platybasia/hearing loss

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[Sudden hearing loss and the cranio-cervical junction].

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Morphological alterations of the craniocervical junction as basilar impressions, a ponticulus posterior, an atlas assimilation, an intervertebral narrowing and spondylosis deformans, were found radiologically in patients with sudden hearing loss. There were no radiological differences to a healthy

A novel mutation in the LIM homeobox 3 gene is responsible for combined pituitary hormone deficiency, hearing impairment, and vertebral malformations.

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BACKGROUND The LIM homeobox 3 (LHX3) LIM-homeodomain transcription factor gene, found in both man and mouse, is required for development of the pituitary and motor neurons, and is also expressed in the auditory system. OBJECTIVE The objective of this study was to determine the cause of, and further

[The differential diagnosis in Menières disease: the basilar impression (author's transl)].

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We examined the cranio-cervical region in 180 patients with nonspecific dizziness and in most cases unilateral sensory-neural hearing loss. In 32 cases we found malformations of the cranio-cervical region. The sympatomatology of the basilar impression is probably caused by the compression of the

Basilar impression. A differential diagnosis of Menier'es disease.

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We examined the craniocervical region in 180 patients with nonspecific dizziness and unilateral sensorineural hearing loss in most of them. In 32 cases, we found malformations of the craniocervical region. The symptoms of the basilar impression are probably caused by compression of the vessels of

The Wildervanck syndrome: cervico-oculo-acoustic dysplasia.

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The Wildervanck syndrome consists of the Klippel-Feil deformity of the spine, eyeball retraction, lateral gaze weakness, and hearing loss. Conductive hearing loss, as well as a more frequently occurring sensorineural hearing loss, is caused by dysplasia of the inner ear. Typically, polytomograms

Vestibular dysfunction in adult patients with osteogenesis imperfecta.

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Progressive hearing loss is a major symptom in osteogenesis imperfecta (OI), a genetic brittle bone disease. Vertigo is frequently associated with otosclerosis in which the hearing loss clinically resembles that in OI. Vertigo is also common in basilar impression (BI) found in up to 25% of adult OI

Symptomatic intracranial hypertension and prolonged hypocalcemia following treatment of Paget's disease of the skull with zoledronic acid.

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Skull involvement in Paget's disease of bone can lead to neurological symptoms, prompting treatment. Intravenous zoledronic acid (ZA) has emerged as an effective and safe treatment option for patients with Paget's, leading to sustained remission and improved quality of life. A previously untreated

Unique Skeletal Manifestations in Patients With Primrose Syndrome

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Primrose syndrome (OMIM 259050) is a rare disorder characterised by macrocephaly with developmental delay, a recognisable facial phenotype, altered glucose metabolism, and other features such as sensorineural hearing loss, short stature, and calcification of the ear cartilage. It is caused by

The petrous temporal bone and deafness in X-linked hypophosphataemic osteomalacia.

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Radiological anatomy of the petrous bones and skull base has been studied in a group of 11 patients with X-linked hypophosphataemic osteomalacia and hearing loss, and comparison made with a control group of normal subjects. Patients with X-linked hypophosphataemic osteomalacia were found to have

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