Subjective Sound Quality Detection (HISQUI) over Time after Vibrant Soundbridge Implantation

Background: To evaluate the long-term audiological outcomes combined with the Hearing Implant Sound Quality Index (HISQUI) after Vibrant Soundbridge (VSB) implantation. Methods: Prospective recall cohort study of patients who received a VSB in a tertiary academic medical center between 1996 and 2017. Air conduction (AC) and bone conduction (BC), sound field thresholds in aided and unaided conditions, and speech discrimination in noise (Oldenburger sentence test) were measured. Postoperative results were compared with preoperative audiograms. Furthermore, the HISQUI was evaluated. Results: Ten patients (eleven implants) were included, the mean follow up period was nine years. The mean AC threshold preoperatively was between 63 and 70 dB, and the BC was between 38 and 49 dB from 500 to 4000 Hz. In the free-field audiogram, the mean threshold was between 61 and 77 dB unaided vs. between 28 and 52 dB in the aided condition. The average signal to noise ratio (SNR) in the Oldenburger sentence test in the unaided condition was 10 dB ± 6.7 dB vs. 2 dB ± 5.4 dB in the aided condition. Three patients reported a good to very good hearing result, four patients a moderate, and three patients a poor hearing result. There was a significant association between the years of implantation and the HISQUI (p = 0.013), as well as a significant decrease by 14 HISQUI points per 10 dB SPL decline (SE 5.2, p = 0.023). There was a significant difference between the change of BC over the years and the HISQUI, as well as the number of years after implantation and the HISQUI. On average, per dB decrease in BC, the HISQUI decreases by 1.4 points, and every year after implantation the HISQUI decreases by 2.7 points. Conclusions: The aided threshold in free field and speech understanding in noise improved significantly with VSB. An increase over time of BC thresholds was observed as well as a decrease in HISQUI score. This decrease in BC thresholds over time may be due to presbycusis. Therefore, monitoring of these patients over time should be considered to discuss alternative hearing rehabilitation measures in a timely manner

Age Dependent Cost-Effectiveness of Cochlear Implantation in Adults: Is There an Age Related Cut-off?

Objective: To analyze the impact of age at implantation on the cost-effectiveness of cochlear implantation (CI). Study Design: Cost-utility analysis in an adapted Markov model. Setting: Adults with profound postlingual hearing loss in a “high income” country. Intervention: Unilateral and sequential CI were compared with hearing aids (HA). Main Outcome Measure: Incremental cost-effectiveness ratio (ICER), calculated as costs per quality adjusted life year (QALY) gained (in CHF/QALY), for individual age and sex combinations in relation to two different willingness to pay thresholds. 1 CHF (Swiss franc) is equivalent to 1.01 USD. Results: When a threshold of 50,000 CHF per QALY is applied, unilateral CI in comparison to HA is cost-effective up to an age of 91 for women and 89 for men. Sequential CI in comparison to HA is cost-effective up to an age of 87 for women and 85 for men. If a more contemporary threshold of 100,000 CHF per QALY is applied, sequential CI in comparison to unilateral CI is cost-effective up to an age of 80 for women and 78 for men. Conclusions: Performing both sequential and unilateral CI is cost-effective up to very advanced ages when compared with hearing aids

Changes of Electrocochleographic Responses During Cochlear Implantation Presented at the Annual Meeting of ADANO 2016 in Berlin

OBJECTIVE: To assess by electrocochleography (ECoG) at which times during cochlear implantation changes of cochlear function occur. METHODS: Tone bursts with a frequency of 500 or 750 Hz were used as acoustic stimuli. The recording electrode was placed on the promontory and left in an unchanged position for all ECoG recordings. RESULTS: Eight subjects were included. After opening the cochlea, an increase of the amplitude of the ECoG signal was detectable in four subjects (mean change 3.9 dB, range from 0.2 to 10.8 dB). No decreases were detectable after opening the cochlea or during the first half of the insertion of the CI electrode array (mean change 0.5 dB, range from -2.2 to 1.6 dB). During the second half of the insertion, the amplitude of the ECoG signal decreased in four subjects (mean change -2.5 dB, range from -0.04 to -4.8 dB). If a decrease occurred during the second half of the insertion, then the decrease continued in the earliest phase after insertion of the CI electrode array (mean change -2.1 dB, range from -0.5 to -5.8 dB). CONCLUSION: Pressure changes inside the cochlea can lead to an increase of ECoG signals after opening the cochlea. If detectable, then a decrease of ECoG signals occurs during the second half of the insertion of the CI electrode array and continues in the earliest phase after completed insertion. These findings suggest that cochlear trauma occurs toward the end of the insertion and that trauma-dependent postoperative mechanisms contribute to postoperative hearing loss

CRIM-negative infantile Pompe disease: 42-month treatment outcome

Pompe disease is a rare lysosomal glycogen storage disorder characterized by deficiency of acid α-glucosidase enzyme (GAA) and caused by mutations in the GAA gene. Infantile-type Pompe disease is a multiorgan disorder presenting with cardiomyopathy, hypotonia, and muscular weakness, which is usually fatal. Enzyme replacement therapy (ERT) with recombinant human GAA (rhGAA) has recently been shown to be effective and subsequently yielded promising results in cross-reactive immunologic material (CRIM)-positive patients. CRIM-negative patients showed a limited response to ERT and died or were ventilator dependant. Over a period of 44months, we monitored cognitive and motor development, behavior, auditory function, and brain imaging of a CRIM-negative infantile Pompe disease patient on rhGAA and monoclonal anti-immunoglobulin E (anti-IgE) antibody (omalizumab) treatment due to severe allergic reaction. Cardiorespiratory and skeletal muscle response was significant, with almost normal motor development. Cognitive development—in particular, speech and language—deviated increasingly from normal age-appropriate development and was markedly delayed at 44months, unexplained by moderate sensorineural hearing impairment. Brain magnetic resonance imaging (MRI) at 18, 30, and 44months of age revealed symmetrical signal alteration of the deep white matter. Titer values of IgG antibodies to rhGAA always remained <1:800. The potential role of omalizumab in immune modulation remains to be elucidated; however, this is the first report presenting a ventilator-free survival of a CRIM-negative patient beyond the age of 36months. The central nervous system (CNS) findings are hypothesized to be part of a yet not fully described CNS phenotype in treated patients with longer surviva

Hearing Loss in Cancer Patients with Skull Base Tumors Undergoing Pencil Beam Scanning Proton Therapy: A Retrospective Cohort Study.

To assess the incidence and severity of changes in hearing threshold in patients undergoing high-dose pencil-beam-scanning proton therapy (PBS-PT). This retrospective cohort study included fifty-one patients (median 50 years (range, 13-68)) treated with PBS-PT for skull base tumors. No chemotherapy was delivered. Pure tone averages (PTAs)were determined before (baseline) and after PBS-PT as the average hearing thresholds at frequencies of 0.5, 1, 2, and 4 kHz. Hearing changes were calculated as PTA differences between pre-and post-PBS-PT. A linear mixed-effects model was used to assess the relationship between the PTA at the follow-up and the baseline, the cochlea radiation dose intensity, the increased age, and the years after PBS-PT. Included patients were treated for chordoma (n = 24), chondrosarcoma (n = 9), head and neck tumors (n = 9), or meningioma (n = 3), with a mean tumor dose of 71.1 Gy (RBE) (range, 52.0-77.8), and a mean dose of 37 Gy (RBE) (range, 0.0-72.7) was delivered to the cochleas. The median time to the first follow-up was 11 months (IQR, 5.5-33.7). The PTA increased from a median of 15 dB (IQR 10.0-25) at the baseline to 23.8 (IQR 11.3-46.3) at the first follow-up. In the linear mixed-effect model, the baseline PTA (estimate 0.80, 95%CI 0.64 to 0.96, p ≤ 0.001), patient's age (0.30, 0.03 to 0.57, p = 0.029), follow-up time (2.07, 0.92 to 3.23, p ≤ 0.001), and mean cochlear dose in Gy (RBE) (0.34, 0.21 to 0.46, p ≤ 0.001) were all significantly associated with an increase in PTA at follow-up. The applied cochlear dose and baseline PTA, age, and time after treatment were significantly associated with hearing loss after proton therapy

Hearing Loss in Cancer Patients with Skull Base Tumors Undergoing Pencil Beam Scanning Proton Therapy: A Retrospective Cohort Study

To assess the incidence and severity of changes in hearing threshold in patients undergoing high-dose pencil-beam-scanning proton therapy (PBS-PT). This retrospective cohort study included fifty-one patients (median 50 years (range, 13–68)) treated with PBS-PT for skull base tumors. No chemotherapy was delivered. Pure tone averages (PTAs)were determined before (baseline) and after PBS-PT as the average hearing thresholds at frequencies of 0.5, 1, 2, and 4 kHz. Hearing changes were calculated as PTA differences between pre-and post-PBS-PT. A linear mixed-effects model was used to assess the relationship between the PTA at the follow-up and the baseline, the cochlea radiation dose intensity, the increased age, and the years after PBS-PT. Included patients were treated for chordoma (n = 24), chondrosarcoma (n = 9), head and neck tumors (n = 9), or meningioma (n = 3), with a mean tumor dose of 71.1 Gy (RBE) (range, 52.0–77.8), and a mean dose of 37 Gy (RBE) (range, 0.0–72.7) was delivered to the cochleas. The median time to the first follow-up was 11 months (IQR, 5.5–33.7). The PTA increased from a median of 15 dB (IQR 10.0–25) at the baseline to 23.8 (IQR 11.3–46.3) at the first follow-up. In the linear mixed-effect model, the baseline PTA (estimate 0.80, 95%CI 0.64 to 0.96, p ≤ 0.001), patient’s age (0.30, 0.03 to 0.57, p = 0.029), follow-up time (2.07, 0.92 to 3.23, p ≤ 0.001), and mean cochlear dose in Gy (RBE) (0.34, 0.21 to 0.46, p ≤ 0.001) were all significantly associated with an increase in PTA at follow-up. The applied cochlear dose and baseline PTA, age, and time after treatment were significantly associated with hearing loss after proton therap

Dynamic Postural Stability and Hearing Preservation after Cochlear Implantation

OBJECTIVES (1) To assess dynamic postural stability before and after cochlear implantation using a functional gait assessment (FGA). (2) To evaluate the correlation between loss of residual hearing and changes in dynamic postural stability after cochlear implantation. METHODS Candidates for first-sided cochlear implantation were prospectively included. The FGAs and pure-tone audiograms were performed before and 4-6 weeks after cochlear implantation. RESULTS Twenty-three subjects were included. Forty-eight percent (n = 11) showed FGA performance below the age-referenced norm before surgery. One subject had a clinically relevant decrease of the FGA score after cochlear implantation. No significant difference between the mean pre- and postoperative FGA scores was detectable (p = 0.4). Postoperative hearing loss showed no correlation with a change in FGA score after surgery (r = 0.3, p = 0.3, n = 16). CONCLUSION Single-sided cochlear implantation does not adversely affect dynamic postural stability 5 weeks after surgery. Loss of functional residual hearing is not correlated with a decrease in dynamic postural stability

Der „Professional Ear User“ – Implikationen für die Prävention, Diagnostik und Therapie von Ohrerkrankungen

Zusammenfassung Hintergrund Ein vollständig intaktes Hörvermögen ist zentral für die Ausübung verschiedener Berufe wie Instrumentenbaumeister, Musiker, Tonmeister sowie für weitere Berufsgruppen ohne Bezug zu Musik wie beispielsweise Sonar-Techniker. Für Personen all dieser Berufsgruppen schlagen wir in Analogie zum „Professional Voice User“ den Begriff „Professional Ear User“ (PEU) vor. PEU haben spezielle Anforderungen an ihre Ohrgesundheit, da sie über eine überdurchschnittliche auditive Wahrnehmungsfähigkeit verfügen, von der sie beruflich abhängig sind. Fragestellung Die vorliegende narrative Übersichtsarbeit hat zum Ziel, die sich daraus ergebenden speziellen Aspekte der Prävention, Diagnostik und Therapie von Ohrerkrankungen bei PEU zusammenzufassen. Ergebnisse und Schlussfolgerung Die Prävention von Hörstörungen und weiteren Ohrerkrankungen umfasst den Schutz vor zu hohen Schallpegeln, die Vermeidung von Ototoxinen oder Nikotin sowie die korrekte Durchführung einer Gehörgangsreinigung. Die Abklärung von Hörstörungen kann sich bei PEU herausfordernd gestalten, da subklinische, jedoch einschränkende Veränderungen des Hörvermögens mit konventionellen audiometrischen Methoden nicht zuverlässig objektiviert werden können. Schließlich kann das Vorliegen einer Ohrerkrankung bei einem PEU Therapieentscheidungen beeinflussen. Weiter muss bei PEU auch eine hohe Wachsamkeit bezüglich nichtorganischer Ohrerkrankungen bestehen. Abschließend werden Möglichkeiten diskutiert, um bei PEU eine umfassende Ohrgesundheit im Rahmen eines edukativen Programms zu fördern und mittels einer spezialisierten ohrenärztlichen Sprechstunde zu erhalten. Im Gegensatz zu bestehenden Konzepten ist der Fokus dabei auf die Gesamtheit der Berufsgruppen gerichtet, welche in professionellem Rahmen speziell von der Ohrgesundheit abhängig sind. Außerdem soll der Schwerpunkt hierbei nicht nur auf Hörstörungen und deren Prävention, sondern auch auf der Erhaltung einer ganzheitlichen Ohrgesundheit liegen. Abstract Background Perfect hearing is crucial to the practice of various professions, such as instrument makers, musicians, sound engineers, and other professions not related to music, such as sonar technicians. For people of these occupational groups, we propose the term “professional ear user” (PEU) in analogy to “professional voice user”. PEUs have special requirements for their hearing health, as they have well-known above-average auditory perceptual abilities on which they are professionally dependent. Objective The purpose of this narrative review is to summarize selected aspects of the prevention, diagnosis, and treatment of ear disorders in PEUs. Results and conclusion Prevention of hearing disorders and other ear diseases includes protection from excessive sound levels, avoidance of ototoxins and nicotine, and a safe manner of cleaning the outer auditory canal. Diagnosing hearing disorders in PEUs can be challenging, since subclinical but relevant changes in hearing cannot be reliably objectified by conventional audiometric methods. Moreover, the fact that a PEU is affected by an ear disease may influence treatment decisions. Further, physicians must be vigilant for non-organic ear diseases in PEUs. Lastly, measures to promote comprehensive ear health in PEUs as part of an educational program and to maintain ear health by means of a specialized otolaryngology service are discussed. In contrast to existing concepts, we lay the attention on the entirety of occupational groups that are specifically dependent on their ear health in a professional setting. In this context, we suggest avoiding a sole focus on hearing disorders and their prevention, but rather encourage the maintenance of a comprehensive ear health

Der „Professional Ear User“ – Implikationen für die Prävention, Diagnostik und Therapie von Ohrerkrankungen

BACKGROUND Perfect hearing is crucial to the practice of various professions, such as instrument makers, musicians, sound engineers, and other professions not related to music, such as sonar technicians. For people of these occupational groups, we propose the term "professional ear user" (PEU) in analogy to "professional voice user". PEUs have special requirements for their hearing health, as they have well-known above-average auditory perceptual abilities on which they are professionally dependent. OBJECTIVE The purpose of this narrative review is to summarize selected aspects of the prevention, diagnosis, and treatment of ear disorders in PEUs. RESULTS AND CONCLUSION Prevention of hearing disorders and other ear diseases includes protection from excessive sound levels, avoidance of ototoxins and nicotine, and a safe manner of cleaning the outer auditory canal. Diagnosing hearing disorders in PEUs can be challenging, since subclinical but relevant changes in hearing cannot be reliably objectified by conventional audiometric methods. Moreover, the fact that a PEU is affected by an ear disease may influence treatment decisions. Further, physicians must be vigilant for non-organic ear diseases in PEUs. Lastly, measures to promote comprehensive ear health in PEUs as part of an educational program and to maintain ear health by means of a specialized otolaryngology service are discussed. In contrast to existing concepts, we lay the attention on the entirety of occupational groups that are specifically dependent on their ear health in a professional setting. In this context, we suggest avoiding a sole focus on hearing disorders and their prevention, but rather encourage the maintenance of a comprehensive ear health.Hintergrund Ein vollständig intaktes Hörvermögen ist zentral für die Ausübung verschiedener Berufe wie Instrumentenbaumeister, Musiker, Tonmeister sowie für weitere Berufsgruppen ohne Bezug zu Musik wie beispielsweise Sonar-Techniker. Für Personen all dieser Berufsgruppen schlagen wir in Analogie zum „Professional Voice User“ den Begriff „Professional Ear User“ (PEU) vor. PEU haben spezielle Anforderungen an ihre Ohrgesundheit, da sie über eine überdurchschnittliche auditive Wahrnehmungsfähigkeit verfügen, von der sie beruflich abhängig sind. Fragestellung Die vorliegende narrative Übersichtsarbeit hat zum Ziel, die sich daraus ergebenden speziellen Aspekte der Prävention, Diagnostik und Therapie von Ohrerkrankungen bei PEU zusammenzufassen. Ergebnisse und Schlussfolgerung Die Prävention von Hörstörungen und weiteren Ohrerkrankungen umfasst den Schutz vor zu hohen Schallpegeln, die Vermeidung von Ototoxinen oder Nikotin sowie die korrekte Durchführung einer Gehörgangsreinigung. Die Abklärung von Hörstörungen kann sich bei PEU herausfordernd gestalten, da subklinische, jedoch einschränkende Veränderungen des Hörvermögens mit konventionellen audiometrischen Methoden nicht zuverlässig objektiviert werden können. Schließlich kann das Vorliegen einer Ohrerkrankung bei einem PEU Therapieentscheidungen beeinflussen. Weiter muss bei PEU auch eine hohe Wachsamkeit bezüglich nichtorganischer Ohrerkrankungen bestehen. Abschließend werden Möglichkeiten diskutiert, um bei PEU eine umfassende Ohrgesundheit im Rahmen eines edukativen Programms zu fördern und mittels einer spezialisierten ohrenärztlichen Sprechstunde zu erhalten. Im Gegensatz zu bestehenden Konzepten ist der Fokus dabei auf die Gesamtheit der Berufsgruppen gerichtet, welche in professionellem Rahmen speziell von der Ohrgesundheit abhängig sind. Außerdem soll der Schwerpunkt hierbei nicht nur auf Hörstörungen und deren Prävention, sondern auch auf der Erhaltung einer ganzheitlichen Ohrgesundheit liegen

Radiological feature heterogeneity supports etiological diversity among patient groups in Meniere's disease

We aimed to determine the prevalence of radiological temporal bone features that in previous studies showed only a weak or an inconsistent association with the clinical diagnosis of Meniere's disease (MD), in two groups of MD patients (n = 71) with previously established distinct endolymphatic sac pathologies; i.e. the group MD-dg (ES degeneration) and the group MD-hp (ES hypoplasia). Delayed gadolinium-enhanced MRI and high-resolution CT data were used to determine and compare between and within (affected vs. non-affected side) groups geometric temporal bone features (lengths, widths, contours), air cell tract volume, height of the jugular bulb, sigmoid sinus width, and MRI signal intensity alterations of the ES. Temporal bone features with significant intergroup differences were the retrolabyrinthine bone thickness (1.04 ± 0.69 mm, MD-hp; 3.1 ± 1.9 mm, MD-dg; p < 0.0001); posterior contour tortuosity (mean arch-to-chord ratio 1.019 ± 0.013, MD-hp; 1.096 ± 0.038, MD-dg; p < 0.0001); and the pneumatized volume (1.37 [0.86] cm3, MD-hp; 5.25 [3.45] cm3, MD-dg; p = 0.03). Features with differences between the affected and non-affected sides within the MD-dg group were the sigmoid sinus width (6.5 ± 1.7 mm, affected; 7.6 ± 2.1 mm, non-affected; p = 0.04) and the MRI signal intensity of the endolymphatic sac (median signal intensity, affected vs. unaffected side, 0.59 [IQR 0.31-0.89]). Radiological temporal bone features known to be only weakly or inconsistently associated with the clinical diagnosis MD, are highly prevalent in either of two MD patient groups. These results support the existence of diverse-developmental and degenerative-disease etiologies manifesting with distinct radiological temporal bone abnormalities