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1.
Am J Otolaryngol ; 45(5): 104360, 2024 Apr 29.
Artículo en Inglés | MEDLINE | ID: mdl-38754261

RESUMEN

INTRODUCTION: Robot-assisted cochlear implant surgery (RACIS) as defined by the HEARO®-procedure performs minimal invasive cochlear implant (CI) surgery by directly drilling a keyhole trajectory towards the inner ear. Hitherto, an entirely robotic automation including electrode insertion has not been described yet. The feasability of using a newly developed, dedicated motorised device for automated electrode insertion in the first clinical case of entirely robotic cochlear implant surgery was investigated. AIM: The aim is to report the first experience of entirely robotic cochlear implantation surgery. INTERVENTION: RACIS with a straight flexible lateral wall electrode. PRIMARY OUTCOME MEASUREMENTS: Electrode cochlear insertion depth. SECONDARY OUTCOME MEASUREMENTS: The audiological outcome in terms of mean hearing thresholds. CONCLUSION: Here, we report on a cochlear implant robot that performs the most complex surgical steps to place a cochlear implant array successfully in the inner ear and render similar audiological results as in conventional surgery. Robots can execute tasks beyond human dexterity and will probably pave the way to standardize residual hearing preservation and broadening the indication for electric-acoustic stimulation in the same ear with hybrid implants.

2.
Eur Arch Otorhinolaryngol ; 281(5): 2313-2325, 2024 May.
Artículo en Inglés | MEDLINE | ID: mdl-38180606

RESUMEN

OBJECTIVE: To investigate the hypothesis that day-case cochlear implantation is associated with equal quality of life, hearing benefits and complications rates, compared to inpatient cochlear implantation. STUDY DESIGN: A single-center, non-blinded, randomized controlled, equivalence trial in a tertiary referral center. METHODS: Thirty adult patients with post-lingual bilateral sensorineural hearing loss eligible for unilateral cochlear implantation surgery were randomly assigned to either the day-case or inpatient treatment group. The effect on general quality of life, patient satisfaction, (subjective) hearing improvement, postoperative complications and causes of crossover and/or readmission were assessed using questionnaires, auditory evaluations and patients' charts over a follow-up period of 1 year. RESULTS: Overall quality of life measured by the HUI3 was equal between the day-case (n = 14) and inpatient group (n = 14). The overall patients' satisfaction showed a slight favor towards an inpatient approach. There was no significant difference in the subjective and objective hearing improvement between both treatment groups. During the 1-year follow-up period no major complications occurred. Minor complications occurred intraoperatively in three day-case patients resulting in three out of nine admissions of day-case patients. Other causes of admission of day-case patients were nausea and vomiting (n = 1), drowsiness (n = 1), late scheduled surgery (n = 2), social reasons (n = 1), or due to an unclear reason (n = 1). No patients required readmission. CONCLUSION: We found equal outcomes of QoL, patient satisfaction, objective, and subjective hearing outcomes between day-case and inpatient unilateral cochlear implantation. Nine out of 14 day-case patients were admitted for at least one night postoperatively (crossover). No major complications occurred in both groups. A day-case approach seems feasible when using specific patient selection, surgical planning and the preoperative provision of patient information into account. Besides this, the familiarity with a day-case approach of both patient and the surgical team can increase the feasibility of day-case surgery.


Asunto(s)
Implantación Coclear , Implantes Cocleares , Pérdida Auditiva Sensorineural , Percepción del Habla , Adulto , Humanos , Implantación Coclear/efectos adversos , Implantación Coclear/métodos , Satisfacción del Paciente , Calidad de Vida , Pacientes Internos , Implantes Cocleares/efectos adversos , Resultado del Tratamiento , Audición , Pérdida Auditiva Sensorineural/cirugía , Complicaciones Posoperatorias/epidemiología , Complicaciones Posoperatorias/cirugía
3.
Am J Otolaryngol ; 44(4): 103926, 2023.
Artículo en Inglés | MEDLINE | ID: mdl-37229977

RESUMEN

INTRODUCTION: Minimal invasive Robotic Assisted Cochlear Implant Surgery (RACIS) is a keyhole surgery by definition. It is therefore not possible to visualize the electrode array during insertion in the scala tympani. Hitherto, surgeons visualised the round window via the external auditory canal by folding over the tympanic membrane. However, the opening of a tympanomeatal flap is not minimal invasive and is especially in conventional cochlear implantation surgery not even necessary. Here we prove that image guided and robot assisted surgery can also allow correct electrode array insertion without opening the tympanomeatal flap. AIM: The aim is to report the first experience of robotic cochlear implantation surgery fully based on image guided surgery and without the opening of a tympanomeatal flap for electrode array insertion. INTERVENTION: RACIS with a straight flexible lateral wall electrode. PRIMARY OUTCOME MEASUREMENTS: Electrode cochlear insertion depth with RACIS and autonomous inner ear access with full electrode insertion of a flexible lateral wall electrode array. SECONDARY OUTCOME MEASUREMENTS: The audiological outcome in terms of mean hearing thresholds. CONCLUSION: After a series of 33 cases and after fine-tuning the insertion angles and yet another new version of planning software to depict the round window approach, a new clinical routine for inserting electrodes fully based on image guided surgery without opening a tympanomeatal flap was developed in robotic-assisted cochlear implant surgery.


Asunto(s)
Implantación Coclear , Implantes Cocleares , Humanos , Implantación Coclear/métodos , Cóclea/cirugía , Ventana Redonda/cirugía , Audición , Electrodos Implantados
4.
Am J Otolaryngol ; 44(1): 103668, 2023.
Artículo en Inglés | MEDLINE | ID: mdl-36323158

RESUMEN

AIM: To report the experience of an image-guided and navigation-based robot arm as an assistive surgical tool for cochlear implantation in a case with a labyrinthitis ossificans. PATIENT: A 55-years-old man with a history of childhood meningitis whose hearing deteriorated progressively to bilateral profound sensorineural hearing loss. INTERVENTION: Robotic Assisted Cochlear Implant Surgery (RACIS) with a straight flexible lateral wall electrode. PRIMARY OUTCOME MEASURES: Electrode cochlear insertion depth with RACIS with facial recess approach and autonomous inner ear access with full electrode insertion of a flexible straight cochlear implant array. CONCLUSIONS: Intra cochlear ossifications pose a challenge for entering the cochlea and full-length insertion of a cochlear implant. RACIS has shown that computations of radiological images combined with navigation-assisted robot arm drilling can provide efficient access to the inner ear.


Asunto(s)
Implantación Coclear , Implantes Cocleares , Pérdida Auditiva Sensorineural , Meningitis , Procedimientos Quirúrgicos Robotizados , Robótica , Masculino , Humanos , Persona de Mediana Edad , Implantación Coclear/métodos , Osteogénesis , Cóclea/diagnóstico por imagen , Cóclea/cirugía , Pérdida Auditiva Sensorineural/etiología , Pérdida Auditiva Sensorineural/cirugía , Pérdida Auditiva Bilateral/cirugía , Meningitis/cirugía
5.
Eur Arch Otorhinolaryngol ; 280(10): 4433-4444, 2023 Oct.
Artículo en Inglés | MEDLINE | ID: mdl-37043021

RESUMEN

PURPOSE: The main objective of this study is to evaluate the short-term and long-term audiological outcomes in patients who underwent cochlear implantation with a robot-assisted system to enable access to the cochlea, and to compare outcomes with a matched control group of patients who underwent cochlear implantation with conventional access to the cochlea. METHODS: In total, 23 patients were implanted by robot-assisted cochlear implant surgery (RACIS). To evaluate the effectiveness of robotic surgery in terms of audiological outcomes, a statistically balanced control group of conventionally implanted patients was created. Minimal outcome measures (MOM), consisting of pure-tone audiometry, speech understanding in quiet and speech understanding in noise were performed pre-operatively and at 3 months, 6 months, 12 months and 2 years post-activation of the audioprocessor. RESULTS: There was no statistically significant difference in pure-tone audiometry, speech perception in quiet and speech perception in noise between robotically implanted and conventionally implanted patients pre-operatively, 3 months, 6 months, 12 months and 2 years post-activation. A significant improvement in pure-tone hearing thresholds, speech understanding in quiet and speech understanding in noise with the cochlear implant has been quantified as of the first measurements at 3 months and this significant improvement remained stable over a time period of 2 years for HEARO implanted patients. CONCLUSION: Clinical outcomes in robot-assisted cochlear implant surgery are comparable to conventional cochlear implantation. CLINICALTRAILS. GOV TRAIL REGISTRATION NUMBERS: NCT03746613 (date of registration: 19/11/2018), NCT04102215 (date of registration: 25/09/2019).


Asunto(s)
Implantación Coclear , Implantes Cocleares , Pérdida Auditiva Sensorineural , Procedimientos Quirúrgicos Robotizados , Robótica , Humanos , Pérdida Auditiva Sensorineural/cirugía , Pérdida Auditiva Sensorineural/rehabilitación
6.
J Craniofac Surg ; 34(3): 1085-1088, 2023 May 01.
Artículo en Inglés | MEDLINE | ID: mdl-36217219

RESUMEN

Fossa navicularis (FN) is defined as bony depression that is not always present and is located anterior to the foramen magnum and pharyngeal tubercle on the inferior aspect of the basilar part of the occipital bone. It has been reported that FN can create an infection spread path from the pharynx to the intracranial structures. Therefore, the diagnosis of this variation is important. Although cone beam computed tomography (CBCT) diagnostic accuracy has mostly been verified in detection and quantification particularly on human skulls, there is no study comparing morphometric measurements between direct measurement on the skull and CBCT measurement. The main object of this study is to evaluate the presence of FN on dry bones and CBCT images of the same dry skull and to examine the morphometric and morphological features of this formation. Thirty-two random craniums that were made available for this study that did not have any fractures or deformities of the cranial base were examined. The sagittal diameter, transverse diameter, and depth of the FN was measured both directly on dry skulls and radiologically on CBCT images of dry skull. In addition, the shape of FN (SFN) was determined. FN was detected in 10 (31.25%) of 32 craniums examined with both modalities. It was determined that sagittal diameter of the FN, transverse diameter of the FN, depth of the FN, and the shape of FN did not show a statistically significant difference between the 2 measurements. Unlike the literature, FN was investigated on dry bones both directly and in CBCT images in this study. In contrast to previously thought the FN may be smaller according to this findings, and this small variation can be detected with CBCT images. According to this findings, it can be said that morphometric evaluations on CBCT are accurate and reliable, and CBCT is a safe method for clinical diagnosis and treatment.


Asunto(s)
Tomografía Computarizada de Haz Cónico Espiral , Humanos , Cráneo/diagnóstico por imagen , Cráneo/anatomía & histología , Base del Cráneo/anatomía & histología , Hueso Occipital/anatomía & histología , Foramen Magno/diagnóstico por imagen , Foramen Magno/anatomía & histología , Tomografía Computarizada de Haz Cónico
7.
Hum Genet ; 141(3-4): 951-963, 2022 Apr.
Artículo en Inglés | MEDLINE | ID: mdl-34410490

RESUMEN

In this study, we investigated the association of ACAN variants with otosclerosis, a frequent cause of hearing loss among young adults. We sequenced the coding, 5'-UTR and 3'-UTR regions of ACAN in 1497 unrelated otosclerosis cases and 1437 matched controls from six different subpopulations. The association between variants in ACAN and the disease risk was tested through single variant and gene-based association tests. After correction for multiple testing, 14 variants were significantly associated with otosclerosis, ten of which represented independent association signals. Eight variants showed a consistent association across all subpopulations. Allelic odds ratios of the variants identified four predisposing and ten protective variants. Gene-based tests showed an association of very rare variants in the 3'-UTR with the phenotype. The associated exonic variants are all located in the CS domain of ACAN and include both protective and predisposing variants with a broad spectrum of effect sizes and population frequencies. This includes variants with strong effect size and low frequency, typical for monogenic diseases, to low effect size variants with high frequency, characteristic for common complex traits. This single-gene allelic spectrum with both protective and predisposing alleles is unique in the field of complex diseases. In conclusion, these findings are a significant advancement to the understanding of the etiology of otosclerosis.


Asunto(s)
Otosclerosis , Regiones no Traducidas 3' , Regiones no Traducidas 5' , Agrecanos/genética , Susceptibilidad a Enfermedades , Frecuencia de los Genes , Predisposición Genética a la Enfermedad , Humanos , Otosclerosis/genética , Fenotipo , Polimorfismo de Nucleótido Simple
8.
Eur Arch Otorhinolaryngol ; 279(4): 1875-1883, 2022 Apr.
Artículo en Inglés | MEDLINE | ID: mdl-34131770

RESUMEN

OBJECTIVE: To investigate the effect of frequency-to-place mismatch, i.e. the mismatch between the tonotopic frequency map in the cochlea and the frequency band that is assigned to an electrode contact of a cochlear implant (CI) at the same cochlear location on speech perception outcomes, using postoperative CT images. STUDY DESIGN: Retrospective observational single-centre study. METHODS: Retrospective pre- and postoperative clinical CT data of 39 CI recipients with normal cochlear anatomy were analysed in an otological surgical planning software. The tonotopic frequency at each electrode position was estimated using the Greenwood function. For each patient, frequency-to-place mismatch between the tonotopic frequency and the fitted centre frequency for each electrode contact was calculated. The influence of frequency-to-place mismatch on speech perception in noise at 6 and 12 months after CI activation was studied. RESULTS: A significant linear correlation was found between the frequency-to-place mismatch and speech perception in noise 6 months after cochlear implantation (p < 0.05). The smaller the frequency-to-place mismatch, the better the initial speech perception in noise results of the CI recipients. The significant effect disappeared after 12 months CI experience. CONCLUSION: The study findings support the idea of minimizing the frequency-to-place mismatch in CI recipients in order to pursue better initial speech perception in noise. Further research is needed to investigate the prospect of tonotopic fitting strategies based upon postoperative CT images of the exact locations of the electrode contacts.


Asunto(s)
Implantación Coclear , Implantes Cocleares , Percepción del Habla , Implantación Coclear/métodos , Audición , Humanos , Estudios Retrospectivos , Percepción del Habla/fisiología
9.
Eur Arch Otorhinolaryngol ; 279(2): 739-750, 2022 Feb.
Artículo en Inglés | MEDLINE | ID: mdl-33683447

RESUMEN

PURPOSE: To determine the usefulness of the Health Utilities Index (HUI) in older cochlear implant (CI) recipients, the primary aims were: (1) to assess health-related quality of life (HRQoL), measured with HUI, in older CI candidates while comparing with age- and gender-matched normal-hearing controls; (2) to compare HRQoL after CI with the pre-operative situation, using HUI and the Nijmegen cochlear implant questionnaire (NCIQ). The difference between pre- and postoperative speech intelligibility in noise (SPIN) and in quiet (SPIQ) and the influence of pre-operative vestibular function on HRQoL in CI users were also studied. METHODS: Twenty CI users aged 55 years and older with bilateral severe-to-profound postlingual sensorineural hearing loss and an age- and gender-matched normal-hearing control group were included. HRQoL was assessed with HUI Mark 2 (HUI2), HUI Mark 3 (HUI3) and NCIQ. The CI recipients were evaluated pre-operatively and 12 months postoperatively. RESULTS: HUI3 Hearing (p = 0.02), SPIQ (p < 0.001), SPIN (p < 0.001) and NCIQ (p = 0.001) scores improved significantly comparing pre- and postoperative measurements in the CI group. No significant improvement was found comparing pre- and postoperative HUI3 Multi-Attribute scores (p = 0.07). The HUI3 Multi-Attribute score after CI remained significantly worse (p < 0.001) than those of the control group. Vestibular loss was significantly related to a decrease in HUI3 Multi-Attribute (p = 0.037) and HUI3 Emotion (p = 0.021) scores. CONCLUSION: The HUI is suitable to detect differences between normal-hearing controls and CI users, but might underestimate HRQoL changes after CI in CI users over 55.


Asunto(s)
Implantación Coclear , Implantes Cocleares , Percepción del Habla , Anciano , Audición , Humanos , Calidad de Vida , Encuestas y Cuestionarios
10.
J Craniofac Surg ; 33(2): e130-e133, 2022.
Artículo en Inglés | MEDLINE | ID: mdl-34320590

RESUMEN

ABSTRACT: In Somalia, which is located in the horn of Africa, a fragile and insecure state structure allowed the strengthening of terrorist groups provoking armed conflicts. Stray bullet injuries can be defined as an accidental bullet wound caused by an anonymous attacker and are usually associated with celebratory gunfire or urban violence. The anatomy of the pterygopalatine fossa (PPF) is complex and penetrating foreign body injuries pose even a greater challenge for the surgeon to operate in this area. Endoscopic approaches facilitate the removal of foreign bodies from the paranasal sinuses, orbital cavity, and aerodigestive system, minimizing potential risks. This study presents a series of removal of stray bullets found in the PPF, as a result of urban violence in Somalia in a period of 6 months. Patient demographics, foreign body origin, treatment modalities, and surgery details were evaluated and assessed. All patients were male and aged 16, 2, and 24 years, respectively. The surgeries were quite straightforward with surgery times recorded as 25, 44, and 22 minutes, respectively. The endoscopic endonasal approach proved to provide safe and sufficient access for removal. Surprisingly, even the foreign body in the PPF of a 2-year-old patient could be removed with an endoscopic endonasal approach and did not require an external approach. The management of foreign body removal in the PPF is challenging due to the potential risks of iatrogenic vascular and nervous tissue injury. The endoscopic endonasal approach for removal proved efficient in 3 cases regardless of age and anatomical dimensions.


Asunto(s)
Cuerpos Extraños , Cavidad Nasal , Adolescente , Preescolar , Endoscopía/métodos , Cuerpos Extraños/cirugía , Humanos , Masculino , Cavidad Nasal/cirugía , Fosa Pterigopalatina/cirugía , Somalia , Adulto Joven
11.
Audiol Neurootol ; 26(2): 76-84, 2021.
Artículo en Inglés | MEDLINE | ID: mdl-32877897

RESUMEN

BACKGROUND AND OBJECTIVES: A rare type of nonsyndromic autosomal recessive hereditary hearing loss is caused by pathogenic mutations in the TRIOBP gene mostly involving exons 6 and 7. These mutations cause hearing loss originating from dysfunction of sensory inner ear hair cells. Of all the affected siblings, 2 brothers and 1 sister, part of an Afghan family, were referred to our clinic for diagnostic workup and candidacy selection for cochlear implantation (CI). METHODS: Molecular analysis showed a homozygous c.1342C > T p. (Arg448*) pathogenic variant in exon 7 of the TRIOBP gene (reference sequence NM_001039141.2) in all 3 affected siblings. Clinical audiometry demonstrated profound sensorineural hearing loss in all 3 affected siblings (2 males and 1 female), and they were implanted unilaterally. RESULTS: One month after activation, the pure-tone averages with the CI processor were between 30 and 23 dBHL. Ten months after the first activation of the implant, open-set speech audiometry test could be performed for the first time in the 2 younger CI recipients (S5 and S9), and they could identify up to a maximum 77% phonemes correctly. The oldest brother (S12) could not yet perform open-set speech audiometry at that moment. CONCLUSIONS: Implant outcomes are better with normal inner ear anatomy in general. The earlier congenital patients are implanted, the better their outcomes. Here, we demonstrate both statements are true in a homozygous c.1342C > T p. (Arg448*) pathogenic variant in the TRIOBP gene in all 3 affected siblings.


Asunto(s)
Implantación Coclear , Implantes Cocleares , Pérdida Auditiva Sensorineural/genética , Proteínas de Microfilamentos/genética , Mutación , Femenino , Pérdida Auditiva Sensorineural/cirugía , Pruebas Auditivas , Humanos , Masculino , Persona de Mediana Edad , Percepción del Habla/fisiología , Resultado del Tratamiento
12.
Audiol Neurootol ; 26(2): 61-75, 2021.
Artículo en Inglés | MEDLINE | ID: mdl-32653882

RESUMEN

Cochlear implants (CI) have increasingly been adopted in older adults with severe to profound sensorineural hearing loss as a result of the growing and aging world population. Consequently, researchers have recently shown great interest in the cost-effectiveness of cochlear implantation and its effect on quality of life (QoL) in older CI users. Therefore, a systematic review and critical evaluation of the available literature on QoL in older adult CI users was performed according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. Studies were selected by searching MEDLINE (PubMed) and the Cochrane Library and by checking the reference lists of relevant articles. Inclusion criteria were as follows: (1) the study sample were adults aged 50 years and older with postlingual onset of bilateral severe to profound hearing loss, (2) all subjects received a multi-electrode CI, and (3) QoL was assessed before and after implantation. Out of 1,093 records, 18 articles were accepted for review. Several studies demonstrated significant positive effects of cochlear implantation on QoL in older adults, but high-level evidence-based medicine is lacking. An improvement of QoL was generally reported when using disease-specific instruments, which are designed to detect treatment-specific changes, whereas the outcomes of generic QoL questionnaires, assessing general health states, were rather ambiguous. However, only generic questionnaires would be able to provide calculations of the cost-effectiveness of CI and comparisons across patient populations, diseases, or interventions. Hence, generic and disease-specific QoL instruments are complementary rather than contradictory. In general, older CI users' QoL was assessed using a variety of methods and instruments, which complicated comparisons between studies. There is a need for a standardized, multidimensional, and comprehensive QoL study protocol including all relevant generic and disease-specific instruments to measure and compare QoL, utility, and/or daily life performance in CI users.


Asunto(s)
Implantación Coclear/psicología , Implantes Cocleares , Pérdida Auditiva Sensorineural/cirugía , Calidad de Vida/psicología , Anciano , Femenino , Pérdida Auditiva Sensorineural/psicología , Humanos , Masculino , Persona de Mediana Edad , Encuestas y Cuestionarios
13.
Ear Hear ; 42(6): 1508-1524, 2021.
Artículo en Inglés | MEDLINE | ID: mdl-34369416

RESUMEN

INTRODUCTION: DFNA9 is characterized by adult-onset progressive sensorineural hearing loss (SNHL) and vestibular impairment. More than 15 years ago, genotype-phenotype correlation studies estimated the initial age of hearing deterioration in the fourth to fifth decade (ranging from 32 to 43 years). However, these analyses were based on relatively limited numbers of mainly symptomatic carriers using markedly different methodologies. The starting point for the hearing deterioration is more correctly determined with larger numbers of carriers and with a more clearly defined starting point of the hearing deterioration. AIM: The aim of this study was to determine milestone ages (start and maximal hearing deterioration, potential eligibility for hearing aids and cochlear implants based on pure-tone average [PTA]) in a large series of p.Pro51Ser COCH variant carriers. The degree of individual interaural asymmetry and the degree of variability (interquartile range) with which the hearing deterioration progresses across ages were also studied, and age-related typical audiograms (ARTA) were constructed. MATERIAL AND METHODS: One hundred eleven Belgian and Dutch p.P51S variant carriers were identified and recruited for audiological investigation. Their hearing thresholds were compared with p50th, p95th, and p97.5th percentile values of presbyacusis (ISO 7029 standards). The onset and degree of hearing deterioration were defined and assessed for each frequency and with three PTAs (PTA0.5-4 [0.5, 1, 2, and 4 kHz]; PTA4-8 [4 and 8 kHz]; and PTA6-8 [6 and 8 kHz]). The milestones ages were derived from nonlinear regression model of hearing thresholds against age, for male and female carriers separately, because of different age-referenced limits. Interaural right-left asymmetry was assessed, and variability of hearing thresholds were calculated using interquartile range. ARTAs were built with both observed data and a prediction model. RESULTS: Hearing dysfunction in p.P51S carriers begins at about 38 years of age (ranging from 28 to 43 years) on average in female and 46 years (ranging from 42 to 49 years) in male carriers (third decade: female, fifth decade: male carriers), depending on the hearing frequency and with differences in deterioration sequence between both genders. These differences, however, were mainly due to more stringent age-referenced limits for men. In contrast, predictions (ARTA) did not show any difference of phenotypic expression between genders. At about 48 to 50 years of age on average, the majority of DFNA9 patients may need conventional hearing aids (PTA ≥ 40 dB HL), whereas this is about 56 to 59 years for cochlear implants (PTA ≥ 70 dB HL). There is a high degree of individual interaural asymmetry and interindividual variability throughout all ages. CONCLUSION: This study demonstrates that the onset of sensorineural hearing deterioration starts in the third decade and probably even earlier. Regardless of differences in estimates, DFNA9 expresses similarly in male and female carriers, but male carriers are much more difficult to identify in early stages of the disease. Comprehensive assessment of the natural course of DFNA9 is of particular interest to predict the age of onset or critical period of most significant function deterioration in individual carriers of the pathogenic variant. This will help to design studies in the search for disease-modifying therapies.


Asunto(s)
Proteínas de la Matriz Extracelular , Pérdida Auditiva Sensorineural , Adulto , Audiometría de Tonos Puros , Estudios Transversales , Proteínas de la Matriz Extracelular/genética , Femenino , Estudios de Asociación Genética , Audición , Humanos , Masculino , Persona de Mediana Edad
14.
Ear Hear ; 42(6): 1525-1543, 2021.
Artículo en Inglés | MEDLINE | ID: mdl-34369417

RESUMEN

INTRODUCTION: DFNA9 is characterized by adult-onset hearing loss and evolution toward bilateral vestibulopathy (BVP). The genotype-phenotype correlation studies were conducted 15 years ago. However, their conclusions were mainly based on symptomatic carriers and the vestibular data exclusively derived from the horizontal (lateral) semicircular canal (SCC). The last decade was marked by the emergence of new clinical diagnostic tools, such as the video head impulse test (vHIT) and vestibular-evoked myogenic evoked potentials (VEMPs), expanding our evaluation to all six SCCs and the otolith organs (saccule and utricule). AIM: The aim of this study was to comprehensively evaluate vestibular function in the largest series presymptomatic as well as symptomatic p.P51S variant carriers, to determine which labyrinthine part shows the first signs of deterioration and which SCC function declines at first and to determine the age at which p.P51S variant carriers develop caloric areflexia on VNG and vHIT vestibulo-ocular reflex (VOR)-gain dysfunction as defined by the Barany Society criteria for BVP. MATERIAL AND METHODS: One hundred eleven p.P51S variant carriers were included. The following vestibular function tests were applied in two different centers: ENG/VNG, vHIT, and VEMPs. The following parameters were analyzed: age (years), hearing loss (pure-tone average of 0.5-4 kHz [PTA0.5-4, dB HL]), sum of maximal peak slow-phase eye velocity obtained with bi-thermal (30°C and 44°C, water irrigation; 25°C and 44°C, air irrigation) caloric test (°/s), vHIT VOR-gain on LSCC, superior SCC and posterior SCC, C-VEMP both numerical (threshold, dB nHL) and categorical (present or absent), and O-VEMP as categorical (present or absent). The age of onset of vestibular dysfunction was determined both with categorical (onset in decades using Box & Whisker plots) and numeric approach (onset in years using regression analysis). The same method was applied for determining the age at which vestibular function declined beyond the limits of BVP, as defined by the Barany Society. RESULTS: With the categorical approach, otolith function was declining first (3rd decade), followed by caloric response (5th decade) and vHIT VOR-gains (5th-6th decade). Estimated age of onset showed that the deterioration began with C-VEMP activity (31 years), followed by caloric responses (water irrigation) (35 years) and ended with vHIT VOR-gains (48-57 years). Hearing deterioration started earlier than vestibular deterioration in female carriers, which is different from earlier reports. BVP was predicted at about 53 years of age on average with VNG caloric gain (water irrigation) and between 47 and 57 years of age for the three SCCs. Loss of C-VEMP response was estimated at about 46 years of age. CONCLUSION: Former hypothesis of vestibular decline preceding hearing deterioration by 9 years was confirmed by the numeric approach, but this was less obvious with the categorical approach. Wide confidence intervals of the regression models may explain deviation of the fits from true relationship. There is a typical vestibular deterioration hierarchy in p.P51S variant carriers. To further refine the present findings, a prospective longitudinal study of the auditory and vestibular phenotype may help to get even better insights in this matter.


Asunto(s)
Vestibulopatía Bilateral , Prueba de Impulso Cefálico , Estudios Transversales , Proteínas de la Matriz Extracelular , Femenino , Estudios de Asociación Genética , Prueba de Impulso Cefálico/métodos , Pérdida Auditiva Sensorineural , Humanos , Estudios Longitudinales , Fenotipo , Estudios Prospectivos , Reflejo Vestibuloocular/fisiología , Agua
15.
Ear Hear ; 42(3): 606-614, 2021.
Artículo en Inglés | MEDLINE | ID: mdl-33055579

RESUMEN

OBJECTIVE: To compare the cognitive evolution of older adults with severe or profound hearing impairment after cochlear implantation with that of a matched group of older adults with severe hearing impairment who do not receive a cochlear implant (CI). DESIGN: In this prospective, longitudinal, controlled, and multicenter study, 24 older CI users were included in the intervention group and 24 adults without a CI in the control group. The control group matched the intervention group in terms of gender, age, formal education, cognitive functioning, and residual hearing. Assessments were made at baseline and 14 months later. Primary outcome measurements included the change in the total score on the Repeatable Battery for the Assessment of Neuropsychological Status for Hearing impaired individuals score and on its subdomain score to assess cognitive evolution in both groups. Secondary outcome measurements included self-reported changes in sound quality (Hearing Implant Sound Quality Index), self-perceived hearing disability (Speech, Spatial, and Qualities of Hearing Scale), states of anxiety and depression (Hospital Anxiety and Depression Scale), and level of negative affectivity and social inhibition (Type D questionnaire). RESULTS: Improvements of the overall cognitive functioning (p = 0.05) and the subdomain "Attention" (p = 0.02) were observed after cochlear implantation in the intervention group; their scores were compared to the corresponding scores in the control group. Significant positive effects of cochlear implantation on sound quality and self-perceived hearing outcomes were found in the intervention group. Notably, 20% fewer traits of Type D personalities were measured in the intervention group after cochlear implantation. In the control group, traits of Type D personalities increased by 13%. CONCLUSION: Intervention with a CI improved cognitive functioning (domain Attention in particular) in older adults with severe hearing impairment compared to that of the matched controls with hearing impairment without a CI. However, older CI users did not, in terms of cognition, bridge the performance gap with adults with normal hearing after 1 year of CI use. The fact that experienced, older CI users still present subnormal cognitive functioning may highlight the need for additional cognitive rehabilitation in the long term after implantation.


Asunto(s)
Implantación Coclear , Implantes Cocleares , Pérdida Auditiva , Percepción del Habla , Anciano , Cognición , Pérdida Auditiva/cirugía , Humanos , Estudios Prospectivos , Calidad de Vida , Resultado del Tratamiento
16.
Eur Arch Otorhinolaryngol ; 278(11): 4225-4233, 2021 Nov.
Artículo en Inglés | MEDLINE | ID: mdl-33788034

RESUMEN

PURPOSE: Magnetic resonance imaging (MRI) is often used to visualize and diagnose soft tissues. Hearing implant (HI) recipients are likely to require at least one MRI scan during their lifetime. However, the MRI scanner can interact with the implant magnet, resulting in complications for the HI recipient. This survey, which was conducted in two phases, aimed to evaluate the safety and performance of MRI scans for individuals with a HI manufactured by MED-EL (MED-EL GmbH, Innsbruck, Austria). METHODS: A survey was developed and distributed in two phases to HEARRING clinics to obtain information about the use of MRI for recipients of MED-EL devices. Phase 1 focused on how often MRI is used in diagnostic imaging of the head region of the cochlear implant (CI) recipients. Phase 2 collected safety information about MRI scans performed on HI recipients. RESULTS: 106 of the 126 MRI scans reported in this survey were performed at a field strength of 1.5 T, on HI recipients who wore the SYNCHRONY CI or SYNCHRONY ABI. The head and spine were the most frequently imaged regions. 123 of the 126 scans were performed without any complications; two HI recipients experienced discomfort/pain. One recipient required reimplantation after an MRI was performed using a scanner that had not been approved for that implant. There was only one case that required surgical removal of the implant to reduce the imaging artefact. CONCLUSION: Individuals with either a SYNCHRONY CI or SYNCHRONY ABI from MED-EL can safely undergo a 1.5 T MRI when it is performed according to the manufacturer's safety policies and procedures.


Asunto(s)
Implantación Coclear , Implantes Cocleares , Audición , Humanos , Imagen por Resonancia Magnética , Imanes
17.
J Craniofac Surg ; 32(7): e672-e676, 2021 Oct 01.
Artículo en Inglés | MEDLINE | ID: mdl-34705372

RESUMEN

ABSTRACT: Ear surgery requires magnified imaging of anatomical structures from its beginning to achieve safe and successful surgical outcomes. The historical evolution of magnification in otology has developed from monocular to binocular, and to three-dimensional and even to digital in modern times. Current technological advancements pursue high-quality visualization for the best surgical outcomes but also ergonomic for surgeons. Here, we evaluated the usability of such new technology in common otological surgeries like cochlear implantation and stapedectomy for the first time in patients. A three-dimensional camera mounted to a robot arm has hands-free control by goggles worn by the surgeon on a head mount. The camera at a distance of the patients but can also be draped in a sterile way that it forms a barrier tent between patient and surgical personnel in the theatre. The main reason to evaluate the feasibility of this new exoscope was driven by COVID-19 obligate measures for elective surgery such as hearing restoration. This new technology can be considered an important advantage for the surgeons working in microsurgery to perform their elective operations without aerosolization of the drill rinsing water possibly containing contaminated tissue. From a subjective point of view, the image quality is equivalent to conventional microscopes to provide safe otologic surgery.


Asunto(s)
COVID-19 , Procedimientos Quirúrgicos Otológicos , Robótica , Humanos , Microcirugia , SARS-CoV-2
18.
Med J Islam Repub Iran ; 35: 99, 2021.
Artículo en Inglés | MEDLINE | ID: mdl-34956945

RESUMEN

Since the discovery of coronavirus disease 2019 (COVID-19), which started in Wuhan, China, the epidemic has not only swept through China but also spread throughout the world in spite of the concerted attempts from the governments to contain it. Thus, prevention and control of COVID-19 infection is very effective in ensuring the safety of medical specialists, health care workers, and patients. Audiology clinics are also crucial in the fight against the infection epidemic, as audiologists provide their diagnostic and rehabilitative services in an environment with different contaminated objects that come in either direct or indirect contact with multiple patients. The current article explains the importance of infection control in audiology and priority setting for audiologic evaluation in COVID-19 pandemic.

19.
Paediatr Anaesth ; 30(9): 1033-1040, 2020 09.
Artículo en Inglés | MEDLINE | ID: mdl-32506586

RESUMEN

BACKGROUND: Cochlear implantation in children with sensorineural hearing loss is preferably performed at youngest age because early auditory input is essential to prevent neural plasticity decline. In contrast, the rate of anesthetic adverse events is increased during infancy. Therefore, to provide recommendations regarding an optimal pediatric implantation age, these possible anesthetic risks in infants need to be taken into account. AIMS: This study aimed at assessing the relation between the age at cochlear implant surgery and anesthetic and surgical adverse events. Secondary aims were to evaluate anesthetic and surgical adverse events in relation to (a) the number of preoperative anesthesia-related procedures for cochlear implant candidacy assessment and (b) the anesthetic maintenance agent (total intravenous anesthesia versus inhalation anesthesia) during implantation. METHODS: We executed a retrospective cohort study to evaluate cochlear implantation performed in infants and toddlers between January 2008 and July 2015 in a tertiary pediatric center. We compared anesthetic and surgical adverse events between age-at-implantation (0-12 and 12-24 months of age) groups. Furthermore, we assessed whether anesthetic adverse events occurred during preoperative anesthesia-related procedures for cochlear implant candidacy assessment. RESULTS: Forty-six cochlear implantations were performed in 43 patients requiring 42 preoperative anesthesia-related procedures. Nineteen cochlear implantations (41.3%) were performed during infancy. During implantation, the maintenance agent was either sevoflurane (n = 22) or propofol (n = 24). None of the patients encountered major anesthetic adverse events, whereas minor adverse events occurred during 34 cochlear implantations. Those attributed to surgery occurred following six implantations. Neither the age at implantation nor the anesthetic maintenance agent was significantly related to the occurrence of both types of adverse events. CONCLUSIONS: Adverse events occur independent of the age at implantation, the number of anesthetic preoperative procedures, and the type of anesthetic maintenance agent in patients who received a cochlear implant before 24 months of age.


Asunto(s)
Anestesia , Implantación Coclear , Implantes Cocleares , Pérdida Auditiva Sensorineural , Anestesia/efectos adversos , Niño , Preescolar , Humanos , Lactante , Estudios Retrospectivos
20.
Mol Genet Genomics ; 294(4): 1001-1006, 2019 Aug.
Artículo en Inglés | MEDLINE | ID: mdl-30968248

RESUMEN

Otosclerosis is a common form of hearing loss (HL) due to abnormal remodeling of the otic capsule. The genetic causes of otosclerosis remain largely unidentified. Only mutations in a single gene, SERPINF1, were previously published in patients with familial otosclerosis. To unravel the contribution of genetic variation in this gene to otosclerosis, this gene was re-sequenced in a large population of otosclerosis patients and controls. Resequencing of the 5' and 3' UTRs, coding regions, and exon-intron boundaries of SERPINF1 was performed in 1604 unrelated otosclerosis patients and 1538 unscreened controls, and in 62 large otosclerosis families. Our study showed no enrichment of rare variants, stratified by type, in SERPINF1 in patients versus controls. Furthermore, the c.392C > A (p.Ala131Asp) variant, previously reported as pathogenic, was identified in three patients and four controls, not replicating its pathogenic nature. We could also not find evidence for a pathogenic role in otosclerosis for 5' UTR variants in the SERPINF1-012 transcript (ENST00000573763), described as the major transcript in human stapes. Furthermore, no rare variants were identified in the otosclerosis families. This study does not support a pathogenic role for variants in SERPINF1 as a cause of otosclerosis. Therefore, the etiology of the disease remains largely unknown and will undoubtedly be the focus of future studies.


Asunto(s)
Proteínas del Ojo/genética , Factores de Crecimiento Nervioso/genética , Otosclerosis/genética , Análisis de Secuencia de ADN/métodos , Serpinas/genética , Regiones no Traducidas 3' , Regiones no Traducidas 5' , Estudios de Casos y Controles , Femenino , Humanos , Masculino , Linaje
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