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Biomedical subjects

R J Ruben

Publications and source records attributed to R J Ruben.

At least 37 records · Page 2Linked to original sources

Otitis media, communication style of primary caregivers, and language skills of 2 year olds: a preliminary report.

The association between early otitis media, styles of primary caregivers, and language development was prospectively examined in 26 2-year-old children from lower socioeconomic backgrounds (of mixed perinatal status) followed prospectively since birth. Based on otoscopic evaluations during the first year of life, there were 14 children who were classified as bilaterally otitis-free (OM-) and 12 children who were classified as bilaterally otitis-positive (OM+). At 2 years of age, all children were administered standardized measures of cognitive and language function along with a 20-minute videotaped play session with one of their primary caregivers. The language of both the children and caregivers was analyzed. Results indicate that caregivers of OM+ and OM- children used language nearly identically when interacting with their children. However, caregivers of OM+ children whose communication style was marked by a high percentage of directives and a corresponding low percentage of both questions and information-giving had the children with the lowest scores on all measures of naturalistic language. Controlling for neonatal illness did not alter the relationships found. The results suggest that caregiver language may compensate for some of the auditory deprivations associated with otitis media.

Brain Damage, Chronic↗

Auditory consequences of early mild hearing loss associated with otitis media.

Two groups of children received periodic documentation of their middle ear status by pneumatic otoscopy in the first year of life and had hearing sensitivity estimated by multiple auditory brainstem response (ABR) assessments during the same period. One group was considered otitis media (OM)-positive, with repeated bilateral episodes of OM and mild conductive hearing loss. The second group was considered OM-free with normal middle ear function bilaterally and normal hearing sensitivity in the first year of life. Children's higher-order auditory abilities were examined at 4, 6 and 9 years of age. Various measures such as speech recognition-in-competition, visual-auditory learning, sound blending, auditory memory, and masking level difference estimates were utilized to probe the children's auditory abilities. Results show that children with a first-year history of otitis media demonstrate deficits in the long term in some aspects of higher-order auditory processing. Performance appears to be associated with the mild hearing loss experienced during an important period of early development.

Child↗

Effect of neurotrophic factors on the inner ear: clinical implications.

Loss of auditory neurons is commonly associated with sensorineural deafness, and may result from either direct neuronal injury or be a consequence of sensory hair cell loss (i.e. loss of source of trophic factors). Developmental studies and in vitro studies of adult neurons have begun to identify growth factors important for the development, maintenance, and rescue/repair of auditory neurons. Specific neurotrophic factors have been shown to enhance the auditory neurons' ability to withstand traumatic loss of target tissue connections and toxic injury. Promising initial in vivo studies confirm that specific neurotrophins are able to support neuronal survival and promote neuronal repair in an intact animal following injury to the cochlea. Further study into unique methods and routes of growth factor delivery will provide insights into the possibility of neurotrophic growth factors to act as drugs for the treatment of injured or stressed auditory neurons.

Animals↗

Early otitis media and later educational risk.

Fourteen children aged 6 years received screening for educational risk status and formal measures of academic performance. These children have been followed prospectively from birth for their otitis media status. Seven of the children were considered positive for otitis media in year one (recurrent episodes bilaterally), while 7 were considered otitis free. Academic status was related to early hearing acuity (as determined by repeated ABR assessment). Moreover, teachers' ratings of academic risk status were related to a measure at 4 years that examined the children's capacity to listen in background noise.

Achievement↗

Acute Streptococcus pneumoniae meningogenic labyrinthitis. An experimental guinea pig model and literature review.

OBJECTIVE: To create an experimental model of Streptococcus pneumoniae type 3 meningogenic labyrinthitis (a leading cause of deafness) similar to that in human disease. DESIGN: Cohort analytic study of guinea pigs that were inoculated intrathecally with varying dilutions of S pneumoniae type 3; the progress of the disease was compared with that in saline solution-inoculated control animals. SUBJECTS: Healthy adult Hartley guinea pigs without clinical evidence of middle ear disease that were conveniently sampled. INTERVENTIONS: Intrathecal inoculation of 10(4) to 10(6) colony-forming units of S pneumoniae type 3 into 13 guinea pigs; signs and symptoms of meningitis/labyrinthitis were observed for 15 days and compared with those in two saline solution-inoculated control animals. MAIN OUTCOME MEASURES: Morbidity--labyrinthitis, meningitis; end point--death. RESULTS: The 10(4) to 10(6) colony-forming units of S pneumoniae type 3 caused inflammation that extended from the meninges to the inner ear via the cochlear aqueduct within 3 days after inoculation; a dose of 10(7) killed animals within 12 hours after inoculation. Three of five animals that were inoculated with a 10(6) dose died 3 days after inoculation; two of three animals that were inoculated with a 10(5) dose lived to 15 days after inoculation. One of two animals that were inoculated with a 10(4) dose did not become infected. Inflammation extended to the middle ear by round-window destruction. Reactive bone formation simulated labyrinthine osteosclerosis. Observers assessed histologic slides "blindly." CONCLUSION: Guinea pigs can survive 15 days after intrathecal inoculation of a 10(5) dose, with morphologic features similar to those in human disease. This is an effective model for this study of meningogenic labyrinthitis.

Acute Disease↗

Reconstruction of the pediatric airway with an open stented tracheotomy tube.

Reconstructive techniques for the stenotic pediatric airway have greatly enhanced the otolaryngologists ability to decannulate the tracheotomy-dependent child. Stents have played an important role in this process by both maintaining the reconstructed lumen and acting as a scaffold. However, decannulation is often not achieved after the first surgical intervention with some patients requiring two, three or more procedures. In an effort to improve upon the design of today's stents, an open stented tracheotomy tube was evaluated at our institution. This stent is designed to provide advantages of immediate postoperative voice and nutrition, and ease of evaluation, as well as to provide for safe and adequate healing. Four patients underwent laryngotracheal reconstruction with this stent over a 10-month period. All patients could vocalize and eat immediately postoperatively but recalcitrant granulation tissue and restenosis has prevented decannulation in three of the cases. The experience with this stent exemplifies the persistent difficulties of stent use in pediatric airway reconstruction. It also points out that progress is still needed in current reconstructive techniques as well as in the development of new techniques.

Adolescent↗

Communicative disorders. The first year of life.

Disorders of communication have high incidence and prevalence in the fetus, neonate, and infant, and optimal care is dependent on early recognition and intervention. This article reviews receptive disorders, expressive disorders, and their diagnoses.

Communication Disorders↗

Evidence of abnormal stromelysin mRNA expression in suspected carriers of otosclerosis. A possible molecular marker.

OBJECTIVE: There is strong evidence that otosclerosis is a genetic disease affecting bone remodeling. We propose that, if otosclerosis is genetic, it will manifest itself universally, particularly at the mRNA transcription level. DESIGN: Skin biopsy specimens were taken in a single blind from human subjects who had been clinically and surgically identified as having otosclerosis. SETTING: Subjects were volunteers from the community, identified through hospital records. All procedures were carried out in a clinical research facility. PATIENTS: Twenty-one volunteers underwent a biopsy, including those positively identified as having otosclerosis (n = 4), their blood relatives (n = 8), or nonrelatives with normal hearing and no known history of otosclerosis (n = 9). INTERVENTION: Three connective tissue remodeling factors, procollagenase, prostromelysin, and tissue inhibitor of metalloprotease, were analyzed. The mRNA was extracted from each biopsy specimen, hybridized against radiolabeled cDNA, and quantitatively measured by radioautography. MAIN OUTCOME MEASURE: We expected to see significant differences in the pattern of mRNA expression for one or more of the three measured bone remodeling factors in otosclerotics, compared with age- and sex-matched negative controls. RESULTS: Two of the otosclerotic subjects had abnormally low levels of mRNA for prostromelysin and two had higher than normal levels. In three (75%) of the four, variability of mRNA expression among procollagenase, prostromelysin, and metalloprotease tissue inhibitor was higher than normal. Three (38%) of the eight relatives showed a similar pattern and two (22%) of the nine control subjects also tested as abnormal. CONCLUSION: This observed variability in otosclerotic subjects might be a manifestation of a genetic control defect, and abnormal stromelysin mRNA expression could serve as a genetic marker for otosclerosis.

Adolescent↗

Communication disorders in children: a challenge for health care.

BACKGROUND: Today's children will participate in a society whose economic basis will be information and communication and they must have optimal communication skills to be able to be functional optimally within the information society. Heretofore, in the United States, there has been no systematic attempt to diagnose and care for children with communication disorders. METHODS: It is essential that the child be diagnosed early because there appears to be a critical period for language development. CONCLUSION: If interventions do not occur early, in the area of communication disorders, there is a high probability of significant morbidity because of impaired communication abilities. This morbidity can manifest itself in a social activity and loss of economic opportunity.

Adolescent↗

The ontogeny of human hearing.

The development of human hearing from the 26th week of fetal life to the third year post natal life is reviewed. The human fetus can perceive, react, and store auditory information from the 26th week of fetal life. This ability appears to play a role in the development of language. Infants by the 2nd to 3rd month of postpartum life have categorical perception for most phonemes whether or not these phonemes are native to their own language. The infant has a difference limen similar to that of the adult. The infant threshold is higher; the single-to-noise ratio is greater in infants than adults; and the infant's temporal processing is slower than that of the adult.

Auditory Pathways↗

Nerve growth factor stimulates neurite regeneration but not survival of adult auditory neurons in vitro.

Injury to either the peripheral or central nervous system results in the accumulation of growth factors at the wound site. Some of these growth factors have been shown to participate in the neural repair process. Adult auditory neurons grown in dissociated spiral ganglion cell cultures are injured (i.e. bilateral axotomy) as a result of the initial preparation of these cultures. Therefore, cell cultures of dissociated spiral ganglia provide a model for the study of repair processes of adult auditory neurons (e.g. effects of exogenous growth factors on the process of neuritogenesis by injured neurons). Auditory neurons do not survive in these dissociated ganglion cell cultures when only exogenous NGF is added to the defined culture medium. Previous work has identified substrate bound basic fibroblast growth factor (bFGF) as a survival factor for adult auditory neurons in vitro. Auditory neurons cultured on substrate bound bFGF also do not show increased survival in response to the addition of increasing concentrations of nerve growth factor (NGF) to the defined medium. This is in sharp contrast to the pronounced neurite outgrowth-promoting effects (concentration dependent) observed when exogenous NGF is added to adult auditory neurons cultured on substrate bound bFGF. We propose that several neuronotrophic factors (e.g. TGFB1, bFGF, NGF and other neurotrophins) are active in the spiral ganglions' response to injury. Several of these growth factors (i.e. bFGF, NGF) act in cooperation to promote the regeneration or repair of severed or traumatized neuritic processes.

Animals↗

Aberration of the tissue collagenase system in association with otosclerosis.

Studies of aural and other body tissues suggest that otosclerosis represents the local manifestation of a general disorder of connective tissue. In particular, collagen abnormalities have been described. We have undertaken a pilot study of the in vivo messenger RNA (mRNA) transcription for procollagenase (precursor of collagenase), as well as for stromelysin and tissue inhibitor of metalloprotease (TIMP), an activator and a specific inhibitor of tissue collagenase activity, respectively. Human skin from individuals with surgically confirmed otosclerosis was compared to skin from their family members (clinically positive and clinically negative) and from unrelated normal controls. Preliminary data indicate that on average there are significantly lower levels of mRNA production for stromelysin among individuals with otosclerosis as compared to all others tested. Similar trends were demonstrated for TIMP and procollagenase, although these did not achieve statistical significance. In addition to suggesting a pathogenetic mechanism for the development of the disease, these data could serve as the basis of possible confirmatory tests for early diagnosis of otosclerosis and as a method for evaluating the genotype of offspring of affected individuals prior to their age of clinical manifestation. This could translate into the application of prophylactic treatment regimens in the future. The proposed abnormalities also suggest candidate genes for otosclerosis.

Audiometry↗

Language screening as a factor in the management of the pediatric otolaryngic patient. Effectiveness and efficiency.

The Early Language Milestone Scale, a screening test for language in children from birth to 36 months of age, was applied to the total population of pediatric otolaryngic patients. Ninety-seven percent of the patients were found to have a completed screen. Thirty percent of the patients failed one or more portions of the Early Language Milestone screen. Practice management was modified by the results of the Early Language Milestone in all the patients who failed the screen. The Early Language Milestone was found to be efficient and effective in contributing to the management of the children with pediatric otolaryngic disorders and diseases.

Child, Preschool↗