Cases reported "Hearing Disorders"

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1/12. Pineal region tumor manifesting initially as hearing impairment.

    An 18-year-old male presented with a pineal region germinoma with hearing impairment as the chief complaint. Magnetic resonance image demonstrated a well-enhanced multi-cystic tumor extending into the upper fourth ventricle and wall of the bilateral lateral ventricles. audiometry revealed bilateral mild hearing impairment in the low frequencies. Auditory brainstem response recording showed low amplitudes in all waves (IV-V/I ratio < 1) with prolong latencies (I-V and III-V) on the right but no discernable wave at 60 dB clicks on left. Hearing impairment and audiometric findings were improved after ventriculoperitoneal shunt operation. The hearing impairment appeared to be a mixed (conductive and sensorineural) type. The tumor was responsible for the sensorineural deafness because of invasion and compression of the central auditory structures. The inferior brachium was maximally compressed anterolaterally by the dilated bilateral lateral ventricles and posteromedially by the tumor. hydrocephalus caused conductive deafness by halting or arresting the footplate of stapes movement, as a consequence of high-pressure transmission through the cochlear aqueduct.
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ranking = 1
keywords = aqueduct
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2/12. magnetic resonance imaging of the large vestibular aqueduct.

    The large vestibular aqueduct syndrome describes an abnormally large endolymphatic duct and sac with associated sensorineural hearing loss. This entity was originally reported in 1978 and has since been identified as a finding in children with progressive hearing loss. The original description of the large vestibular aqueduct employed hypocycloidal polytomography of temporal bone. Subsequent reports studied patients identified with this syndrome using computed tomographic scans. We report magnetic resonance imaging of two patients diagnosed with the large vestibular aqueduct syndrome. The magnetic resonance imaging and computed tomographic scans are compared and the significant findings on magnetic resonance imaging are reviewed. This should assist the otolaryngologist and radiologist with establishing the appropriate diagnosis.
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ranking = 7
keywords = aqueduct
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3/12. Traumatic perilymphatic fistula: how long can symptoms persist? A follow-up report.

    In the past 18 years 68 ears (average 3.8 per year) were explored for perilymphatic fistula (PLF). A total of nine (13%) ears had a fistula identified at operation. patients with a previous history of otologic surgery were excluded from this review. The most common etiology for PLF was head trauma (4 of 9). Most patients had persistent symptoms lasting months (average 6.7). Eighty-three percent of all patients had sudden or fluctuating hearing loss, 77 percent had vertigo or dysequilibrium, and 61 percent had tinnitus. vertigo was the most commonly improved symptom postoperatively, and only 25 percent of patients had improved hearing. There were no major complications. The authors discuss indications for operation, criteria for diagnosis of PLF, and audiometric and electronystagmographic findings. This report agrees with other recent data indicating that exploration for fistula is an uncommon procedure performed by otologists.
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ranking = 167.70437960095
keywords = perilymphatic
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4/12. Hypoplastic endolymphatic sac, hydrops, and Mondini deformity: a case report.

    The Mondini deformity of the inner ear is usually associated with a large vestibular aqueduct and endolymphatic sac. The authors present a case with a hypoplastic sac and endolymphatic hydrops, which are presumed to be the cause of the Meniere's syndrome symptoms that occurred in mid-life.
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ranking = 1
keywords = aqueduct
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5/12. Repair of a cerebrospinal fluid perilymph fistula primarily through the middle ear and secondarily by occluding the cochlear aqueduct.

    A 35-year-old man had a 5-year history of fluctuating hearing loss in his only hearing ear. history and diagnostic tests indicated a perilymph fistula, a diagnosis subsequently confirmed by exploration. Primary and secondary repairs temporarily ameliorated symptoms. A spinal fluid to middle ear fluid pathway was identified by radioactive tracer. A patent cochlear aqueduct indicated on computed tomography scan was found and repaired through a posterior cranial fossa approach. Hearing was preserved, remaining relatively stable during the 2-year follow-up period.
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ranking = 5
keywords = aqueduct
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6/12. Perilymphatic fistula: a new hampshire experience.

    Thirty-five patients with perilymphatic fistulas (PLFs) are presented. Of this group of 35 patients (39 ears), 4 patients did not have fistulas that could be observed with certainty but were presumed to have fistulas by virtue of their excellent response to surgical repair. Our case reports provide examples of the great variety and possible classifications of presentations and symptom complexes that lead one to suspect the diagnosis of perilymphatic fistula. Comments on diagnostic and therapeutic modalities and on postoperative care and counseling are included. The age range of patients in our series is 3 to 67 years. Four patients are under age 20, and an additional three patients probably developed their symptoms prior to age 20 but presented later. Twenty-three (79%) of 29 patients with spontaneous PLFs began having symptoms closely related to some event involving physical or mechanical stress, and a high percentage (76%) had symptoms aggravated by physical stress. Six are believed to have fistulas of congenital origin. There is a sibling pair and a mother and son in the series; these four people had bilateral fistulas.
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ranking = 67.08175184038
keywords = perilymphatic
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7/12. Pneumolabyrinth in perilymphatic fistula: report of three cases.

    In three patients with perilymphatic fistula, exploratory tympanotomy revealed air bubbles emerging through the ruptured round window membrane. review of the literature disclosed three case reports in which air was demonstrated in the labyrinth in association with perilymphatic fistula. Experimental evidence that air could enter the labyrinth through a defect of the round window membrane was described in two articles. In our patients, the perilymphatic fistula was produced by implosive force. When a perilymphatic fistula was produced by implosive force, or in the case of a pre-existing perilymphatic fistula, we assume, air may enter the scala tympani through the defect of the round window membrane if the middle ear pressure rises beyond a certain limit. Sudden onset of deafness and reversibility of hearing in perilymphatic fistula could be attributable to the presence of air bubble in the scala tympani--pneumolabyrinth--which might disturb propagation of the traveling wave of the basilar membrane.
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ranking = 335.4087592019
keywords = perilymphatic
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8/12. ataxia and hearing loss secondary to perilymphatic fistula.

    ataxia is rarely attributed to lesions of the peripheral vestibular system. In 1973, the first case of ataxia and hearing loss secondary to a labyrinthine fistula was reported. Until now, this syndrome has not been reported in patients under the age of 10 years. A case is presented of a 5-year-old boy with symptoms of ataxia and hearing loss as well as vertigo and tinnitus after head trauma. Three physical findings appear to be most characteristic of patients with perilymphatic fistulas: a positive fistula response, positive positional testing with the involved ear down, and evidence of vestibular ataxia when testing station and gait. The absolute diagnosis of perilymphatic fistula can only be established by exploration of the middle ear space. If a fistula is found, it may be sealed with soft tissue and, if this fails, actual stapedectomy may be required.
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ranking = 201.24525552114
keywords = perilymphatic
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9/12. rupture of the round window membrane.

    The mechanism of development of rupture of the round window membrane appears to be an increase in pressure in the cerebrospinal fluid transmitted to the inner ear via the cochlear aqueduct. Two cases of rupture of the round window membrane in divers are described in detail. One diver had ruptures of both round window membranes following difficulty in autoinflating when diving, and the other had a rupture of one round window membrane, having presented with acute prostating vertigo and normal hearing. Surgical repair restored the hearing in the former case and preserved normal hearing in the latter. The similarity between ruptures of the round window membrane and poststapedectomy fistula is emphasized, and it is suggested that spontaneous healing of ruptures may well occur. A case history supporting this hypothesis is presented. The technique of repair of ruptures of the round window membrane is described as well as postoperative management. Normal nasal function in divers is essential if inner ear barotrauma and ruptures of the round window membranes are to be avoided.
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ranking = 1
keywords = aqueduct
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10/12. cerebrospinal fluid otorrhea.

    Cerebrospinal fluid (CSF) otorrhea is a dangerous and potentially life threatening occurrence for which the otolaryngologist is often consulted. CSF otorrhea occurs on the basis of skull fracture, tumor, infections, congenital anomalies, and operative trauma. Forty-three patients with CSF otorrhea of varied etiology are reviewed in this paper. Eight cases are of congenital or labyrinthine origin confirming at surgery the probable connection between the subarachnoid and perilymphatic spaces. Eleven cases had spinal fluid otorrhea due to infection. All cases presented with symptoms of chronic infection: 4 cases had a history of previous surgery for chronic ear disease; 7 cases had temporal lobe abscess; 1 case had a cerebellar abscess; 8 had tegmen defects secondary to cholesteatoma; in 1 case the tegmen defect was due to previous surgery for chronic infection. Nine of 11 cases have serviceable hearing postoperatively. Fourteen cases of spinal fluid otorrhea resulted from trauma: 1 case was due to traumatic stapes footplate fracture in a congenitally malformed ear; 4 were due to transverse temporal bone fracture; and 9 were due to longitudinal temporal bone fractures. All transverse fractures resulted in nonhearing ears. Three cases were due to a combination of temporal bone fracture and infection. In 2 of these cases chronic infection preceded the fracture; in 1 case the fracture led to chronic ear disease with spinal fluid leakage. One patient required 1 surgical procedure for closure of the otorrhea, 1 patient 2 procedures, and 1 patient 3 procedures. Ten cases are due to translabyrinthine acoustic neuroma removal: 7 cases had resolution of the spinal fluid leakage after conservative nonsurgical treatment; and 3 required surgical intervention using muscle, fat and fascia obliteration of the spinal fluid pathway.
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ranking = 33.54087592019
keywords = perilymphatic
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