aim of this report is to present a new surgical technique for the BaHa ® system implant and to di... more aim of this report is to present a new surgical technique for the BaHa ® system implant and to discuss the operational techniques and complications related to this type of surgery. The common technique for the positioning of the Bone-anchored Hearing aid (BaHa ® , Cochlear Limited, Englewood, CO, uSa) titanium implant into the temporal bone is based on the use of either a free or a pedunculated skin flap. Reported complications of this type of surgery include skin flap necrosis with healing by second intention, infection of the flap, skin growth over the abutment, failure of osseointegration and implant extrusion. In order to reduce the incidence of these problems, different types of surgery have already been presented over the years. Herewith, a new technique is proposed for implanting a BaHa ® screw in the temporal bone, that is simple, rapid to perform, and does not require the use of a flap. This technique appears to offer two main advantages: i) the speeding up of the procedure...
Dear Editor: We read with great interest the article entitled “A Modern Case Sheds Light on a Cla... more Dear Editor: We read with great interest the article entitled “A Modern Case Sheds Light on a Classical Enigma: Beethoven’s Deafness” by Brotto et al. Researchers have continuously sought to uncover underlying causes for hearing loss of the great composer and pianist, Ludwig van Beethoven. Authors explore this subject and ultimately support the lead poisoning hypothesis, given Beethoven’s dependence on alcohol, often infused with lead at his time, presence of elevated lead in his hair and deep bone, and a case of a modern patient who presents with hearing loss similar to Beethoven’s due to lead poisoning. We commend authors’ robust exploration of this enigma and concur that lead poisoning remains a highly probable cause. Given such, we would like to provide few insights from our previous study on this topic. As authors referred to in their study, Beethoven suffered from progressive hearing loss, hyperacusis, tinnitus, and diminished speech discrimination without vertigo that eventua...
Aim of this report is to present a new surgical technique for the BAHA system implant and to disc... more Aim of this report is to present a new surgical technique for the BAHA system implant and to discuss the operational techniques and complications related to this type of surgery. The common technique for the positioning of the Bone-Anchored Hearing Aid (BAHA, Cochlear Limited, Englewood, CO, USA) titanium implant into the temporal bone is based on the use of either a free or a pedunculated skin flap. Reported complications of this type of surgery include skin flap necrosis with healing by second intention, infection of the flap, skin growth over the abutment, failure of osseointegration and implant extrusion. In order to reduce the incidence of these problems, different types of surgery have already been presented over the years. Herewith, a new technique is proposed for implanting a BAHA screw in the temporal bone, that is simple, rapid to perform, and does not require the use of a flap. This technique appears to offer two main advantages: i) the speeding up of the procedure; ii) t...
In Reply: We would like to thank Prof. Stevens for the specifications reported in his letter. His... more In Reply: We would like to thank Prof. Stevens for the specifications reported in his letter. His previous article, about the theory of chronic poisoning determined by consumption of wine tainted with lead as a possible explanation of Beethoven’s deafness, should be considered a milestone in this field. In our previous article, we performed a wide revision of papers and theories to explain not only the audiological course but also the overall clinical status of the great composer. Over the decades, some theories have become public knowledge (i.e., otosclerosis or syphilis), despite missing conclusive evidence. It is our belief that, among the multiple hypotheses proposed over the centuries, chronic lead poisoning seems to be the most founded, probable, and in the end realistic. Nonetheless, we cannot exclude that some pieces of Ludwig van Beethoven’s history will be discovered in the future. In absence of new documents, our brainstorming led to ... the lead; but we have the feeling that this debate will not be over soon. DAVIDE BROTTO, MD, FLAVIA SORRENTINO, MD, FLAVIA GHELLER, Dr, PATRIZIA TREVISI, Prof, ROBERTO BOVO, Prof, Otorhinolaryngology Section, Neurosciences Department, Università degli Studi di Padova, Padova, Italy
Two hundred and fifty years have passed since the birth of Ludwig van Beethoven, and the enigma a... more Two hundred and fifty years have passed since the birth of Ludwig van Beethoven, and the enigma about his hearing loss and overall health status seems to be not completely solved. However, the admission to the hospital of a 64‐year‐old woman in 2018 with symptoms extremely similar to those experienced by the great composer may add further evidence to a theory previously underestimated. The health issues of the modern patient were found to be due to chronic lead intoxication. The lead was released during daily cooking using a ceramic‐coated frying pan with worn surface that poisoned her breakfast most probably for years.
To assess preoperative features that could predict the audiological outcome after cochlear implan... more To assess preoperative features that could predict the audiological outcome after cochlear implantation in the elderly, in terms of pure tone audiometry, speech audiometry, and speech perception performance. All available records of patients with cochlear implants aged 65 or more at the time of their implantation at our Institution were reviewed (50 patients, mean age 70.76 ± 4.03 years), recording preoperative clinical features. Pure tone audiometry, speech audiometry, and speech perception performance 1 year after cochlear implant activation and fitting were used as outcome measures. No statistically significant association emerged between clinical features and pure tone audiometry. On univariate analysis, progressive sensorineural hearing loss of unknown origin was associated with a better outcome in terms of speech audiometry and speech perception performance (p = 0.035 and p = 0.033, respectively). On multivariate analysis, progressive sensorineural hearing loss retained its independent prognostic significance in terms of speech perception performance (p = 0.042). The discriminatory power of a two-variable panel (age and etiology of hearing loss) featured an AUC (ROC) of 0.738 (an acceptable discriminatory power according to the Hosmer–Lemeshow scale). A progressive sensorineural hearing loss of unknown origin was associated with a better outcome in terms of speech perception in the elderly in our case study. Further features that can predict audiological outcome achievable with cochlear implants in the elderly are desirable to perform adequate counselling and rehabilitation programs.
ABSTRACT Introduction: Cogan’s syndrome (CS) is a rare autoimmune disease, characterized by ocula... more ABSTRACT Introduction: Cogan’s syndrome (CS) is a rare autoimmune disease, characterized by ocular and vestibulo-auditory involvement. Treatment of CS could be challenging, and the only evidence-based data comes from case reports or series. Areas covered: There have recently been several reports of new treatment strategy involving the use of biological disease-modifying anti-rheumatic drugs such as TNFα inhibitors, anti-CD20 or anti-IL6 receptor antibodies, in cases resistant to first- or second-line drugs. Expert commentary: Corticosteroids are the cornerstone of CS therapy at disease onset and during acute phases of the disease. Conventional immunosuppressive therapy, such as methotrexate, could be used in relapsing patients or as a glucocorticoid sparing agent, but efficacy is often modest. The anti-TNFα monoclonal antibody Infliximab appears to be the most frequently used, leading to an improvement in hearing loss in 89% of the cases and allow corticosteroid tapering in 86% of the patients. The appropriate timing of Infliximab treatment has yet to be thoroughly investigated, but it seems to be more effective when started at an early stage of the disease. Efficacy of others anti-TNFα agents is controversial. Rituximab and Tocilizumab are a safe option, but results on hearing loss have still to be confirmed on larger patients’ cohorts.
Importance Common cavity deformity is a rare congenital inner ear malformation associated with pr... more Importance Common cavity deformity is a rare congenital inner ear malformation associated with profound hearing loss and attributed to an early developmental arrest of the cochlear-vestibular structures. This narrative review highlights the need to identify reliable indicators of hearing rehabilitation outcome. Observations Despite its relatively simple definition, common cavity deformity varies widely in morphologic features, presence of cochlear nerve fibers and remnants of Corti organ, and outcome after cochlear or brainstem implant. Conclusions and Relevance Cochlear implant has been shown to be a valid option for common cavity deformity, but its outcome remains variable and poor. Identification of specific neuroradiologic, audiologic, and neurophysiologic prognostic features; tailoring of the surgical approach; and standardization of outcome measures are needed to optimize the management of common cavity deformity and hearing rehabilitation after implant.
This contribution is part of a wider project aiming at the creation of a phonological discriminat... more This contribution is part of a wider project aiming at the creation of a phonological discrimination test for preschool subjects (48to 72-months-old). Children with primary language impairment are usually affected by deficits in speech production and/or in the phonological representation of speech sounds causing discrimination disorders. The proposed test is designed to evaluate the ability to discriminate pairs of non-words through a “same/different” judgment. The test items combined in pairs examine all the possible contrasts’ simplifications (i.e. “phonological processes”) that may accompany the speech production of typical developing preschool children even if these processes are not always (and all) present during language development, and even if there is currently no consensus in the literature on which processes belong to typical or atypical development.
Inner ear malformations are associated with different degrees of hearing loss. Some of these anom... more Inner ear malformations are associated with different degrees of hearing loss. Some of these anomalies require cochlear implantation because of the occurrence of profound hearing loss. The radiological imaging necessary for the evaluation of the congenital hearing loss might also reveal the concomitant presence of important collateral findings such as the central nervous system and cranial nerves malformations. These neurological features might be extremely important in the surgical planning of the cochlear implantation and also have a role in the hearing and speech development of the affected children. In addition, anomalies affecting specific structures of the inner ear should be considered predisposing factors for otogenic meningitis and they should be identified as early as possible. The present review aims to summarize the available literature about this specific topic crucial for the patients’ neuropsychological and auditory development.
Introduction: Non-syndromic hereditary hearing loss is characterized by extreme genetic heterogen... more Introduction: Non-syndromic hereditary hearing loss is characterized by extreme genetic heterogeneity. So far, more than 100 pathogenic or likely pathogenic variants in TMC1 gene have been reported in patients with autosomal recessive hearing loss (HL) DFNB7/11. The prevailing auditory phenotype of individuals with DFNB7/11 is congenital, profound, bilateral HL, but the functional outcome after cochlear implantation (CI) described in the literature is variable. The objective of this work is to evaluate the auditory outcome after CI in pediatric patients with DFNB7/11, born to non-consanguineous parents. Methods: A retrospective analysis of genetic and audiological data of DFNB7/11 patients followed up in a single Italian otolaryngology clinic was performed. Cases with biallelic pathogenic variants in TMC1 were selected from the cohort of children with non-syndromic hearing loss who had undergone CI and had been molecularly characterized by multigene panel testing. All patients under...
Inner ear malformations are present in 20% of patients with sensorineural hearing loss. Although ... more Inner ear malformations are present in 20% of patients with sensorineural hearing loss. Although the first descriptions date to the 18th century, in recent years the knowledge about these conditions has experienced terrific improvement. Currently, most of these conditions have a rehabilitative option. Much less is known about the etiology of these anomalies. In particular, the evolution of genetics has provided new data about the possible relationship between inner ear malformations and genetic anomalies. In addition, in syndromic condition, the well-known presence of sensorineural hearing loss can now be attributed to the presence of an inner ear anomaly. In some cases, the presence of these abnormalities should be considered as a characteristic feature of the syndrome. The present paper aims to summarize the available knowledge about the possible relationships between inner ear malformations and genetic mutations.
Pediatric hearing loss early diagnosis and treatment have been limited by the current restriction... more Pediatric hearing loss early diagnosis and treatment have been limited by the current restrictions due to the coronavirus disease 2019 pandemic. The difficulty in accessing the multiple facilities required for the rehabilitative process is influencing the timing of each step of the process. Auditory hearing screening programs, etiological characterization, surgical timing, and speech therapies have all been limited in the past year. The current conditions have forced us to adopt different strategies to overcome the necessary social distancing prescriptions. Although their efficacy should be proved over time, some of these resources will be probably useful even in a nonpandemic future.
Objectives: West Nile virus (WNV) has been spreading over the last 20 years. Human infection is a... more Objectives: West Nile virus (WNV) has been spreading over the last 20 years. Human infection is asymptomatic in most cases. When the disease becomes clinically manifest, it may involve a range of issues, from a mild infection with flu-like symptoms to a neuroinvasive disease. Albeit rarely, WNV-associated sensorineural hearing loss (SNHL) has also been reported. Here we describe two new cases of SNHL and balance impairment caused by WNV infection. Methods: The patients were investigated with repeated audiometric tests and, for the first time, videonystagmography was also used. Results: Unlike findings in the few other published cases, an improvement in audiometric thresholds and vestibular function was documented in both of our patients. Conclusions: In the light of our findings, a prospective study would be warranted on a large series of patients with WNV infection in order: (i) to better define the epidemiology of the related cochlear-vestibular involvement; and (ii) to elucidate ...
aim of this report is to present a new surgical technique for the BaHa ® system implant and to di... more aim of this report is to present a new surgical technique for the BaHa ® system implant and to discuss the operational techniques and complications related to this type of surgery. The common technique for the positioning of the Bone-anchored Hearing aid (BaHa ® , Cochlear Limited, Englewood, CO, uSa) titanium implant into the temporal bone is based on the use of either a free or a pedunculated skin flap. Reported complications of this type of surgery include skin flap necrosis with healing by second intention, infection of the flap, skin growth over the abutment, failure of osseointegration and implant extrusion. In order to reduce the incidence of these problems, different types of surgery have already been presented over the years. Herewith, a new technique is proposed for implanting a BaHa ® screw in the temporal bone, that is simple, rapid to perform, and does not require the use of a flap. This technique appears to offer two main advantages: i) the speeding up of the procedure...
Dear Editor: We read with great interest the article entitled “A Modern Case Sheds Light on a Cla... more Dear Editor: We read with great interest the article entitled “A Modern Case Sheds Light on a Classical Enigma: Beethoven’s Deafness” by Brotto et al. Researchers have continuously sought to uncover underlying causes for hearing loss of the great composer and pianist, Ludwig van Beethoven. Authors explore this subject and ultimately support the lead poisoning hypothesis, given Beethoven’s dependence on alcohol, often infused with lead at his time, presence of elevated lead in his hair and deep bone, and a case of a modern patient who presents with hearing loss similar to Beethoven’s due to lead poisoning. We commend authors’ robust exploration of this enigma and concur that lead poisoning remains a highly probable cause. Given such, we would like to provide few insights from our previous study on this topic. As authors referred to in their study, Beethoven suffered from progressive hearing loss, hyperacusis, tinnitus, and diminished speech discrimination without vertigo that eventua...
Aim of this report is to present a new surgical technique for the BAHA system implant and to disc... more Aim of this report is to present a new surgical technique for the BAHA system implant and to discuss the operational techniques and complications related to this type of surgery. The common technique for the positioning of the Bone-Anchored Hearing Aid (BAHA, Cochlear Limited, Englewood, CO, USA) titanium implant into the temporal bone is based on the use of either a free or a pedunculated skin flap. Reported complications of this type of surgery include skin flap necrosis with healing by second intention, infection of the flap, skin growth over the abutment, failure of osseointegration and implant extrusion. In order to reduce the incidence of these problems, different types of surgery have already been presented over the years. Herewith, a new technique is proposed for implanting a BAHA screw in the temporal bone, that is simple, rapid to perform, and does not require the use of a flap. This technique appears to offer two main advantages: i) the speeding up of the procedure; ii) t...
In Reply: We would like to thank Prof. Stevens for the specifications reported in his letter. His... more In Reply: We would like to thank Prof. Stevens for the specifications reported in his letter. His previous article, about the theory of chronic poisoning determined by consumption of wine tainted with lead as a possible explanation of Beethoven’s deafness, should be considered a milestone in this field. In our previous article, we performed a wide revision of papers and theories to explain not only the audiological course but also the overall clinical status of the great composer. Over the decades, some theories have become public knowledge (i.e., otosclerosis or syphilis), despite missing conclusive evidence. It is our belief that, among the multiple hypotheses proposed over the centuries, chronic lead poisoning seems to be the most founded, probable, and in the end realistic. Nonetheless, we cannot exclude that some pieces of Ludwig van Beethoven’s history will be discovered in the future. In absence of new documents, our brainstorming led to ... the lead; but we have the feeling that this debate will not be over soon. DAVIDE BROTTO, MD, FLAVIA SORRENTINO, MD, FLAVIA GHELLER, Dr, PATRIZIA TREVISI, Prof, ROBERTO BOVO, Prof, Otorhinolaryngology Section, Neurosciences Department, Università degli Studi di Padova, Padova, Italy
Two hundred and fifty years have passed since the birth of Ludwig van Beethoven, and the enigma a... more Two hundred and fifty years have passed since the birth of Ludwig van Beethoven, and the enigma about his hearing loss and overall health status seems to be not completely solved. However, the admission to the hospital of a 64‐year‐old woman in 2018 with symptoms extremely similar to those experienced by the great composer may add further evidence to a theory previously underestimated. The health issues of the modern patient were found to be due to chronic lead intoxication. The lead was released during daily cooking using a ceramic‐coated frying pan with worn surface that poisoned her breakfast most probably for years.
To assess preoperative features that could predict the audiological outcome after cochlear implan... more To assess preoperative features that could predict the audiological outcome after cochlear implantation in the elderly, in terms of pure tone audiometry, speech audiometry, and speech perception performance. All available records of patients with cochlear implants aged 65 or more at the time of their implantation at our Institution were reviewed (50 patients, mean age 70.76 ± 4.03 years), recording preoperative clinical features. Pure tone audiometry, speech audiometry, and speech perception performance 1 year after cochlear implant activation and fitting were used as outcome measures. No statistically significant association emerged between clinical features and pure tone audiometry. On univariate analysis, progressive sensorineural hearing loss of unknown origin was associated with a better outcome in terms of speech audiometry and speech perception performance (p = 0.035 and p = 0.033, respectively). On multivariate analysis, progressive sensorineural hearing loss retained its independent prognostic significance in terms of speech perception performance (p = 0.042). The discriminatory power of a two-variable panel (age and etiology of hearing loss) featured an AUC (ROC) of 0.738 (an acceptable discriminatory power according to the Hosmer–Lemeshow scale). A progressive sensorineural hearing loss of unknown origin was associated with a better outcome in terms of speech perception in the elderly in our case study. Further features that can predict audiological outcome achievable with cochlear implants in the elderly are desirable to perform adequate counselling and rehabilitation programs.
ABSTRACT Introduction: Cogan’s syndrome (CS) is a rare autoimmune disease, characterized by ocula... more ABSTRACT Introduction: Cogan’s syndrome (CS) is a rare autoimmune disease, characterized by ocular and vestibulo-auditory involvement. Treatment of CS could be challenging, and the only evidence-based data comes from case reports or series. Areas covered: There have recently been several reports of new treatment strategy involving the use of biological disease-modifying anti-rheumatic drugs such as TNFα inhibitors, anti-CD20 or anti-IL6 receptor antibodies, in cases resistant to first- or second-line drugs. Expert commentary: Corticosteroids are the cornerstone of CS therapy at disease onset and during acute phases of the disease. Conventional immunosuppressive therapy, such as methotrexate, could be used in relapsing patients or as a glucocorticoid sparing agent, but efficacy is often modest. The anti-TNFα monoclonal antibody Infliximab appears to be the most frequently used, leading to an improvement in hearing loss in 89% of the cases and allow corticosteroid tapering in 86% of the patients. The appropriate timing of Infliximab treatment has yet to be thoroughly investigated, but it seems to be more effective when started at an early stage of the disease. Efficacy of others anti-TNFα agents is controversial. Rituximab and Tocilizumab are a safe option, but results on hearing loss have still to be confirmed on larger patients’ cohorts.
Importance Common cavity deformity is a rare congenital inner ear malformation associated with pr... more Importance Common cavity deformity is a rare congenital inner ear malformation associated with profound hearing loss and attributed to an early developmental arrest of the cochlear-vestibular structures. This narrative review highlights the need to identify reliable indicators of hearing rehabilitation outcome. Observations Despite its relatively simple definition, common cavity deformity varies widely in morphologic features, presence of cochlear nerve fibers and remnants of Corti organ, and outcome after cochlear or brainstem implant. Conclusions and Relevance Cochlear implant has been shown to be a valid option for common cavity deformity, but its outcome remains variable and poor. Identification of specific neuroradiologic, audiologic, and neurophysiologic prognostic features; tailoring of the surgical approach; and standardization of outcome measures are needed to optimize the management of common cavity deformity and hearing rehabilitation after implant.
This contribution is part of a wider project aiming at the creation of a phonological discriminat... more This contribution is part of a wider project aiming at the creation of a phonological discrimination test for preschool subjects (48to 72-months-old). Children with primary language impairment are usually affected by deficits in speech production and/or in the phonological representation of speech sounds causing discrimination disorders. The proposed test is designed to evaluate the ability to discriminate pairs of non-words through a “same/different” judgment. The test items combined in pairs examine all the possible contrasts’ simplifications (i.e. “phonological processes”) that may accompany the speech production of typical developing preschool children even if these processes are not always (and all) present during language development, and even if there is currently no consensus in the literature on which processes belong to typical or atypical development.
Inner ear malformations are associated with different degrees of hearing loss. Some of these anom... more Inner ear malformations are associated with different degrees of hearing loss. Some of these anomalies require cochlear implantation because of the occurrence of profound hearing loss. The radiological imaging necessary for the evaluation of the congenital hearing loss might also reveal the concomitant presence of important collateral findings such as the central nervous system and cranial nerves malformations. These neurological features might be extremely important in the surgical planning of the cochlear implantation and also have a role in the hearing and speech development of the affected children. In addition, anomalies affecting specific structures of the inner ear should be considered predisposing factors for otogenic meningitis and they should be identified as early as possible. The present review aims to summarize the available literature about this specific topic crucial for the patients’ neuropsychological and auditory development.
Introduction: Non-syndromic hereditary hearing loss is characterized by extreme genetic heterogen... more Introduction: Non-syndromic hereditary hearing loss is characterized by extreme genetic heterogeneity. So far, more than 100 pathogenic or likely pathogenic variants in TMC1 gene have been reported in patients with autosomal recessive hearing loss (HL) DFNB7/11. The prevailing auditory phenotype of individuals with DFNB7/11 is congenital, profound, bilateral HL, but the functional outcome after cochlear implantation (CI) described in the literature is variable. The objective of this work is to evaluate the auditory outcome after CI in pediatric patients with DFNB7/11, born to non-consanguineous parents. Methods: A retrospective analysis of genetic and audiological data of DFNB7/11 patients followed up in a single Italian otolaryngology clinic was performed. Cases with biallelic pathogenic variants in TMC1 were selected from the cohort of children with non-syndromic hearing loss who had undergone CI and had been molecularly characterized by multigene panel testing. All patients under...
Inner ear malformations are present in 20% of patients with sensorineural hearing loss. Although ... more Inner ear malformations are present in 20% of patients with sensorineural hearing loss. Although the first descriptions date to the 18th century, in recent years the knowledge about these conditions has experienced terrific improvement. Currently, most of these conditions have a rehabilitative option. Much less is known about the etiology of these anomalies. In particular, the evolution of genetics has provided new data about the possible relationship between inner ear malformations and genetic anomalies. In addition, in syndromic condition, the well-known presence of sensorineural hearing loss can now be attributed to the presence of an inner ear anomaly. In some cases, the presence of these abnormalities should be considered as a characteristic feature of the syndrome. The present paper aims to summarize the available knowledge about the possible relationships between inner ear malformations and genetic mutations.
Pediatric hearing loss early diagnosis and treatment have been limited by the current restriction... more Pediatric hearing loss early diagnosis and treatment have been limited by the current restrictions due to the coronavirus disease 2019 pandemic. The difficulty in accessing the multiple facilities required for the rehabilitative process is influencing the timing of each step of the process. Auditory hearing screening programs, etiological characterization, surgical timing, and speech therapies have all been limited in the past year. The current conditions have forced us to adopt different strategies to overcome the necessary social distancing prescriptions. Although their efficacy should be proved over time, some of these resources will be probably useful even in a nonpandemic future.
Objectives: West Nile virus (WNV) has been spreading over the last 20 years. Human infection is a... more Objectives: West Nile virus (WNV) has been spreading over the last 20 years. Human infection is asymptomatic in most cases. When the disease becomes clinically manifest, it may involve a range of issues, from a mild infection with flu-like symptoms to a neuroinvasive disease. Albeit rarely, WNV-associated sensorineural hearing loss (SNHL) has also been reported. Here we describe two new cases of SNHL and balance impairment caused by WNV infection. Methods: The patients were investigated with repeated audiometric tests and, for the first time, videonystagmography was also used. Results: Unlike findings in the few other published cases, an improvement in audiometric thresholds and vestibular function was documented in both of our patients. Conclusions: In the light of our findings, a prospective study would be warranted on a large series of patients with WNV infection in order: (i) to better define the epidemiology of the related cochlear-vestibular involvement; and (ii) to elucidate ...
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