RESUMEN
BACKGROUND: Necessity of new and alternative treatments in traumatic facial nerve injury. AIMS/OBJECTIVE: In this experimental study, we aimed to evaluate the histopathologic and functional effects of methylprednisolone, hyperbaric oxygen and hesperidin + diosmin treatments on traumatic facial nerve regeneration in rats. METHODS: After facial nerve injury, five groups were formed with eight rats in each group: Group 1 (negative control), 2 (operation), 3 (corticosteroid), 4 (hyperbaric oxygen), 5 (hesperidin + diosmin). Blink reflex of rats evaluated a day after the operation and at the first, second and third weeks. Facial nerve samples from sacrificed animals were examined under a light microscope. RESULTS: According to our results, in group 4; axonal degeneration and vascular congestion were significantly lower than group 2 and 3, and myelin sheath thickness was significantly higher than group 3. In group 5; axonal degeneration was significantly lower than group 2 and vascular congestion was significantly lower than group 2 and 3. In terms of functional recovery; there was no statistically significant difference between the groups. CONCLUSIONS AND SIGNIFICANCE: It has been shown that both hyperbaric oxygen and hesperidin + diosmin treatments have positive effects on facial nerve regeneration. Both treatments may be good alternatives for ameliorating traumatic nerve injury, but these treatment modalities need to be further explored.
Asunto(s)
Diosmina , Traumatismos del Nervio Facial , Hesperidina , Oxigenoterapia Hiperbárica , Ratas , Animales , Metilprednisolona/farmacología , Hesperidina/farmacología , Diosmina/farmacología , Modelos Animales , Nervio Facial/patología , Regeneración NerviosaRESUMEN
Following facial nerve axotomy, nerve function is not fully restored even after reconstruction. This may be attributed to axon degeneration/neuronal death and sustained neuroinflammation. CD38 is an enzyme that catalyses the hydrolysis of nicotinamide adenine dinucleotide (NAD+) and is a candidate molecule for regulating neurodegeneration and neuroinflammation. In this study, we analyzed the effect of CD38 deletion and NAD+ supplementation on neuronal death and glial activation in the facial nucleus in the brain stem, and on axon degeneration and immune cell infiltration in the distal portion of the facial nerve after axotomy in mice. Compared with wild-type mice, CD38 knockout (KO) mice showed reduced microglial activation in the facial nucleus, whereas the levels of neuronal death were not significantly different. In contrast, the axon degeneration and demyelination were delayed, and macrophage accumulation was reduced in the facial nerve of CD38 KO mice after axotomy. Supplementation of NAD+ with nicotinamide riboside slowed the axon degeneration and demyelination, although it did not alter the level of macrophage infiltration after axotomy. These results suggest that CD38 deletion and supplementation of NAD+ may protect transected axon cell-autonomously after facial nerve axotomy.
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ADP-Ribosil Ciclasa 1/metabolismo , Axones/fisiología , Axotomía/métodos , Enfermedades del Nervio Facial/metabolismo , Nervio Facial/patología , NAD/metabolismo , ADP-Ribosil Ciclasa 1/genética , Animales , Recuento de Células , Células Cultivadas , Suplementos Dietéticos , Modelos Animales de Enfermedad , Enfermedades del Nervio Facial/genética , Enfermedades del Nervio Facial/terapia , Humanos , Ratones , Ratones Endogámicos ICR , Ratones Noqueados , Degeneración NerviosaRESUMEN
BACKGROUND: Bell's palsy is an idiopathic, acute peripheral palsy of the facial nerve that supplies the muscles of facial expression. Despite an expected 70% full recovery rate, up to 30% of patients are left with potentially disfiguring facial weakness, involuntary movements, or persistent lacrimation. The most frequently used treatment options are corticosteroids and antiviral drugs. However, accompanying clinical conditions, such as uncontrolled diabetes, hypertension, gastrointestinal disturbances, polypharmacy of geriatric patients, and significant sequelae ratios, indicate the need for safe and effective complementary therapies that would enhance the success of the conventional interventions. CASE SUMMARY: A 26-year-old female presented with numbness and earache on the left side of the face; these symptoms had been ongoing for 8-10h. Physical examination revealed peripheral facial paralysis of House-Brackmann grade III and corticosteroid-valacyclovir treatment was initiated. On the same day, Kinesio Taping was applied to the affected nerve and muscle area with the aim of primarily neurofacilitation and edema-pain relief. On the fifth day, acupuncture treatment was started and was continued for 3 consecutive days. A physical therapy program was administered for the subsequent 10days. At the 3-week follow-up examination, Bell's palsy was determined as grade I, and the treatment was stopped. CONCLUSION: Acupuncture and Kinesio Taping, in conjunction with physical therapy modalities, are safe and promising complementary therapies for the acute management of Bell's palsy. However, further large scale and randomized controlled studies are necessary to assess whether these complementary interventions have significant additive or synergistic effect for complete recovery of patients with Bell's palsy.
Asunto(s)
Terapia por Acupuntura , Cinta Atlética , Parálisis de Bell/terapia , Nervio Facial/patología , Modalidades de Fisioterapia , Aciclovir/análogos & derivados , Aciclovir/uso terapéutico , Corticoesteroides/uso terapéutico , Adulto , Antivirales/uso terapéutico , Parálisis de Bell/tratamiento farmacológico , Femenino , Humanos , Valaciclovir , Valina/análogos & derivados , Valina/uso terapéuticoRESUMEN
This research evaluated the influence of Photobiomodulation Therapy (PBMT) on lesions of the facial nerve repaired with the end-to-side technique or coaptation with a new heterologous fibrin sealant. Thirty-two Wistar rats were separated into 5 groups: Control group (CG), where the buccal branch of the facial nerve was collected; Experimental Suture Group (ESG) and Experimental Fibrin Group (EFG), in which the buccal branch was end-to-side sutured to the zygomatic branch on the right side of the face or coaptated with fibrin sealant on the left side; Experimental Suture Laser Group (ESLG) and Experimental Fibrin Laser Group (EFLG), in which the same procedures were performed as the ESG and EFG, associated with PBMT (wavelength of 830nm, energy density 6.2J/cm2, power output 30mW, beam area of 0.116cm2, power density 0.26W/cm2, total energy per session 2.16J, cumulative dose of 34.56J). The laser was applied for 24s/site at 3 points on the skin's surface, for a total application time of 72s, performed immediately after surgery and 3 times a week for 5weeks. A statistically significant difference was observed in the fiber nerve area between the EFG and EFLG (57.49±3.13 and 62.52±3.56µm2, respectively). For the area of the axon, fiber diameter, axon diameter, myelin sheath area and myelin sheath thickness no statistically significant differences were found (p<0.05). The functional recovery of whisker movement occurred faster in the ESLG and EFLG, which were associated with PBMT, with results closer to the CG. Therefore, PBMT accelerated morphological and functional nerve repair in both techniques.
Asunto(s)
Traumatismos del Nervio Facial/terapia , Adhesivo de Tejido de Fibrina/uso terapéutico , Animales , Nervio Facial/patología , Nervio Facial/fisiología , Nervio Facial/ultraestructura , Traumatismos del Nervio Facial/radioterapia , Adhesivo de Tejido de Fibrina/química , Láseres de Estado Sólido/uso terapéutico , Terapia por Luz de Baja Intensidad , Masculino , Microscopía Electrónica de Transmisión , Ratas , Ratas Wistar , Venenos de Serpiente/químicaRESUMEN
Objetivos: Determinar las características de los grados de afectación del nervio facial y la relación con la discapacidad facial en pacientes con parálisis facial periférica de un año de evolución. Materiales y Métodos: Estudio de tipo observacional, correlacional y transversal. Se estudió una población de pacientes con diagnóstico de parálisis facial periférica durante el año 2014. Se seleccionó una muestra inicial de 56 pacientes a través de historias clínicas. Los criterios de inclusión fueron pacientes con lesión nerviosa del tipo axonotmesis (Clasificación de Seddon), ser mayores de 18 años, sin antecedentes de parálisis facial periférica, sin alteración de la capacidad mental o haber recibido tratamiento por toxina botulínica. Se evaluó a 43 de ellos 22 mujeres y 21 varones, usando la escala House-Brackmann 2.0 y el índice de Discapacidad Facial, posteriormente se describieron las características según sexo, edad y se determinó la relación entre ambas medidas de evaluación usando el coeficiente correlación de Pearson. Resultados: Se encontró una fuerte correlación entre los grados de afectación del nervio facial y el índice de discapacidad facial subescala física (r= -0.85, p<0,001), para los grados de afectación del nervio facial y el índice de discapacidad facial subescala social (r= -0.74, p<0,001). Los participantes mayores de 60 años reflejaron puntajes superiores en el grado de afectación nerviosa y mayor discapacidad facial. Así mismo no se halló diferencia importante entre varones y mujeres para los grados de afectación del nervio facial. Conclusiones: Se demostró la correlación negativa entre los grados de afectación del nervio facial y la Discapacidad Facial, a mayor grado de afección nerviosa facial se obtendrá puntajes inferiores en el cuestionario de autoevaluación de discapacidad. La inclusión de estas herramientas en la evaluación fisioterapéutica permitiría conocer además los cambios físico - sociales que conllevan a la discapacidad...
Objectives: To determine the characteristics of the degrees of affection of the facial nerve and the relation with disability in patients with facial paralysis with 1 year of evolution. Materials and methods: Observational, correlational and cross-sectional study. A population of patients diagnosed with peripheral facial paralysis was studied during 2014. A first sample of 56 patients was chose through clinical records. Inclusion criteria were: patients with Axonotmesís type injury (Seddon's classification), older than 18 years, without history of peripheral facial paralysis, not mental capacity disorder or having received treatment for botulinum toxin. 43 of them were evaluated - 21 women and 22 men - using the Facial Nerve Grading System 2.0 (FNGS2.0) and the Facial Disability Index. Characteristics according to sex and age were subsequently described and the relation between both evaluation measures was determined using the Pearson correlation coefficient. Results: A strong correlation between the degrees of affection of facial nerve and Facial Disability Index for the physical subscale was found, (r= -0.85, p<0.001) for the degrees of affection of the facial nerve and the Facial Disability Index for social subscale (r= -0.74, p<0.001). The participants older than 60 years reflected higher scores in the degrees of affection and a higher facial disability. In addition, there were no differences between degrees of affection in men and women. Conclusions: The level of nerve affection is associated with facial disability. And this association is higher with the physic disability dimension. Including these tools in physiotherapeutic evaluation would make possible to acknowledge physical and social changes that lead to disability as a result of facial nerve injury.
Asunto(s)
Masculino , Femenino , Humanos , Adulto , Persona de Mediana Edad , Anciano , Evolución Clínica , Manifestaciones Neurológicas , Nervio Facial/patología , Parálisis Facial , Estudios Observacionales como Asunto , Estudios TransversalesRESUMEN
Bell palsy is an acute affliction of the facial nerve, resulting in sudden paralysis or weakness of the muscles on one side of the face. Testing patients with unilateral facial paralysis for diabetes mellitus or Lyme disease is not routinely recommended. Patients with Lyme disease typically present with additional manifestations, such as arthritis, rash, or facial swelling. Diabetes may be a comorbidity of Bell palsy, but testing is not needed in the absence of other indications, such as hypertension. In patients with atypical symptoms, magnetic resonance imaging with contrast enhancement can be used to rule out cranial mass effect and to add prognostic value. Steroids improve resolution of symptoms in patients with Bell palsy and remain the preferred treatment. Antiviral agents have a limited role, and may improve outcomes when combined with steroids in patients with severe symptoms. When facial paralysis is prolonged, surgery may be indicated to prevent ocular desiccation secondary to incomplete eyelid closure. Facial nerve decompression is rarely indicated or performed. Physical therapy modalities, including electrostimulation, exercise, and massage, are neither beneficial nor harmful.
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Antivirales/uso terapéutico , Parálisis de Bell , Nervio Facial/patología , Glucocorticoides/uso terapéutico , Prednisona/uso terapéutico , Parálisis de Bell/diagnóstico , Parálisis de Bell/tratamiento farmacológico , Parálisis de Bell/fisiopatología , Medicina Basada en la Evidencia , Nervio Facial/cirugía , Humanos , Imagen por Resonancia Magnética , Modalidades de FisioterapiaRESUMEN
Patients suffering from facial paralysis are on the hazard of disfigurement and loss of vision due to loss of blink function. Functional-electrical stimulation (FES) is one possible way of restoring blink and other functions in these patients. A blink restoration system for uni-lateral facial paralyzed patients is described in this paper. The system achieves restoration of synchronized blink through processing the myoelectric signal of orbicularis oculi at the normal side in real-time as the trigger to stimulate the paralyzed eyelid. Design issues are discussed, including EMG processing, stimulating strategies and real-time artifact blanking. Two artifact removal approaches based on sample and hold and digital filtering technique are proposed and implemented. Finally, the whole system has been verified on rabbit models.
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Parpadeo/fisiología , Terapia por Estimulación Eléctrica , Electromiografía , Procesamiento de Señales Asistido por Computador , Algoritmos , Animales , Artefactos , Estimulación Eléctrica , Electrónica , Párpados/fisiopatología , Nervio Facial/patología , Parálisis Facial/fisiopatología , Parálisis Facial/terapia , Músculos Oculomotores/fisiopatología , Conejos , Reproducibilidad de los Resultados , Programas InformáticosRESUMEN
Using a combined morphofunctional approach, we recently found that polyinnervation of the neuromuscular junction (NMJ) is the critical factor for recovery of function after transection and suture of the facial nerve. Since polyinnervation is activity-dependent and can be manipulated, we tried to design a clinically feasible therapy by electrical stimulation or by soft tissue massage. First, electrical stimulation was applied to the transected facial nerve or to paralyzed facial muscles. Both procedures did not improve vibrissal motor performance (video-based motion analysis of whisking), failed to diminish polyinnervation, and even reduced the number of innervated NMJ to one-fifth of normal values. In contrast, gentle stroking of the paralyzed vibrissal muscles by hand resulted in full recovery of whisking. Manual stimulation depended on the intact sensory supply of the denervated muscle targets and was also effective after hypoglossal-facial anastomosis, after interpositional nerve grafting, when applied to the orbicularis oculi muscle and after transection and suture of the hypoglossal nerve. From these results, we conclude that manual stimulation is a noninvasive procedure with immediate potential for clinical rehabilitation following facial nerve reconstruction.
Asunto(s)
Músculos Faciales/patología , Músculos Faciales/fisiopatología , Parálisis/fisiopatología , Parálisis/rehabilitación , Animales , Axones/metabolismo , Terapia por Estimulación Eléctrica , Músculos Faciales/inervación , Nervio Facial/patología , Nervio Facial/fisiopatología , Humanos , Microtúbulos/metabolismo , Actividad Motora/fisiología , Desnervación Muscular , Ratas , Recuperación de la Función/fisiología , Vibrisas/patologíaRESUMEN
CONCLUSION: Hyperbaric oxygen treatment (HBOT) promoted an increase of the mean axonal diameter in the group evaluated 2 weeks after lesion induction, which suggests a more advanced regeneration process. However, the number of myelin nerve fibers of the facial nerve of the rabbits was similar when compared to the control and treatment groups, in both evaluation periods. OBJECTIVE: To evaluate the effect of HBOT on the histological pattern of the facial nerve in rabbits exposed to a nerve crush injury. MATERIALS AND METHODS: Twenty rabbits were exposed to facial nerve crush injury. Ten rabbits received HBOT, 10 rabbits comprised the control group. The rabbits were sacrificed 2 and 4 weeks after the trauma. Qualitative morphological analysis, measurement of the external axonal diameters and myelin fiber count were carried out in an area of 185 000 microm2. RESULTS: There was an increase in the area of the axons and thicker myelin in the 2 weeks treatment group in comparison with the control group. The mean diameter of the axons was of 2.34 microm in the control group and of 2.81 microm in the HBOT group, with statistically significant differences. The 2 week control group had a mean number of myelin fibers of 1865.2 +/- 664, and the HBOT group had a mean number of 2026.3 +/- 302; this was not statistically significant. The 4 week control group presented a mean of 2495.1 +/- 479 fibers and the HBOT group presented a mean of 2359.9 +/- 473; this was not statistically significant.
Asunto(s)
Traumatismos del Nervio Facial/patología , Traumatismos del Nervio Facial/terapia , Oxigenoterapia Hiperbárica , Regeneración Nerviosa , Animales , Axones/patología , Axones/fisiología , Nervio Facial/patología , Nervio Facial/fisiopatología , Traumatismos del Nervio Facial/fisiopatología , Masculino , Vaina de Mielina/patología , Vaina de Mielina/fisiología , Compresión Nerviosa , Conejos , Factores de TiempoRESUMEN
OBJECTIVE: To investigate the protective effect of Mailuoning on the facial nerve demyelination of Hemifacial spasm and provide the data for therapy of Hemifacial spasm. METHOD: 24 New Zealand white rabbits were divided into control group, Saline group and Mailuoning group, on the latter two groups the models of Hemifacial spasm were made by the temporal superficial artery closely contacting the main trunk of facial nerve at stylomastoid foramen. From the 5th week, the Saline and Mailuoning were injected intravenously into ear margin for 2 weeks on Saline and Mailuoning group respectively. At the 7th week, the MDA and SOD in serum were measured, mean while the microstructure and ultrastructure of facial nerve were observed on 3 animal groups. RESULT: The MDA decreased obviously (P < 0.05) and SOD increased significantly (P < 0.01) in Mailuoning group comparing with that of Saline group, while the MDA and SOD showed insignificant changes of Mailuoning group and control group. The facial nerve severely demyelinated and axons retrogressively changed in Saline group but mild in Mailuoning group. CONCLUSION: Mailuoning injection has a significant protective effect on the facial nerve demyelination of Hemifacial spasm and the very important applied value for therapy of Hemifacial spasm.
Asunto(s)
Enfermedades Desmielinizantes/tratamiento farmacológico , Medicamentos Herbarios Chinos/farmacología , Nervio Facial/ultraestructura , Espasmo Hemifacial/tratamiento farmacológico , Fármacos Neuroprotectores/farmacología , Plantas Medicinales , Animales , Enfermedades Desmielinizantes/patología , Combinación de Medicamentos , Medicamentos Herbarios Chinos/administración & dosificación , Medicamentos Herbarios Chinos/aislamiento & purificación , Nervio Facial/patología , Femenino , Espasmo Hemifacial/patología , Inyecciones Intravenosas , Masculino , Fármacos Neuroprotectores/administración & dosificación , Plantas Medicinales/química , ConejosRESUMEN
Therapeutic vaccination with Copaxone (glatiramer acetate, Cop-1) protects motor neurons against acute and chronic degenerative conditions. In acute degeneration after facial nerve axotomy, the number of surviving motor neurons was almost two times higher in Cop-1-vaccinated mice than in nonvaccinated mice, or in mice injected with PBS emulsified in complete Freund's adjuvant (P < 0.05). In mice that express the mutant human gene Cu/Zn superoxide dismutase G93A (SOD1), and therefore simulate the chronic human motor neuron disease amyotrophic lateral sclerosis, Cop-1 vaccination prolonged life span compared to untreated matched controls, from 211 +/- 7 days (n = 15) to 263 +/- 8 days (n = 14; P < 0.0001). Our studies show that vaccination significantly improved motor activity. In line with the experimentally based concept of protective autoimmunity, these findings suggest that Cop-1 vaccination boosts the local immune response needed to combat destructive self-compounds associated with motor neuron death. Its differential action in CNS autoimmune diseases and neurodegenerative disorders, depending on the regimen used, allows its use as a therapy for either condition. Daily administration of Cop-1 is an approved treatment for multiple sclerosis. The protocol for non-autoimmune neurodegenerative diseases such as amyotrophic lateral sclerosis, remains to be established by future studies.
Asunto(s)
Esclerosis Amiotrófica Lateral/terapia , Enfermedad de la Neurona Motora/terapia , Péptidos/inmunología , Vacunas/uso terapéutico , Enfermedad Aguda , Esclerosis Amiotrófica Lateral/genética , Esclerosis Amiotrófica Lateral/inmunología , Animales , Axotomía , Muerte Celular , Enfermedad Crónica , Modelos Animales de Enfermedad , Nervio Facial/inmunología , Nervio Facial/patología , Nervio Facial/fisiopatología , Femenino , Acetato de Glatiramer , Ácido Glutámico/toxicidad , Humanos , Ratones , Ratones Endogámicos C57BL , Ratones Mutantes , Enfermedad de la Neurona Motora/genética , Enfermedad de la Neurona Motora/inmunología , Neuronas Motoras/inmunología , Neuronas Motoras/patología , Neuronas Motoras/fisiología , Degeneración Nerviosa/inmunología , Degeneración Nerviosa/patología , Degeneración Nerviosa/fisiopatología , Degeneración Nerviosa/prevención & control , Péptidos/uso terapéutico , Superóxido Dismutasa/genética , Superóxido Dismutasa-1RESUMEN
Many of the acupuncture points on the face, according to classic acupuncture textbooks, could be used to treat facial pain, paralysis, and toothache. But it is not specified which acupuncture point would be effective for which tooth. Many of these points, when used for the treatment of dental pain, often fail. From an anatomical point of view, we describe which acupuncture point to use for each specific area for effective treatment of dental pain. Also, we emphasize the importance of the depth of needle insertion, especially the necessity to touch the facial bone, for effective pain treatment. When performed properly, acupuncture can be very effective for relief of dental pain.
Asunto(s)
Analgesia por Acupuntura/métodos , Puntos de Acupuntura/clasificación , Nervio Facial/patología , Dolor Facial/terapia , Cabeza/inervación , Cuello/inervación , Odontalgia/terapia , Nervio Trigémino/patología , Cabeza/patología , Humanos , Cuello/patología , Pautas de la Práctica en MedicinaRESUMEN
OBJECT: Neuronal survival is an important factor in the achievement of functional restitution after peripheral nerve injuries. Intracranial tumors or trauma may cause patients to exhibit a temporary or permanent facial nerve palsy. Nimodipine, which acts as an antagonist to L-type voltage-gated calcium channels, has been shown to be neuroprotective in various lesion models of the central and peripheral nervous systems. The aim of the present study was to evaluate the effect of nimodipine on motor neuron survival in the facial motor nucleus following intracranial transection of the adult rat facial nerve. METHODS: The facial nerve was cut intracranially in the posterior cranial fossa. Nimodipine was administered orally preoperatively for 3 days and postoperatively for up to 1 month, after which the number of neuronal profiles was quantified. The glial reaction was studied in the facial nucleus for up to 1 month by using immunocytochemical analysis. There was a significantly larger proportion of surviving motor neurons 1 month postinjury in animals treated with nimodipine (61+/-6.7%) in comparison with untreated animals (26.8+/-11.3%). Immunocytochemical analysis showed an increase in the amount of OX42 (microglia), ED1 (macrophages), and anti-glial fibrillary acidic protein (astrocytes) ipsilateral to the nerve injury; however, there was no difference between the two experimental groups of animals 2 to 28 days after surgery. CONCLUSIONS: The authors propose a neuroprotective role for nimodipine, which may be useful as a "cranial nerve protective agent" following insults such as head injury or skull base surgery.
Asunto(s)
Tronco Encefálico/cirugía , Bloqueadores de los Canales de Calcio/uso terapéutico , Nervio Facial/efectos de los fármacos , Neuronas Motoras/efectos de los fármacos , Fármacos Neuroprotectores/uso terapéutico , Nimodipina/uso terapéutico , Administración Oral , Animales , Astrocitos/efectos de los fármacos , Astrocitos/patología , Tronco Encefálico/efectos de los fármacos , Bloqueadores de los Canales de Calcio/administración & dosificación , Recuento de Células , Supervivencia Celular , Fosa Craneal Posterior , Nervio Facial/patología , Nervio Facial/cirugía , Parálisis Facial/prevención & control , Estudios de Seguimiento , Proteína Ácida Fibrilar de la Glía/análisis , Inmunohistoquímica , Macrófagos/efectos de los fármacos , Macrófagos/patología , Masculino , Microglía/efectos de los fármacos , Microglía/patología , Neuronas Motoras/patología , Vías Nerviosas/efectos de los fármacos , Vías Nerviosas/cirugía , Neuroglía/efectos de los fármacos , Neuroglía/patología , Fármacos Neuroprotectores/administración & dosificación , Nimodipina/administración & dosificación , Ratas , Ratas Sprague-DawleyRESUMEN
An interesting case is reported of an 11-year-old girl who experienced a sudden, profound right-sided hearing loss at the age of 5 years after hearing a "pop" and the sound of rushing water in her right ear. At that time the patient was thought to have Mondini's deformity, and a perilymphatic fistula of the oval window in the right ear. Six years later she was diagnosed with a juvenile pilocytic astrocytoma of the pons with an exophytic component extending into the cerebellopontine angle. Of particular interest in this case is the presence of evoked otoacoustic emissions in the right ear with a profound neural hearing loss. The presence of transient evoked and distortion product otoacoustic emissions confirmed normal sensory outer hair cell function and an intact peripheral auditory system in a clinically deaf ear, thus indicating hearing loss due to a neural component instead of a sensory component, which was previously assumed.