Measurements of Trunk Sway for Stance and Gait Tasks 2 Years after Vestibular Neurectomy.

The aim of this study was to investigate changes in balance control for stance and gait tasks in patients 2 years before and after vestibular neurectomy (VN) performed to alleviate intractable Meniere's disease. Amplitudes of trunk sway in roll and pitch directions were measured for stance and gait tasks in 19 patients using gyroscopes mounted at the lower-back. Measurements before VN and 2 years later were compared to those of healthy age-matched controls (HC). We also examined if changes in trunk sway amplitudes were correlated with patients' subjective assessment of disability using the AAO-HNS scale. For patients with low AAO-HNS scores 0-2 (n = 14), trunk roll and pitch sway velocities, standing eyes closed on foam, increased 2 years post VN compared to HC values (p < 0.01). Trunk sway amplitudes remained at levels of HC for simple gait tasks, but task durations were longer and therefore gait slower. For complex gait tasks (stairs), balance control remained impaired at 2 years. In patients with AAO-HNS high scores level 6 (n = 5), balance control remained abnormal, compared to HC, 2 years postoperatively for all stance, several simple and all complex gait tasks. Trunk sway in the pitch and roll directions for stance tasks was correlated with clinical (AAO-HNS) scores (p ≤ 0.05). These results indicate that VN leads to chronic balance problems for stance and complex gait tasks. The problems are greater for patients with high compared to low AAO-HNS scores, thereby explaining the different symptoms reported by these patients. The lack of balance recovery in VN patients to levels of HCs after 2 years contrasts with the 3 months average recovery period for acute vestibular neuritis patients and is indicative of the effects of neurectomy on central compensation processes.

Novel Bone-Anchored Vascular Access on the Mastoid for Hemodialysis: Concept and Preclinical Trials.

GOAL: We present the development of a bone-anchored port for the painless long-term hemodialytic treatment of patients with renal failure. This port is implanted behind the ear. METHODS: The port was developed based on knowledge obtained from long-term experience with implantable hearing devices, which are firmly anchored to the bone behind the ear. This concept of bone anchoring was adapted to the requirements for a vascular access during hemodialysis. The investigational device is comprised of a base plate that is firmly fixed with bone screws to the bone behind the ear (temporal bone). A catheter leads from the base plate valve block through the internal jugular vein and into the right atrium. The valves are opened using a special disposable adapter, without any need to puncture the blood vessels. Between hemodialysis sessions, the port is protected with a disposable cover. RESULTS: Flow rate, leak tightness, and purification were tested on mockups. Preoperative planning and the surgical procedure were verified in 15 anatomical human whole head specimens. CONCLUSION: Preclinical evaluations demonstrated the technical feasibility and safety of the investigational device. SIGNIFICANCE: Approximately 1.5 million people are treated with hemodialysis worldwide, and 25% of the overall cost of dialysis therapy results from vascular access problems. New approaches toward enhancing vascular access could potentially reduce the costs and complications of hemodialytic therapy.

[A new bone anchored hemodialysis access]. / Neuartiger knochenverankerter Hämodialysezugang.

When classic arteriovenous fistulas or grafts fail, dialysis patients have a vital requirement for a catheter to ensure vascular access. Permanent central venous catheters penetrate the cervical and thoracic soft tissues and the skin without rigid fixation. The infection rate for such devices is high, often requiring explantation. Bone anchored hearing aids are an established treatment in patients with conductive hearing loss. The implant is firmly fixed on the temporal bone and the abutment permanently penetrates the skin. Severe infections requiring explantation are very rare. We suppose that one of the main reasons for the low complication rate is the firm fixation of the implant to the temporal bone, which minimizes the movement of the skin relative to the underlying bone. Based on the experience with implantable hearing devices we developed a percutaneous bone anchored port fixed to the skull in the region of the temporal bone. Such a bone anchored port could be a beneficial alternative to conventional central venous catheters for patients undergoing hemodialysis. In the course of the development process we investigated the individual anatomy to locate the correct implantation site with sufficient bone thickness; we studied screw stability in bone; we developed the titanium implant that houses the port system as well as the surgical tools and procedure for save implantation; we tested flow rate, leak tightness and purification on mockups; we defined the Seldinger-insertion of the catheter into the internal jugular vein via a small neck incision. Our results show the technical feasibility of a temporal bone anchored port and form the basis of a now-approved clinical pilot study.

Functional results and quality of life after retrosigmoid vestibular neurectomy in patients with Ménière's disease.

BACKGROUND: Severe Ménière's disease (MD) may be debilitating and compromising, despite intensive medical treatment. Vestibular neurectomy (VN) is considered an effective surgical treatment for disabling MD. Our aim was to analyse the medium- to long-term outcome after retrosigmoid VN with special regard to vertigo, quality of life (American Academy of Otolaryngology-Head and Neck Surgery criteria), and pure tone average (PTA). METHODS: Retrospective evaluation of patients with disabling MD treated with retrosigmoid VN at the university hospital of Bern (1992-2009), after unsuccessful attempts at medical treatment. Demographics, clinical signs and symptoms, quality of life, thresholds of hearing, and adverse events were documented at baseline, 1 week, 12 months, and 24 months after surgery. RESULTS: Medium to long-term follow-up data were available from 44 of 78 patients, who had undergone retrosigmoid VN (19 men; mean age, 50.3 ± 11.0 yr). Vertigo disappeared in 34 (77.3%) of 44 patients and improved in 6 (13.6%) of 44 patients. Quality of life significantly improved postoperatively (mean American Academy of Otolaryngology-Head and Neck Surgery, 0.68 ± 1.14 [1 yr] and 0.57 ± 1.19 [2 yr] versus 5.11 ± 0.66). Mean PTA decreased (52.3 ± 19.2 dB versus 56.2 ± 21.6 dB [1 wk] and 60.4 ± 20.5 dB [1 yr]; p < 0.001). Ten (22.7%) of 44 patients showed improved PTA after VN. These patients had significantly higher baseline PTA (69.4 dB versus 47.9 dB; p = 0.001). CONCLUSION: Retrosigmoid VN is a valuable and safe surgical option to treat disabling MD that has proved resistant to medical treatments. It may also be indicated for patients with preoperative severely impaired thresholds of hearing, in whom a certain hearing gain may be observed.

Mutation analysis of Netrin 1 and HMX3 genes in patients with superior semicircular canal dehiscence syndrome.

CONCLUSION: In spite of its absence in the control population, there is questionable evidence for the alteration c.114C->T in the HMX3 gene being implicated in the development of superior semicircular canal dehiscence (SSCD). However, the concept of a complex disease is valid for SSCD and a possible molecular origin can neither be confirmed nor excluded by the results of this study. OBJECTIVES: SSCD was first described in 1998 by Minor et al. While the etiology is not clear, findings from both temporal bone CT and histologic studies suggest a congenital or developmental origin. In recent years, a couple of genes regulating inner ear morphogenesis have been described. Specifically, Netrin-1 and HMX3 have been shown to be critically involved in the formation of the SCC. Molecular alterations in these two genes might lead to a disturbed development of this canal and might represent an explanation for SSCD. METHODS: DNA was extracted from whole blood of 15 patients with SSCD. The coding sequences of Netrin-1 and HMX3 were amplified by PCR and sequenced. RESULTS: One sequence alteration, heterozygous c.114C->T (conservative change without alteration of amino acid) in exon 1 of HMX3, was detected in 2 of 15 patients but not in 300 control chromosomes. The study was supported in part by the Emilia-Guggenheim-Schnurr-Foundation, Basel, Switzerland.

Retrograde cochlear implantation in postmeningitic basal turn ossification.

OBJECTIVES/HYPOTHESIS: Postmeningitic basal turn ossification is a challenge for successful cochlear implantation despite the availability of sophisticated implants and advanced drill-out procedures. A less complex concept consisting of a cochleostomy near the apex with retrograde array insertion is evaluated clinically and experimentally with emphasis on imaging of intracochlear array morphology. STUDY DESIGN: Retrospective case-control study. METHODS: Outcome, hearing performance, and radiological findings including three-dimensional (3D) reconstructions were assessed in the long term in eight retrograde implanted ears of seven postmeningitic deaf patients and compared to an etiology- and device-matched control group of 17 basal turn implanted ears of 14 patients. Experimental insertions into three autopsy-derived human temporal bones were evaluated using high-resolution microtomography, 3D reconstruction, and histology. RESULTS: No complications occurred. At the long-term follow-up, the average monosyllabic word test scores were 41% for the study group and 67% for the control group (P = .03). Radiological follow-up revealed insertion sites into either the apical or middle turn and frequent intracochlear array direction changes (n = 5). Experimental implantations in temporal bones resulted in folding-free, retrograde, middle turn insertions (n = 3). CONCLUSIONS: The retrograde cochlear implantation is a safe and efficient alternative approach in basal turn ossification. Despite a high occurrence of intracochlear array direction changes, open set speech discrimination was achieved in all patients. Postoperative computed tomography is recommended for fitting the speech processor according to intracochlear array positions. The experimental insertion in temporal bones helped to optimize the approach.

In-the-canal versus behind-the-ear microphones improve spatial discrimination on the side of the head in bilateral cochlear implant users.

OBJECTIVE: To test whether in-the-canal (ITC) microphones have an impact on spatial discrimination and speech perception by taking advantage of auricular cues. STUDY DESIGN: Experimental clinical study. SETTING: Audiologic laboratory in a tertiary referral center. PATIENTS: Seven bilateral adult cochlear implant (CI) users were selected from the institution's database. INTERVENTION: The adaptive German Oldenburg sentence test and minimum audible angle measurements were performed binaurally with experimental ITC and behind-the-ear (BTE) microphones. MAIN OUTCOME MEASURES: Spatial discrimination and speech perception scores assessed for each microphone position. RESULTS: Spatial discrimination on the side of the head was superior using ITC compared with BTE microphone positions; the differences on the side of the first CI were statistically significant (mean minimum audible angle ± standard deviation, 28.4 ± 7.61 degrees versus 34.1 ± 14.4 degrees; p = 0.031). Speech perception scores with ITC versus BTE microphone positions also were improved, although the differences were not statistically significant. CONCLUSION: ITC microphones could help to improve CI performance in the future.

Meniere's disease in the elderly.

Menière disease usually begins in adults from 20 to 60 years old, and occurs in more than 10% of patients older than 65. The treatment of Menière disease in the elderly represents a challenge because of polymedication. Antivertiginous drugs such as betahistine and cinnarizin give good results with minor secondary effects. In contrast, major vestibular suppressor drugs such as thiethylperazin must be avoided as long-term treatment because of their side effects. Definitive vestibular surgical deafferentations such as labyrinthectomy and selective vestibular neurectomy represent optional procedures but must be carefully evaluated from case to case. Ablative procedures remain the efficient treatment of drop attacks, which represent a high potential risk of severe injuries by older patients sometimes with important social consequences.

The floating mass transducer at the round window: direct transmission or bone conduction?

The round window placement of a floating mass transducer (FMT) is a new approach for coupling an implantable hearing system to the cochlea. We evaluated the vibration transfer to the cochlear fluids of an FMT placed at the round window (rwFMT) with special attention to the role of bone conduction. A posterior tympanotomy was performed on eleven ears of seven human whole head specimens. Several rwFMT setups were examined using laser Doppler vibrometry measurements at the stapes and the promontory. In three ears, the vibrations of a bone anchored hearing aid (BAHA) and an FMT fixed to the promontory (pFMT) were compared to explore the role of bone conduction. Vibration transmission to the measuring point at the stapes was best when the rwFMT was perpendicularly placed in the round window and underlayed with connective tissue. Fixation of the rwFMT to the round window exhibited significantly lower vibration transmission. Although measurable, bone conduction from the pFMT was much lower than that of the BAHA. Our results suggest that the rwFMT does not act as a small bone anchored hearing aid, but instead, acts as a direct vibratory stimulator of the round window membrane.

Revision stapedectomy: an analysis of 201 operations.

INTRODUCTION: Recurrent or persistent conductive hearing impairment as well as vertigo or tinnitus after a stapes operation can be treated by means of stapes revision surgery. We analyzed stapes revisions performed during the last 15 years at our institution. The aim was to ascertain the causes that led to the failure, to analyze the postrevision results, and to compare them with data from the literature. MATERIALS AND METHODS: This retrospective study analyzes 201 stapes revisions performed in 175 patients between 1992 and 2006. The indications, intraoperative findings, and surgical techniques were analyzed. Functional results were evaluated by means of hearing tests before and after surgery (1-15 yr). Revisions were mainly performed by the transcanal approach through the fixed speculum under local anesthesia using the fiber-optic argon laser and the Skeeter microdrill. RESULTS: Indications for revision in 172 operations included lack of hearing improvement or recurrent conductive hearing loss. Six patients additionally had symptoms of vertigo. Sixteen patients experienced isolated vertigo despite improved hearing, 1 patient experienced intolerable tinnitus, and in 6 patients, the indication was deafness after stapedotomy.The most common intraoperative observations were prosthesis lateralization (53%), partial or total incus necrosis (33%), reossification of the footplate (31%), and loosening of the loop on the incus (9%). Hearing improved postoperatively in 88% of the patients. In 55% of the patients, there was a residual air-bone gap of 10 dB or less, and in 84%, the gap was 20 dB or less. DISCUSSION: Stapes revisions are more challenging procedures with less perfect results compared with primary operations. Nevertheless, a postoperative hearing improvement was achieved in 88% of our patients. Modern techniques, such as the laser and microdrill, demonstrate their full usefulness.

Prospective effectiveness of stapes surgery for otosclerosis in a multicenter audit setting: feasibility of the Common Otology Database as a benchmark database.

INTRODUCTION: The Common Otology Database (COD) is a joint effort by an international group of otologists to organize audit with a standardized reporting method in middle ear surgery. The first results on hearing outcome of the COD are presented in this article. OBJECTIVE: The primary objective was to confirm the validity of the benchmark group by comparing hearing outcome results with previously reported results. The secondary objective was to describe the population, technical aspects, and hearing outcomes of stapes surgery. STUDY DESIGN: Nonrandomized prospective multicenter audit. SETTING: Twenty tertiary-referral otologic centers. PATIENTS AND INTERVENTION: Primary and revision stapes operations in patients with otosclerosis. MAIN OUTCOME MEASURES: Air-bone gap (ABG), bone-conduction (BC) thresholds, and air-conduction (AC) thresholds were evaluated at 3 and 12 months according to the guidelines of the Committee on Hearing and Equilibrium for the evaluation of conductive hearing loss. Raw data were displayed in an Amsterdam Hearing Evaluation Plot. RESULTS: In primary stapes surgeries, the postoperative ABG was closed to 10 dB or less in 63.6% and to 20 dB or less in 92.6% (median, 8.75 dB). In revision stapes surgeries, the postoperative ABG was closed to 10 dB in 41.2% and to 20 dB in 76.5% of cases (median, 11.25 dB). The overall mean postoperative ABG at 12 months was 10.38 dB compared with 28.75 dB preoperatively. Using laser to perform the fenestration results in a less pronounced BC improvement when compared with procedures without laser assistance. No statistically significant difference in ABG pure-tone average at 3 months could be demonstrated between the different prosthesis types. CONCLUSION: Results of hearing outcome are similar to previous, primarily retrospective, single-center studies. Our data confirm the effectiveness of stapes surgery in patients with otosclerosis.

Otologic manifestations in Samter's syndrome.

OBJECTIVE: It was the aim of this study to investigate the prevalence of otologic manifestations in a cohort of Samter's syndrome patients (nasal polyps with chronic rhinosinusitis, aspirin intolerance and asthma). METHODS: We analyzed a retrospective and prospective case review from 1995 to 2005, performed in the otorhinolaryngology outpatient clinic of a tertiary referral center. Ear history, clinical examinations, treatment and outcome were evaluated using office and hospital records in 23 cases. All subjects completed a questionnaire. RESULTS: In 6 cases (26%), otological manifestations with ear pressure and conductive hearing loss occurred during an advanced stage of Samter's syndrome (>5 years after onset of the first nasal symptoms) partially responsive to systemic steroids. CONCLUSION: Recognition of the association between Samter's syndrome and otological disease is important (26% of the cases) because it could also be responsive to systemic steroids which prevent progression to irreversible hearing loss or infectious otomastoiditis.

Cholesterol granuloma of the petrous apex: benefit of computer-aided surgery.

The following is an analysis of the role of computer aided surgery by infralabyrinthine-subcochlear approach to the petrous apex for cholesterol granulomas with hearing preservation. In a retrospective case review from 1996 to 2008 six patients were analysed in our tertiary referral centre, otorhinolaryngology outpatient clinic. Excellent intraoperative localisation of the carotid artery, facial nerve and the entrance into the cholesterol cyst of the bone by means of the navigation system was seen. Additionally, the operation time decreased from an initial 4 h down to 2 h. The application of computer-aided surgery allows intraoperative monitoring of the position of the tip of the microsurgical instruments in case of a rare disease and in the delicate area of the petrous apex giving a high security level.

External auditory canal cholesteatoma: reassessment of and amendments to its categorization, pathogenesis, and treatment in 34 patients.

OBJECTIVE: External auditory canal cholesteatoma (EACC) is a rarity. Although there have been numerous case reports, there are only few systematic analyses of case series, and the pathogenesis of idiopathic EACC remains enigmatic. STUDY DESIGN: In a tertiary referral center for a population of 1.5 million inhabitants, 34 patients with 35 EACC (13 idiopathic [1 bilateral] and 22 secondary) who were treated between 1994 and 2006 were included in the study. RESULTS: EACC cardinal symptoms were longstanding otorrhea (65%) and dull otalgia (12%). Focal bone destruction in the external auditory canal with retained squamous debris and an intact tympanic membrane were characteristic. Only 27% of the patients showed conductive hearing loss exceeding 20 dB. Patients with idiopathic EACC had lesions typically located on the floor of the external auditory canal and were older, and the mean smoking intensity was also greater (p < 0.05) compared with patients with secondary EACC. The secondary lesions were assigned to categories (poststenotic [n = 6], postoperative [n = 6], and posttraumatic EACC [n = 4]) and rare categories (radiogenic [n = 2], postinflammatory [n = 1], and postobstructive EACC [n = 1]). In addition, we describe 2 patients with EACC secondary to the complete remission of a Langerhans cell histiocytosis of the external auditory canal. Thirty of 34 patients were treated surgically and became all free of recurrence, even after extensive disease. DISCUSSION: For the development of idiopathic EACC, repeated microtrauma (e.g., microtrauma resulting from cotton-tipped applicator abuse or from hearing aids) and diminished microcirculation (e.g., from smoking) might be risk factors. A location other than in the inferior portion of the external auditory canal indicates a secondary form of the disease, as in the case of 2 patients with atypically located EACC after years of complete remission of Langerhans cell histiocytosis, which we consider as a new posttumorous category and specific late complication of this rare disease.

International Common Otology Database: taste disturbance after stapes surgery.

OBJECTIVE: To use a study on dysgeusia to assess the usefulness of an otology database. STUDY DESIGN: Data were extracted from the international Common Otology Database. INTERVENTION: Primary stapes operations. MAIN OUTCOME MEASURE AND RESULTS: From a cohort of 14 otologists, only 8 (57%) were able to satisfy external validation and maintain data input for a period of at least 6 months. The rates of dysgeusia varied from 0 to 39% at 3 months and 0 to 27% at 6 months. The percentages of patients with taste disturbance at 6 months in the "nerve-cut" and "nerve-preserved" groups were 22.7 and 10.9%, respectively, although this was not statistically significant (chi2; p = 0.325). CONCLUSION: Many surgeons found it difficult to maintain a prospective otology database. The rates of certain subjective symptoms such as dysgeusia are influenced by how vigorously the reviewers prompt the response from the patients. Dysgeusia after stapes surgery is common even if the chorda tympani nerve is preserved. Many patients whose chorda tympani nerve is divided may not complain of dysgeusia.

Ultrastructural changes in otoconia of osteoporotic rats.

The etiology of benign paroxysmal positional vertigo (BPPV) remains obscure in many cases and women are affected more often than men. A recent prospective study, performed in women >50 years of age suffering from recurrent BPPV, showed associated osteopenia or osteoporosis in a large percentage of these patients. These results suggested the possible relationship between recurrent BPPV and a decreased fixation of calcium in bone in women >50 years. To test this hypothesis, an experimental study was performed in adult female rats. Utricular otoconia of female rats in which osteopenia/osteoporosis was induced by bilateral ovariectomy (OVX) were compared to those of sham-operated adult females rats (SHAM), as control group. FIRST STUDY: The morphology of theutricles of OVX and SHAM rats was analyzed with scanning electron microscopy. In osteopenic/osteoporotic rats, the density of otoconia (i.e. the number of otoconia per unit area) was decreased (p = 0.036)and their size was increased (p = 0.036) compared to the control group. SECOND STUDY: To test the role of calcium turnover in such morphological changes, utricular otoconia of 2 other groups of OVX and SHAM rats, previously injected with calcein subcutaneously, were examined by conventional and epifluorescence microscopy. In epifluorescence microscopy, labeling with calcein showed no significant fluorescence in either group. This finding was interpreted as a lack of external calcium turnover into otoconia of adult female rats. The ultrastructural modifications of otoconia in osteopenic/osteoporotic female adult rats as well as the role of estrogenic receptors in the inner ear are discussed. The possible pathophysiological mechanisms which support the relationship between recurrent BPPV in women and the disturbance of the calcium metabolism of osteopenia/osteoporosis are debated.

A novel implantable hearing system with direct acoustic cochlear stimulation.

A new implantable hearing system, the direct acoustic cochlear stimulator (DACS) is presented. This system is based on the principle of a power-driven stapes prosthesis and intended for the treatment of severe mixed hearing loss due to advanced otosclerosis. It consists of an implantable electromagnetic transducer, which transfers acoustic energy directly to the inner ear, and an audio processor worn externally behind the implanted ear. The device is implanted using a specially developed retromeatal microsurgical approach. After removal of the stapes, a conventional stapes prosthesis is attached to the transducer and placed in the oval window to allow direct acoustical coupling to the perilymph of the inner ear. In order to restore the natural sound transmission of the ossicular chain, a second stapes prosthesis is placed in parallel to the first one into the oval window and attached to the patient's own incus, as in a conventional stapedectomy. Four patients were implanted with an investigational DACS device. The hearing threshold of the implanted ears before implantation ranged from 78 to 101 dB (air conduction, pure tone average, 0.5-4 kHz) with air-bone gaps of 33-44 dB in the same frequency range. Postoperatively, substantial improvements in sound field thresholds, speech intelligibility as well as in the subjective assessment of everyday situations were found in all patients. Two years after the implantations, monosyllabic word recognition scores in quiet at 75 dB improved by 45-100 percent points when using the DACS. Furthermore, hearing thresholds were already improved by the second stapes prosthesis alone by 14-28 dB (pure tone average 0.5-4 kHz, DACS switched off). No device-related serious medical complications occurred and all patients have continued to use their device on a daily basis for over 2 years.

Echinococcosis presenting as an otogenic brain abscess: an unusual lesion of the middle ear cleft and temporal lobe.

This paper presents a case of a 28-year-old male with a seizure episode and a 4-year history of intermittent tinnitus on the left ear. On computed tomography and magnetic resonance imaging, a density with rim enhancement was found at the temporal lobe, associated with mastoid tegmen destruction and middle ear mass, indicating cholesteatoma with complicating brain abscess. Evacuation of the brain abscess was performed with a combined otolaryngologic and neurosurgical procedures (canal wall-down mastoidectomy and temporal craniotomy). The pathology turned out to be infestation with Echinococcus granulosus.

Speech understanding in quiet and in noise with the bone-anchored hearing aids Baha Compact and Baha Divino.

CONCLUSIONS: Speech understanding is better with the Baha Divino than with the Baha Compact in competing noise from the rear. No difference was found for speech understanding in quiet. Subjectively, overall sound quality and speech understanding were rated better for the Baha Divino. OBJECTIVES: To compare speech understanding in quiet and in noise and subjective ratings for two different bone-anchored hearing aids: the recently developed Baha Divino and the Baha Compact. PATIENTS AND METHODS: Seven adults with bilateral conductive or mixed hearing losses who were users of a bone-anchored hearing aid were tested with the Baha Compact in quiet and in noise. Tests were repeated after 3 months of use with the Baha Divino. RESULTS: There was no significant difference between the two types of Baha for speech understanding in quiet when tested with German numbers and monosyllabic words at presentation levels between 50 and 80 dB. For speech understanding in noise, an advantage of 2.3 dB for the Baha Divino vs the Baha Compact was found, if noise was emitted from a loudspeaker to the rear of the listener and the directional microphone noise reduction system was activated. Subjectively, the Baha Divino was rated statistically significantly better in terms of overall sound quality.