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Biomedical subjects

R J Ruben

Publications and source records attributed to R J Ruben.

At least 19 recordsLinked to original sources

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

Effectiveness and efficacy of early detection of hearing impairment in children.

Throughout the industrial world, technology and techniques are now available so that any child, no matter how young or how impaired, can have an accurate and precise assessment of middle ear function, auditory reactivity, and physiological processing of auditory stimuli. Yet, a major problem exists in the lack of timely identification of many children with hearing impairments. Presently, identification systems are primarily proactive and are based on technology. These consist of testing of infants with biological risk factors and the use of hearing screening programs at various times during the first decade of life. The reactive sources of referral appear to be inadequate, an impression that is supported by the data on the delay of diagnosis. These inadequacies appear to be due to a lack of awareness on the part of health providers as to the potential hearing losses; ignorance concerning the ability to diagnose them; and a lack of awareness of the potential of effective intervention. Two additional approaches are suggested which would be added to those already existing for improving the number of children who will have their diagnoses made in a timely fashion. The first of these is educational: health providers should have required and continuing education concerning the effects of hearing loss, the ability to diagnose, and to intervene effectively. A parallel educational program should be provided for the public. The second is the periodic assessment of speech and language from early infancy through the first few years of life for all children. This would enable children with suspected impairments to be referred for definitive testing.(ABSTRACT TRUNCATED AT 250 WORDS)

Age Factors

Auditory brain stem responses to bone-conducted tones in infants.

The auditory brain stem responses (ABRs) to 500- and 2,000-Hz bone-conducted (BC) tones were recorded from 48 infants with ears exhibiting various external and middle ear states (normal, otitis media, auditory meatal atresia). Amplitudes were greater, wave V latencies longer, and detectability better for responses to 500-Hz BC tones compared to 2,000-Hz BC tones. Overall, most (94% to 100%) infants with normal cochlear sensitivity demonstrate ABRs to 20-dB normal hearing level (nHL) 500-Hz BC tones and 30-dB nHL 2,000-Hz BC tones. In cases in which masking is difficult (eg, bilateral atresia), infant ipsilateral/contralateral ABR asymmetries may help determine from which cochlea a response to the BC tones originates. In conclusion, two-channel ABR recordings to BC tones appear to be feasible for demonstrating normal cochlear sensitivity in infants.

Audiometry, Evoked Response

Sex predilection in patients with acute frontal sinusitis.

We performed a retrospective analysis of 50 patients with acute frontal sinusitis who came to the Albert Einstein College of Medicine affiliated hospitals between 1981 and 1984. Cases were analyzed for age and sex in an attempt to determine the sex predilection for this disease in various age groups. Of the 50 patients studied, 38 (76%) were male and 12 (24%) were female. When subdivided into adult (older than 21 years of age) and adolescent (11 to 21 years of age) subgroups, a similar male predominance was noted. Among adults (28 patients), 21 (75%) were male and 7 (25%) were female. Among adolescents (22 patients), 17 (77%) were male and 5 (23%) were female. Of the 50 patients studied, 3 developed intracranial complications. All of these latter patients (all males) were in the adolescent group. Our findings indicate that male adolescents may be a high risk subgroup for developing intracranial complications secondary to their sinus infections.

Acute Disease

Spontaneous perilymphatic fistula in children.

Treatment of fistulae in children should be targeted at four different problem areas. The surgical intervention of these fistulae is most efficacious in controlling recurrent meningitis, which has not been discussed in this paper, and in the controlling of vestibular symptoms. Whether or not it will stop the progression of hearing loss or restore hearing is not known in a group of patients. It has been documented in this and other reports that the closure of the fistula will in specific cases appear to be associated in time with a stopping of the progression of the hearing loss and is associated also in time with the restoration of hearing function. Various factors must be considered in developing criteria for intervention. A decision to explore the ear should be based on history, audiometric change, vestibular testing including a fistula test, and malformation of bony labyrinth.

Adolescent

Treatment of recurrent respiratory papillomatosis with human leukocyte interferon. Results of a multicenter randomized clinical trial.

Recurrent respiratory papillomatosis is a relentless disease of viral origin in which squamous papillomas frequently obstruct the respiratory tract of children and young adults. No therapy has been proved to be curative for this process. Recent reports have suggested that interferon may cure or dramatically control airway papillomatosis. We evaluated the efficacy of human leukocyte interferon in the treatment of respiratory papillomatosis. One hundred twenty-three patients were randomly assigned to receive treatment with either surgery plus interferon or surgery alone. Interferon (2 X 10(6) IU per square meter of body-surface area) was given daily for one week, then three times per week for one year; treatment was followed by a year of observation, without the drug. Both study groups underwent serial endoscopy to remove papillomas and to document the efficacy of treatment during the two years of study. During the first six months, the growth rate of papillomas in the interferon group was significantly lower than in the control group (P = 0.0007). This difference diminished during the second six months and was no longer statistically significant (P = 0.68). Our data do not show that interferon is either curative or of substantial value as an adjunctive agent in the long-term management of recurrent respiratory papillomatosis. The initial benefit of interferon is not sustained.

Child, Preschool

Hearing results with the use of different tympanostomy tubes: a prospective study.

The audiograms of 59 patients who had 15 different types of tubes implanted in a prospective study from 1982 to 1985 were reviewed. The audiograms were analyzed for changes in air and bone thresholds at 250, 500, 1000, 2000, 4000 and 8000 Hz. The first post-operative audiogram was tabulated for all the initial post-operative visits and for only the initial post-operative visits in which the tubes were functioning. The change in pure tone average air conduction showed a range in improvement from 22.1 dB for the Armstrong Grommet Tube to 1.9 dB for the Canoe Tube. A total of 8 tube types showed mean improvements of 10 dB or better. With the exception of the Reuter Bobbin, all mean air conduction thresholds in functioning tubes were below 20 dB. For 5 tubes the mean threshold was less than 10 dB. The Armstrong Beveled Grommet, Donaldson, and Richard's Wing tubes showed the largest improvements in air conduction threshold with mean decreases of 22.1, 18.7 and 18.3 dB, respectively. Pure tone average bone conduction for all functioning tubes improved 2.8 dB.

Adolescent

Otitis media and language development at 1 year of age.

The effect of otitis media on emerging language was examined in a group of 1-year-olds. Based on pneumatic otoscopy, 15 babies were considered to be free of otitis media in both ears at 80% or more of their first year medical visits (otitis free) and 12 infants had bilaterally positive otoscopy results at 30% or more of their visits (otitis positive). Outcome measures at 1 year included the Bayley Scales of Infant Development (Bayley, 1969) and the Sequenced Inventory of Communication Development (SICD; Hedrick, Prather, & Tobin, 1984) Receptive and Expressive scales. No significant differences were detected on either the Bayley or the SICD Receptive scale. However, the otitis positive group exhibited significantly lower expressive language scores than the otitis free group, suggesting that impairments in language expression may be evident as early as 1 year of age in children with otitis media.

Acoustic Impedance Tests

Otitis media, auditory sensitivity, and language outcomes at one year.

The relationship among otitis media, auditory sensitivity, and emerging language was examined in a group of 1-year-old children who were prospectively followed since birth. Pneumatic otoscopy was used to document the otologic status of the children's ears at each medical visit. There were 13 babies with normal ratings in each ear at 80% more of their visits (designated as "otitis free") and 12 babies with bilaterally positive otoscopy results at 30% or more of their first year visits (designated "otitis positive"). In comparison to the otitis free infants, the group of otitis positive babies demonstrated reduced auditory sensitivity as measured by auditory brain stem response (ABR) and poorer expressive language abilities. However, differences in receptive language were not detected. These results suggest that otitis media may have an impact on auditory sensitivity and developing language as early as 1 year of age.

Audiometry, Evoked Response

Otitis media in neonatal intensive care unit graduates: a 1-year prospective study.

A group of 46 high-risk infants (graduates of a neonatal intensive care unit) and 19 full-term infants were observed prospectively for middle ear status beginning at 40 weeks' postconceptional age. All infants were born to families living in low socioeconomic urban neighborhoods. Pneumootoscopy was used to determine the presence or absence of middle ear effusion during periodic medical and nonmedical visits throughout a 1-year period. Of all infants studied, 91% had at least one episode of otitis media with effusion during the observation interval. There were no differences in the percentages of visits during which high-risk and full-term infants experienced either normal middle ears bilaterally or otitis media with effusion in one or both ears. Furthermore, the age of onset of otitis media with effusion was similar for the two groups of babies. No differences were found between boys and girls in the age of onset for otitis media or in the percentage of visits at which otitis media with effusion was detected. Hispanic infants experienced their initial episode at significantly younger ages than did black infants in the sample. Both groups had similar percentages of visits attributable to otitis media with effusion during the observation period. The results indicate a high incidence among the infants studied and similar otologic courses for neonatal intensive care unit graduates and full-term infants during the first year of life.

Age Factors