Inner ear cholesteatoma: an embryologic aberration.
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Cholesteatoma↗
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Intralabyrinthine schwannomas.
Intralabyrinthine schwannomas are unusual tumors of the vestibule, cochlea, semicircular canals, or some combination of these three, which in the past have been reported as incidental findings at autopsy or surgery. We summarize eight cases of intralabyrinthine schwannomas diagnosed by magnetic resonance imaging at the House Ear Clinic during the past 3 years. We discuss the typical clinical presentation and treatment of cochlear vs. vestibular intralabyrinthine schwannomas.
Adolescent↗
Aberrant internal carotid artery causing erosion of the otic capsule: an unusual cause of pulsatile tinnitus.
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Bone Resorption↗
Temporal bone histopathology in dominantly inherited audiovestibular syndrome.
OBJECTIVE: To describe the clinical and pathologic features of a new dominantly inherited audiovestibular syndrome. METHODS: History, examination, and audiometric testing in the proband, brother, and son; quantitative rotational testing in the proband and son; histopathology of the cochlea and vestibular labyrinth in the proband; sequencing candidate genes COCH and MYO7A in the brother and son. RESULTS: Affected family members developed slowly progressive hearing loss beginning in their late 30s and progressive imbalance in their early 70s. Three of four affected had brief (minutes) episodes of vertigo typically occurring a few times per year. Auditory and vestibular function testing documented a slowly progressive loss of auditory and vestibular function. Postmortem examination showed a loss of hair cells in the cochlea and vestibular receptor organs. There were no cellular infiltrates or acidophilic deposits. No mutations were found in the COCH or MYO7A genes. CONCLUSIONS: This dominantly inherited audiovestibular syndrome results in a selective loss of hair cells in the auditory and vestibular end organs. Finding the causative gene could have important implications for understanding the pathophysiology of presbycusis and dysequilibrium of aging.
Adult↗
Hyperacusis in Williams syndrome: characteristics and associated neuroaudiologic abnormalities.
BACKGROUND: Hyperacusis and phonophobia are common, debilitating symptoms in Williams syndrome (WS), yet little is known about their underlying audiologic and neurologic processes. METHODS: The mothers of 49 subjects with WS were asked to complete the Hyperacusis Screening Questionnaire. Subjects with reported hyperacusis and sufficient developmental capacity underwent comprehensive audiological and brain auditory evoked response (BAER) testing. Findings were compared with those from pair-matched typically developing control subjects. RESULTS: Forty-one of the 49 children with WS (84%) had hyperacusis of moderate to severe degree, which began in infancy. Of these, 21 (mean age 15.8 +/- 5.5 years) were quantitatively tested. Subjects with WS reported discomfort at sound intensities on average 20 dB lower than control subjects. Pure-tone audiometry and distortion products otoacoustic emission test revealed a high-frequency cochlear hearing loss. An absence of ipsilateral acoustic reflex responses to maximum stimulation was significantly more common in the subjects with WS than controls. On BAER testing, the WS group had a significant prolongation in wave I latency. CONCLUSIONS: Hyperacusis in Williams syndrome (WS) is associated with a high-frequency hearing loss resembling the configuration of noise-induced hearing loss. The hyperacusis and hearing loss in WS may stem from a deficiency in the acoustic reflex resulting from auditory nerve dysfunction. Additional mechanisms that may mediate hyperacusis in WS and should be evaluated in future studies include recruitment, malformation of the facial canal, and haploinsufficiency of the elastin gene.
Acoustic Stimulation↗
Bacterial meningitis in adults: demonstration of inner ear involvement using high-resolution MRI.
OBJECTIVE: To visualize the sites involved in audiovestibular dysfunction during bacterial meningitis in adults and to relate these findings to the extent of hearing impairment and vestibular dysfunction. BACKGROUND: Hearing impairment is among the most frequent complications of bacterial meningitis. METHODS: High-resolution MRI (HR-MRI) of the inner ear was performed in seven adult patients with hearing loss as a complication of bacterial meningitis. RESULTS: Five patients had unilateral (n = 1) or bilateral (n = 4) contrast enhancement of vestibulocochlear structures. The structures most frequently involved were the cochlear nerve (n = 9), the first cochlear turn (n = 9), the vestibulum (n = 9), and the semicircular canals (n = 7). There was a significant correlation between clinical and MRI findings: all nine ears with cochlear enhancement were deaf (hearing loss >90 dB), whereas none of the five ears with normal MRI findings had hearing losses of more than 90 dB (range, 30 to 70 dB; p = 0.0005). Vestibular dysfunction as revealed clinically and by quantitative vestibular function testing was found in six of seven patients (11 of 14 ears). Five of these patients (nine ears) also demonstrated enhancement of the vestibular organ on high-resolution MRI of the inner ear. CONCLUSIONS: High-resolution MRI can visualize the involvement of vestibulocochlear structures in bacterial meningitis in both cooperative and consciously impaired patients. These findings suggest a correlation between abnormalities on MRI and the extent of cochlear dysfunction.
Adolescent↗
Hearing impairment in 18q deletion syndrome.
The 18q-syndrome is associated with hearing impairment in 50-80 per cent of cases. The hearing loss may be sensorineural or conductive. A high proportion of cases are associated with narrow or stenosed external auditory canals. This may be a useful clinical pointer to the syndrome. Two cases with impaired hearing are presented in this paper including one case with complex external ear and middle ear malformations. The clinical and audiological features in each case are described.
Adolescent↗
Effects of persistent perilymph fistula on the inner ear.
To study the effects of a persistent perilymph fistula on the cochlea, a small cannula was inserted into the scala tympani of the basal turn of cochlea in guinea pigs. A month later, cochlear morphology and blood flow were studied using either histological evaluation or the microsphere surface preparation technique. Some animals showed no cochlear morphologic changes or no cochlear blood-flow reduction, even if tubal patency was maintained and perilymph leakage lasted for 1 month. This suggests that a prolonged perilymph fistula, per se, causes no permanent cochlear damage. However, in some animals, hair cell damage and cochlear blood-flow disorders were observed. These observations and the causes of hearing loss in clinical cases of perilymph fistula were studied.
Animals↗
Manipulating the mobile stapes during tympanoplasty: the risk of stapedial luxation.
Manipulating the mobile stapes is a central procedure during tympanoplasty, i.e., mounting a prosthesis onto the stapedial head or dissecting cholesteatoma matrix off the oval window niche. An excessive displacement of the stapedial suprastructure as caused by these manipulations with hand-held instruments might result in a rupture of the annular ligament. Bacteria invading through this perilymphatic fistula threaten the inner ear. In experiments with temporal bones, the author investigated the rupture mechanisms of the annular ligament with definite stapedial manipulations. Leaks, which became detectable by exerting pressure on the inner ear fluid, occur only when all suspension fibers are completely ruptured. Further clinical aspects with different directions of stapedial manipulations are demonstrated.
Cochlear Diseases↗
Frequency selectivity in patients with acoustic neuroma.
Frequency selectivity was compared in subjects with hearing loss due to acoustic neuroma and cochlear pathology, and normal listeners. A particular interest was the role of probe tone parameters on the shape of the tuning curve. Psychophysical tuning curves (PTCs) were measured for each of two equal energy 2000-Hz probe tones (10 dB SL/300 msec and 17 dB SL/60 msec), using simultaneous 1/3-octave narrow-band noise maskers centered at 1, 1.25, 1.6, 2.5, 3.15, and 4 kHz. The results showed that the critical masker levels obtained for impaired listeners were significantly greater than those from normal subjects. The slope of the low-frequency limb of the PTC was steeper for normal compared to hearing-impaired listeners but there was no difference due to site of lesion. In all three groups, the critical masker levels obtained with the short probe were significantly greater than those for the long probe, negating the hypothesis that equal energy probes would yield the same outcomes. Tuning in listeners with hearing loss was highly correlated with audiometric threshold but not with tumor size, width of the internal auditory canal, or tumor location within the cerebellopontine angle. The main conclusion was that cochlear and retrocochlear hearing loss are similar with respect to their effect on frequency selectivity.
Acoustic Stimulation↗
[Neuro-otological findings on the posterior fossa lesions. Part VI. Auditory evoked potentials and its clinical significance (author's transl)].
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Adolescent↗
Apparently accidental degenerative changes in the guinea-pig cochlea.
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Cochlea↗
Pathophysiology of inner ear fluid imbalance.
Maintenance of homeostasis of inner ear fluids and biochemical integrity of inner ear tissue are essential for proper functioning of the auditory and vestibular end organs. Although various regulatory mechanisms exist in a different portion of the labyrinth, the inner ear is known to respond to systemic challenges. The association of Meniere's syndrome with an imbalance of inner ear fluid homeostasis has been hypothesized for the past century. Among many factors, the effects of hormonal imbalance on inner ear fluid composition and inner ear function have however scarcely been studied. The purpose of this study was to explore the relationship between the autonomic nervous system and inner ear function and possible mechanisms of functional disturbances in an experimental condition. An infusion of supraphysiologic amounts of epinephrine, a stress related hormone, resulted in an elevation of osmolality in serum and perilymph. Furthermore, the infusion of epinephrine resulted in elevation of threshold, prolongation of latency, and depression of amplitude in the compound action potential of the auditory nerve. These findings were most marked at high frequencies. We hypothesized that the epinephrine-induced hearing loss was brought about by an increase in perilymphatic osmolality, as well as by the ionic imbalance caused by the osmotic gradient. Since emotional stress has been implicated as a mechanism of inducing a Meniere's attack, evaluation of the relationship between the autonomic system and cochlear function may contribute to the understanding of possible mechanisms of inner ear dysfunction caused by hormonal imbalances.
Animals↗
Postoperative complications in acoustic neuroma surgery by the extended middle cranial fossa approach.
The complications of acoustic neuroma (AN) surgery by the extended middle cranial fossa approach were studied in 160 cases. Death during surgery occurred in 3 cases (1.9%); 2 of these involved large tumors and the third was a case of recurrence. Transient temporal lobe symptoms were seen in 24 cases (15.3%); the average tumor diameter in these cases was 36.0 mm. Beside symptoms of 7th and 8th nerve disturbance, hemiparesis was seen 7.6%; 3rd, 4th, and 6th cranial nerve symptoms in 7.0%; and cerebellar symptoms in 7.6% of the patients. The incidence of these disturbances increased with tumor size. Cerebrospinal fluid (CSF) leakage occurred in 20.4% of the cases and this necessitated surgical treatment in 5% of the cases.
Cerebral Hemorrhage↗
Changes in clinical features of acoustic neuroma.
During a 14-year period from 1976 to 1989, the authors experienced 160 cases of acoustic neuroma (AN). The clinical features were investigated in 132 of these cases (excluding those cases of recurrence and neurofibromatosis 2). The cases were divided into three periods for study. There were no significant differences in the patients' age, sex, or affected side among the three periods. The number of intracanalicular tumors have increased in recent years. The proportion of medium-sized and large tumors, however, has not changed, comprising about 70% of the total number of cases. Tumors of 20 mm or smaller in diameter were found in 24% of the cases in the early period, but increased to 58% in the most recent period. There was no difference among the periods in the length of time elapsed from the onset of symptoms to the first examination. A higher proportion of cases were diagnosed in otolaryngology departments in the middle and most recent periods than in the early period. Fewer cases in the most recent period presented initially with disequilibrium or trigeminal nerve symptoms. This was believed to be due to the increase in early stage cases. Patients are increasingly presenting with early stage AN. We feel that in order to support this favorable tendency, more emphasis needs to be placed on early diagnosis.
Adolescent↗
The effect of furosemide on the endocochlear potential in ears with experimentally induced endolymphatic hydrops.
The endocochlear potential (EP) was measured in 38 guinea pigs with experimentally induced endolymphatic hydrops at the 3rd, 6th, 12th and 24th postoperative weeks, and the effects of furosemide (FUR, 50 or 80 mg/kg) on the EP were examined. A time-related reduction of the EP from the normal value and increased susceptibility to FUR were disclosed in the hydropic animals. Furthermore, 24-week animals given 80 mg/kg FUR showed a significantly slower recovery rate of the EP than the other groups, indicating impairment of the strial function progressive with post-operative time. The negative component of the EP was considered to be unimpaired until at least 12 weeks after the surgery.
Animals↗
Spontaneous otoacoustic emissions and sensori-neural hearing loss.
This study sought to clarify the clinical relevance of spontaneous otoacoustic emissions (SOAEs) and to define the hearing loss level (and frequency) at which absence of SOAE is found. Findings from 126 ears of patients with sensori-neural hearing loss showed an incidence of SOAEs in 18.25% of the cases (23 out of 126 ears). SOAEs were never found when hearing loss at 1,000 Hz exceeded 10 dB. The presence of SOAE seems to indicate a good cochlear functioning at least in the mid-frequencies. Although the incidence of SOAEs is markedly lower than that of evoked otoacoustic emissions (EOAEs), SOAE recording is shown to be a good test, rapid, non-invasive for audiological screening, the presence of SOAE confirming a hearing threshold of less than 10 dB at 1,000 Hz, the absence of SOAE being inconclusive.
Acoustic Impedance Tests↗
Degeneration of the organ of Corti following intravenous administration of cisplatin.
The development of acute morphological changes in the cochlea was studied in guinea pigs given one intravenous high-dose injection of cisplatin. In the light microscope three major stages of degeneration in the organ of Corti could be recognized: 1) an initial swelling of the Hensen's cells and protrusion of the Deiters' cells into the space of Nuel enclosing the outer hair cells, 2) a gradual degeneration of the outer hair cells together with a vacuolization in the region of the base of the inner hair cells, 3) a collapse of the Reissner's membrane and the entire organ of Corti with different degrees of damage to the inner hair cells. Sporadic bulging of the marginal cells of the stria vascularis into the endolymphatic space could be observed 4 days after injection.
Animals↗