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At least 19 recordsLinked to original sources
PubMed1990

Hyperacusis and otitis media in individuals with Williams syndrome.

Williams syndrome is characterized by cardiac defects, varying degrees of physical and developmental delay, stellate eye pattern, possible elevated serum calcium level, and elfin/pixie facial features. A problem perhaps unique to these children is hyperacusis that can be severe enough to disrupt many routine daily activities. Parental questionnaires were used to determine the prevalence of hyperacusis and otitis media in individuals with Williams syndrome. Prevalences of 95% for hyperacusis and 61% for otitis media were found. This was significantly higher than in the general population. Despite the prevalence of hyperacusis, parents of these children were not counseled about management of the problem. The audiologist may become involved with Williams syndrome patients through hearing assessment and management, parental counseling, and research.

Adolescent↗
PubMed1987

Pathophysiology of tinnitus: a special case--hyperacusis and a proposed treatment.

The occurrence of hyperacusis is rare. In our Tinnitus Clinic, where more than 4,000 patients have been seen, hyperacusis has been seen only four times. Treatment of hyperacusis has been a combination of protection from external sounds along with desensitization to sound. Desensitization has been produced by very low intensity masking which is gradually increased every two to four weeks. The process of desensitization requires a great deal of time.

Adult↗
PubMed1976

Audiotympanometric findings in myasthenia gravis.

Myasthenia gravis can be a difficult diagnostic and therapeutic problem. Our study on six patients consisted of pure-tone audiograms, tympanometry, and acoustic reflex tests. Positive findings in the myasthenia gravis patients prior to medication included hyperacusis and increase in the intensity of sound required to elicit an acoustic reflex. Following medication, the hyperacusis lessened and the intensity of sound required to elicit the acoustic reflex decreased. During follow-up of two of the patients we encountered cholinergic (overdosage) crisis in one patient and myasthenic (underdosage) crisis in the other patient. Muscle weakness can be a common factor in both conditions. The acoustic reflex test can differentiate between the two types of crises. We believe audiotympanometric tests can aid in diagnosis and treatment of myasthenia gravis patients.

Acoustic Stimulation↗
PubMed1992

GM1 gangliosidosis type 2 in two siblings.

A sister and brother, now aged 7 and 9 years, presented with developmental arrest, gait disturbance, dementia, and a progressive myoclonic epilepsy syndrome with hyperacusis in the second year of life. Then, spastic quadriparesis led to a decerebrate state. In the absence of macular or retinal degeneration, organomegaly, and somatic-facial features suggesting mucopolysaccharidosis, the presence of hyperacusis together with sea-blue histiocytes in bone marrow biopsies and deficient beta-galactosidase activity but normal glucosidase, hexosaminidase, and neuraminidase activity on lysosomal enzyme assays constitutes the clinical-pathologic-biochemical profile of GM1 gangliosidosis type 2. This is a rare, late infantile onset, progressive gray-matter disease in which beta-galactosidase deficiency is largely localized to the brain, though it can be demonstrated in leukocytes and cultured skin fibroblasts. It must be distinguished from the Jansky-Bielschowsky presentation of neuronal ceroid lipofuscinosis, mitochondrial encephalopathy, lactic acidosis, strokelike episodes (MELAS) and myoclonic epilepsy with ragged-red fibers (MERRF) syndromes, atypical presentations of GM2 gangliosidoses (Tay-Sachs and Sandhoff's diseases), primary sialidosis (neuraminidase deficiency), galactosialidosis, and Alpers' disease.

Biopsy↗
PubMed1992

Prediction of recovery of Bell's palsy from clinical manifestations.

Sixty patients of Bell's palsy aged between 8 and 72 years, comprising 31 males and 29 females, were studied clinically to find out a method of prediction of recovery in early stage. It was found that young patients with incomplete palsy, unaccompanied by postauricular pain, loss of taste sensation over anterior 2/3rds of tongue hyperacusis and dry eye and recovery beginning within 4 weeks of onset of palsy are likely to make complete recovery, while older patients with complete palsy accompanied by severe postauricular pain, loss of taste sensation, hyperacusis and dry eye and beginning of recovery after 4 weeks of onset of palsy are most likely to have incomplete recovery.

Adolescent↗
PubMed1978

Acoustic reflex and loudness discomfort in acute facial paralysis.

An extensive research protocol was used to evaluate the conditions of 48 consecutive patients with acute facial paralysis. The results indicated that after nerve excitability testing, the acoustic reflex was the most efficient indicator of impending nerve degeneration and predictor of recovery. The presence of increased sensitivity to intense acoustic stimuli (loudness discomfort level) indicated poor prognosis. The data demonstrate that dysacusis (hyperacusis) is not related to stapedial muscle paralysis and also question the validity of "topographic diagnosis" in determination of the site of the lesion in facial paralysis.

Adolescent↗
PubMedDate not supplied

Tay-Sachs disease: B1 variant.

This first child of non-Jewish parents had nystagmus at 4 months of age, bilateral cherry-red macular spots at 7 months of age, and hyperacusis at 8 months of age; the patient has deteriorated progressively following a clinical course typical of Tay-Sachs disease B variant. Total beta-N-acetylhexosaminidase assayed with 4-methylumbelliferyl-beta-glucosamine (4 MU GlcNAc) as substrate was within the normal range in plasma and cultured dermal fibroblasts and 2/3 the normal mean in leukocytes. The hexosaminidase A activity, assayed with the same substrate in plasma and cultured fibroblasts, approximated Tay-Sachs disease heterozygote levels; however, the activity of hexosaminidase A assayed with 4 MU Glc NAc-6-sulfate in the plasma, leukocytes, and cultured fibroblasts was less than 8, 2, and 1%, respectively of the control mean. This female infant with the B1 variant of Tay-Sachs disease demonstrated an earlier onset and more rapidly progressive course than was observed in 4 of the 5 previously reported patients with this Tay-Sachs disease variant.

Female↗
PubMed1990

Audiological manifestations of Ramsay Hunt syndrome.

Ramsay Hunt syndrome is known to cause audiological signs and symptoms, including sudden, unexpected hearing loss. We carried out a retrospective review of the audiological manifestations of 186 patients with Ramsay Hunt syndrome, measuring their hearing loss patterns, hyperacusis, tinnitus, herpetic rash, facial paralysis, pain and vertigo. Statistical correlations of these parameters were equated with prognosis. Prognosis for eventual hearing recovery is, in general, excellent. Prognostic indicators of poor hearing recovery include advanced age, retrocochlear hearing loss, male gender, vertigo, and speech frequency hearing loss.

Adolescent↗
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↗
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↗
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↗
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↗