Paul Van de Heyning

A basic protocol for functional assessment of voice pathology, especially for investigating the efficacy of (phonosurgical) treatments and evaluating new assessment techniques

Abstract The proposal of this basic protocol is an attempt to reach better agreement and uniformity concerning the methodology for functional assessment of pathologic voices. The purpose is to allow relevant comparisons with the literature when presenting / publishing the results of voice treatment, e.g. a phonosurgical technique, or a new / improved instrument or procedure for investigating the pathological voice. Meta-analyses of the results of voice treatments are generally limited and may even be impossible owing to the major diversity in the ways functional outcomes are assessed. A multidimensional set of minimal basic measurements suitable for all “common” dysphonias is proposed. It includes five different approaches: perception (grade, roughness, breathiness), videostroboscopy (closure, regularity, mucosal wave and symmetry), acoustics (jitter, shimmer, Fo-range and softest intensity), aerodynamics (phonation quotient), and subjective rating by the patient. The protocol is elaborated on the basis of an exhaustive review of the literature, of the experience of the Committee members, and of plenary discussions within the European Laryngological Society. Instrumentation is kept to a minimum, but it is considered essential for professionals performing phonosurgery.

GJB2 Mutations and Degree of Hearing Loss: A Multicenter Study

Hearing impairment (HI) affects 1 in 650 newborns, which makes it the most common congenital sensory impairment. Despite extraordinary genetic heterogeneity, mutations in one gene, GJB2, which encodes the connexin 26 protein and is involved in inner ear homeostasis, are found in up to 50% of patients with autosomal recessive nonsyndromic hearing loss. Because of the high frequency of GJB2 mutations, mutation analysis of this gene is widely available as a diagnostic test. In this study, we assessed the association between genotype and degree of hearing loss in persons with HI and biallelic GJB2 mutations. We performed cross-sectional analyses of GJB2 genotype and audiometric data from 1,531 persons, from 16 different countries, with autosomal recessive, mild-to-profound nonsyndromic HI. The median age of all participants was 8 years; 90% of persons were within the age range of 0-26 years. Of the 83 different mutations identified, 47 were classified as nontruncating, and 36 as truncating. A total of 153 different genotypes were found, of which 56 were homozygous truncating (T/T), 30 were homozygous nontruncating (NT/NT), and 67 were compound heterozygous truncating/nontruncating (T/NT). The degree of HI associated with biallelic truncating mutations was significantly more severe than the HI associated with biallelic nontruncating mutations (P<.0001). The HI of 48 different genotypes was less severe than that of 35delG homozygotes. Several common mutations (M34T, V37I, and L90P) were associated with mild-to-moderate HI (median 25-40 dB). Two genotypes--35delG/R143W (median 105 dB) and 35delG/dela(GJB6-D13S1830) (median 108 dB)--had significantly more-severe HI than that of 35delG homozygotes.

Test-retest study of the GRBAS scale: Influence of experience and professional background on perceptual rating of voice quality

Perceptual evaluation was performed by means of the GRBAS scale for a series of nine pathological voice samples that were presented twice to a group of 23 judges, consisting of experienced and inexperienced otolaryngologists (ENTs) and speech-language pathologists (S/LPs). The time interval between ratings was 14 days. Test-retest reliability was moderate. The best agreement between the observers was obtained for the G (grade) parameter and the worst for the S (strained) parameter. Considering the medians of the GRBAS ratings, no significant influence was measured for level of experience or professional background. Yet, based on means, the results show that professional background has a greater impact on perceptual rating than experience.

The neural correlates of tinnitus-related distress

Tinnitus is an auditory phantom percept with a tone, hissing, or buzzing sound in the absence of any objective physical sound source. About 6% to 25% of the affected people report interference with their lives as tinnitus causes a considerable amount of distress. However, the underlying neurophysiological mechanism for the development of tinnitus-related distress remains not well understood. Hence we focus on the cortical and subcortical source differences in resting-state EEG between tinnitus patients with different grades of distress using continuous scalp EEG recordings and Low Resolution Electromagnetic Tomography (LORETA). Results show more synchronized alpha activity in the tinnitus patients with a serious amount of distress with peaks localized to various emotion-related areas. These areas include subcallosal anterior cingulate cortex, the insula, parahippocampal area and amygdala. In addition, less alpha synchronized activity was found in the posterior cingulate cortex, precuneus and DLPFC. A comparison between the tinnitus group with distress and the Nova Tech EEG (NTE) normative database demonstrated increased synchronized alpha and beta activity and less synchronized delta and theta activity in the dorsal anterior cingulate cortex in tinnitus patients with distress. It is interesting that the areas found show some overlap with the emotional component of the pain matrix and the distress related areas in asthmatic dyspnea. Unpleasantness of pain activates the anterior cingulate and prefrontal cortices, amygdala, and insula. As such, it might be that distress is related to alpha and beta activity in the dorsal anterior cingulate cortex, the amount of distress perceived to an alpha network consisting of the amygdala-anterior cingulate cortex-insula-parahippocampal area.

Incapacitating Unilateral Tinnitus in Single-Sided Deafness Treated by Cochlear Implantation

Objectives: Tinnitus is a well-known, difficult-to-treat symptom of hearing loss. Users of cochlear implants (CIs) have reported a reduction in tinnitus following implantation for bilateral severe-to-profound deafness. This study assessed the effect of electrical stimulation via a CI on tinnitus in subjects with unilateral deafness and ipsilateral tinnitus who underwent implantation in an attempt to treat tinnitus with the CI. Methods: Twenty-one subjects who complained of severe intractable tinnitus that was unresponsive to treatment received a CI. Tinnitus loudness was measured with a Visual Analog Scale; loudness percepts were recorded with the device activated and deactivated. Tinnitus distress was measured with the Tinnitus Questionnaire before and after implantation. Results: Electrical stimulation via a CI resulted in a significant reduction in tinnitus loudness (mean ± SD; 1 year after implantation, 2.4 ± 1.8; 2 years after implantation, 2.5 ± 1.9; before implantation, 8.5 ± 1.3). With the device deactivated, tinnitus loudness was still reduced to between 6.1 and 7.0 over 24 months. The Tinnitus Questionnaire revealed a significant positive effect of CI stimulation. Conclusions: Unilateral tinnitus resulting from single-sided deafness can be treated with electrical stimulation via a CI. The outcomes of this pilot study demonstrate a new method for treatment of tinnitus in select subjects, perhaps an important new indication for cochlear implantation.

Transcranial magnetic stimulation for tinnitus: influence of tinnitus duration on stimulation parameter choice and maximal tinnitus suppression.

Objective: Tinnitus is a distressing symptom for which few treatments exist. It leads to an important decrease in quality of life in 2 to 3% of the population. Tinnitus is considered a phantom sound, the result of cortical reorganization. Transcranial magnetic stimulation (TMS) is a noninvasive method to modulate cortical reorganization and has been shown to be able to influence tinnitus perception. Study Design: Retrospective analysis. Setting: Tertiary referral center. Patients: The effect of TMS of the contralateral auditory cortex in 114 patients with unilateral tinnitus is investigated as one of the selection criteria used for surgical implantation of electrodes on the auditory cortex. Intervention: TMS is performed at 90% of motor threshold at 1, 3, 5, 10, and 20 Hz, with each stimulation session consisting of 200 pulses. Results were classified as no effect (0-19% improvement), partial effect (20-79% improvement), and good effect (80-100 suppression). Main Outcome Measures: TMS had a good effect in 25% of the patients studied, partial effect in 28% patients, and no effect in 47%. Results: TMS at 200 pulses is capable of tinnitus suppression for seconds only. The results were influenced by tinnitus duration: the longer the tinnitus exists, the lower the stimulation frequency that yields maximal tinnitus suppression (p < 0.001). The maximal amount of tinnitus suppression decreases in time (p < 0.01), resulting in a 2% decrease of potential tinnitus suppression per year. Conclusion: TMS of the auditory cortex is capable of modifying tinnitus perception for a very short time. The maximal amount of suppression and best stimulation frequency depends on the tinnitus duration.

Tinnitus Intensity Dependent Gamma Oscillations of the Contralateral Auditory Cortex

Background Non-pulsatile tinnitus is considered a subjective auditory phantom phenomenon present in 10 to 15% of the population. Tinnitus as a phantom phenomenon is related to hyperactivity and reorganization of the auditory cortex. Magnetoencephalography studies demonstrate a correlation between gamma band activity in the contralateral auditory cortex and the presence of tinnitus. The present study aims to investigate the relation between objective gamma-band activity in the contralateral auditory cortex and subjective tinnitus loudness scores. Methods and Findings In unilateral tinnitus patients (N = 15; 10 right, 5 left) source analysis of resting state electroencephalographic gamma band oscillations shows a strong positive correlation with Visual Analogue Scale loudness scores in the contralateral auditory cortex (max r = 0.73, p<0.05). Conclusion Auditory phantom percepts thus show similar sound level dependent activation of the contralateral auditory cortex as observed in normal audition. In view of recent consciousness models and tinnitus network models these results suggest tinnitus loudness is coded by gamma band activity in the contralateral auditory cortex but might not, by itself, be responsible for tinnitus perception.

Binaural Hearing after Cochlear Implantation in Subjects with Unilateral Sensorineural Deafness and Tinnitus

The aim of this clinical study was to assess speech recognition in noise after cochlear implantation in subjects with single-sided deafness and incapacitating tinnitus. 20 subjects complaining of severe intractable tinnitus unresponsive to treatment received a MED-EL cochlear implant (CI). 11 subjects had normal hearing (NH group) on the contralateral side, while 9 used a hearing aid (HA group). The subjects were tested in noise in two listening conditions, i.e. with their acoustic hearing only and with adding the CI to the acoustic hearing (binaural). Subjective improvement in daily life was evaluated using the Speech Spatial and Qualities Hearing Scale (SSQ). The summation effect (3.3 dB for the HA group and 0.6 dB for the NH group) is not significant in both groups. A significant squelch effect of adding the CI was seen for the HA users (3.8 dB), but not for the NH group (1.2 dB). Additionally, a significant effect of adding the CI was found for the spatial configuration where noise is presented in front and speech on the CI side for both the HA group (6.5 dB) and the NH group (1.7 dB). Results of the SSQ show a significant overall benefit of wearing the CI for both groups. The preliminary results of these 20 subjects suggest that cochlear implantation can improve hearing in people suffering from single-sided deafness combined with tinnitus.

Occupational Noise, Smoking, and a High Body Mass Index are Risk Factors for Age-related Hearing Impairment and Moderate Alcohol Consumption is Protective: A European Population-based Multicenter Study

A multicenter study was set up to elucidate the environmental and medical risk factors contributing to age-related hearing impairment (ARHI). Nine subsamples, collected by nine audiological centers across Europe, added up to a total of 4,083 subjects between 53 and 67 years. Audiometric data (pure-tone average [PTA]) were collected and the participants filled out a questionnaire on environmental risk factors and medical history. People with a history of disease that could affect hearing were excluded. PTAs were adjusted for age and sex and tested for association with exposure to risk factors. Noise exposure was associated with a significant loss of hearing at high sound frequencies (>1 kHz). Smoking significantly increased high-frequency hearing loss, and the effect was dose-dependent. The effect of smoking remained significant when accounting for cardiovascular disease events. Taller people had better hearing on average with a more pronounced effect at low sound frequencies (<2 kHz). A high body mass index (BMI) correlated with hearing loss across the frequency range tested. Moderate alcohol consumption was inversely correlated with hearing loss. Significant associations were found in the high as well as in the low frequencies. The results suggest that a healthy lifestyle can protect against age-related hearing impairment.

Factors Affecting Auditory Performance of Postlinguistically Deaf Adults Using Cochlear Implants: An Update with 2251 Patients

Objective: To update a 15-year-old study of 800 postlinguistically deaf adult patients showing how duration of severe to profound hearing loss, age at cochlear implantation (CI), age at onset of severe to profound hearing loss, etiology and CI experience affected CI outcome. Study Design: Retrospective multicenter study. Methods: Data from 2251 adult patients implanted since 2003 in 15 international centers were collected and speech scores in quiet were converted to percentile ranks to remove differences between centers. Results: The negative effect of long duration of severe to profound hearing loss was less important in the new data than in 1996; the effects of age at CI and age at onset of severe to profound hearing loss were delayed until older ages; etiology had a smaller effect, and the effect of CI experience was greater with a steeper learning curve. Patients with longer durations of severe to profound hearing loss were less likely to improve with CI experience than patients with shorter duration of severe to profound hearing loss. Conclusions: The factors that were relevant in 1996 were still relevant in 2011, although their relative importance had changed. Relaxed patient selection criteria, improved clinical management of hearing loss, modifications of surgical practice, and improved devices may explain the differences.