1/25. Cutaneous-evoked tinnitus. I. Phenomenology, psychophysics and functional imaging.DC00166e and acute unilateral deafferentation of the auditory periphery (auditory and vestibular afferents) can induce changes in the central nervous system that may result in unique forms of tinnitus. These tinnitus perceptions can be controlled (turned on and off) or modulated (changed in pitch or loudness) by performing certain overt behaviors in other sensory/motor systems. Clinical reports from our laboratory and several other independent sources indicate that static change in eye gaze, from a neutral head-referenced position, is one such behavior that can evoke, modulate and/or suppress these phantom auditory events. This report deals with a new clinical entity and a form of tinnitus that can be evoked directly by cutaneous stimulation of the upper hand and fingertip regions. In 2 adults, cutaneous-evoked tinnitus was reported following neurosurgery for space-occupying lesions at the base of the skull and posterior craniofossa, where hearing and vestibular functions were lost completely and acutely in one ear (unilateral deafferentation) and facial nerve paralysis (unilateral deefferentation) was present either immediately following neurosurgery or had occurred as a delayed-onset event. Herein, we focus on the phenomenology of this discovery, provide perceptual correlates using contemporary psychophysical methods and document in one individual cutaneous-evoked tinnitus-related neural activity using functional magnetic resonance imaging. In a companion paper, neuroanatomical and physiological interactions between auditory and somatosensory systems, possible mechanistic accounts and relevant functional neuroimaging studies are reviewed.- - - - - - - - - - ranking = 1keywords = physical (Clic here for more details about this article) |
2/25. noise-induced hearing loss.hearing loss caused by exposure to recreational and occupational noise results in devastating disability that is virtually 100 percent preventable. noise-induced hearing loss is the second most common form of sensorineural hearing deficit, after presbycusis (age-related hearing loss). Shearing forces caused by any sound have an impact on the stereocilia of the hair cells of the basilar membrane of the cochlea; when excessive, these forces can cause cell death. Avoiding noise exposure stops further progression of the damage. noise-induced hearing loss can be prevented by avoiding excessive noise and using hearing protection such as earplugs and earmuffs. patients who have been exposed to excessive noise should be screened. When hearing loss is suspected, a thorough history, physical examination and audiometry should be performed. If these examinations disclose evidence of hearing loss, referral for full audiologic evaluation is recommended.- - - - - - - - - - ranking = 7.035625745518keywords = physical examination, physical (Clic here for more details about this article) |
3/25. High-resolution three-dimensional magnetic resonance imaging of the vestibular labyrinth in patients with atypical and intractable benign positional vertigo.Benign paroxysmal positional vertigo (BPPV) is a most common cause of dizziness and usually a self-limited disease, although a small percentage of patients suffer from a permanent form and do not respond to any treatment. This persistent form of BPPV is thought to have a different underlying pathophysiology than the generally accepted canalolithiasis theory. We investigated 5 patients who did not respond to physical treatment, presented with an atypical concomitant nystagmus or both with high-resolution three-dimensional magnetic resonance imaging of the inner ear. This method provides an excellent imaging of the inner ear fluid spaces. In all 5 patients, we found structural changes such as fractures or filling defects in the semicircular canals which we did not find in control groups. One patient clinically presented with the symptoms of a 'heavy cupula'. Whereas crosssections through the ampullary region and the adjoining utricle showed no abnormalities, there were significant structural changes in the semicircular canals, which are able to provide an explanation for the symptoms of a heavy cupula.- - - - - - - - - - ranking = 1keywords = physical (Clic here for more details about this article) |
4/25. vertigo and the enlarged vestibular aqueduct syndrome.An enlarged vestibular aqueduct (EVA) is one of the most commonly identified inner ear bony malformations in children with sensorineural hearing loss of unknown cause. Most previous reports have focused on hearing loss, but individuals with EVA may also experience paroxysmal vertigo lasting minutes to hours. We report the clinical vestibular features and vestibular function testing of two children and one adult with EVA who had a history of sensorineural hearing loss and presented to our neurotology Clinic for the evaluation of episodic vertigo. All the patients had an antecedent history of profound bilateral sensorineural hearing loss that had been present since early childhood. The onset of vertigo was delayed into adulthood in one patient. Episodes of vertigo could be triggered by minor head trauma or vigorous physical activity. Despite recurrent episodes of vertigo, vestibular function was normal or moderately impaired compared with the severe auditory deficit. Careful analysis of temporal bone CT demonstrated EVA. Associated enlargement of the membranous endolymphatic sac was evident on brain MRI. While hearing loss is a prominent symptom in patients with EVA, vestibular symptoms may cause referral to a neurologist. Although hearing loss occurs early in childhood, vestibular symptoms can be delayed into adulthood, a finding not previously reported.- - - - - - - - - - ranking = 1keywords = physical (Clic here for more details about this article) |
5/25. genetic counseling for the deaf.genetic counseling is a process that emphasizes accurate diagnosis of hereditary conditions and communication of information to families. genetic counseling involves systematic collection of family and medical history, a physical examination by a certified clinical geneticist, sharing of information with the family, and follow-up and support services. The issues that arise in genetic counseling can differ for every family and are often dependent on the degree of deafness present in the family, age of onset, and linguistic and cultural orientation. It is important for the genetic counselor to consider these factors in the provision of genetic services. With the increasing application of molecular genetics to the diagnosis and management of hereditary deafness and the increasing participation of families with deafness in research studies, the involvement of genetic counselors to provide information and education to consumers as well as medical professionals and researchers is becoming even more critical. The success of genetic counseling for the provision of information to families and the delineation of types of hereditary deafness through clinical and laboratory research is dependent on appropriate referrals by medical professionals, including otolaryngologists. A working relationship between otolaryngologists and clinical geneticists for the referral and evaluation of patients with hereditary deafness or deafness of "unknown" etiology is important.- - - - - - - - - - ranking = 7.035625745518keywords = physical examination, physical (Clic here for more details about this article) |
6/25. Wegener's granulomatosis diagnosed by testicular biopsy.Urogenital involvement, other than the kidneys, is extremely rare in Wegener's granulomatosis (WG) and occurs in less than 1% of the cases. When encountered it is confined to prostate, bladder, urethra, cervix, and vagina. Granulomatous infiltration of the testis from WG has not been cited in the medical literature. We report a case of WG in a Hispanic male who presented with sensorineural hearing loss and hemoptysis. He had a pulmonary lesion and a painless right testicular mass, which was found to have necrotizing granulomas on excisional biopsy. This may be the first reported case of WG diagnosed by testicular biopsy as testicular involvement is rare in WG. We believe that the actual incidence of testicular involvement in WG may be higher as genital examination may be ignored during routine physical examinations.- - - - - - - - - - ranking = 7.035625745518keywords = physical examination, physical (Clic here for more details about this article) |
7/25. Childhood-onset osteoarthritis, tall stature, and sensorineural hearing loss associated with Arg75-Cys mutation in procollagen type II gene (COL2A1).OBJECTIVE: To define the clinical, radiologic, and molecular genetic characteristics of a family with early progressive osteoarthritis mimicking childhood rheumatoid arthritis, Scheuermann-like changes of the spine, tall stature, short 3 and 4 metatarsals, and moderate sensorineural hearing loss. methods: We describe a 22-year-old woman and her 54-year-old mother with early progressive osteoarthritis mimicking childhood rheumatoid arthritis. The index case, her mother, and 3 other family members underwent a physical examination, anthropometric measurements, and radiologic studies. Their dna was sequenced for the procollagen type II (COL2A1) gene. RESULTS: Mild scoliosis was noticed in the proband at the age of 6 years, and at the age of 7 years large Schmorl's nodes were found in the vertebrae L1-2. At the age of 11 years, changes resembling Scheuermann's disease were seen, mostly in the thoracic vertebrae. At the same age, she began to have arthralgia in the weight-bearing joints and osteoarthritis progressed fast, necessitating a hip prosthesis at the age of 18 years. The proband and her mother had bilateral sensorineural hearing loss of moderate degree. Both mother and daughter had an Arg75-Cys mutation in the COL2A1 gene. CONCLUSION: This family is the fourth example of the Arg75-Cys mutation in the COL2A1 gene, which appears to lead to a clearly recognizable phenotype. The finding suggests that sensorineural hearing loss may be a part of this syndrome.- - - - - - - - - - ranking = 7.035625745518keywords = physical examination, physical (Clic here for more details about this article) |
8/25. Progressive cone dystrophy and sensorineural hearing loss.A 39-year old man presented 13 years ago with a history of progressive loss of vision and photophobia. A full ophthalmological and ENT work-up during several years of follow-up, including psychophysical as well as electrophysiological tests, revealed a progressive cone dystrophy in combination with sensorineural hearing loss. His younger sister presented with very similar features and underwent the same work-up. A novel syndrome of progressive cone dystrophy and sensorineural hearing loss is described in both siblings. Both also suffered from non-ocular disease possibly related to ciliary dysfunction. The condition is likely to be inherited as an autosomal recessive trait.- - - - - - - - - - ranking = 1keywords = physical (Clic here for more details about this article) |
9/25. temporal bone histopathology of a patient with a nucleus 22-channel cochlear implant.The temporal bone histopathology of a patient with profound sensorineural hearing loss attributable to cochlear otosclerosis is presented. The patient had a Nucleus 22-channel cochlear prosthesis implanted in the right ear prior to his demise. Unintentional scala media insertion of the electrode array resulted from labyrinthitis ossificans obliteration of the scala tympani. Inadvertent facial nerve stimulation necessitated program exclusion of electrodes 11 to 16 located in proximity to the labyrinthine and geniculate segments of the facial nerve. The patient received significant benefit from use of the device. The histopathologic findings are correlated with psychophysical and speech perception results.- - - - - - - - - - ranking = 1keywords = physical (Clic here for more details about this article) |
10/25. Congenital rubella infection from reinfection of previously immunised mothers.Two children developed congenital rubella infection when their mothers had been proven to be satisfactorily immunised against rubella before the affected pregnancy. One child was severely affected with heart lesions, brain damage, severe deafness, physical retardation, cataracts and rubella retinopathy. The other child had moderately severe sensorineural deafness and a mild reduction in visual acuity due to rubella retinopathy.- - - - - - - - - - ranking = 1keywords = physical (Clic here for more details about this article) |
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