Potential Contributions of Skeletal Muscle Contractile Dysfunction to Altered Biomechanics in Obesity

Publication date: Available online 13 May 2017
Source:Gait & Posture
Author(s): Lance M. Bollinger
BackgroundObesity alters whole body kinematics and joint kinetics during activities of daily living which are thought to contribute to increased risk of musculoskeletal injury, development of lower extremity joint osteoarthritis (OA), and physical disability. To date, it has widely been accepted that excess adipose tissue mass is the major driver of biomechanical alterations in obesity. However, it is well established that obesity is a systemic disease affecting numerous, if not all, organ systems of the body. Indeed, obesity elicits numerous adaptations within skeletal muscle, including alterations in muscle structure (ex. myofiber size, architecture, lipid accumulation, and fiber type), recruitment patterns, and contractile function (ex. force production, power production, and fatigue) which may influence kinematics and joint kinetics. This review discusses the specific adaptations of skeletal muscle to obesity, potential mechanisms underlying these adaptations, and how these adaptations may affect biomechanics.



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Kinematic Strategies for Obstacle-Crossing in Patients with Isolated Posterior Cruciate Ligament Deficiency

Publication date: Available online 13 May 2017
Source:Gait & Posture
Author(s): Mei-Ying Kuo, Shih-Wun Hong, Tsai-Hsueh Leu, Chien-Chung Kuo, Tung-Wu Lu, Jyh-Horng Wang
The posterior cruciate ligament (PCL) plays an important role in the structural stability and sensory feedback at the knee. Altered structural and proprioceptive function at the PCL-deficient knee may affect the joint motions and the end-point control during functional activities. The current study identified the effects of unilateral PCL deficiency (PCLD) on the end-point control and joint kinematics of the lower limbs during obstacle-crossing. Eighteen patients with unilateral PCLD and eighteen healthy controls were each asked to walk and cross obstacles of heights of 10%, 20% and 30% of their leg lengths, with the affected and the unaffected limb leading, while their kinematic data were measured. Patients with PCLD were found to cross obstacles with significantly increased toe-clearance (p <0.01), increased trailing toe-obstacle distance (p <0.05) and reduced crossing speed (p <0.01) when compared to the controls. Similar end-point control was observed in the PCLD group whether leading with the affected or unaffected limb, which appears to be as a result of bilateral kinematic accommodation to reduce the risk of tripping. To achieve similar toe-clearances, crossing strategies with the unaffected limb leading involved angular changes at more joints than those with the affected limb leading. The PCLD group appeared to adopt a conservative strategy to reduce the risk of tripping over the obstacle during obstacle-crossing, using different joint kinematic changes depending on whether the affected or unaffected limb was leading. It is suggested that monitoring of the kinematic strategies adopted by patients with PCLD during obstacle-crossing may be needed in future rehabilitation programs with the aim of reducing tripping risks during obstacle-crossing.



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Potential Contributions of Skeletal Muscle Contractile Dysfunction to Altered Biomechanics in Obesity

Publication date: Available online 13 May 2017
Source:Gait & Posture
Author(s): Lance M. Bollinger
BackgroundObesity alters whole body kinematics and joint kinetics during activities of daily living which are thought to contribute to increased risk of musculoskeletal injury, development of lower extremity joint osteoarthritis (OA), and physical disability. To date, it has widely been accepted that excess adipose tissue mass is the major driver of biomechanical alterations in obesity. However, it is well established that obesity is a systemic disease affecting numerous, if not all, organ systems of the body. Indeed, obesity elicits numerous adaptations within skeletal muscle, including alterations in muscle structure (ex. myofiber size, architecture, lipid accumulation, and fiber type), recruitment patterns, and contractile function (ex. force production, power production, and fatigue) which may influence kinematics and joint kinetics. This review discusses the specific adaptations of skeletal muscle to obesity, potential mechanisms underlying these adaptations, and how these adaptations may affect biomechanics.



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Kinematic Strategies for Obstacle-Crossing in Patients with Isolated Posterior Cruciate Ligament Deficiency

Publication date: Available online 13 May 2017
Source:Gait & Posture
Author(s): Mei-Ying Kuo, Shih-Wun Hong, Tsai-Hsueh Leu, Chien-Chung Kuo, Tung-Wu Lu, Jyh-Horng Wang
The posterior cruciate ligament (PCL) plays an important role in the structural stability and sensory feedback at the knee. Altered structural and proprioceptive function at the PCL-deficient knee may affect the joint motions and the end-point control during functional activities. The current study identified the effects of unilateral PCL deficiency (PCLD) on the end-point control and joint kinematics of the lower limbs during obstacle-crossing. Eighteen patients with unilateral PCLD and eighteen healthy controls were each asked to walk and cross obstacles of heights of 10%, 20% and 30% of their leg lengths, with the affected and the unaffected limb leading, while their kinematic data were measured. Patients with PCLD were found to cross obstacles with significantly increased toe-clearance (p <0.01), increased trailing toe-obstacle distance (p <0.05) and reduced crossing speed (p <0.01) when compared to the controls. Similar end-point control was observed in the PCLD group whether leading with the affected or unaffected limb, which appears to be as a result of bilateral kinematic accommodation to reduce the risk of tripping. To achieve similar toe-clearances, crossing strategies with the unaffected limb leading involved angular changes at more joints than those with the affected limb leading. The PCLD group appeared to adopt a conservative strategy to reduce the risk of tripping over the obstacle during obstacle-crossing, using different joint kinematic changes depending on whether the affected or unaffected limb was leading. It is suggested that monitoring of the kinematic strategies adopted by patients with PCLD during obstacle-crossing may be needed in future rehabilitation programs with the aim of reducing tripping risks during obstacle-crossing.



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Potential Contributions of Skeletal Muscle Contractile Dysfunction to Altered Biomechanics in Obesity

Publication date: Available online 13 May 2017
Source:Gait & Posture
Author(s): Lance M. Bollinger
BackgroundObesity alters whole body kinematics and joint kinetics during activities of daily living which are thought to contribute to increased risk of musculoskeletal injury, development of lower extremity joint osteoarthritis (OA), and physical disability. To date, it has widely been accepted that excess adipose tissue mass is the major driver of biomechanical alterations in obesity. However, it is well established that obesity is a systemic disease affecting numerous, if not all, organ systems of the body. Indeed, obesity elicits numerous adaptations within skeletal muscle, including alterations in muscle structure (ex. myofiber size, architecture, lipid accumulation, and fiber type), recruitment patterns, and contractile function (ex. force production, power production, and fatigue) which may influence kinematics and joint kinetics. This review discusses the specific adaptations of skeletal muscle to obesity, potential mechanisms underlying these adaptations, and how these adaptations may affect biomechanics.



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Kinematic Strategies for Obstacle-Crossing in Patients with Isolated Posterior Cruciate Ligament Deficiency

Publication date: Available online 13 May 2017
Source:Gait & Posture
Author(s): Mei-Ying Kuo, Shih-Wun Hong, Tsai-Hsueh Leu, Chien-Chung Kuo, Tung-Wu Lu, Jyh-Horng Wang
The posterior cruciate ligament (PCL) plays an important role in the structural stability and sensory feedback at the knee. Altered structural and proprioceptive function at the PCL-deficient knee may affect the joint motions and the end-point control during functional activities. The current study identified the effects of unilateral PCL deficiency (PCLD) on the end-point control and joint kinematics of the lower limbs during obstacle-crossing. Eighteen patients with unilateral PCLD and eighteen healthy controls were each asked to walk and cross obstacles of heights of 10%, 20% and 30% of their leg lengths, with the affected and the unaffected limb leading, while their kinematic data were measured. Patients with PCLD were found to cross obstacles with significantly increased toe-clearance (p <0.01), increased trailing toe-obstacle distance (p <0.05) and reduced crossing speed (p <0.01) when compared to the controls. Similar end-point control was observed in the PCLD group whether leading with the affected or unaffected limb, which appears to be as a result of bilateral kinematic accommodation to reduce the risk of tripping. To achieve similar toe-clearances, crossing strategies with the unaffected limb leading involved angular changes at more joints than those with the affected limb leading. The PCLD group appeared to adopt a conservative strategy to reduce the risk of tripping over the obstacle during obstacle-crossing, using different joint kinematic changes depending on whether the affected or unaffected limb was leading. It is suggested that monitoring of the kinematic strategies adopted by patients with PCLD during obstacle-crossing may be needed in future rehabilitation programs with the aim of reducing tripping risks during obstacle-crossing.



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Phonak and VA Collaborate to Develop Hearing Aid Distance Fitting for Veterans

Phonak (http://ift.tt/1TpqSBL) is starting a pilot program with the U.S. Department of Veterans Affairs (VA) to explore opportunities to use telehealth in real-life audiology settings and simultaneously improve veterans' access to remote fitting. Phonak's smartphone app VA HearAssist allows hearing aid wearers to connect with their audiologists through the internet. The app is connected to the hearing aids via Bluetooth, and links the hearing aids to an audiologist's fitting software through this connection. Audiologists can then directly access the hearing aids and adjust their settings in real-time without having to use an additional intermediary device. They can also give advice to their patients through video conference calls.

VA HearAssist will explore base line clinical trials at three VA facilities in Cleveland, Chicago, and Miami from this summer to the fall. If successful, the company will evaluate the app for private use. Phonak has worked with the VA on various projects regarding distance fitting, with the most recent one being the Technical Feasibility Study for Distance Support in 2012. This collaboration on VA HearAssist is made possible through Iron Bow Technologies, the company that provides IT services to the VA. 

​

Published: 5/12/2017 10:48:00 AM

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Phonak and VA Collaborate to Develop Hearing Aid Distance Fitting for Veterans

Phonak (http://ift.tt/1TpqSBL) is starting a pilot program with the U.S. Department of Veterans Affairs (VA) to explore opportunities to use telehealth in real-life audiology settings and simultaneously improve veterans' access to remote fitting. Phonak's smartphone app VA HearAssist allows hearing aid wearers to connect with their audiologists through the internet. The app is connected to the hearing aids via Bluetooth, and links the hearing aids to an audiologist's fitting software through this connection. Audiologists can then directly access the hearing aids and adjust their settings in real-time without having to use an additional intermediary device. They can also give advice to their patients through video conference calls.

VA HearAssist will explore base line clinical trials at three VA facilities in Cleveland, Chicago, and Miami from this summer to the fall. If successful, the company will evaluate the app for private use. Phonak has worked with the VA on various projects regarding distance fitting, with the most recent one being the Technical Feasibility Study for Distance Support in 2012. This collaboration on VA HearAssist is made possible through Iron Bow Technologies, the company that provides IT services to the VA. 

​

Published: 5/12/2017 10:48:00 AM

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Phonak and VA Collaborate to Develop Hearing Aid Distance Fitting for Veterans

Phonak (http://ift.tt/1TpqSBL) is starting a pilot program with the U.S. Department of Veterans Affairs (VA) to explore opportunities to use telehealth in real-life audiology settings and simultaneously improve veterans' access to remote fitting. Phonak's smartphone app VA HearAssist allows hearing aid wearers to connect with their audiologists through the internet. The app is connected to the hearing aids via Bluetooth, and links the hearing aids to an audiologist's fitting software through this connection. Audiologists can then directly access the hearing aids and adjust their settings in real-time without having to use an additional intermediary device. They can also give advice to their patients through video conference calls.

VA HearAssist will explore base line clinical trials at three VA facilities in Cleveland, Chicago, and Miami from this summer to the fall. If successful, the company will evaluate the app for private use. Phonak has worked with the VA on various projects regarding distance fitting, with the most recent one being the Technical Feasibility Study for Distance Support in 2012. This collaboration on VA HearAssist is made possible through Iron Bow Technologies, the company that provides IT services to the VA. 

​

Published: 5/12/2017 10:48:00 AM

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Defining the Hook Region Anatomy of the Guinea Pig Cochlea for Modeling of Inner Ear Surgery.

Hypothesis: The aim of this study was to describe the hook region anatomy of the guinea pig cochlea to identify the optimal surgical approach for cochlear implantation and to determine what anatomical structures are at risk. Background: Animal studies investigating hearing loss after cochlear implantation surgery are currently constrained by the lack of a reproducible implantation model. Methods: Guinea pig cochleae were imaged using thin-sheet laser imaging microscopy. Images were stitched, reconstructed, and segmented for analysis. Insertion vectors were determined by tracing their paths to the outer wall and converting to Cartesian coordinates. Spherical surface and multiplane views were generated to analyze outer wall and radial forces of the insertion vector. Results: Thin-sheet laser imaging microscopy enabled quantitative, whole specimen analysis of the soft and bony tissue relationships of the complex cochlear hook region in any desired plane without loss of image quality. Round window or cochleostomy approaches in the anteroinferior plane avoided direct damage to cochlear structures. Cochleostomy approach had large interindividual variability of angular depth and outer wall forces but predictable radial force. Conclusion: The guinea pig hook region and lower basal turn have similar structural relationships to humans. Careful cochleostomy placement is essentially for minimizing cochlear trauma and for ensuring a straight insertion vector that successfully advances around the outer wall. Experiments with guinea pigs that control for the surgical approach are likely to provide useful insights into the aetiology and the development of therapies directed at postimplantation hearing loss. Copyright (C) 2017 by Otology & Neurotology, Inc. Image copyright (C) 2010 Wolters Kluwer Health/Anatomical Chart Company

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The Effect of Systemic Steroid on Hearing Preservation After Cochlear Implantation via Round Window Approach: A Guinea Pig Model.

Hypothesis: When administered perioperatively, systemic dexamethasone will reduce the hearing loss associated with cochlear implantation (CI) performed via the round window approach. Background: The benefits of electroacoustic stimulation have led to interest in pharmacological interventions to preserve hearing after CI. Methods: Thirty guinea pigs were randomly divided into three experimental groups: a control group; a 3-day infusion group; and a 7-day infusion group. Dexamethasone was delivered via a mini-osmotic pump for either 3 or 7 days after CI via the round window. Pure tone-evoked auditory brainstem response (ABR) thresholds were monitored for a period of 12 weeks after CI. The cochleae were then collected for histology. Results: At 4 and 12 weeks after CI, ABR threshold shifts were significantly reduced in both 7-day and 3-day infusion groups compared with the control group. Furthermore, the 7-day infusion group has significantly reduced ABR threshold shifts compared with the 3-day infusion group. The total tissue response, including fibrosis and ossification, was significantly reduced in the 7-day infusion group compared with the control group. On multiple regression the extent of fibrosis predicted hearing loss across most frequencies, while hair cell counts predicted ABR thresholds at 32 kHz. Conclusion: Hearing protection after systemic administration of steroids is more effective when continued for at least a week after CI. Similarly, this treatment approach was more effective in reducing the fibrosis that encapsulates the CI electrode. Reduced fibrosis seemed to be the most likely explanation for the hearing protection. Copyright (C) 2017 by Otology & Neurotology, Inc. Image copyright (C) 2010 Wolters Kluwer Health/Anatomical Chart Company

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Use of Positive Airway Pressure Following Middle Ear Surgery: A Practice Survey of Otologists.

Background: Positive airway pressure (PAP) devices are used as treatment for obstructive sleep apnea (OSA). PAP may increase middle ear pressure which is of interest to otologic surgeons. There is a lack of data to guide management of PAP therapy after middle ear surgery. Objective: To elucidate how otologic surgeons manage PAP in their patients after ear surgery. Study Design: A survey e-mailed to practicing members of the American Neurotology Society. Results: Among 60 respondents, the most common recommendations to patients were to avoid PAP use for 1 week (40%), return to normal use immediately (23.3%), avoid PAP for 1 month (13.3%), or avoid PAP for 1 day (13.3%) after surgery. Twenty percent of providers reported that they change their advice on the basis of the PAP pressure settings (i.e., shorter hiatus for higher settings). Among respondents, 47% think that they have patients with middle ear issues because of PAP and 13% attributed surgical failures to PAP use. One-third of providers routinely pack the Eustachian tube during surgery. Providers who attributed a negative surgical outcome to PAP use were more likely to routinely pack/plug the Eustachian tube during otologic surgery (p = 0.001). Conclusion: Treatment recommendations regarding postoperative PAP treatment for obstructive sleep apnea vary greatly among practicing otologists. Providers who think that an adverse outcome was attributed to PAP use were more likely to prophylactically plug the Eustachian tube during surgery. Future research will provide additional information which will allow a better understanding of the effect of PAP on the middle ear, especially after otologic surgery. Copyright (C) 2017 by Otology & Neurotology, Inc. Image copyright (C) 2010 Wolters Kluwer Health/Anatomical Chart Company

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Endoscopic Infracochlear Approach for Drainage of Petrous Apex Cholesterol Granulomas: A Case Series.

Objective: To describe the feasibility and technical nuances of a transcanal endoscopic infracochlear approach for drainage of petrous apex cholesterol granulomas. Study Design: Retrospective case review. Setting: Tertiary care university hospital. Patients: A 32-year-old man with bilateral petrous apex cholesterol granulomas and a 54-year-old man with a left-sided petrous apex granuloma each with symptoms necessitating surgical intervention. Interventions: Transcanal endoscopic infracochlear approach for drainage of the cholesterol granulomas. Main Outcome Measures: Operation efficacy, corridor size, and perioperative morbidity. Results: All three cholesterol granulomas were successful drained without violating the cochlea, jugular bulb, or carotid artery. The dimensions of the infracochlear surgical corridor measured 5 mm x 6 mm, 3.5 mm x 3.5 mm, and 6 mm x 4 mm, respectively. All corridors facilitated visualization within the cyst and allowed lyses of adhesions for additional cyst content eradication. All patients had resolution of their acute symptoms. Two of the three subjects had serviceable hearing before and after their procedures. One patient required revision surgery 2-months after their initial procedure secondary to recurrent symptoms from acute hemorrhage within the cyst cavity. The infracochlear tract in this patient was noted to be patent. Conclusions: A transcanal endoscopic infracochlear approach is feasible for the management of cholesterol granuloma. The surgical access was wide enough to introduce the endoscope into the petrous apex cavity in each case. Further studies are needed to compare the efficacy and perioperative morbidity versus the traditional postauricular transtemporal approaches. Copyright (C) 2017 by Otology & Neurotology, Inc. Image copyright (C) 2010 Wolters Kluwer Health/Anatomical Chart Company

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Transcanal Endoscopic Ear Surgery for Excision of a Facial Nerve Venous Malformation With Interposition Nerve Grafting: A Case Report.

Objective: To illustrate a novel approach for the surgical management of a venous malformation of the facial nerve, including interposition nerve grafting, via an exclusively transcanal endoscopic ear surgery (TEES) approach. Patient: Thirty nine-year-old woman with a preoperative House-Brackmann (HB) grade IV facial paresis secondary to a facial nerve tumor. Intervention(s): Surgical excision and interposition nerve graft via a transcanal endoscopic approach. Main Outcome Measure(s): Completeness of resection, approach morbidities, and facial nerve outcome. Results: The TEES approach provided wide exposure of the facial nerve from the geniculate ganglion through the mastoid segment. This visualization facilitated gross total tumor resection, incus interposition ossicular reconstruction, and placement of an interposition nerve graft. The nerve graft was positioned in the fallopian canal and was secured at both ends with surgicel. The patient had no postoperative complications. At 11-month follow-up her facial function had returned to HB grade IV. Conclusions: This is the first report of resecting a venous malformation of the facial nerve with concomitant interposition nerve graft reconstruction via an exclusively endoscopic approach. This report adds to the growing body of evidence that TEES can manage diverse middle ear and lateral skull base pathology. Additional studies are needed to fully elucidate the risk-benefit profile of this technique. Copyright (C) 2017 by Otology & Neurotology, Inc. Image copyright (C) 2010 Wolters Kluwer Health/Anatomical Chart Company

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Real-time Intracochlear Electrocochleography Obtained Directly Through a Cochlear Implant.

Hypothesis: Utilizing the cochlear implant to record electrophysiologic responses during device placement is a feasible and efficacious technique for monitoring near real-time cochlear physiology during and following electrode insertion. Background: Minimizing intracochlear trauma during cochlear implantation has emerged as a highly researched area to help improve patient performance. Currently, conventional cochlear implant technology allows for the recording of electrically evoked compound action potentials (eCAPs). Acoustically evoked potentials may be more sensitive in detecting physiologic changes occurring as a result of electrode insertion. Electrocochleography obtained from within the cochlea allows hair cell and neural response monitoring along the cochlear spiral at locations where changes most likely would occur. Methods: Intracochlear electrocochleography (ECochG) was recorded from the cochlear implant during surgery in 14 subjects. A long acquisition time (54.5 ms), capable of measuring potentials from the low frequency-serving apical region of the cochlea (125 and 500 Hz) was employed. Two distinct intracochlear processing methods were used and compared in obtaining electrophysiologic data. Results: Measureable intracochlear ECochG responses were obtained from all 14 participants. The 1st harmonic distortions (cochlear microphonic and auditory nerve neurophonic) generally increased steadily with electrode insertion. Electrode and frequency scan following insertion revealed that response amplitude varied based on location of recording electrode and frequency of stimulation. Exquisite sensitivity to manipulation during round window muscle packing was demonstrated. Conclusion: Intracochlear ECochG recorded from the electrode array of the cochlear implant is a highly feasible technique that sheds light on cochlear micromechanics during cochlear implant electrode placement. Copyright (C) 2017 by Otology & Neurotology, Inc. Image copyright (C) 2010 Wolters Kluwer Health/Anatomical Chart Company

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Age-related Increase in Blood Levels of Otolin-1 in Humans.

Objective: To test the hypothesis that age-related demineralization of otoconia will result in an age-related increase in blood levels of otoconia matrix protein, otolin-1. Study Design: Cross-sectional observational clinical trial. Setting: Clinical research center. Patients: Seventy nine men and women ranging in age from 22 to 95 years old. Interventions: Diagnostic. Main Outcome Measures: Blood levels of otolin-1 in relation to age. Results: Levels of otolin-1 of subjects divided into four age groups (1: 20-30 [n = 20], 2: 50-65 [n = 20], 3: 66-80 [n = 20], 4: 81-95 [n = 19] years old) demonstrated an increasing trend with age. The difference between otolin levels of groups 2 and 3, as well as, (p = 0.04) and 2 and 4 (p = 0.031) were statistically significant, but there was no significant difference between the two oldest groups. Conclusions: Otolin-1 blood levels are significantly higher in patients older than 65 years of age. This is consistent with previous scanning electron microscopy findings of age-related otoconia degeneration and increased prevalence of benign paroxysmal positional vertigo (BPPV) with age. Normative data provided here can serve as important reference values against which levels from BPPV patients can be compared with further evaluate otolin-1 as a circulatory biomarker for otoconia degeneration. Copyright (C) 2017 by Otology & Neurotology, Inc. Image copyright (C) 2010 Wolters Kluwer Health/Anatomical Chart Company

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Epidemiology of Dizzy Patient Population in a Neurotology Clinic and Predictors of Peripheral Etiology.

Objective: To compare the proportion of peripheral versus nonperipheral dizziness etiologies among all patients, inclusive of those presenting primarily or as referrals, to rank diagnoses in order of frequency, to determine whether or not age and sex predict diagnosis, and to determine which subgroups tended to undergo formal vestibular testing. Study Design: Retrospective cohort. Setting: Academic neurotology clinic. Patients: Age greater than 18 neurotology clinic patients with the chief complaint of dizziness. Intervention(s): None. Main Outcome Measure(s): Age, sex, diagnosis, record of vestibular testing. Results: Two thousand seventy-nine patients were assigned 2,468 diagnoses, of which 57.7 and 42.3% were of peripheral and nonperipheral etiologies, respectively. The most common diagnoses were Meniere's (23.0%), vestibular migraine (19.3%), benign paroxysmal positional vertigo (BPPV) (19.1%), and central origin, nonmigraine (16.4%). Peripheral diagnoses are more likely to be found in men than in women (odds ratio [OR] 1.59). Peripheral diagnoses were most likely to be found in the 60 to 69 age group (OR 3.82). There was not a significant difference in rate of vestibular testing between women and men. Among patients with two diagnoses, the most common combinations were vestibular migraine and BPPV then vestibular migraine and Meniere's. Conclusions: A large proportion of patients seen for the chief complaint of dizziness in the neurotology clinic were found not to have a peripheral etiology of their symptoms. These data challenge a prevalent dogma that the most common causes of dizziness are peripheral: BPPV, vestibular neuritis, and Meniere's disease. Age and sex are statistically significant predictors of peripheral etiology of dizziness. Copyright (C) 2017 by Otology & Neurotology, Inc. Image copyright (C) 2010 Wolters Kluwer Health/Anatomical Chart Company

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Paget's Disease of the Temporal Bone: A Single-Institution Contemporary Review of 27 Patients.

Objectives: To report a contemporary review from a single-institution series on Paget's disease of the temporal bone (PDTB). Study Design: Retrospective chart review of patients evaluated from 1998 to 2016. Setting: Quaternary referral center. Patients: Patients with radiographically confirmed PDTB. Main Outcome Measures: Clinical, audiological, and radiological features and management strategies of PDTB. Results: A total of 50 temporal bones in 27 patients (15 men) were diagnosed with PDTB. Symptoms at presentation included hearing loss (n = 23, 85%), headache (n = 18, 67%), dizziness (n = 14, 52%), tinnitus (n = 5, 19%), chronic otitis media (n = 2, 7%), hemifacial spasm without facial paralysis (n = 1, 4%), multiple cranial neuropathies (n = 1, 4%), and neoplastic transformation (n = 1, 4%). Of the 23 ears with audiometric data available for review, 65% exhibited sensorineural hearing loss, and 35% mixed hearing loss. Long-term audiometric follow-up was available on two patients, both of whom demonstrated hearing loss at a rate greater than would be expected for normal aging. Two patients underwent successful cochlear implantation, achieving open-set speech recognition. Radiographic features of temporal bone involvement are reviewed and illustrated. Conclusion: This is the largest single-institution clinical series examining patients with PDTB in the English literature. Variable patterns of temporal bone involvement by Paget's disease are observed leading to a diverse set of clinical symptoms, including slowly progressive hearing loss, tinnitus, compressive cranial neuropathies, and benign or malignant tumorigenesis. Involvement typically begins in the petrous apex and progresses laterally. Otic capsule bone demineralization occurs late in the disease process. Cochlear implantation appears to be an effective management strategy for patients with severe-to-profound hearing loss. Copyright (C) 2017 by Otology & Neurotology, Inc. Image copyright (C) 2010 Wolters Kluwer Health/Anatomical Chart Company

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Cochlear Implantation in Patients With Usher Syndrome Type IIa Increases Performance and Quality of Life.

Objectives: Usher syndrome type IIa (USH2a) is characterized by congenital moderate to severe hearing impairment and retinitis pigmentosa. Hearing rehabilitation starts in early childhood with the application of hearing aids. In some patients with USH2a, severe progression of hearing impairment leads to insufficient speech intelligibility with hearing aids and issues with adequate communication and safety. Cochlear implantation (CI) is the next step in rehabilitation of such patients. This study evaluates the performance and benefit of CI in patients with USH2a. Design: Retrospective case-control study to evaluate the performance and benefit of CI in 16 postlingually deaf adults (eight patients with USH2a and eight matched controls). Performance and benefit were evaluated by a speech intelligibility test and three quality-of-life questionnaires. Results: Patients with USH2a with a mean age of 59 years at implantation exhibited good performance after CI. The phoneme scores improved significantly from 41 to 87% in patients with USH2a (p = 0.02) and from 30 to 86% in the control group (p = 0.001). The results of the questionnaire survey demonstrated a clear benefit from CI. There were no differences in performance or benefit between patients with USH2a and control patients before and after CI. Conclusions: CI increases speech intelligibility and improves quality of life in patients with USH2a. Copyright (C) 2017 by Otology & Neurotology, Inc. Image copyright (C) 2010 Wolters Kluwer Health/Anatomical Chart Company

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