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PubMed2005

Superior canal dehiscence: review of a new condition.

A new cause of sound and pressure induced vertigo, superior canal dehisence, is described. Auditory manifestations include hyperacusis to bone-conducted sounds and conductive hearing loss with normal acoustic reflexes. The diagnosis is reached by a directed history, documentation of upward and torsional nystagmus evoked by sound and pressure, and radiology. Acoustic reflexes and VEMP (vestibular evoked myogenic potentials) aid in the identification of patients with an apparent conductive loss with normal acoustic reflexes or have an asymptomatic dehiscense on radiology. Treatment involves avoidance of the precipitating stimuli. Surgical treatment, by resurfacing the dehiscence, is considered in patients with more severe symptoms.

Acoustic Impedance Tests↗
PubMed1977

A case of musicogenic epilepsy.

1) A case of musicogenic epilepsy or psychomotor seizures supervening whenever the patient hears a certain tume has been presented. 2) The EEG features of the seizure are such as are often seen in psychomotor seizures. 3) Auditory evoked response may be left out of consideration. 4) There is no appreciable relationship between the disease and the life history of the patient. The attempt of our patient to cure herself by making use of conditioned reflex proved to be a failure. The patient is more liable to the disease when she is on the strain while hearing a tune. 5) Our consideration of the mechanism of the disease has led to the presumptive conclusion that hyperacusis, conditioned reflexes and the impact of life history may be involved, and the fragility of the memory function of the temporal lobe underlie the genesis of the disease.

Aged↗
PubMed2001

Symptoms and signs in superior canal dehiscence syndrome.

Patients with superior canal dehiscence (SCD) syndrome experience vertigo and oscillopsia in response to loud sounds and to stimuli that result in changes in middle ear or intracranial pressure. They may also experience hyperacusis to bone-conducted sounds. The evoked eye movements in this syndrome align with the plane of the dehiscent superior canal. The symptoms and signs can be understood in terms of the effect of the dehiscence in creation of a third mobile window into the inner ear. The SCD syndrome has been diagnosed in 28 patients who were examined in the neuro-otology clinics at the Johns Hopkins Medical Institutions from May 1995 through January 2001. The diagnosis is best established based upon the symptoms that are characteristic for the syndrome, the vertical-torsional eye movements evoked by sound or pressure stimuli noted on examination performed with Frenzel goggles, the lowered thresholds for responses to vestibular-evoked myogenic potentials, and CT imaging of the temporal bones.

Adult↗
PubMed1984

Idiopathic infantile hypercalcaemia--a continuing enigma.

Seventy six children with documented Fanconi-type idiopathic infantile hypercalcaemia were studied and compared with 41 with the Williams-Beuren syndrome. Clinical comparison showed, as expected, very close similarities but also considerable differences, particularly in the severity of feeding problems and the degree of failure to thrive. The estimated incidence of idiopathic infantile hypercalcaemia alone has remained constant for the past 20 years, at approximately 18 cases per year in the United Kingdom (1 per 47 000 total live births). Long term morbidity in these children is mainly due to mental handicap and arteriopathy, but hypertension (29%), kyphoscoliosis (19%), hyperacusis (75%), and obesity (50%) may be added complications. In one child, hypercalcaemia recurred during adolescence but this seems to be excessively rare. More detailed investigation before treatment is required to discover the aetiology of hypercalcaemia in this condition.

Aortic Valve Stenosis↗
PubMed1972

A case of G M2 gangliosidosis of late onset.

A case of G(M2)-gangliosidosis commencing by the age of 5 years is described, in which hyperacusis, dementia, and fits were prominent clinical features. In addition to the typical ganglioside pattern on thin layer chromatography and the presence of membranous bodies in electron microscopic studies and characteristic histology and histochemistry, there was biochemical evidence of a gross reduction in heat-labile hexosaminidase activity in white blood cells and brain. A younger unaffected sibling showed the same enzyme defect in white blood cells.

Age Factors↗
PubMed1998

Tinnitus after head injury: evidence from otoacoustic emissions.

OBJECTIVE: Tinnitus may be caused by a lesion or dysfunction at any level of the auditory system. This study explores cochlear mechanics using otoacoustic emissions in patients with tinnitus after head injury, in whom there seems to be evidence to support dysfunction within the CNS. METHODS: The study included 20 patients with tinnitus and other auditory symptoms, such as hyperacusis and difficulty in listening in background noise, after head injury, in the presence of an "intact" auditory periphery (normal or near normal audiometric thresholds). They were compared with 20 normal subjects and 12 subjects with head injury, but without tinnitus, who had similar audiometric thresholds. In all subjects otoacoustic emissions, including transient click-evoked (TEOAEs) and spontaneous otoacoustic emissions (SOAEs), were recorded, and a test of efferent medial olivocochlear suppression, consisting of recording of TEOAEs under contralateral stimulation, was performed. RESULTS: A significantly higher prevalence of SOAEs (100%), higher TEOAE response amplitudes, and reduced medial olivocochlear suppression in patients with tinnitus in comparison with subjects without tinnitus have been found. CONCLUSION: These findings have been interpreted to be an extracochlear phenomenon, in which the reduction in central efferent suppression of cochlear mechanics, leading to an increase in cochlear amplifier gain, was subsequent to head injury. Auditory symptoms in these patients seemed to constitute the "disinhibition syndrome".

Acoustic Stimulation↗
PubMed2003

Acute peripheral vestibular deficits after whiplash injuries.

We report 3 patients who had acute peripheral vestibular dysfunction minutes to hours after a car collision with whiplash injury without head trauma. The accident was a frontal collision in 1 case, a rear impact in the second, and lateral in the third. All patients complained immediately of cervicalgia, headache, acute vertigo with a sensation of erroneous body movements, and slipping of image with head movements. A sudden sensation of tilting of the environment when driving, tinnitus, and hyperacusis were also described. The otoneurologic findings showed bilateral canalolithiasis in 1 patient and an acute peripheral vestibular deficit in 2 patients. Tilt of the subjective visual vertical was measured in all patients. Cerebral magnetic resonance imaging yielded normal findings. As angular and linear accelerometers, the vestibular organs are directly exposed to high forces generated by whiplash mechanisms. Vertigo generated by peripheral vestibular lesions is probably underestimated in whiplash injuries and may often be incorrectly attributed to cervical or cerebral lesions.

Accidents, Traffic↗
PubMed1995

Effects of L-baclofen and D-baclofen on the auditory system: a study of click-evoked potentials from the inferior colliculus in the rat.

The drug baclofen is a potential treatment for severe tinnitus, but its action in relieving tinnitus is not known. Baclofen is available as an approved drug only in racemic form with about equal content of the two enantiomers. In the present paper we show that L-baclofen causes a considerable (40.7%) suppression of the amplitude of the second peak in the click-evoked response from the cochlear nucleus. Bipolar recordings from the external nucleus of the inferior colliculus showed that L-baclofen caused a reduction in the amplitude of three or four distinct peaks in this response. D-Baclofen had no detectable effect on the response from the cochlear nucleus, and had only a slight effect on one component of the response from the external nucleus of the inferior colliculus. The demonstrated effect of L-baclofen on excitation in the ascending auditory pathway indicates that this drug may be a potential treatment for hyperactive auditory disorders such as tinnitus and hyperacusis.

Animals↗
PubMed1978

Treatment of Bell palsy with prednisone: a prospective, randomized study.

Two hundred thirty-nine patients with Bell palsy were randomly distributed into prednisone-treated and control groups. Patients were followed until complete recovery or for 1 year. In the steroid-treated and control groups, respectively, incomplete recovery of facial strength occurred in 12 percent and 20 percent; motor synkinesis in 9 percent and 15 percent; autonomic synkinesis in 1 percent and 10 percent; and electromyographic evidence of severe denervation in 9 percent and 17 percent. The differences reached statistical significance only for autonomic synkinesis. In the total series recovery of facial strength was incomplete in 16 percent. Residual weakness was mild in 14 percent and moderate in 2 percent. No patient remained with severe weakness. Age, hyperacusis, and severity of the initial palsy were associated with an increased risk of poor outcome.

Adolescent↗
PubMed1998

Auditory illusions caused by a small lesion in the right medial geniculate body.

Although the medial geniculate body (MGB) is a well-known integration center for the central auditory pathways, auditory symptoms caused by MGB damage have not been described. We report the appearance of the auditory illusions of hyperacusis and palinacousis in a 49-year-old man without previous psychiatric or epileptic illness after a small hemorrhagic infarction located mainly in the right MGB.

Auditory Perception↗
PubMed2003

A small vestibular schwannoma arising from the inferior vestibular nerve.

The investigation of a complaint of unilateral right sensorineural hearing loss led to the identification of a right 3 mm vestibular schwannoma arising from the inferior division of the right vestibular nerve. On investigation the patient was found to have normal caloric function, this being mediated by the superior vestibular nerve. Both transient and distortion product otoacoustic emissions were absent in the right ear, and ABR was abnormal on this side. These findings are of interest as they indicate that this small lesion produced a hearing loss that was both cochlear and retrocochlear. The anatomical finding that the medial auditory efferents run within the inferior vestibular nerve is considered. This patient did not experience tinnitus or hyperacusis despite the site of the lesion arising from the inferior vestibular nerve.

Gadolinium↗
PubMed1976

The use of steroids in Bell's palsy: a prospective controlled study.

A prospective, controlled, double-blind study was designed to evaluate the effect of steroid treatment on the natural history of Bell's palsy. Fifty-one patients were included in the study between 1972 and 1974. The patients were evaluated and started on treatment within two days of onset of Bell's palsy and followed for six months. Treatment was given in randomized double-blind fashion and consisted of either vitamins or a total of 410 mg of prednisone plus vitamins in descending doses over 10 days. The recovery of facial motor function was determined by three physicians who had no knowledge of the treatment received by the patients. They examined photographs of the patients taken six months after onset of paralysis in eight positions of facial function and categorized them as to complete fair, or poor recovery of facial function. These results of this evaluation were submitted to the biostatistician who broke the treatment code. The results of this study demonstrate no statistically significant beneficial effect of steroid therapy upon recovery from Bell's palsy. Factors considered included the patients' age, sex, the presence of pain, ageusia, hyperacusis, diabetes, hypertension, the progression and degree of palsy, the results of nerve excitability and salivary flow tests, and the time at which recovery was first noted or became complete. Bell's palsy remains without a proven efficacious treatment.

Adult↗
PubMed1977

Auditory symptoms associated with herpes zoster or idiopathic facial paralysis.

Auditory symptoms (hyperacusis, tinnitus, decreased hearing) have long been recognized to accompany herpetic or idiopathic facial paralysis. Twenty-nine percent of 1,080 patients with idiopathic facial paralysis and 37 percent of 172 with herpes zoster oticus facial paralysis had auditory symptoms. Abnormal related sensori-neural hearing loss was documented in only 11 of these 377 patients with auditory complaints. All of the 11 had a diagnosis of herpes zoster oticus. Sensori-neural hearing loss occurs in only about 6.5 percent of patients with herpes zoster facial paralysis, and no confirmed case of such loss in idiopathic facial paralysis has been reported. In patients presenting with sensori-neural hearing loss accompanying facial paralysis believed to be idiopathic, herpes zoster should be suspected even in the absence of vesicles. Factors favorable for recovery of auditory function include age 64 years or younger, mild initial hearing loss, a cochlear pattern of hearing loss, and absence of vertigo. Recovery of auditory function does take place; however, a high-tone sensori-neural loss may persist except in younger patients.

Adolescent↗
PubMed2004

Magnetic resonance imaging of neuronal ceroid lipofuscinosis in a border collie.

A castrated male border collie 23 months of age weighing 19.4 kg was referred to the Animal Medical Center of Nihon University with complaints of visual disturbance and behavioral abnormality, hyperacusis and morbid fear. The MRI examination revealed the slight dilated cerebral sulci and cerebellar fissures and left ventricular enlargement. This is the first report of MRI findings of canine neuronal ceroid lipofuscinosis.

Animals↗
PubMed1997

Behavioral and emotional disturbance in individuals with Williams syndrome.

Behavioral and emotional disturbance was assessed in 70 children and adolescents with Williams syndrome. They were compared with an epidemiological control population, which was statistically controlled for age, gender, and level of mental retardation. Those with Williams syndrome were more likely to be diagnosed as suffering psychiatric disorder. The disorder was characterized by anxiety, hyperactivity, preoccupations, and inappropriate interpersonal relating. Significantly increased rates of other individual symptoms were also found, including sleep disturbance and hyperacusis. These results, considered with earlier findings, suggest that there is a valid behavior phenotype of Williams syndrome. This is frequently associated with sufficient impairment to consider inclusion of the behavior phenotype in future official taxonomies of mental disorders.

Analysis of Variance↗
PubMedDate not supplied

Acute acoustic trauma.

Acute acoustic trauma is a clinical condition with immediate persistent hearing loss after impulse or blast wave noise. This condition is not well recognized in occupational medicine and probably not even in otolaryngology. We report 52 cases of acute acoustic trauma including information concerning the traumatic event. Most cases occurred within military service and in the shipbuilding industry. Except for immediate hearing loss, many patients experienced tinnitus and some pain and hyperacusis. Relatively few patients report immediately. Most patients have been met by a nihilistic approach to therapy, in most cases due to the fact that patients report long after the trauma. The aim of the report is to focus attention on this clinical condition, since there is some indication that the final outcome may improve if patients are taken care of and treated early.

Acute Disease↗
PubMedDate not supplied

Discriminant analysis in predicting prognosis of Bell's palsy.

A prospective study was carried out to objectively quantificate the most important predictive factors in idiopathic facial paralysis (Bell's palsy). Stepwise discriminant analysis was applied to data prospectively obtained from 570 patients with Bell's palsy treated at the ENT Department of La Paz Hospital between January 1983 and January 1986. Due to statistical requirements the final number of patients included for discriminant analysis was 140. In all, 37 variables were studied in each patient. With data obtained from these variables a linear discriminant function was obtained, with an overall accuracy of 95%. The rate of correct prediction is 95.8% for patients with an expected complete facial recovery, and 90.9% for the group of patients with an expected recovery of less than 100%. The 8 predictor variables selected are: ENoG amplitude, stapedius reflex, familial incidence, Hilger test, maximum degree of the palsy, recurrent facial paralysis, facial pain, and hyperacusis. The discriminant function obtained is an easily adaptable method for routine practice in order to objectively assess the prognosis of patients with Bell's palsy.

Adult↗
PubMed1988

Audiological aspects of idiopathic perilymphatic fistula.

Audiological findings in 40 patients with surgically confirmed idiopathic perilymphatic fistula were investigated. Most patients complained of roaring tinnitus, hearing impairment, ear fullness, and hyperacusis. However, a popping sound and a streaming water-like sound were noticed only in 8. Pure-tone sensitivity included every kind of hearing impairment, from sudden profound, to normal sensitivity. Development of hearing impairment was sudden, and deteriorated further, fluctuating in order of frequency. In the early stage of perilymphatic fistula patients had low-frequency hearing impairment and evidenced dominant-negative SP in the electrocochleogram. These findings suggest that a fistula of the inner ear window is not solely responsible for the wide variety of signs and symptoms, and that there must be simultaneous lesions in the membranous labyrinth.

Adolescent↗