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Biomedical subjects

S A Telian

Publications and source records attributed to S A Telian.

At least 19 recordsLinked to original sources

Multichannel cochlear implantation in visually impaired patients.

OBJECTIVE: To evaluate the outcome of cochlear implantation in patients with severe to profound hearing loss and visual impairment. STUDY DESIGN: Retrospective case review. SETTING: Tertiary referral center with a large cochlear implant program. PATIENTS: Six adults and two children with severe or profound hearing loss and significant visual impairment underwent multichannel cochlear implantation. Follow-up period ranged from 6 months to 9 years. Case history, etiology of visual and hearing loss, and benefit from cochlear implant were evaluated. INTERVENTIONS: Cochlear implantation and subsequent rehabilitation. MAIN OUTCOME MEASURES: Speech perception measures were selected based on the patient age and cognitive abilities. Identical measures were used in each patient before and after implantation. RESULTS: As a group, patients did well after cochlear implantation. There was significant improvement in speech perception when compared with the score before implantation. CONCLUSIONS: Cochlear implants can play a significant rehabilitative role in patients with severe visual and auditory impairment. Additional skills are required by the implant team for rehabilitation of patients with multiple sensory deficits.

Adult↗

Labyrinthectomy versus vestibular neurectomy: long-term physiologic and clinical outcomes.

OBJECTIVE: To determine whether long-term vestibular compensation (VC) and clinical outcomes differ after transmastoid labyrinthectomy (TML) versus retrolabyrinthine vestibular neurectomy (RVNS). STUDY DESIGN: Prospective, observational study. SETTING: Tertiary care, university hospital. PATIENTS: Twenty-one subjects were studied several years after they were relieved of spontaneous episodic vertigo caused by peripheral vestibular disease by TML or RVNS. INTERVENTIONS: All patients had undergone TML or RVNS more than 2.5 years before the study and returned for physiologic and functional studies of vestibular compensation. MAIN OUTCOME MEASURES: Completeness of physiologic VC, as assessed by electronystagmography and rotational chair testing; performance on computerized dynamic posturography; pure-tone and speech audiometry; self-assessment of balance and hearing function with validated survey instruments. RESULTS: There were no differences in the incidence of physiologic VC or functional recovery between the TML and RVNS subjects. Although a majority of subjects in each group had evidence of incomplete vestibular compensation, there was no difference in self-assessment of balance or hearing handicap at long-term follow-up. CONCLUSIONS: Long-term clinical balance and hearing outcomes are equivalent when TML and RVNS successfully cure spontaneous, episodic vertigo. There is a high incidence of incomplete VC after both procedures, though this does not usually produce a significant balance handicap.

Auditory Threshold↗

Implantation of the malformed cochlea.

OBJECTIVE: This study assesses the results of cochlear implantation in children with cochlear malformations. STUDY DESIGN: Retrospective, matched-pairs analysis of prospectively collected data. SETTING: University-based regional cochlear implant center. PATIENTS: Seventeen children with osseous cochlear malformations who had undergone implantation were matched and compared with a group of children with normal cochleae who had undergone implantation. INTERVENTIONS: All subjects received a multichannel cochlear implant and habilitation. MAIN OUTCOME MEASURES: All subjects were tested with the Early Speech Perception test, and the Glendonald Auditory Speech Perception tests for words and sentences. Patients were classified in a standardized speech perception category based on performance on the Early Speech Perception test. RESULTS: All subjects demonstrated improving performance on all measures of speech perception over time. Overall, the two groups showed no statistically significant differences in performance at 6 and 24 months. However, subjects with malformed cochleae evidenced slower rates of improvement than did their matched control subjects. Subjects with more severe malformations demonstrated poorer performance, but this may have been attributable to preoperative factors rather than to implant performance. CONCLUSIONS: Children with radiographic cochlear malformations benefit from cochlear implantation with multichannel devices. They ultimately perform as well as their matched counterparts with normal cochleae, although they may improve more slowly over time.

Child↗

Diagnosis and initiating treatment for peripheral system disorders: imbalance and dizziness with normal hearing.

Disorders affecting the peripheral vestibular system commonly involve the peripheral auditory system causing hearing loss. There are a number of disorders, however, that selectively involve the peripheral vestibular system causing dizziness without hearing loss. These disorders include benign paroxysmal positional vertigo, vestibular neuritis, recurrent vestibulopathy, familial vestibulopathy, and bilateral idiopathic vestibulopathy. This article reviews these disorders and their diagnosis and management.

Diagnosis, Differential↗

Recurrence of acoustic neuroma after incomplete resection.

OBJECTIVE: To determine the risk of recurrent tumor in patients after incomplete resection of acoustic neuroma. STUDY DESIGN: Retrospective case review. SETTING: A tertiary referral center. PATIENTS: Thirty-nine patients were identified who underwent incomplete resection of acoustic neuroma between January 1988 and December 1993, and had a minimum follow-up of 3.5 years, at a mean of 6.2 years (range 3.5-10.2 years). INTERVENTION: Regular follow-up. using either computed axial tomography or magnetic resonance imaging yearly. MAIN OUTCOME MEASURES: Growth of residual tumor as determined by the increase in its greatest dimension on follow-up imaging studies and the necessity for additional treatment. RESULTS: Tumor regrowth occurred in 17 patients (44%). Ten patients (26%) required additional treatment during the follow-up period. CONCLUSION: This study suggests that incomplete resection of acoustic neuroma is associated with a significant risk of recurrent tumor requiring subsequent intervention.

Adult↗

Successful cochlear implantation in a patient with MELAS syndrome.

OBJECTIVE: To describe methods of assessing cochlear implant candidacy in patients with potentially significant peripheral and central nervous system (CNS) degeneration. STUDY DESIGN: A patient with a degenerative CNS disease (MELAS syndrome) undergoing evaluation for cochlear implantation is described. SETTING: This study took place at a tertiary care center. PATIENT: A patient with mitochondrial encephalopathy, lactic acidosis, and stroke-like episodes (MELAS) who had cortical blindness and profound sensorineural hearing loss was evaluated and rehabilitated with cochlear implantation. INTERVENTIONS: Pure-tone audiogram, behavioral responses to promontory stimulation electrical auditory brainstem response, and electrically evoked middle-latency responses (MLRs) were used to assess eighth nerve, auditory brainstem, and cortical auditory pathways. Cochlear implantation with Cochlear Corporation mini 22 implant was performed. RESULTS: Repeatable electrically evoked MLRs and behavioral responses to promontory stimulation documented the presence of auditory cortical responses. Successful implantation resulted in open set speech recognition and communication using the auditory/oral mode. CONCLUSION: This report describes successful implantation in a patient with MELAS syndrome and demonstrates the ability to preoperatively confirm the integrity of brainstem and cortical auditory pathways despite significant CNS degeneration.

Adult↗

Minimizing wound complications in cochlear implant surgery.

OBJECTIVE: An extended postauricular incision has replaced the standard C-shaped scalp flap for cochlear implant surgery at our institution. The postoperative wound complication rates of the two incisions were evaluated. STUDY DESIGN: This study was a retrospective case review. SETTING: This study was performed in a tertiary referral center. PATIENTS: A total of 256 adult and pediatric patients who underwent cochlear implantation during a 10-year period (1986 to 1996) were reviewed. MAIN OUTCOME MEASURE: Postoperative wound complications were identified. Major complications included flap necrosis, wound dehiscence with or without implant exposure, and wound infection requiring hospitalization. Hematoma, seroma, or superficial wound infections were considered minor complications. RESULTS: There were 6 major and 6 minor complications among 116 patients with the standard scalp flap (complication rate, 10.3%). There was only 1 minor complication among 140 implants using the postauricular incision (0.7%). CONCLUSION: The extended postauricular incision appears to significantly reduce the incidence of wound complications in cochlear implant surgery.

Adolescent↗

AP30, a differential protein marker for perilymph and cerebrospinal fluid in middle ear fluid, has been purified and identified as human apolipoprotein D.

Using two-dimensional (2-D) gel electrophoresis, human perilymph and cerebrospinal fluid have been shown to be highly enriched for an acidic protein with MR 30,000, we designated it as AP30. The protein exhibits charge heterogeneity, with at least eight isoforms visible between pI 4.5 to 5.5 on 2-D gels. Purification of the protein was carried out by ammonium sulfate precipitation, polybuffer exchanger column chromatofocusing, and acetone fractional precipitation. The resulting preparation also contains eight spots in the acidic area of 2-D gels, and one broad band located at Mr 30,000 by SDS-PAGE. Digestion of AP30 with neuraminidase causes the isoforms to shift to a more basic position and to consolidate into two primary spots, indicating that AP30 is a variably sialylated glycoprotein. Amino acid analysis of AP30 revealed an amino acid content very similar to that of human apolipoprotein D. Attempts to determine the amino acid sequence demonstrated that the N-terminus is blocked. Edman sequencing of two peptide fragments, generated by cyanogen bromide cleavage of AP30, both revealed sequences having 100% identity to human apolipoprotein D. Western blot analysis of AP30 with the antibody against authentic human apolipoprotein D demonstrated a high degree of cross-reactivity. These studies indicate that AP30 from human perilymph and cerebrospinal fluid is a member of the apolipoprotein D family.

Amino Acid Sequence↗

Evaluation of clinical measures of equilibrium.

OBJECTIVE: Evaluate the clinical utility of several simple measures of static and dynamic equilibrium in human subjects. In particular, one proposed clinical measure, the Clinical Test of Sensory Integration and Balance (CTSIB) was compared with dynamic posturography for the measurement of postural control capabilities. STUDY DESIGN: Cross-sectional study of normal subjects and prospective observational study of the same performance measures in vestibular disorder patients. SETTING: Academic tertiary care referral center. PARTICIPANTS: Data were collected for all test measures from a group of normal subjects (ages, 20 to 79 years), as well as for a group of patients undergoing treatment for vestibular dysfunction. RESULTS: Data suggest that several semiquantitative clinical tests of static and dynamic equilibrium can be helpful in evaluating and monitoring patients with chronic vestibular dysfunction. The CTSIB results seem to correlate well with dynamic posturography, suggesting that this measure may be useful in identifying patients with abnormal postural control. Formal dynamic posturography testing appears to be more sensitive in detecting abnormal postural control and more exact in defining the specific pattern of dysfunction. CONCLUSION: Simple clinical measures of static and dynamic equilibrium can reliably distinguish vestibular disorder patients from normal subjects. Dynamic posturography continues to play an important role in the functional evaluation and management of vestibular disorder patients.

Adult↗

Familial Mondini dysplasia.

OBJECTIVES/HYPOTHESIS: To determine the mode of inheritance of familial nonsyndromic Mondini dysplasia. STUDY DESIGN: Correlative clinical genetic analysis of a single kindred. METHODS: Clinical history, physical examination, audiologic analysis, computed tomography of the temporal bones, and cytogenetic analysis. RESULTS: The male proband, three affected sisters, and an affected brother are offspring of unaffected parents. The mother and an unaffected brother have audiologic findings suggestive of heterozygous carrier status for a recessive hearing loss gene. CONCLUSIONS: Pedigree analysis indicates autosomal recessive inheritance in this family. The observed inheritance and clinical, audiologic, and radiologic findings are different from those previously described for another family with nonsyndromic Mondini dysplasia. The phenotype in this study family therefore represents a distinct subtype, indicating clinical and genetic heterogeneity of this disorder. This information should facilitate future molecular linkage analyses and genetic counselling of patients with inner ear malformations.

Adult↗

Spontaneous chronic epidural pneumocephalus resulting from hyperpneumatization of the cranium causing mass effect: case report.

OBJECTIVE AND IMPORTANCE: We report a rare case of spontaneous epidural pneumocephalus resulting from an unusual boney defect caused by hyperpneumatization of the cranium. The pneumocephalus was also unusual for its chronicity and significant mass effect. CLINICAL PRESENTATION: A 49-year-old man presented with a 3-year history of diffuse headaches and sensation of air movement in his sinuses on the right side. An evaluation for suspected sinusitis using computed tomography determined extensive pneumatization of most of the cranium, a large accumulation of epidural air, and a critical degree of brain shift. INTERVENTION: A right frontoparietal-temporal craniotomy with an osteoplastic bone flap was performed. The floor of the middle cranial fossa and the inner table of the bone flap were extensively debrided of air cells. A large pericranial flap was turned down over the floor of the middle cranial fossa and was held in place by a fat graft. After closing the craniotomy, a tympanostomy tube was placed into the right tympanic membrane. CONCLUSION: The incidence of hyperpneumatization of the cranium seems to be very low. Hyperpneumatization when present, however, can cause spontaneous intracranial pneumocephalus. Based on the literature and the success of this case, the optimal management is surgical obliteration of the involved air cells.

Air↗

Disability from vestibular symptoms after acoustic neuroma resection.

OBJECTIVE: This study aimed to evaluate the recovery of balance function after acoustic neuroma resection. STUDY DESIGN: This study was a retrospective case review with patient survey. SETTING: The surgery was conducted at a tertiary referral center. PATIENTS: Patients who underwent surgical resection of acoustic neuroma and had preoperative vestibular function testing were eligible for entering the study. INTERVENTIONS: All patients received surgical resection of acoustic neuroma. Patients treated since 1990 received postoperative vestibular habituation exercises. MAIN OUTCOME MEASURES: These included postoperative symptom and disability scores, dizziness handicap inventory (DHI) total and subset scores, time after surgery at which patients were able to walk independently, whether patients returned to their usual professional responsibility, and time to return to full activities at work. RESULTS: Significant correlation was found between several preoperative symptoms and vestibular testing results and the resulting postoperative disability from dizziness. CONCLUSIONS: This information may be helpful in counseling patients before surgery with respect to the degree of postoperative dysequilibrium and may suggest that the clinician should initiate more aggressive vestibular rehabilitation exercises in patients who may be at greater risk of having persistent dysequilibrium develop after surgery.

Adolescent↗

Performance with the 20 + 2L lateral wall cochlear implant.

OBJECTIVE: The purpose of this study was to investigate the operating characteristics of the Nucleus 20 + 2L lateral wall cochlear implant including speech recognition results. STUDY DESIGN: This was a prospective randomized study involving five different modes of stimulation. SETTING: This study was carried out in an academic, tertiary referral center. PATIENTS: The patients included in this study were adult cochlear implant candidates ages 44-74 years. Length of deafness ranged from 1 to 58 years with a variety of etiologies. INTERVENTIONS: All patients were implanted with a 20 + 2L implant, which includes an intrascalar electrode array and an apically placed extracochlear, titanium encased ball electrode in contact with the endosteum of the apical turn. All patients underwent psychophysical and speech recognition testing in five different modes of electrode configuration. MAIN OUTCOME MEASURE(S): The main outcome measures included thresholds, comfort levels, dynamic ranges, and speech recognition results obtained in five electrode configuration modes. RESULTS: Thresholds were significantly lower (repeated measures ANOVA) in both monopolar conditions when compared to bipolar mode of stimulation. A binomial statistical analysis indicated that in five of nine patients activated in all five modes of stimulation, the monopolar modes of stimulation resulted in improved speech recognition scores. CONCLUSIONS: The results of the study demonstrate the feasibility of the use of an apical lateral cochlear wall electrode in conjunction with an intrascalar electrode array. It further demonstrated the superiority of monopolar stimulation in selected patients.

Adult↗

Hearing improvement after resection of cerebellopontine angle meningioma: case study of the preoperative role of transient evoked otoacoustic emissions.

In a retrospective case study of a patient with a right-sided cerebellopontine angle mass lesion, transient evoked otoacoustic emissions were robustly present despite a severe to profound sensorineural hearing loss and abnormal auditory brainstem response. These results were interpreted as suggestive of a neural site of lesion, and the potential for planned, preserved, or improved hearing by a suboccipital surgical craniotomy was considered. A gross total resection was successful. Three years postoperatively, the patient has normal hearing sensitivity and word recognition ability.

Acoustic Stimulation↗

Human autoantibodies and monoclonal antibody KHRI-3 bind to a phylogenetically conserved inner-ear-supporting cell antigen.

Autoimmunity is thought to be one cause of sensorineural hearing loss (SNHL). Sera from patients with rapidly progressive hearing loss have been shown to contain antibodies to a 68-kD protein in heterologous inner-ear tissue. Using guinea pig inner-ear tissue as the antigenic substrate and either Western blot or immunofluorescence (IF) or both, we tested sera from 74 patients suspected to have autoimmune hearing loss for inner-ear antibodies. Sera from 73 patients were tested by Western blot, and sera from 36 were tested by IF. Thirty-seven of 73 (51%) had antibody to a 68-70-kD protein by Western blot. Sera positive by IF stained supporting cells with a staining pattern like that previously observed with the KHRI-3 monoclonal antibody. There was concordance between Western blot and IF assays. Of 36 patients tested by both assays, 29/31 (94%) that were positive in Western blot were also positive by IF, three were negative by both tests, and two each were positive by one assay but negative by the other. Absorption of patient sera with human inner-ear tissue removed antibody reactivity to the guinea pig supporting cells, indicating that the antigen detected by the autoantibody is also present in the human inner ear. Absorption with an equal volume of white or red blood cells from the tissue donor did not remove the antibody reactivity to inner ear, showing that the absorption by inner-ear tissue is specific. Sera from three patients positive in both assays also stained a 68-70-kD inner-ear protein immunoprecipitated by the KHRI-3 monoclonal antibody, indicating that the monoclonal and human antibodies recognize the same antigen. The results support the hypothesis that patients with autoimmune sensorineural hearing loss produce autoantibodies to an inner-ear supporting cell antigen that is phylogenetically conserved and defined by the murine monoclonal antibody KHRI-3. Since KHRI-3 can induce hearing loss after infusion into the inner ear, it is likely that autoantibodies with the same antigenic target are also pathogenic in humans.

Animals↗

Impaired vocal cord mobility in the setting of acute suppurative thyroiditis.

BACKGROUND: Acute suppurative thyroiditis is an increasingly rare entity, accounting for less than 0.1% of thyroid surgery. This is the first report in the literature of acute thyroiditis causing a unilateral reversible vocal cord paralysis. METHODS: A 41-year-old woman was initially seen with a unilateral vocal cord paralysis resulting from acute suppurative thyroiditis. RESULTS: The vocal cord paralysis resolved following surgical drainage of the thyroid abscess and treatment with intravenous antibiotics. CONCLUSIONS: We present this case to emphasize the need for a complete and thorough head and neck exam including preoperative documentation of vocal cord mobility.

Acute Disease↗

Cochlear implantation of children with minimal open-set speech recognition skills.

OBJECTIVE: The purpose of this study was to evaluate the postoperative performance of 12 children who demonstrated some open-set speech recognition skills before receiving a Nucleus multichannel cochlear implant with a view toward expanding the selection criteria for cochlear implant candidacy to include children who derive minimal benefit from amplification. DESIGN: Pre- and postoperative performance of two groups of children were compared. Group 1 consisted of 12 children who demonstrated some open-set speech recognition skills before receiving a Nucleus multichannel cochlear implant (Borderline group). Group 2 consisted of 12 children who demonstrated no open-set speech recognition skills before implantation with a Nucleus device (Traditional group). In all children, candidacy was determined based on preimplant binaural aided performance. For most subjects, the poorer ear was selected for implantation. Mean pre- and postoperative speech recognition scores of the Borderline subjects were compared to determine the benefit provided by their cochlear implants. Secondly, matched-pair analyses were used to compare the mean speech recognition scores obtained by the Borderline and Traditional subjects. RESULTS: The scores of the Borderline group improved significantly on five of six speech recognition measures when 6 mo postoperative scores obtained with the implant were compared with preoperative test scores obtained with hearing aids. By the 12 mo postoperative interval, the scores of the Borderline group had improved significantly (p < 0.05) on all six measures. In contrast, scores obtained by the Traditional group had improved significantly on three of six measures at both the 6 and 12 mo postoperative intervals. Comparison of postoperative test scores revealed that the Borderline group scored significantly higher than the Traditional group on three of six measures at the 6 mo test interval and on six of six measures at the 12 mo test interval (p < 0.05). CONCLUSIONS: The findings of this study indicate that both groups derive significant benefit from their cochlear implants. Although the mean preoperative audiograms for the implanted ears did not differ significantly for the two groups of subjects, members of the Borderline group exhibited significantly better speech recognition skills than the Traditional group during the first year after implantation. These findings suggest that the increased auditory experience of the Borderline subjects positively influenced their performance with a cochlear implant. The authors advocate that the selection criteria used to determine pediatric cochlear implant candidacy be broadened to include consideration of children who demonstrate minimal open-set speech recognition skills.

Child↗