Preemptive strategies and lessons learned from complications encountered with microvascular decompression for hemifacial spasm.

OBJECTIVE: Microvascular decompression (MVD) is the only curative treatment modality for hemifacial spasm (HFS). Although generally considered to be safe, this surgical procedure is surrounded by many risks and possible complications. The authors present the spectrum of complications that they met in their case series, the possible causes, and the strategies recommended to minimize them. METHODS: The authors reviewed a prospectively maintained database for MVDs performed from 2005 until 2021 and extracted relevant data including patient demographics, offending vessel(s), operative technique, outcome, and different complications. Descriptive statistics with uni- and multivariable analyses for the factors that may influence the seventh, eighth, and lower cranial nerves were performed. RESULTS: Data from 420 patients were obtained. Three hundred seventeen of 344 patients (92.2%) with a minimum follow-up of 12 months had a favorable outcome. The mean follow-up (standard deviation) was 51.3 ± 38.7 months. Immediate complications reached 18.8% (79/420). Complications persisted in only 7.14% of patients (30/420) including persistent hearing deficits (5.95%) and residual facial palsy (0.95%). Temporary complications included CSF leakage (3.10%), lower cranial nerve deficits (3.57%), meningitis (0.71%), and brainstem ischemia (0.24%). One patient died because of herpes encephalitis. Statistical analyses showed that the immediate postoperative disappearance of spasms and male gender are correlated with postoperative facial palsy, whereas combined vessel compressions involving the vertebral artery (VA) and anterior inferior cerebellar artery can predict postoperative hearing deterioration. VA compressions could predict postoperative lower cranial nerve deficits. CONCLUSIONS: MVD is safe and effective for treating HFS with a low rate of permanent morbidity. Proper patient positioning, sharp arachnoid dissection, and endoscopic visualization under facial and auditory neurophysiological monitoring are the key points to minimize the rate of complications in MVD for HFS.

Cribaje neonatal precoz de déficit auditivo mediante el registro de otoemisiones acústicas

La alta prevalencia de déficit auditivo, establecido ya antes del nacimiento del niño o adquirido en el periodo perinatal o posnatal inmediato, es alarmante, unido a la repercusión de esta discapacidad sobre el lenguaje y otros procesos cognitivos, la convierten en un serio problema de salud, por lo que un diagnóstico precoz, es esencial para una rehabilitación oportuna. El objetivo de nuestro trabajo es caracterizar los resultados del registro de otoemisiones acústicas en recién nacidos. Se utilizó el método de investigación descriptiva, retrospectiva y transversal de serie de 4176 neonatos, a los cuales se le realizó registro de otoemisiones acústicas en el hospital provincial general "Carlos Manuel de Céspedes" de Bayamo, Granma, durante el periodo comprendido del 1ro de enero hasta el 31 de diciembre del 2022.Más del 96 % de los neonatos fueron pesquisados; la prevalencia de pruebas fallidas, sugestivas de déficit auditivo fue de 0,4 %. El comportamiento de los hallazgos del examen, fue próximo al 50 % en cada sexo y la afectación de ambos oídos se encontró en el 53 % de los casos. Los neonatos con factores de riesgo auditivo, representaron cerca del 5 % del total de recién nacidos y de este subgrupo, el 5,3 % no pasaron el tamizaje. El bajo peso al nacer, fue el factor de riesgo más comúnmente encontrado. Podemos considerar que el cribaje realizado, cumple los criterios establecidos para calificarlo de, universal. La prevalencia de pruebas fallidas fue alta, aunque acorde con los criterios de la Junta del Comité de Audición infantil (JCIH) y no mostró diferencia alguna según el sexo. La presencia de factores de riesgo, fue baja, aunque en correspondencia con resultados a nivel global, destacándose como el más común, el bajo peso al nacer.

The high prevalence of hearing deficit, established already before the birth of the child or acquired in the immediate perinatal or postnatal period, is alarming, together with the impact of this disability on language and other cognitive processes, make it a serious health problem, so an early diagnosis is essential for timely rehabilitation. The objective of our work is to characterize the results of the registration of otoacoustic emissions in newborns. The descriptive, retrospective and cross-sectional research method of series of 4176 neonates was used, to which acoustic otoemissions were recorded in the general provincial hospital "Carlos Manuel de Céspedes" of Bayamo, Granma, during the period from January 1 to December 31, 2022. More than 96% of neonates were screened; the prevalence of failed tests suggestive of hearing deficit was 0.4%. The behavior of the examination findings was close to 50% in each sex and the involvement of both ears was found in 53% of cases. Neonates with auditory risk factors represented about 5% of all newborns and of this subgroup, 5.3% did not pass screening. Low birth weight was the most commonly found risk factor. We can consider that the screening carried out meets the criteria established to qualify it as universal. The prevalence of failed tests was high, although in line with the criteria of the Board of the Child Hearing Committee (JCIH) and showed no difference by sex. The presence of risk factors was low, although in correspondence with results at the global level, standing out as the most common, low birth weight.

A alta prevalência do déficit auditivo, estabelecido já antes do nascimento da criança ou adquirido no período perinatal oupós-natal imediato, é alarmante, juntamente com o impacto desta deficiência na linguagem e em outros processos cognitivos, tornando-a um grave problema de saúde, sendo essencial o diagnóstico precoce para a reabilitação em tempo hábil. O objetivo do nosso trabalho é caracterizar os resultados do registro das emissões otoacústicas em recém-nascidos. Utilizou-se o método de pesquisa descritiva, retrospectiva e transversal de série de 4176 neonatos, para a qual foram registradas otoemissões acústicas no hospital geral provincial "Carlos Manuel de Céspedes" de Bayamo, Granma, durante o período de 1º de janeiro a 31 de dezembro de 2022. Mais de 96% dos neonatos foram triados; A prevalência de falha nos testes sugestivos de déficit auditivo foide 0,4%. O comportamento dos achados do exame foi próximo de 50% em cada sexo e o acometimento de ambas as orelhas foi encontrado em 53% dos casos. Neonatos com fatores de risco auditivo representaram cerca de 5% de todos os recém-nascidos e, deste subgrupo, 5,3% não passaram na triagem. O baixo peso ao nascer foi o fator de risco mais encontrado. Podemos considerar que a triagem realizada atende aos critérios estabelecidos para qualificá-la como universal. A prevalência de reprovação nos testes foi alta, embora de acordo com os critérios do Board of the Child Hearing Committee (JCIH) e não mostrou diferença por sexo. A presença de fatores de risco foi baixa, embora em correspondência com os resultados em nível global, destacando-se como o mais comum, o baixo peso ao nascer.

Genetic and Non-genetic Workup for Pediatric Congenital Hearing Loss.

Hearing loss is one of the most common concerns for presentation for a geneticist. Presentation prior to the age of one (congenital hearing loss), profound sensorineural hearing loss (SNHL), and bilateral hearing loss are sensitive and should raise concern for genetic causes of hearing loss and prompt referral for genetic testing. Genetic testing particularly in this instance offers the opportunity for anticipatory guidance including possible course of the hearing loss over time and also connection and evaluation for additional congenital anomalies that may be associated with an underlying syndrome vs. isolated genetic hearing loss.

Sensorineural and conductive hearing loss in infants diagnosed in the program of universal newborn hearing screening.

OBJECTIVE: The aim of this study was to analyze infants diagnosed with sensorineural or conductive hearing deficit and to identify risk factors associated with these defects. MATERIAL AND METHODS: A retrospective analysis of infants diagnosed with hearing deficit based on the database of the universal newborn hearing screening program and medical records of the patients. RESULTS: 27 935 infants were covered by the universal neonatal hearing screening program. 109 (0.39%) were diagnosed with hearing deficit and referred for treatment and rehabilitation. 56 (51.4%) children were diagnosed with conductive, 38 (34.9%) with sensorineural and 15 (13.8%) with mixed type of hearing deficit. Children with sensorineural hearing deficit more frequently suffered from hyperbilirubinemia (p < 0.05), while infants with conductive hearing loss were more frequently diagnosed with isolated craniofacial anomalies (p < 0.05). The prevalence of other risk factors did not differ between the groups. Sensorineural hearing deficit occurred almost 3 times more often bilaterally than unilaterally (p < 0.05). In other types of hearing deficit, the difference was not significant. In children with conductive and mixed type of hearing loss the impairment was mainly mild while among those with sensorineural hearing deficit in almost 45% it was severe and profound (p < 0.05). When analyzing the consistency between hearing screening test by means of otoacoustic emissions and the final diagnosis of hearing deficit we found that the highest agreement rate was observed in children with sensorineural hearing loss (p < 0.01). CONCLUSIONS: The prevalence of most risk factors of hearing deficit was similar in children with sensorineural, conductive and mixed type of hearing loss, only hyperbilirubinemia seemed to predispose to sensorineural hearing deficit and isolated craniofacial malformations seemed to be associated with conductive hearing loss. Sensorineural hearing deficit usually occurred bilaterally and was severe or profound, while conductive and mixed type of hearing deficit were most often of mild degree. Most children with the final diagnosis of sensorineural hearing deficit had positive result of hearing screening by means of otoacoustic emissions.

The results of newborn hearing screening by means of transient otoacoustic emissions - has anything changed over 10 years?

OBJECTIVES: Universal newborn hearing screening (UNHS) has become the standard of care in many countries. The aim of this study was to evaluate the results of UNHS after ten years of the program in Poland and to compare them with the results of 2003. METHODS: In the study, we analyze the results of UNHS in the University Hospital in Poznan, Poland. Between 01.01.2013 and 31.12.2013, 6827 children were examined by means of otoacoustic emissions. RESULTS: Risk factors (RF) were identified in 772 (11.3%) newborns, which is significantly less than 10 years ago (p < 0.05). The most frequent RF were: ototoxic medications, treatment in neonatal intensive care unit (NICU) and prematurity < 33 weeks of gestation. In 2003, the most frequent were ototoxic medications and prematurity, less frequent was treatment in NICU and more common was low Apgar score. In 51 (6.6%) newborns with RF, the result of OAE was positive either unilaterally or bilaterally. In infants without RF the result was positive unilaterally in 22 (0.4%) and bilaterally in 14 (0.2%) patients. These results are significantly lower than in our former study. The relative risk of positive result was the highest in infants with complex congenital anomalies (RR = 44.99), craniofacial anomalies (RR = 17.46) and mechanical ventilation for > 5 days (RR = 10.69). In our previous study, the highest RR of positive test results was in infants with family history, congenital malformations and low Apgar score. We found that most predictive as to the final diagnosis was bilaterally positive OAE test. In most patients, the second check confirmed the diagnosis, independently of RF. The number of false positive tests at the 1st level of screening is significantly lower now than 10 years ago, probably due to better staff training. CONCLUSIONS: Long term monitoring and the appropriate management of hearing deficit in children is essential. UNHS seems to be the most efficient way of finding children who require treatment of hearing impairment. The prevalence of most risk factors of hearing deficit has significantly changed over the years. The number of false positive results has significantly decreased over the years thanks to better staff training.

Imported Case of Lassa Fever in Sweden With Encephalopathy and Sensorineural Hearing Deficit.

We describe an imported case of Lassa fever with both encephalopathy and bilateral sensorineural hearing deficit. Absence of fever during hospitalization, initially nonspecific symptoms, and onset of hearing deficit in a late stage of disease probably contributed to delayed diagnosis (14 days after admittance to hospital). The pathogenesis of neurological manifestations of Lassa fever is poorly understood and no specific treatment was given. A total of 118 personnel had close contact with the patient, but no secondary cases occurred. This case highlights the importance of considering Lassa fever as a differential diagnosis in patients with recent travel to endemic areas.

ABER Assessment in Pre-school Children with Developmental Speech and Language Impairment.

INTRODUCTION: Developmental speech and language disorders (DLD) constitute a group of disorders when children with normal intelligence and hearing fail to develop language in an age-appropriate manner. There is no definite or surrogate neurophysiologic laboratory marker to quantitate the extent of speech and language impairment. The current study was designed to evaluate the abnormalities in Auditory Brainstem Evoked Responses (ABER) in children with speech and language impairment who do not have a hearing deficit or autism. MATERIALS AND METHODS: ABER recording was done in a cohort of 94 children (age 2-8 y) with DLD without overt hearing deficit or autism. The mean latencies for waves I, II, III, IV and V along with inter peak latencies for I-III, I-V, III-V and amplitude ratio of wave V/I was measured after click stimulus with intensities 110 db until 40 db and compared to age appropriate normograms. RESULTS: The peak latencies for waves I, III & V, inter-peak latencies I-III & I-V, III-V and wave amplitude ratio V/I was found within normal limits in both ears of all the children when compared to age appropriate normograms. CONCLUSION: The current study therefore emphasizes the fact that ABER may not be used/recommended as diagnostic or prognostic tool in children with speech and language impairment without autism or hearing deficit. The results and the recommendations of this study will definitely reduce the burden on electrophysiologist, laboratories and also save time and financial resources.

A compreensão do texto por crianças deficientes auditivas profundas oralizadas e sinalizadoras / The comprehension of text by oralized and signalizer children with deep hearing deficit

O presente estudo verifica a capacidade de interpretação de texto por crianças surdas oralizadas e crianças surdas sinalizadoras. A amostra se constituiu de 18 indivíduos surdos com perda auditiva profunda bilateral congênita, sendo 10 sinalizadores e 8 oralizados. Texto para leitura silenciosa foi apresentado aos participantes do estudo. Em seguida, eles responderam a um questionário objetivo, sem consulta ao texto, para verificação da sua capacidade de interpretação. Os dois grupos obtiveram boa porcentagem de acertos quanto à análise individual. Já em relação ao número de questões respondidas corretamente, o grupo dos oralizados apresentou mais acertos que os sinalizadores. Confirmou-se que pessoas surdas oralizadas possuem melhor desempenho em habilidades de interpretação de texto do que os surdos sinalizadores, provavelmente pelo contato prévio com a Língua portuguesa. (AU)

The ability for text comprehension was verified in 18 deaf individuals with deep hearing loss (10 sinalizers and 8 oralized). A text was presented to them for silent reading, and after reading it the individuals answered an objective questionnaire with no consulting to the text to verify the ability of interpretation. Both groups had a good percentage of right answers in the individual analysis. Right answers were significantly more frequent in the group of oralized individuals. Oralized deafs had better performance in text interpretation than the signalizers probably because of the previous contact with the Portuguese language. (AU)

A compreensão do texto por crianças deficientes auditivas profundas oralizadas e sinalizadoras / The comprehension of text by oralized and signalizer children with deep hearing deficit

O presente estudo verifica a capacidade de interpretação de texto por crianças surdas oralizadas e crianças surdas sinalizadoras. A amostra se constituiu de 18 indivíduos surdos com perda auditiva profunda bilateral congênita, sendo 10 sinalizadores e 8 oralizados. Texto para leitura silenciosa foi apresentado aos participantes do estudo. Em seguida, eles responderam a um questionário objetivo, sem consulta ao texto, para verificação da sua capacidade de interpretação. Os dois grupos obtiveram boa porcentagem de acertos quanto à análise individual. Já em relação ao número de questões respondidas corretamente, o grupo dos oralizados apresentou mais acertos que os sinalizadores. Confirmou-se que pessoas surdas oralizadas possuem melhor desempenho em habilidades de interpretação de texto do que os surdos sinalizadores, provavelmente pelo contato prévio com a Língua portuguesa.

The ability for text comprehension was verified in 18 deaf individuals with deep hearing loss (10 sinalizers and 8 oralized). A text was presented to them for silent reading, and after reading it the individuals answered an objective questionnaire with no consulting to the text to verify the ability of interpretation. Both groups had a good percentage of right answers in the individual analysis. Right answers were significantly more frequent in the group of oralized individuals. Oralized deafs had better performance in text interpretation than the signalizers probably because of the previous contact with the Portuguese language.