ABSTRACT
OBJECTIVES: Newborn (NB) auditory deficit has a prevalence of 1-2% in the world. Since the 1990s different screening programs have been put into practice. The Newborn Hearing Screening Program has been in operation since 2002 in our hospital (HCUVA) in Murcia (Spain) and two years later it was introduced into the whole of the Autonomous Community of the Region of Murcia as part of universal healthcare. The objective of this study was to analyze and assess its results. METHOD: The newborn (NB) population is divided into two groups: not-at-risk NBs and at-risk NBs. In the first case we carry out acoustic otoemissions (AOEs) 48 h after birth and depending on the result the child is either discharged or, in negative cases, the infant undergoes a series of tests in a period of 30-45 days to confirm or rule out the existence of hearing anomalies. In the at-risk group we combine AOEs with brain trunk potentials (BERA) following the stages in a decision tree diagram similar to the ones for not-at-risk children in order to provide a clinical diagnosis in the first three months of life. RESULTS: The screening performance was assessed for the 156,122 children studied, of which 151,258 belonged to the group of not-at-risk children; and 4864 to the group at risk of hypoacusia. As a result of the screening only 410 (0.26%) were sent to consultation, 213 in the not-at-risk group (0.14%) and 197 (24.7%) in the at-risk group. A total of 7452 false positives were identified (4.7%), 6951 (4.5%) in the not-at-risk group; and 501 (10.3%) in the group with risk factors; and there were 53 false negatives (0.03%). Sensitivity in the screening program was 88.5%, with a specificity of 95%. CONCLUSIONS: The Region of Murcia has a Newborn Hearing Screening Program with tests that provide a high level of sensitivity and specificity in accordance with the findings of the literature. Our results endorse the program and the patients were treated in a way that met the objective of providing a correct diagnosis and the appropriate therapeutic action.
Subject(s)
Hearing Loss/diagnosis , Hearing Tests , Neonatal Screening , Evoked Potentials, Auditory, Brain Stem , Female , Humans , Infant, Newborn , Male , Referral and Consultation , Risk Factors , Sensitivity and Specificity , SpainABSTRACT
An ischaemic infarction of the anterior inferior cerebellar artery (AICA) is usually associated with vertigo, hearing loss, facial palsy, nystagmus, or truncal ataxia; it is often accompanied by other brainstem or cerebellar signs. Sudden- onset bilateral hearing loss without associated neurological symptoms is infrequent in the literature. We report a case of sudden bilateral hearing loss, later diagnosed as AICA infarction without other symptoms.
Subject(s)
Cerebellum/blood supply , Cerebral Arteries/pathology , Hearing Loss, Bilateral/diagnosis , Hearing Loss, Bilateral/etiology , Aged , Audiometry, Pure-Tone , Brain Ischemia/pathology , Cerebellum/diagnostic imaging , Cerebrovascular Circulation/physiology , Female , Humans , Magnetic Resonance Imaging , Severity of Illness Index , Tomography, X-Ray Computed , Vertigo/diagnosis , Vertigo/etiologyABSTRACT
El infarto isquémico en el territorio de la arteria cerebelosa anteroinferior (AICA) se asocia a vértigo, hipoacusia, parálisis facial, nistagmus o ataxia, suele acompañarse de síntomas neurológicos o cerebelosos y no es infrecuente encontrar afección auditiva concomitante. La hipoacusia bilateral como signo casi único de presentación de infarto cerebeloso es infrecuente y escasamente relatado en la literatura. El caso clínico presentado plantea el diagnóstico de un infarto en el territorio de la AICA, que se inicia como una sordera brusca bilateral
An ischaemic infarction of the anterior inferior cerebellar artery (AICA) is usually associated with vertigo, hearing loss, facial palsy, nystagmus, or truncal ataxia; it is often accompanied by other brainstem or cerebellar signs. Sudden- onset bilateral hearing loss without associated neurological symptoms is infrequent in the literature. We report a case of sudden bilateral hearing loss, later diagnosed as AICA infarction without other symptoms
Subject(s)
Humans , Female , Aged , Hearing Loss, Sudden/etiology , Hearing Loss, Bilateral/diagnosis , Hearing Loss, Bilateral/etiology , Cerebral Arteries/pathology , Cerebellum/blood supply , Vertigo/diagnosis , Cerebrovascular Circulation/physiology , Brain Ischemia/pathology , Tomography, X-Ray ComputedABSTRACT
OBJECTIVE: The primary goal of this study is to determine the frequency of onset of this disorder in our infant population. Its clinical importance is due, among other reasons, to the fact that idiopathic cases constitute false negatives in the auditory screening programmes based on the performance of otoacoustic emissions to all newborn children and brainstem auditory evoked response only to those presenting these risk factors, for which reason another goal is to establish the prevalence of these pure cases and the diagnostic delay they cause. PATIENTS AND METHOD: Prospective study of all newborn children at the Virgen de la Arrixaca Mother and Child Hospital (Murcia, Spain) in the period between June 1, 2000 and June 30, 2006. RESULTS: Our screening programme, with a coverage of 95.68 %, detected 114 patients with unilateral or bilateral sensorineural hearing loss, with 6 presenting hearing loss attributable to auditory neuropathy. CONCLUSIONS: The estimated prevalence of auditory neuropathy in our infant population turned out to be 1406 for every 10 000 children, ie 5.26 % of all sensorineural hearing loss diagnosed. Another significant finding was the delay in diagnosis, since the mean age at the moment of diagnosis was of 11.5 months, mainly due to idiopathic cases not detected during neonatal screening. Two clear conclusions can be inferred from these data: a) auditory neuropathy does not constitute an extraordinarily rare disorder, and b) idiopathic cases constitute false negatives in the universal auditory screening programmes based on the performance of otoacoustic emissions, which habitually leads to a delay in diagnosis and treatment.
Subject(s)
Cochlear Nerve , Hearing Loss, Sensorineural/epidemiology , Vestibulocochlear Nerve Diseases/epidemiology , Female , Hearing Loss, Sensorineural/etiology , Humans , Infant , Male , Prevalence , Prospective Studies , Vestibulocochlear Nerve Diseases/complicationsABSTRACT
Objetivo: El objetivo principal de este estudio es determinar la frecuencia de aparición de este trastorno en nuestra población infantil. Su importancia clínica se debe, entre otros motivos, a que sus casos idiopáticos son falsos negativos de los programas de cribado auditivo basados en la realización de otoemisiones acústicas a todos los recién nacidos y potenciales evocados auditivos de tronco cerebral sólo de los que presentan dichos factores de riesgo, por lo que otro objetivo es la prevalencia de estos casos puros y el retraso diagnóstico que ocasionan. Pacientes y método: Estudio prospectivo de todos los recién nacidos del Hospital Materno-Infantil Virgen de la Arrixaca (Murcia) en el período comprendido entre el 1 de junio del 2000 y el 30 de junio del 2006. Resultados: Nuestro programa de cribado, con un índice de cobertura del 95,68 %, detectó a 114 niños con hipoacusia neurosensorial unilateral o bilateral, de los que 6 presentaron una hipoacusia neurosensorial atribuible a la neuropatía auditiva. Conclusiones: La prevalencia estimada de la neuropatía auditiva en nuestra población infantil resultó ser de 1,406 cada 10.000 niños, es decir, un 5,26 % de todas las hipoacusias neurosensoriales diagnosticadas. Otro dato relevante es el retraso diagnóstico, ya que la media de edad al momento del diagnóstico fue de 11,5 meses, fundamentalmente por los casos idiopáticos que pasaron el cribado neonatal. De estos datos podemos deducir 2 claras conclusiones: a) la neuropatía auditiva no es un trastorno extraordinariamente raro, y b) los casos idiopáticos son falsos negativos de los programas de cribado auditivo universal basados en la realización de otoemisiones acústicas, lo que habitualmente conduce a un retraso diagnóstico y, por lo tanto, terapéutico
Objective: The primary goal of this study is to determine the frequency of onset of this disorder in our infant population. Its clinical importance is due, among other reasons, to the fact that idiopathic cases constitute false negatives in the auditory screening programmes based on the performance of otoacoustic emissions to all newborn children and brainstem auditory evoked response only to those presenting these risk factors, for which reason another goal is to establish the prevalence of these pure cases and the diagnostic delay they cause. Patients and method: Prospective study of all newborn children at the Virgen de la Arrixaca Mother and Child Hospital (Murcia, Spain) in the period between June 1, 2000 and June 30, 2006. Results: Our screening programme, with a coverage of 95.68 %, detected 114 patients with unilateral or bilateral sensorineural hearing loss, with 6 presenting hearing loss attributable to auditory neuropathy. Conclusions: The estimated prevalence of auditory neuropathy in our infant population turned out to be 1406 for every 10 000 children, ie 5.26 % of all sensorineural hearing loss diagnosed. Another significant finding was the delay in diagnosis, since the mean age at the moment of diagnosis was of 11.5 months, mainly due to idiopathic cases not detected during neonatal screening. Two clear conclusions can be inferred from these data: a) auditory neuropathy does not constitute an extraordinarily rare disorder, and b) idiopathic cases constitute false negatives in the universal auditory screening programmes based on the performance of otoacoustic emissions, which habitually leads to a delay in diagnosis and treatment
