Contributions to the study of spinocerebellar ataxia type 38 (SCA38).

Objective: To report clinical and ancillary findings in a kindred with spinocerebellar ataxia 38 (SCA38).

Patients And Methods: Five family members spanning two generations developed gait ataxia and intermittent diplopia. On examination, a cerebellar syndrome accompanied by downbeat nystagmus and a saccadic head impulse test (HIT) were found.

Regulation of Fn14 Receptor and NF-κB Underlies Inflammation in Meniere's Disease.

Meniere's disease (MD) is a rare disorder characterized by episodic vertigo, sensorineural hearing loss, tinnitus, and aural fullness. It is associated with a fluid imbalance between the secretion of endolymph in the cochlear duct and its reabsorption into the subarachnoid space, leading to an accumulation of endolymph in the inner ear. Epidemiological evidence, including familial aggregation, indicates a genetic contribution and a consistent association with autoimmune diseases (AD).

Ontogenetic explanation for tegmen tympani dehiscence and superior semicircular canal dehiscence association.

Objectives: To analyze the ontogeny of the superior semicircular canal and tegmen tympani and determine if there are common embryological factors explaining both associated dehiscence.

Methods: We analyzed 77 human embryological series aged between 6 weeks and newborn. Preparations were serially cut and stained with Masson's trichrome technique.

Mitochondrial ribosome and Ménière's disease: a pilot study.

Ménière's disease patients experience vestibular disability. When most of medical treatments fail, a chemical labyrinthectomy using aminoglycosides is indicated. However, this process frequently causes hearing damage.

[Subacute necrotizing histiocytic lymphadenitis. An almost unknown condition].

The etiology of neck masses is multifactorial, including genetical troubles, infections or tumoral lesions. A detailed medical history and a thorough physical check-up with the aid of analyses and imaging pictures properly selected, provide the doctor with the needle information in order to establish a correct diagnosis (table 1). We present the case of a young woman with lateral neck and supraclavicular adenopathies which diagnosis although sporadic must be keep in mind in daily clinic owing its growing incidence in last years.

Otorhinolaryngologic manifestations in Chiari malformation.

The Chiari malformation causes herniation of the cerebellar amygdalae through the foramen magnum, resulting in the descent of the brain stem and/or traction on the lower cranial pairs. It is important for otolaryngologists to recognize Chiari malformations as part of the differential diagnosis of balance disorders, because patients may initially exhibit symptoms related to the vestibular system, including ataxia, nystagmus, or vertigo. We report 2 cases.