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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. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

Background: Expedient resuscitation and emergent damage control interventions remain critical tools of modern combat casualty care. Although fortunately rare, the requirement for life and limb salvaging surgical intervention prior to arrival at traditional deployed medical treatment facilities may be required for the care of select casualties. The optimal employment of a surgical resuscitation team (SRT) may afford life and limb salvage in these unique situations., Methods: Fifteen years of after-action reports (AARs) from a highly specialized SRTs were reviewed. Patient demographics, specific details of encounter, team role, advanced emergent life and limb interventions, and outcomes were analyzed., Results: Data from 317 casualties (312 human, five canines) over 15 years were reviewed. Among human casualties, 20 had no signs of life at intercept, with only one (5%) surviving to reach a Military Treatment Facility (MTF). Among the 292 casualties with signs of life at intercept, SRTs were employed in a variety of roles, including MTF augmentation (48.6%), as a transport capability from other aeromedical platforms, critical care transport (CCT) between MTFs (27.7%), or as an in-flight damage control capability directly to point of injury (POI) (18.2%). In the context of these roles, the SRT performed in-flight life and limb preserving surgery for nine patients. Procedures performed included resuscitative thoracotomy (7/9; 77.8%), damage control laparotomy (1/9; 11.1%) and extremity fasciotomy for acute lower extremity compartment syndrome (1/11; 11%). Survival following in-flight resuscitative thoracotomy was 33% (1/3) when signs of life (SOL) were absent at intercept and 75% (3/4) among patients who lost SOL during transport., Conclusion: In-flight surgery by a specifically trained and experienced SRT can salvage life and limb for casualties of major combat injury. Additional research is required to determine optimal SRT utilization in present and future conflicts., (2020.)

Background: Early resuscitation and damage control surgery (DCS) are critical components of modern combat casualty care. Early and effective DCS capabilities can be delivered in a variety of settings through the use of a mobile surgical resuscitation team (SRT)., Methods: Twelve years of after-action reports from SRTs were reviewed. Demographics, interventions, and outcomes were analyzed., Results: Data from 190 casualties (185 human, five canine) were reviewed. Among human casualties, 12 had no signs of life at intercept and did not survive. Of the remaining 173 human casualties, 96.0% were male and 90.8% sustained penetrating injuries. Interventions by the SRT included intravascular access (50.9%) and advanced airway establishment (29.5%). Resuscitation included whole blood (3.5%), packed red blood cells (20.8%), and thawed plasma (11.0%). Surgery was provided for 63 of the 173 human casualties (36.4%), including damage control laparotomy (23.8%) and arterial injury shunting or repair (19.0%). SRTs were effectively used to augment an existing medical treatment facility (70.5%), to facilitate casualty transport (13.3%), as an independent surgical entity at a forward ground structure (9.2%), and in mobile response directly to the point of injury (6.9%). Overall survival was 97.1%., Conclusion: An SRT provides a unique DCS capability that can be successfully used in a variety of flexible roles., (2017.)

Objectives/hypothesis: To describe the clinical experience and characterize the outcomes of cochlear implantation (CI) in children with isolated enlarged vestibular aqueduct (IEVA) as compared to children with enlarged vestibular aqueduct (EVA) associated with other bony labyrinth abnormalities., Study Design: Single, tertiary care, institutional retrospective review over 2 decades., Methods: The clinical course and outcomes of 55 children with EVA undergoing CI between 1991 and 2013 were reviewed. Test measures included open and closed set speech perception tests, and various speech and language measures., Results: In 18 children (32.7%), IEVA was the only defect present. In 33 children (60%), EVA occurred concomitantly with incomplete partition type 2 (IP 2) bilaterally, and three children with incomplete partition type 1 bilaterally. Ninety-two percent (51 of 55) occurred bilaterally and had matching bony defects. Mean age of CI was 73.4 months. A statistically significant defect-related and linguistic-status pattern was noted, impacting the timing of implantation: IEVA = 112.8 months, IP 2 = 58.4 months (P < .001), prelingual deafness = 53.8 months, postlingual deafness = 110.8 months (P < .001). Controlling for implant age and hearing loss severity, IEVA children demonstrated superior performance on speech perception tests (8.2 to 20.3 point differences), though statistical significance was inconsistent (P = .01-.40. Performance was also superior in speech and language tests, though statistical significance was never reached (2.9-13.9 point differences; P = .14-.69)., Conclusions: Children with hearing loss secondary to EVA respond meaningfully to cochlear implantation. However, the severity of temporal bone anomalies in these children has clinical relevance., Level of Evidence: 4., (© 2015 The American Laryngological, Rhinological and Otological Society, Inc.)

Objective: To evaluate hearing outcomes and effects of stimulation rate on performance with the Nucleus Freedom cochlear implant (Cochlear Americas, Denver, CO)., Study Design and Setting: Randomized, controlled, prospective, single-blind clinical study using single-subject repeated measures (A-B-A-B) design at 14 academic centers in the United States and Canada and comparison with outcomes of a prior device by the same manufacturer., Patients: Seventy-one severely/profoundly hearing impaired adults., Results: Seventy-one adult recipients were randomly programmed in two different sets of rate: ACE or higher rate ACE RE. Mean scores for Consonant Nucleus Consonant words is 57%, Hearing in Noise Test (HINT) sentences in quiet 78%, and HINT sentences in noise 64%. Sixty-seven percent of subjects preferred slower rates of stimulation, and performance did not improve with higher rates of stimulation using this device., Conclusions: Subjects performed well, and there was no advantage to higher stimulation rates with this device., Significance: Higher stimulation rates do not necessarily result in improved performance.

Background and Purpose: This paper describes the CT findings that characterize the middle and inner ear anomalies in coloboma, heart defects, choanal atresia, mental retardation, genitourinary, and ear anomalies (CHARGE) syndrome. With this information, neuroradiologists will be better prepared to provide clinically relevant information to their referring physicians regarding this rare syndrome., Materials and Methods: CT studies from 13 patients were reviewed by 2 neuroradiologists with Certificate of Additional Qualification. Each ear was counted separately for a total of 26 ears. Middle and inner ear anomalies associated with CHARGE syndrome were categorized. Investigational review board approval was obtained., Results: Twenty of 26 (77%) ears demonstrated cochlear aperture atresia. Four of these ears were evaluated with MR imaging and were found to lack a cochlear nerve. Twenty-one of 26 (81%) cochlea had some form of dysplasia. Six of 26 (23%) round windows were aplastic. Three of 26 (12%) round windows were hypoplastic. Twenty-one of 26 (81%) oval windows were atretic or aplastic. Fifteen of 26 (58%) vestibules were hypoplastic or dysplastic. There were 5 of 26 (19%) enlarged vestibular aqueducts. Twelve of 26 (46%) vestibular aqueducts had an anomalous course. All cases demonstrated absent semicircular canals. Twenty-three of 26 (88%) facial nerve canals had an anomalous course. Four of 26 (15%) tympanic segments were prolapsed. Three of 26 (12%) temporal bones had an anomalous emissary vein referred to as a petrosquamosal sinus. Twenty-one of 26 (81%) middle ear cavities were small. Twenty-three of 26 (93%) ossicles were dysplastic with ankylosis. Three of 26 (12%) internal auditory canals were small., Conclusion: The CT findings that correlate to the anomalies of CHARGE syndrome affect conductive as well as sensorineural hearing. Stenosis of the aperture for the cochlear nerve aperture on CT is suggestive of hypoplasia or absence of the cochlear nerve, which has been demonstrated in some cases by MR. Absence of the cochlear nerve would be a contraindication to cochlear implantation.

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.

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.

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.

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.

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.

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.

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.

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.

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.

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.

The development of a sensitive and specific diagnostic test for endolymphatic hydrops has eluded investigators for over 30 years. The absence of such a test has hampered basic and clinical research into Meniere's disease and related entities. Presently used tests are limited in their applicability because of their low sensitivity and specificity and a poor understanding of the underlying physiologic principles. Despite this, it is generally agreed that some of these studies have merit in selected situations. This article reviews the present state of diagnostic testing for endolymphatic hydrops. The use of nonspecific studies, such as basic audiometry and tests for recruitment, and "specific" studies, such as electrocochleography and dehydration testing, are critically reviewed.

Objective: To summarize the current applications of auditory evoked potential in children with cochlear implants and candidates for implantation., Patients and Methods: Perioperative transtympanic EABR is used routinely for ear selection and to establish the electrical stimulability of the ear intended to be implanted. The perioperative transtympanic EABR is supplemented with EABR obtained immediately following the insertion of the electrode array and the seating of the implant's receiver. Postoperatively, EABR and averaged electrode voltages are used effectively to properly adjust the implant stimulus parameters and to determine whether the implant is functioning adequately. Postoperatively, cognitive evoked potentials to speech and tonal stimuli may also be obtained., Results: EABR results have contributed to effective implant placement and function. There were several significant correlations between speech recognition and cognitive evoked potential., Conclusion: These measures help assure proper implant function and effective stimulus delivery.

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.

A within-subjects comparison of monopolar versus bipolar stimulation was performed using a modified version of the Nucleus mini-22 cochlear implant, the Nucleus "20 + 2" implant. Six subjects underwent implantation with this device, which is identical to the Nucleus 22 cochlear implant with the addition of two extracochlear indifferent electrodes. These electrodes provide two monopolar modes of stimulation in addition to the standard bipolar modes used with the Nucleus 22 device. One of the indifferent electrodes is a ball placed under the temporalis muscle (MP1), whereas the second electrode is mounted on the lateral aspect of the receiver-stimulator (MP2). After a pre-experimental phase, subjects used each of three stimulation modes (BP + 1, MP1, and MP2) for a total of 4 weeks each. Variables tested with each mode included electric thresholds, comfort levels, dynamic ranges, and speech recognition. Both the MP1 and MP2 modes of stimulation required significantly less current than the bipolar mode (BP + 1) to reach threshold and comfort level. Analysis of dynamic range data also indicated a significant stimulation mode effect. However, stimulation in the various modes did not significantly affect speech recognition scores, although two subjects demonstrated significantly improved speech recognition scores when programmed in a monopolar mode.

Although the concept of using exercises as part of the treatment for patients with persistent vertigo was introduced several decades ago, organized vestibular rehabilitation therapy programs have only recently been introduced. These programs typically involve a three-pronged approach, customized to the needs of the individual patient: (1) habituation exercises designed to facilitate central nervous system compensation by extinguishing pathologic responses to head motion, (2) postural control exercises, and (3) general conditioning activities. This article provides an update regarding the status of vestibular rehabilitation, reviewing the concept of vestibular compensation and patient selection criteria for current rehabilitative techniques.

Labyrinthitis ossificans may complicate the insertion of a multichannel cochlear implant in patients deafened after meningitis. Two children who initially underwent partial insertion of a 22-channel cochlear implant because of severe cochlear ossification required revision surgery after several months of unsuccessful device use. At the time of revision, resection of the car canal, tympanic membrane, malleus, and incus provided access to the lateral wall of the cochlea, permitting extensive drilling of the basal turn and a circumodiolar placement of the electrode. Functional integrity of the electronic components of the original device was documented intraoperatively, avoiding the expense of a new receiver-stimulator. Complete insertion of the active electrodes was accomplished in both cases, and electrophysiologic responsiveness to the implant was documented using intraoperative electrically evoked auditory brainstem response recordings. Postoperative performance has been similar to that of cochlear implant patients with nonossified ears. Experience with these two cases suggests that efforts to optimize electrode insertion at the original surgical procedure are appropriate and may help to avoid the disappointment of an unsuccessful cochlear implant.

Objective: To report operative findings, postoperative course, and postimplantation performance in patients with cochlear malformations who underwent cochlear implantation., Design: Case study and intervention study (before-after trial). Minimum follow-up of 12 months; average follow-up of 24 months., Setting: Academic tertiary referral center., Patients: Six patients, including five children who underwent implantation at ages 3.5 to 13 years and one adult who underwent implantation at age 27 years. malformations included common cavity deformity (n = 1), cochlear hypoplasia (n = 2), and incomplete partition (n = 3). All patients with cochlear malformations who underwent implantation at the University of Michigan, Ann Arbor, are included, selected from a group of 196 patients so treated since 1986., Intervention: Implantation with a standard multichannel cochlear implant., Main Outcome Measures: Operative findings described include round window and facial nerve anatomy and cerebrospinal fluid leak. Postoperative roentgenographic findings, electrode activation, and reason for non-use of electrodes were investigated. Standard tests of speech perception were used to compare preoperative and postoperative performance for each subject., Results: Operative findings included round window abnormalities (three patients), anomalous facial nerve (one patient), and cerebrospinal fluid leak (three patients). No surgical complications occurred. A minimum of 10 electrodes were activated for all patients. Electrode thresholds and discomfort levels were variable for several months after implantation. All patients demonstrated improved performance after implantation. Four subjects demonstrated open-set speech perception. Two other subjects, whose poor language skills precluded administration of standard tests, showed increased awareness of environmental sounds and increased vocalization after implantation., Conclusions: Cochlear implantation can be a successful method of rehabilitation in patients with congenital deafness who have cochlear malformations.

Complete, irreversible failure of the implanted receiver-stimulator of the Cochlear Corporation multichannel implant are relatively rare. However, as the implanted patient population grows, malfunctions may be expected. From the over 200 patients implanted at the University of Michigan Medical Center, 6 patients with a complete and irreversible cochlear implant receiver-stimulator failure have been identified and treated. This represents a 3% failure rate. The amount of time between initial implantation and device failure ranged from 6 months to 3.5 years. Determination of device failure was made using psychophysical, electrophysiologic, and averaged electrode voltage measurements. The measurement of the average electrode voltages proved to be useful in determining the condition of the implant. Physiologic changes causing reduced electrical excitability were ruled out using psychophysical or electrophysiologic promontory testing. All patients were successfully explanted and reimplanted.

Although the use of vestibular exercises for patients with persistent vertigo and dysequilibrium has received some attention for many years, organized vestibular rehabilitation therapy programs have only recently been introduced. The benefits of such programs are becoming widely accepted. They typically involve a three-pronged approach: habituation exercises designed to facilitate central nervous system compensation by extinguishing pathologic responses to head motion, postural control exercises, and general conditioning activities. This article will describe the programmatic approach to vestibular rehabilitation, emphasizing selection criteria and reviewing results that may be anticipated when this treatment modality is used in a variety of patient populations.

Many qualitative studies examining the chondrogenic potential of perichondrium have suggested that it may be useful for airway reconstruction. This study was performed to quantitatively evaluate cartilage formation by free and vascularized rabbit perichondrium placed in sterile and contaminated (airway) environments for 8 weeks. The mean cartilage thickness for free sterile perichondrium was 0.09 mm, for free contaminated perichondrium was 0.13 mm, for vascularized sterile perichondrium was 0.16 mm, and for vascularized contaminated perichondrium was 0.44 mm. Contaminated vascularized grafts produced significantly more cartilage when compared to both sterile vascularized grafts (p = .001) and contaminated free grafts (p < .001). No differences in cartilage formation were seen between sterile and contaminated free grafts (p = .198), or between free and vascularized grafts that were sterile (p = .170). These findings support further investigation of airway reconstruction with vascularized perichondrial grafts.

Objective: To study the feasibility and clinical applicability of preoperative, transtympanic electrically evoked auditory brain-stem response (EABR) in a pediatric patient population with cochlear implants., Design: A descriptive study using repeated measures analyses of variance to determine if EABR measures were affected by response configuration or cochlear status., Setting: The operating room before cochlear implant surgery., Patients: A population-based sample of 43 patients aged 2.5 to 14.5 years who were candidates for cochlear implantation., Intervention: Stimuli consisting of brief balanced biphasic current pulses were provided by a transtympanically placed promontory needle electrode; EABR was recorded with subdermal needle electrodes on the forehead and contralateral mastoid., Main Outcome Measure: Presence or absence of postoperative electrical excitability with a cochlear implant., Results: Electrically evoked auditory brain-stem responses were available from 41 of the 43 patients tested. Mean EABR threshold was 406.5 microA (SD = 118.1) for 31 patients with patent cochleas and 472 microA (SD = 91) for 10 patients with cochlear ossification. Mean wave V latency at threshold was 4.69 milliseconds (SD = 0.57)., Conclusion: Preoperative EABR is an integral component of the preoperative selection process for pediatric patients with cochlear implants.