A New Treatment Option in Incomplete Partition Type III: The Varese Bone-Air Stimulation (B.A.S.).

The incomplete partition type III is a severe cochlear malformation present in X-linked deafness. It is a rare, non-syndromic cause of severe to profound mixed hearing loss, often progressive. The complete absence of bony modiolus and the wide communication between the cochlea and the internal auditory canal make cochlear implantation challenging, with still no consensus on the management of these patients. To the best of our knowledge, no results have ever been published in the literature on the treatment of these patients with hybrid stimulation (bone and air). We present three cases in which this hybrid stimulation gave better audiological results then air stimulation alone. A literature review on audiological results of the current treatment options in children affected by IPIII malformation was conducted independently by two researchers. Ethical considerations on the treatment of these patients were conducted by the Bioethics department of the University of Insubria. In two of the patients, the bone-air stimulation, associated with prosthetic-cognitive rehabilitation, meant that surgery was avoided, obtaining similar communication performances of those present in the literature. We believe that, when the bone threshold appears partially preserved, a stimulation through the bone or hybrid modality, such as the Varese B.A.S. stimulation, should be attempted.

Oscillopsia in labyrinthine defective patients: comparison of objective and subjective measures.

OBJECTIVE: To compare the oscillopsia sensation in vestibular defective patients, using a specific handicap questionnaire and a specific Visual Analog Scale, with objective measure of the vertical vestibulo-ocular reflex efficiency in the pitch plane, using the computerized Dynamic Visual Acuity (DVA) test and Gaze Stabilization Test (GST). DESIGN: Controlled retrospective study. SETTING: Day hospital in ENT Rehabilitation Unit. SUBJECTS: Sixty-five subjects: 35 controls (12 men and 23 women; mean age, 50.77 ± 13.39 years) and 30 patients with chronic dizziness: 18 with unilateral vestibular hypofunction (7 men and 11 women; mean age, 55.50 ± 12.72 years) and 12 with bilateral hypofunction (7 men and 5 women; mean age, 57.25 ± 9.18 years). MAIN MEASURES: Computerize vertical DVA and GST; subjective Visual Analog Scale, Oscillopsia Score questionnaire. RESULTS: Instrumental tests had different means between subject groups; vertical DVA results and subjective measures were significantly correlated. CONCLUSIONS: Vertical DVA and GST test in up and down direction are able to separate healthy and vestibular patients. Moreover, the DVA test in down direction differentiates patients with unilateral vestibular hypofunction and with bilateral vestibular hypofunction. These results show that vertical DVA test can be used for the assessment of the visual field instability referred to as disabling.

The p.Gly130Val mutation in the GJB2 gene: A familiar case of autosomal dominant non-syndromic hearing loss.

Several forms of sensorineural hearing loss (SNHL) have been imputated to connexins mutations and prevalently to connexin 26 (Cx26), codified by the GJB2 gene (gap junction protein, beta 2). Here, we report the first familiar case (heterozygous p. G130V mutation) of non-syndromic (without any dermatological manifestation) dominant profound SNHL. Proband was a 6-years-old male with post-lingual bilateral profound SNHL, clinically identified at the age of 3 with diagnosis of severe SNHL. We confirm that the p. G130V variant of the GJB2 gene is causative of autosomal dominant form of SNHL, although it is not always associated with the presence of skin diseases.

Effects of vestibular rehabilitation therapy on emotional aspects in chronic vestibular patients.

OBJECTIVE: A strong relationship exists between vestibular dysfunction and anxiety disorders. The aim of this study was to assess the anxiety and depression levels in patients with chronic dizziness and to assess the effects of vestibular rehabilitation (VR) therapy, on the anxiety and depression levels, without a behavioural or pharmacological therapy. METHOD: Two groups of 40 patients, each affected by chronic vestibular deficit, were studied. The first one underwent VR, and the latter did not. The psychometric tests used were the State-Trait Anxiety Inventory (STAI) and the Centre for Epidemiological Studies Depression Scale (CES-D). RESULTS: Psychological factors influence the level of handicap experienced by chronic dizziness patients, and disequilibrium influences the anxiety and depression levels. STAI and CES-D scales significantly decrease after VR therapy (P<.001) and remain stable at follow-up. CONCLUSIONS: The VR therapy positively influences the emotional condition of chronic vestibular deficit patients without pharmacological or psychotherapy treatments.

Vestibular rehabilitation outcomes in chronic vertiginous patients through computerized dynamic visual acuity and Gaze stabilization test.

OBJECTIVE: To evaluate the efficiency of the rehabilitative protocols in patients with labyrinthine hypofunction, focusing on computerized dynamic visual acuity test (DVAt) and Gaze stabilization test (GST) specifically evaluating the vestibulo-oculomotor reflex (VOR) changes due to vestibular rehabilitation. DESIGN: Consecutive sample study. SETTING: Day hospital in Ears, Nose, and Throat Rehabilitation Unit. SUBJECTS: Thirty-two patients with chronic dizziness with a mean age of 60.74 years. INTERVENTION: Patients performed one cycle of 12 daily rehabilitation sessions (2 h each) consisting of exercises aimed at improving VOR gain. The rehabilitation program included substitutional and/or habitudinal exercises, exercises on a stability platform, and exercises on a moving footpath with rehabilitative software. MAIN MEASURES: Dizziness Handicap Inventory and Activities-specific Balance Confidence Scale. Computerized dynamic posturography, computerized DVAt, and GST. RESULTS: The patients significantly improved in all the tests. CONCLUSION: Vestibular rehabilitation improved the quality of life by reducing the handicap index and improving the ability in everyday tasks. The recovery of the vestibular-ocular reflex and vestibular-spinal reflex efficiency was objectively proven by instrumental testing. The DVAt and the GST allow to objectively quantify the fixation ability at higher frequencies and speeds (main VOR function). Moreover, these new parameters permit to completely evaluate vestibular rehabilitation outcomes, adding new information to the generally used tests that only assess vestibulospinal reflex.

Balance impairment after acoustic neuroma surgery.

OBJECTIVE: The aim of this study was to assess the incidence of balance problems after acoustic neuroma surgery, evaluating whether disequilibrium is disabling. STUDY DESIGN: Retrospective observational study. SETTING: Rehabilitation center. PATIENTS: A group of 386 patients who underwent acoustic neuroma surgery. INTERVENTIONS: Patients were selected from a population of 459 subjects who had undergone surgery for acoustic neuroma. MEAN OUTCOME MEASURES: The Dizziness Handicap Inventory, The Activities-specific Balance Confidence Scale (ABC), and a specific questionnaire on oscillopsia. RESULTS: The specific questionnaire emphasized that 39 patients (10.10%) perceived disequilibrium as disabling, and the oscillopsia handicap score result was moderate in 73.32% of the sample, mild in 21.50%, and severe in 5.18% of patients. The Dizziness Handicap Inventory and ABC scales revealed the presence of handicap and disability due to disequilibrium and the influence of some variables such as sex and a higher oscillopsia handicap score. Dizziness Handicap Inventory and ABC scores were higher in symptomatic patients. CONCLUSION: Disequilibrium influences handicap and disability after acoustic neuroma surgery. This symptom is also present after several years since surgery, and some patients perceived disequilibrium as disabling.

Quality of life after acoustic neuroma surgery.

OBJECTIVE: The aim of this study is to assess patients' quality of life after acoustic neuroma surgery, and if some patients' characteristics, parameters of tumor and surgical parameters affect patients' quality of life. STUDY DESIGN: Retrospective study. SETTING: Tertiary care center. PATIENTS: A group of 386 patients who underwent acoustic neuroma surgery. INTERVENTION: Patients included were derived from a population of 459 subjects operated for acoustic neuroma by the Otologic Group of Piacenza. MEAN OUTCOME MEASURE: The outcome of the questionnaires on quality of life (SF 36), the Dizziness Handicap Inventory (DHI) and a questionnaire of patients' histories were analyzed. RESULTS: In our sample a group of 231 patients were asymptomatic after surgery, while 155 patients reported very really disabling symptoms. Hearing loss was perceived as the most disabling symptom. The scores of questionnaire on quality of life were lower than scores for the normal population. The indices of disablement influenced the perception of quality of life. CONCLUSION: Our study has shown that patients perceived a worsening of their quality of life, in particular women and patients over 45. Surgery caused a higher percentage of disability. Patients' quality of life can be used as a parameter for the timing of surgery. Our data indicate that an early surgical approach for intrameatal tumor is better than waiting for an increase in the tumor size. A "wait-and-scan" strategy for extrameatal neuromas which do not affect the brainstem is preferable, since these patients have a worsening of their quality of life after surgery independently of the tumor size.

Vestibular function after cochlear implant surgery.

OBJECTIVE: The aim of this study was to analyze vestibular function in cochlear implant (CI) patients for iatrogenic damage to vestibular function. METHODS: Prospective clinical study. Tertiary care audiological center. Twenty-five subjects receiving surgery for cochlear implantation during 2012 and 2013 were analyzed. Both vestibulo-ocular and vestibulo-spinal responses before and 2 months after CI surgery were evaluated using several tests: analysis of spontaneous nystagmus, head shaking test (HST), and head impulse test recorded by videooculography; caloric stimulation at 44° and 30° in both ears; cervical evoked myogenic potentials and static stabilometry. Residual cochlear function was tested by air-conduction pure-tone audiometry. RESULTS: Our tests showed damage to vestibular receptors after CI surgery in 12% of the patients; in particular, horizontal semicircular canal function and saccular function had lower responses after surgery. Audiometric results showed poorer thresholds after CI surgery. The static stabilometry results indicate good vestibulo-spinal responses and patients did not report disequilibrium nor postural deficit. CONCLUSION: All data suggest an efficient vestibular compensation mechanism in CI patients.

Recurrence quantification analysis of gait in normal and hypovestibular subjects.

The study of postural control processes during locomotion may provide useful outcome measures of stability for people with unilateral vestibular hypofunction (UVH). Since nonlinear analysis techniques can characterize complex behaviour of a system, this may highlight mechanisms underlying dynamic stability in locomotion, although only few efforts have been made. In particular, there have been no studies that use recurrence quantification analysis (RQA), which can be applied even to short and non-stationary data. The purpose of this study was to develop a new method for walking balance assessment measuring the complexity of head, trunk and pelvis three-dimensional accelerations and angular velocities during normal overground locomotion by means of RQA in normal subjects and UVH patients. The results showed differential effect of upper body parts on pattern regularity, with better head than pelvis stabilization in both groups of subjects. The RQA outputs such as percent determinism and recurrence were nevertheless significantly lower in the UVH group for all measures, suggesting that body accelerations and angular velocities, although not significantly different in amplitude, were more chaotic in patients. The observed lower regularity of upper body movements in UVH is consistent with an important role of the vestibular system in controlling dynamic stability during walking. The findings suggest that RQA can be used as a quantitative tool to assess walking performance and rehabilitation outcome in patients with different balance disorders.