have an etiologic diagnosis such as Down syndrome or cerebral palsy. Vestibular testing revealed hypoactive labyrinthine function in all 4 of the cases, and careful imaging of the temporal bone showed anomalous development of the cochlea, vestibule, and semicircular canals in 3 of the 4 cases. None of the patients had ataxia, tremor, or significant nystagmus. Over time, the hypotonia improved in all, and none were felt to have cognitive deficits. These cases demonstrate that hypoactive labyrinthine function may be associated with hypotonia that is severe enough to result in delayed acquisition of motor milestones. The patients followed the typical remitting course of "benign congenital hypotonia." The distinguishing clinical feature is the presence of moderate to profound sensorineural hearing loss in all of the patients.

Cochlea↗
PubMed2005

Superior canal dehiscence is not due to cephalic displacement of the labyrinth.

Superior semicircular canal dehiscence syndrome may have a congenital basis. CT scans of 44 control and 21 dehiscent superior canals were examined to determine if dehiscent canals were more cephalically placed or vertically oriented than control canals. Results showed that neither was the case. Instead, the defect may be in the process of ossification above the superior canal.

Ear, Inner↗
PubMed1991

Multichannel cochlear implant and electrically evoked auditory brainstem responses in a child with labyrinthitis ossificans.

Ossification of the cochlea following meningitis presents a surgical challenge. Electrode mapping, especially in the young child, is difficult given the uncertainty of electrode contact with viable neural elements. This paper reviews surgical technique and the use of auditory brainstem responses to map the electrodes. A 4-year-old child deafened by meningitis at age 20 months had bilateral cochlear ossification by computed tomography. At surgery, a canal wall-down mastoidectomy and closure of the ear canal were performed. A trough around the modiolus was drilled, and the electrode array was placed in it. Post-operatively, the patient gave aversive or no responses to electrode stimulation. To assess electrode function, auditory brainstem responses to individual electrode activation were obtained under general anesthesia. Functioning electrodes could thus be selected for mapping. The patient now responds well to sound.

Child, Preschool↗
PubMed1992

The incidence and distribution of cupular deposits in the labyrinth.

Findings of large basophilic staining deposits on the cupula of the posterior semicircular canal ampulla have been used in part to explain the clinical phenomenon of benign positional vertigo (BPV). Although it is generally agreed that cupulolithiasis may involve other canal ampullae, the precise nature, distribution, and origin of these deposits remains unclear. In order to provide a better understanding of this finding, a series of 566 temporal bone specimens from the Ear Pathology Research Laboratory at the University of Toronto were reviewed. The results from this survey and speculations concerning the nature and formation of these deposits are discussed.

Calculi↗
PubMed1993

Development of high endothelial venule-like characteristics in the spiral modiolar vein induced by viral labyrinthitis.

The postcapillary venule is known to be the preferred site of lymphocyte migration into the extravascular compartment. In this study, induction of these high endothelial venule (HEV)-like vessels has been investigated by inoculation of live cytomegalovirus or inactivated virus into scala tympani of guinea pig cochleas. Endothelial cells of the spiral modiolar vein (SMV) were evaluated histologically 2 to 6 days following inoculation. The experimental group endothelial cells had larger nuclei and increased amounts of cytoplasm. In addition, many lymphocytes were adherent to and present within the vascular wall. Control SMV endothelia showed normal structure and morphology throughout the study. These findings reiterate that nonlymphoid tissues may develop HEV-like characteristics and suggest that HEV-like morphology may be acquired during an acute inflammatory condition in the cochlea.

Animals↗
PubMed2006

Otitis interna (labyrinthitis) associated with Salmonella enterica arizonae in turkey poults.

Otitis interna was diagnosed in five 9-to-21-day-old turkey poults with clinical signs of paralysis, opisthotonus, torticollis, blindness, and increased mortality. Gross and microscopic lesions in the poults included omphalitis, typhlitis, hepatitis, meningoencephalitis, ophthalmitis, neuritis and ganglionitis of the vestibulocochlear nerve, and otitis interna. Salmonella enterica arizonae was isolated from the brains, eyes, intestines, yolk sacs, and livers of poults. Birds with otitis interna also had meningoencephalitis. It is most likely that the S. enterica arizonae infection spread from the brain to the internal ears through the vestibulocochlear nerve. This is the first documentation of otitis interna caused by bacteria in an avian species.

Animals↗