1/56. Fibrous dysplasia of the temporal bone.Fibrous dysplasia is characterized by a progressive replacement of normal bone elements by fibrous tissue. It is seen in the two first decades of life and is equally distributed in relation to the sex of patients. The temporal bone is involved in 18% of the cases. Fibrous dysplasia of the temporal bone is more commonly manifested in the external auditory canal (80%) and the inner and medium ear can also be involved. Sarcomatous alterations are rare. diagnosis is based on radiological images. Treatment is conservative and surgery is reserved for preserving function and preventing complications. The case of a male patient, 16 years old, who presented with progressive hearing loss over a 1-year period is reported.- - - - - - - - - - ranking = 1keywords = canal (Clic here for more details about this article) |
2/56. Inner ear abnormalities in Kabuki make-up syndrome: report of three cases.Three patients, a female and two males, 28, 15, and 14 years of age, with Kabuki make-up syndrome (KMS) were studied for middle and inner ear abnormalities by using CT scanning of the petrous bones. All three patients had bilateral dysplasia of the inner ear, i.e., hypodysplasia of the cochlea, vestibule, and semicircular canals (so-called Mondini dysplasia), whereas their middle ears had no abnormalities. audiometry demonstrated a sharp decrease in hearing of the high tone range, bilateral in one and unilateral in another, while the third patient was noncooperative. In view of these findings, it would be advisable to study each individual with KMS and hearing impairment for possible inner ear abnormalities.- - - - - - - - - - ranking = 1keywords = canal (Clic here for more details about this article) |
3/56. Sudden unilateral hearing loss with simultaneous ipsilateral posterior semicircular canal benign paroxysmal positional vertigo: a variant of vestibulo-cochlear neurolabyrinthitis?We describe 4 patients who all simultaneously developed a sudden total or partial unilateral sensorineural hearing loss and an unusual acute peripheral vestibulopathy in the same ear characterized by posterior semicircular canal benign paroxysmal positional vertigo with intact lateral semicircular canal function. Two patients also had ipsilateral loss of otolith function. The vertigo resolved in all 4 patients after particle-repositioning maneuvers. The findings of audiometry and vestibular tests indicated that the lesion responsible for this syndrome was probably located within the labyrinth itself rather than within the vestibulocochlear nerve and that it was more likely a viral vestibulocochlear neurolabyrinthitis than a labyrinthine infarction.- - - - - - - - - - ranking = 6keywords = canal (Clic here for more details about this article) |
4/56. Sigmoid and transverse sinus thrombosis after closed head injury presenting with unilateral hearing loss.Sinus thrombosis has rarely been associated with closed head injury; more often, thrombosis of the sigmoid or transverse sinus is caused by otogenic inflammations or tumours, or occurs during pregnancy. Symptoms are frequently vague, while untreated thrombus progression may be fatal due to venous congestion and infarction. We report a 32-year-old man presenting with right hearing loss, tinnitus and headache 2 days after a closed head injury. Neurological examination showed no additional abnormality. The EEG showed focal bifrontal slowing. CT revealed a fracture of the occipital bone. MRI and MRA demonstrated complete thrombosis of the right sigmoid and transverse sinuses. After 2 weeks of intravenous heparin therapy followed by warfarin, the patient's hearing improved and MRI and MRA showed complete recanalisation of the sigmoid and transverse sinuses. Venous sinus thrombosis can be an undetected sequel to head injury. Appropriate imaging studies should be carried out to enable therapy to be started as soon as possible.- - - - - - - - - - ranking = 1keywords = canal (Clic here for more details about this article) |
5/56. The Wildervanck syndrome: cervico-oculo-acoustic dysplasia.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 demonstrate a bulbous vestibule and dilated lateral semicircular canals with basilar impression of the skull. The Duane's eyeball retraction phenomenon completes the syndrome; this may be explained either by misdirected innervation of the extraocular muscles or by atrophy or fibrosis of the lateral rectus muscles.- - - - - - - - - - ranking = 1keywords = canal (Clic here for more details about this article) |
6/56. Spontaneous intracranial hypotension: a rare cause of labyrinthine hydrops.Spontaneous intracranial hypotension should be considered as a possible cause of cochlear hydrops. We report a case of unilateral hearing loss attributed to spontaneous intracranial hypotension on the basis of characteristic abnormalities seen on magnetic resonance imaging. The diagnostic gold standards for intracranial hypotension are lumbar measurement of cerebrospinal fluid pressure and magnetic resonance imaging. The usual treatment is an autologous blood injection into the peridural spaces. The mechanism of hearing loss is thought to involve secondary perilymph depression due to a patent cochlear aqueduct. This perilymph depression would induce a compensatory expansion of the endolymphatic compartment, with a subsequent decrease in basilar or Reissner's membrane compliance. endolymphatic hydrops can occur in the course of intracranial hypotension, and not only because of abnormal endolymph production or resorption. Hydrops can thus be classified into 1) syndromes of endolymphatic origin and 2) syndromes of perilymphatic origin, in which loss of perilymph induces compensatory expansion of the endolymphatic space.- - - - - - - - - - ranking = 0.0025453564992695keywords = spinal (Clic here for more details about this article) |
7/56. X-linked stapes gusher: CT findings in one patient.A 31-year-old male patient presented with progressive mixed hearing loss since birth. A stapedectomy was attempted and was unsuccessful because of perilymph gushing. CT of the temporal bones showed bulbous dilatation of the fundi of the internal auditory canals and absence of the bone plates separating them from the base of the cochleas. This unusual abnormality was found after the attempted stapedectomy and explains the clinical findings. The findings in male patients are fairly typical X-linked congenital deafness.- - - - - - - - - - ranking = 1keywords = canal (Clic here for more details about this article) |
8/56. De novo inv(2)(p12q34) associated with Klippel-Feil anomaly and hypodontia.The present case report describes a patient with Klippel-Feil anomaly (KFA) and oligodontia, carrying a de novo pericentric inversion of chromosome 2 (p12q34). KFA is characterised by congenital vertebral fusion of the cervical spine and a wide spectrum of associated anomalies. It therefore constitutes a heterogenous group of clinical conditions and has been classified morphologically, although its aetiology remains unclear. We present an 18-year-old female with KFA, associated with congenital impairment of hearing, psychomotor retardation, speech limitation, short stature, spinal scoliosis, facial asymmetry and latent hypothyroidism. No renal anomaly or heart disease was present. In addition, she exhibited oligodontia of both the deciduous and permanent dentition, a unique characteristic that has not yet been reported in any non-cleft palate KFA case. CONCLUSION: The current report of a patient with oligodontia and an inversion on chromosome 2 may aid in the identification of novel genes for oligodontia.- - - - - - - - - - ranking = 0.0025453564992695keywords = spinal (Clic here for more details about this article) |
9/56. Familial early-onset progressive vestibulopathy without hearing impairment.A family with early-onset vestibulopathy is presented. The 34-year-old father had experienced brief attacks of vertigo and currently suffers from unsteadiness and oscillopsia during head movements. His two young sons also experience brief attacks of spontaneous vertigo. In vestibular testing all three subjects showed reduced caloric responses. However, only the father showed reduced otolith function (as reflected by the vestibular-evoked myogenic potentials). Further, all three subjects had walked before the age of 1 year and none of them had had any auditory symptoms. It is suggested that they have familial early-onset progressive vestibulopathy affecting the canals before the otoliths but sparing cochlear function.- - - - - - - - - - ranking = 1keywords = canal (Clic here for more details about this article) |
10/56. facial nerve anomalies in association with congenital hearing loss.OBJECTIVE: facial nerve (FN) anomalies are exceedingly rare. The association between a hearing loss and an anomalous facial nerve has never been touched deeply in the literature. The aim of this study is to report facial nerve anomalies in patients presenting with congenital hearing loss (COHL) and/or facial nerve palsy. methods: A retrospective chart review over 10 years from two academic tertiary referral centers. Eight patients were diagnosed with facial nerve anomalies in association with congenital hearing loss. The age ranged from 7 months to 13 years. RESULTS: Five patients had conductive congenital hearing loss (CCHL). Two had congenital sensorineural hearing loss (CSNHL). The eighth patient had a mixed hearing loss (MHL). Surgical findings for patients with conductive congenital hearing loss included facial nerve passing inferior to oval window (OW) (two patients), facial nerve covering head of stapes (three patients), deformed stapes (two patient), deformed incus (two patient), and absent incus (one patient). Radiological findings for patients with congenital sensorineural hearing loss and mixed hearing loss include, single cavity anomaly (one patient), dilated vestibule and superior semicircular canal (one patient), and absent internal auditory canal (IAC) (two patients). For patients with conductive congenital hearing loss, the radiological findings consistency demonstrates a soft tissue opacification in the middle ear. CONCLUSIONS: Congenital hearing loss may be associated with facial nerve anomalies. Pediatric otolaryngologists should be cautious when exploring patients with conductive congenital hearing loss.- - - - - - - - - - ranking = 2keywords = canal (Clic here for more details about this article) |
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