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1.
J Clin Med ; 13(11)2024 Jun 04.
Artigo em Inglês | MEDLINE | ID: mdl-38893014

RESUMO

Objectives: The purpose of this study was to investigate the hearing characteristics and causes of sudden sensorineural hearing loss (SSNHL) in patients aged from 15 to 40 years, focusing on audiological outcomes one year after the diagnosis. Methods: The medical records of individuals with SSNHL who were referred to our tertiary-level audiologic center were reviewed. All patients had undergone comprehensive diagnostic evaluations, including high-resolution 3D-FLAIR delayed magnetic resonance imaging (MRI), cone beam computed tomography (CBCT), and screening for coagulation, infectious, and autoimmune diseases. Results: Overall, 56 patients (mean age 28.1 ± 7.6 years) were included in the study. The hearing threshold in the affected ear improved significantly from 56.0 ± 18.0 dB at the diagnosis to 46.9 ± 22.3 dB after one year (p = 0.02). The degree of hearing loss, audiometric configurations, hearing improvements, and adherence to hearing treatments showed considerable variability among patients. Aural fullness, tinnitus, and hyperacusis were the predominant symptoms associated with SSNHL, and their prevalence decreased significantly over time. The diagnostic protocol led to the identification of the specific cause of SSNHL in 75% (42/56) of patients. The known etiology was found to be otological (39.3%), infectious (21.4%), autoimmune (7.1%), vascular (5.4%), or neoplastic (1.8%). In particular, Menière's disease (n = 12), isolated cochlear endolymphatic hydrops (n = 6), HSV-1 (n = 5), and EBV (n = 4) infections were the most frequent causes of SSNHL. Conclusions: The identification of the specific etiology of SSNHL may facilitate a more personalized approach to management and treatment.

2.
Artigo em Inglês | MEDLINE | ID: mdl-38613183

RESUMO

OBJECTIVE: To assess the prevalence of otitis media with effusion (OME) among children who attended a tertiary level audiologic center 2 and 3 years after the COVID-19 lockdown, and to determine the impact of temporary interruption of day care center attendance on chronic OME. STUDY DESIGN: Retrospective study. SETTING: Tertiary level referral audiologic center. METHODS: We assessed the prevalence of OME among children aged 6 months to 12 years in 3 different periods (May-June 2022, January-February 2023, and May-June 2023) and compared the results with those of the corresponding periods before the COVID-19 lockdown. We also compared the disease resolution rates between a subgroup of children with chronic OME who interrupted day care center attendance for a 2-month period (Subgroup A) and a similar subgroup who continued attending day care centers (Subgroup B). RESULTS: The prevalence of OME was 38.5% (138/358) in May-June 2022, 51.9% (193/372) in January-February 2023, and 40.9% (149/364) in May-June 2023. No significant prevalence differences were observed between the periods May-June 2019, May-June 2022, and May-June 2023 (P = .78), and between the periods January-February 2020 and January-February 2023 (P = .93). At the May-June 2023 assessment, the children belonging to Subgroup A presented a greater rate of disease resolution (85.7%, 18/21) than the children belonging to Subgroup B (32%, 8/25, P < .001). CONCLUSION: This study suggests that the prevalence of OME has returned to prelockdown levels, and that interrupting day care center attendance for a 2-month period could be effective in resolving most cases of chronic OME.

3.
Acta Otorhinolaryngol Ital ; 43(Suppl. 1): S67-S75, 2023 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-37698103

RESUMO

Objective: To establish the safety and effectiveness of subtotal petrosectomy with cochlear implantation in patients affected by chronic middle ear disorders to refractory to previous surgical treatments. Methods: A multicentre, retrospective study was conducted on patients affected by recalcitrant chronic middle ear disorders who underwent cochlear implantation in combination with subtotal petrosectomy. Patients' details were collected from databases of 11 Italian tertiary referral centres. Additionally, a review of the most updated literature was carried out. Results: 55 patients were included with a mean follow-up time of 44 months. Cholesteatoma was the most common middle ear recurrent pathology and 50.9% of patients had an open cavity. 80% of patients underwent a single stage surgery. One case of explantation for device failure was reported among the 7 patients with post-operative complications. Conclusions: Subtotal petrosectomy with cochlear implantation is a benchmark for management of patients with recalcitrant chronic middle ear disorders. A single stage procedure is the most recommended strategy. Optimal follow-up is still debated. Further studies are required to investigate the role of this surgery in paediatric patients.


Assuntos
Colesteatoma , Implante Coclear , Otite Média Supurativa , Humanos , Orelha Média/cirurgia , Estudos Retrospectivos
4.
Front Surg ; 8: 700271, 2021.
Artigo em Inglês | MEDLINE | ID: mdl-34497826

RESUMO

The diagnosis of "definite" Méniére's disease (MD) relies upon its clinical manifestations. MD has been related with Endolymphatic Hydrops (EH), an enlargement of the endolymphatic spaces (ES) (cochlear duct, posterior labyrinth, or both). Recent advances in Magnetic Resonance (MR) imaging justify its increasing role in the diagnostic workup: EH can be consistently recognized in living human subjects by means of 3-dimensional Fluid-Attenuated Inversion-Recovery sequences (3D-FLAIR) acquired 4 h post-injection of intra-venous (i.v.) Gadolinium-based contrast medium, or 24 h after an intratympanic (i.t.) injection. Different criteria to assess EH include: the comparison of the area of the vestibular ES with the whole vestibule on an axial section; the saccule-to-utricle ratio ("SURI"); and the bulging of the vestibular organs toward the inferior 1/3 of the vestibule, in contact with the stapedial platina ("VESCO"). An absolute link between MD and EH has been questioned, since not all patients with hydrops manifest MD symptoms. In this literature review, we report the technical refinements of the imaging methods proposed with either i.t. or i.v. delivery routes, and we browse the outcomes of MR imaging of the ES in both MD and non-MD patients. Finally, we summarize the following imaging findings observed by different researchers: blood-labyrinthine-barrier (BLB) breakdown, the extent and grading of EH, its correlation with clinical symptoms, otoneurological tests, and stage and progression of the disease.

5.
Otolaryngol Head Neck Surg ; 165(5): 710-715, 2021 11.
Artigo em Inglês | MEDLINE | ID: mdl-33494659

RESUMO

OBJECTIVE: To evaluate the role of social isolation during the lockdown due to the SARS-CoV-2 outbreak (severe acute respiratory syndrome coronavirus 2) in modifying the prevalence of otitis media with effusion (OME) and the natural history of chronic OME. STUDY DESIGN: Retrospective study. SETTING: Tertiary level referral audiologic center. METHODS: We assessed the prevalence of OME among children aged 6 months to 12 years who attended the outpatient clinic for hearing or vestibular disorders during 2 periods before the lockdown, May-June 2019 (n = 350) and January-February 2020 (n = 366), and the period immediately after the lockdown, May-June 2020 (n = 216). We also compared the disease resolution rates between a subgroup of children with chronic OME (n = 30) who were diagnosed in summer 2019 and reevaluated in May-June 2020 and a similar subgroup (n = 29) assessed in 2018-2019. RESULTS: The prevalence of OME in this clinic population was 40.6% in May-June 2019, 52.2% in January-February 2020, and 2.3% in May-June 2020. Children with chronic OME had a higher rate of disease resolution in May-June 2020 (93.3%) than those examined in May-June 2019 (20.7%, P < .001). CONCLUSION: Closure of schools and the physical distancing rules were correlated with a reduction in the prevalence of OME and favored the resolution of its chronic forms among children who attended the outpatient clinic. These data could suggest that in the presence of chronic OME, keeping young children out of group care settings for a period might be beneficial to allow for OME resolution.


Assuntos
COVID-19 , Otite Média com Derrame/epidemiologia , Quarentena , Criança , Pré-Escolar , Doença Crônica , Feminino , Previsões , Humanos , Lactente , Masculino , Otite Média com Derrame/terapia , Estudos Retrospectivos
6.
Ann Otol Rhinol Laryngol ; 130(1): 112-115, 2021 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-32613850

RESUMO

OBJECTIVE: To report presentation, diagnostic process, management and outcome of a case of autoimmune inner ear disease (AIED) related with Churg-Strauss syndrome, also known as eosinophilic granulomatosis with polyangiitis (EGPA), treated with cochlear implantation, and review of relevant literature. CASE PRESENTATION AND MANAGEMENT: A retrospective case report of AIED associated with EGPA treated with cochlear implantation was described. A multi-step approach for diagnosis and confirmation of AIED and hearing rehabilitation was conducted, eventually leading to left cochlear implantation. RESULTS: The surgery was without complications and postoperative course was uneventful. Two years after surgery, pure-tone and speech soundfield audiometry with left cochlear implant switched on showed a good improvement in pure-tone threshold and a word recognition score of 50% at 60 dB nHL. Literature review does not report any previous case of AIED EGPA-related. CONCLUSIONS: Cochlear implantation in AIED EGPA-related have been shown to be a viable treatment option in a stabilized phase of disease.


Assuntos
Síndrome de Churg-Strauss/complicações , Implante Coclear , Perda Auditiva Neurossensorial/etiologia , Perda Auditiva Súbita/etiologia , Adulto , Feminino , Perda Auditiva Neurossensorial/cirurgia , Perda Auditiva Súbita/cirurgia , Humanos
7.
Acta Biomed ; 89(3): 382-388, 2018 10 08.
Artigo em Inglês | MEDLINE | ID: mdl-30333463

RESUMO

BACKGROUND AND AIM: As in other syndromes characterized by craniofacial anomalies, middle ear cholesteatoma is known to have a high prevalence in Turner syndrome. The aim of this study was to review a multicenter experience with the surgical management of middle ear cholesteatoma in children with Turner syndrome. METHODS: We retrospectively analyzed sixteen girls with Turner syndrome who underwent otologic surgery for middle ear cholesteatoma between January 2000 and December 2012. Surgery was performed in 3 tertiary care otologic centers. Four patients had bilateral disease, resulting in a total of 20 ears treated. The following data were recorded: age, history of ventilation tube insertion, status of the controlateral ear, cholesteatoma location and extension, and surgical technique involved. Cholesteatoma recidivism, stable mastoid cavity and hearing levels were the main outcomes measured. RESULTS: Follow-up ranged from 3 to 15 years (mean 7 years). Fourteen ears underwent canal wall down mastoidectomy: no cases of recurrent cholesteatoma were observed in these cases; revision mastoidectomy with cavity obliteration was needed in 2 ears (14.3%) for recurrent otorrhea. In the remaining 6 ears a staged canal wall up mastoidectomy was performed: 1 child showed a recurrent cholesteatoma and required conversion to canal wall down mastoidectomy. A postoperative air-bone gap result of 0 to 20 dB was achieved in 6 ears (30%); in 9 ears (45%) postoperative air-bone gap was between 21 and 30 dB, while in 5 (25%) was >30 dB. Bone conduction thresholds remained unaffected in all cases. CONCLUSIONS: Cholesteatoma in children with Turner syndrome is a challenging entity for the otologic surgeon. Although not mandatory, canal wall down mastoidectomy should be regarded as the technique of choice to achieve a safe and dry ear in TS children with middle ear cholesteatoma. Intact canal wall mastoidectomy should be adopted only in appropriately selected patients such as those with limited attic cholesteatoma that can be regularly followed-up.


Assuntos
Colesteatoma da Orelha Média/cirurgia , Mastoidectomia/métodos , Síndrome de Turner/complicações , Adolescente , Condução Óssea , Criança , Colesteatoma da Orelha Média/diagnóstico por imagem , Colesteatoma da Orelha Média/epidemiologia , Colesteatoma da Orelha Média/etiologia , Feminino , Seguimentos , Humanos , Lactente , Recém-Nascido , Masculino , Recidiva , Estudos Retrospectivos , Cirurgia de Second-Look , Centros de Atenção Terciária , Tomografia Computadorizada por Raios X , Resultado do Tratamento
8.
J Int Adv Otol ; 14(2): 233-238, 2018 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-30256197

RESUMO

OBJECTIVES: The aim of this study was to define the typical pattern for varicella zoster virus (VZV) reactivation in delayed facial palsy (DFP) after stapedectomy for otosclerosis. MATERIALS AND METHODS: Review of the relevant literature, personal casistics, and case-report. RESULTS: In total, 48 cases of DFP after stapes surgery have been described so far, including the reported case with exclusive manifestation of atypical Ramsay Hunt syndrome (RH); in the personal series of 1253 stapedectomies, DFP occurred in only one case (0.08%). Complete DFP (House-Brackmann grade VI) rapidly developed 12 days after surgery; RH appeared 2 days later, confirming the role of VZV. The DFP started improving after 8 weeks and completely recovered 6 months later. CONCLUSION: Acute otalgia prior to DFP should raise the suspicion of VZV reactivation. Atypical RH is the most frequent pattern that occurs in DFP after stapedectomy.


Assuntos
Paralisia Facial/etiologia , Herpes Zoster da Orelha Externa/etiologia , Otosclerose/cirurgia , Cirurgia do Estribo/efeitos adversos , Zoster Sine Herpete/diagnóstico , Aciclovir/administração & dosagem , Aciclovir/uso terapêutico , Antivirais/uso terapêutico , Nervo Facial/patologia , Nervo Facial/virologia , Paralisia Facial/diagnóstico , Paralisia Facial/fisiopatologia , Paralisia Facial/virologia , Feminino , Herpes Zoster da Orelha Externa/classificação , Herpes Zoster da Orelha Externa/diagnóstico , Herpes Zoster da Orelha Externa/tratamento farmacológico , Herpesvirus Humano 3/isolamento & purificação , Herpesvirus Humano 3/patogenicidade , Humanos , Incidência , Pessoa de Meia-Idade , Otosclerose/classificação , Complicações Pós-Operatórias/etiologia , Complicações Pós-Operatórias/virologia , Estudos Retrospectivos , Cirurgia do Estribo/métodos , Fatores de Tempo , Resultado do Tratamento , Ativação Viral , Zoster Sine Herpete/complicações , Zoster Sine Herpete/tratamento farmacológico
9.
Eur J Radiol ; 106: 106-113, 2018 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-30150031

RESUMO

OBJECTIVE: We compared the image quality and radiation dose of flat-panel CT (FPCT) and multi-slice CT (MSCT) performed respectively with an angiographic unit and a 128-slice CT scanner. We investigated whether the higher spatial resolution of FPCT translated into higher image quality and we sought to eliminate inter-subject variability by scanning temporal bone specimens with both techniques. MATERIALS AND METHODS: Fifteen temporal bone specimens were imaged with FPCT and MSCT. Two neuroradiologists experienced in otoradiology evaluated 30 anatomical structures with a 0-2 score; 18 structures important from a clinical perspective were assigned a twofold value in calculation of the overall score. The radiation dose was calculated through the use of an anthropomorphic phantom. RESULTS: The image quality was significantly higher for FPCT than MSCT for 10 of the 30 anatomical structures; the overall score was also significantly higher for FPCT (p = 0.001). The equivalent dose of the two techniques was very similar, but with different effective doses to the organs. CONCLUSION: FPCT performed on an angiographic unit provides higher image quality in temporal bone assessment compared to MSCT performed on a 128-slice CT scanner thanks to its higher spatial resolution, with comparable equivalent doses but different effective doses to the organs.


Assuntos
Doses de Radiação , Osso Temporal/anatomia & histologia , Tomógrafos Computadorizados , Tomografia Computadorizada por Raios X/instrumentação , Tomografia Computadorizada por Raios X/métodos , Cadáver , Humanos
10.
Eur Arch Otorhinolaryngol ; 275(8): 1987-1993, 2018 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-29971494

RESUMO

OBJECTIVE: To report neuroradiologic findings, surgical strategies and clinical and audiological results in a series of children with CHARGE syndrome (CS) who had been evaluated for cochlear implantation (CI). STUDY DESIGN: Retrospective case series. SETTING: Tertiary referral university hospital. PATIENTS: Eight profoundly deafened children with CS were included. Routine audiometric measurements, speech perception categories and speech intelligibility ratings were performed pre- and post-operatively. Neuroradiological and intraoperative findings, surgical planning, and post-operative complications were analyzed. RESULTS: Six children were profoundly deaf from birth and 2 had progressive hearing loss to profound levels. Cochlear nerve deficiency (CND) was noted in 5 out of the 6 patients with congenital sensorineural hearing loss (SNHL). Seven children underwent CI. Surgery was performed using standard transmastoid facial recess approach in 3 ears, subtotal petrosectomy in 3, and transmastoid single-slit labyrinthotomy in one. Temporary facial palsy occurred in one patient. In the group of patients with congenital SNHL, 2 children benefitted from CI and developed spoken language; the remaining 3 children obtained improved access to environmental sounds and used signs and gestures as their main mode of communication. The two patients with progressive SNHL had preoperative verbal language and continued to use verbal language after CI. CONCLUSIONS: The constant presence of temporal bone anomalies in children with CS requires surgical expertise in performing non-standard approaches for safe and effective CI. Patients with progressive SNHL and normal cochlear nerves had satisfactory results with CI. Limited benefits have been observed in presence of CND.


Assuntos
Síndrome CHARGE/cirurgia , Implante Coclear , Surdez/cirurgia , Adolescente , Síndrome CHARGE/complicações , Criança , Pré-Escolar , Nervo Coclear/anormalidades , Feminino , Perda Auditiva Neurossensorial/congênito , Perda Auditiva Neurossensorial/cirurgia , Humanos , Lactente , Desenvolvimento da Linguagem , Masculino , Estudos Retrospectivos , Língua de Sinais , Osso Temporal/anormalidades
11.
Am J Case Rep ; 19: 82-89, 2018 Jan 23.
Artigo em Inglês | MEDLINE | ID: mdl-29358571

RESUMO

BACKGROUND Implantable devices have been proposed as an alternative to hearing aids and auditory canal reconstruction in patients with microtia (congenital aural atresia), which includes a malformation of the external and middle ear. This report is of two rare cases of microtia associated with congenital stapes ankylosis treated with an implantable device and describes the treatment outcomes. CASE REPORT Two siblings from Ecuador, a 29-year-old woman, and her 35-year-old brother, were born with unilateral type II microtia with bilateral external auditory canal atresia and conductive hearing loss. Pre-operatively, high-resolution computed tomography (HRCT) imaging was performed using FastView software to allow placement of a bone conduction-floating mass transducer (BC-FMT) to couple a Bonebridge bone conduction implant (BCI) system in both patients. Pure-tone audiometry (PTA) testing and speech audiology were performed. The Abbreviated Profile of Hearing Aid Benefit (APHAB) and the Speech, Spatial and Qualities (SSQ) of hearing scale questionnaires and scoring systems were used. Following activation of the implantable device, both patients achieved improved bilateral conductive hearing with sound-field (field-free) thresholds >25 dB, and speech recognition scores >90%. In both cases, hearing improvement remained at three years following surgery. CONCLUSIONS To our knowledge, these are the first reported cases of microtia with congenital stapes ankylosis successfully treated with a bone conduction implantable device. Patients with microtia and stapes ankylosis who are reluctant to undergo surgery may benefit from unilateral or bilateral, short-term or long-term use of a Bonebridge bone conduction implantable device.


Assuntos
Anquilose/complicações , Condução Óssea , Microtia Congênita/complicações , Auxiliares de Audição , Perda Auditiva Condutiva/terapia , Estribo , Adulto , Feminino , Perda Auditiva Condutiva/etiologia , Humanos , Masculino
12.
Neuroradiol J ; 31(1): 39-41, 2018 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-29046124

RESUMO

We report a case of a 57-year-old man with bilateral masses in the internal auditory canal. The peculiar findings at magnetic resonance imaging with tridimensional fluid-attenuated inversion recovery sequence combined with clinical data provided new insights into understanding the pathophysiology of the hearing loss.


Assuntos
Orelha Interna/patologia , Perda Auditiva Neurossensorial/diagnóstico por imagem , Perda Auditiva Neurossensorial/etiologia , Perda Auditiva Súbita/diagnóstico por imagem , Perda Auditiva Súbita/etiologia , Imageamento Tridimensional , Imageamento por Ressonância Magnética/métodos , Melanoma/secundário , Neoplasias Cutâneas/patologia , Audiometria de Tons Puros , Meios de Contraste , Diagnóstico Diferencial , Feminino , Humanos , Masculino , Compostos Organometálicos
13.
JAMA Otolaryngol Head Neck Surg ; 141(1): 34-9, 2015 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-25375088

RESUMO

IMPORTANCE: Pars tensa retraction is a frequent condition in chronic otitis media, and there is a large diversity of opinions regarding its management. OBJECTIVE: To report the long-term results and prognostic factors of myringoplasty in pediatric patients with grade 5 pars tensa atelectasis. DESIGN, SETTING, AND PARTICIPANTS: This was an observational, retrospective case review, conducted at a referral university hospital, of children undergoing surgical intervention for dry tympanic disruption after a clinical history of tensa retraction with a transcanal or postauricular approach under general anesthesia. INTERVENTIONS: Underlay myringoplasty with temporalis fascia or tragal perichondrium by transcanal or postauricular approach under general anesthesia. MAIN OUTCOMES AND MEASURES: Anatomical results are reported with a minimum follow-up of 5 years analyzing perforation or retraction of the neotympanum. Age; sex; side, position, and size of the eardrum disruption; surgical approach; graft material; and presence of contralateral disease were correlated with anatomical failure. RESULTS: The population included 33 girls and 28 boys undergoing 65 interventions; the age varied from 4 to 16 years (mean [SD], 10 [3.2] years). An intact tympanic membrane was obtained in 58 cases (89.2%) with a follow-up varying from 5 to 14 years (mean follow-up, 9 years). None of the variables analyzed significantly predisposed patients to tympanic perforation after surgical repair. There were no new progressive retractions. CONCLUSIONS AND RELEVANCE: Tensa retraction resulting in a tympanic disruption can be cured by underlay myringoplasty with perichondrium or temporalis fascia with results similar to those of simple tympanic perforations without recurrent retraction even in the long-term period.


Assuntos
Miringoplastia , Membrana Timpânica/patologia , Adolescente , Criança , Pré-Escolar , Feminino , Seguimentos , Humanos , Masculino , Prognóstico
14.
Eur Arch Otorhinolaryngol ; 271(1): 3-13, 2014 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-23404468

RESUMO

The number of non-neurofibromatosis type 2 (NF2) indications for auditory brainstem implant (ABI) in the literature is increasing. The objective of this study was to analyze and discuss the indications for ABI. Retrospective chart review and systematic review were conducted at Quaternary referral skull base center and referring centers. Analysis of ABI cases with non-NF2 indications and systematic review presenting non-NF2 ABI cases were performed. Fourteen referred cases with ABI were identified. All cases had unsatisfactory results of ABI and all could have been rehabilitated with a cochlear implant (CI). Of these 14 cases, 9 improved with a cochlear implant, and 2 with a hearing aid, two are still planned for CI, one received bilateral CI, no ABI. In literature, we found 31 articles presenting 144 non-NF2 ABI cases with at least 7 different indications other than NF2. ABI should be restricted to those patients who have no other rehabilitation options. Patency of the cochlea and evidence of an intact cochlear nerve should be examined with imaging and electrophysiologic testing. Sometimes a CI trial should be planned prior to proceeding with ABI. We have shown that in many cases a CI is still possible and CI provided better results than ABI. In vestibular schwannoma in the only hearing ear, cochlear otosclerosis, temporal bone fractures, (presumed) bilateral traumatic cochlear nerve disruption, auto-immune inner ear disease and auditory neuropathy primarily CI are indicated. Traumatic bilateral cochlear nerve disruption is exceptionally unlikely. In cochlear nerve aplasia, testing should be performed prior to meeting indications for ABI. In malformations, ABI is indicated only in severe cochlear hypoplasia or cochlear aplasia.


Assuntos
Implantes Auditivos de Tronco Encefálico , Surdez/cirurgia , Perda Auditiva/cirurgia , Implantes Cocleares , Contraindicações , Perda Auditiva Central/cirurgia , Humanos , Neuroma Acústico/cirurgia , Ossificação Heterotópica , Otosclerose/cirurgia , Resultado do Tratamento , Doenças do Nervo Vestibulococlear/patologia
15.
Int J Pediatr Otorhinolaryngol ; 74(8): 962-6, 2010 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-20627414

RESUMO

Pontine tegmental cap dysplasia (PTCD) is an exceptionally rare brain stem and cerebellar malformation characterized by ventral pontine hypoplasia, vaulted pontine tegmentum, hypoplasia of the vermis, subtotal absence of middle cerebellar peduncles, lateralized course of the superior cerebellar peduncles, and absence or alteration of the inferior olivary nucleus. The main clinical features are multiple cranial neurophaties and ataxia. Sensorineural hearing loss of varying severity is almost always present. To date, 14 cases of PTCD have been reported in the literature. We present a child with PTCD and profound bilateral sensorineural hearing loss who underwent cochlear implantation. To the best of our knowledge, cochlear implantation in PTCD has not been previously reported. Functional outcome was assessed using the Speech Perception Categories and the Speech Intelligibility Rating scale. At 22 months' postoperative evaluation, the patient who was placed into speech perception category 0 (no detection of speech) preoperatively progressed to category 3 (beginning word identification). Before implantation, the child had connected speech unintelligible. At the last follow-up, she had connected speech intelligible to a listener who has little experience of a deaf person's speech. Cochlear implantation allowed this child to improve her quality of life, increasing her self-confidence, independence, and social integration.


Assuntos
Implante Coclear/métodos , Perda Auditiva Neurossensorial/etiologia , Perda Auditiva Neurossensorial/cirurgia , Ponte/anormalidades , Audiometria , Criança , Implantes Cocleares , Eletroencefalografia/métodos , Feminino , Seguimentos , Perda Auditiva Neurossensorial/diagnóstico , Humanos , Imageamento por Ressonância Magnética/métodos , Malformações do Sistema Nervoso/diagnóstico , Qualidade de Vida , Percepção da Fala , Tomografia Computadorizada por Raios X/métodos , Resultado do Tratamento
16.
Auris Nasus Larynx ; 35(4): 562-8, 2008 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-18243617

RESUMO

OBJECTIVES: To report of a 65-year-old woman with bilateral Meniere's disease was referred for cochlear implantation (CI) due to severe/profound sensorineural hearing loss. METHODS: During the assessment workup, a vestibular schwannoma in the right ear was found by MR imaging. She underwent a translabyrinthine removal of the acoustic neuroma (AN) with sparing of the cochlear nerve and concurrent ipsilateral CI with a Nucleus Freedom device (Cochlear Ltd., Lane Cove, New South Wales, Australia). RESULTS: Complete removal of the AN was achieved without complications. Neural Response Telemetry (NRT) measurements, which showed poor morphology at the intraoperative tests, rapidly improved after activation, similarly to electrically evoked auditory brainstem responses (E-ABR). The patient reached 100% speech perception performances within 2 months from implantation, in the monaural condition. She was relieved from vertigo spell up to 14 months after the operation. CONCLUSION: Cochlear implantation at the time of acoustic neuroma removal with VIII nerve sparing can be a safe and effective hearing restoration procedure.


Assuntos
Implante Coclear , Perda Auditiva Neurossensorial/cirurgia , Neuroma Acústico/cirurgia , Idoso , Terapia Combinada , Feminino , Seguimentos , Perda Auditiva Neurossensorial/diagnóstico , Humanos , Doença de Meniere/diagnóstico , Neuroma Acústico/diagnóstico , Testes de Discriminação da Fala
17.
Acta Otolaryngol ; 127(5): 452-8, 2007 May.
Artigo em Inglês | MEDLINE | ID: mdl-17453469

RESUMO

CONCLUSIONS: We observed 4 cases of paroxysmal positional vertigo (PPV) among 62 cochlear implant (CI) recipients. They occurred in the implanted ear without chronological relation with the surgical procedure or the implant's activation. All of them relapsed within 3 months after an initially successful repositioning maneuver, and finally recovered after the second one. None of the patients showed labyrinthine weakness in the implanted ear. The outbreak of PPV did not affect the patients' speech perception performances. OBJECTIVES: To report and discuss the occurrence of PPV after cochlear implantation. PATIENTS AND METHODS: Among 32 adult patients who received a Nucleus CI, 4 suffered from PPV on the basis of Dix-Hallpike's maneuvers. After a Semont's repositioning maneuver, recurrences were similarly addressed. An electro-nystagmography (ENG) recording of caloric irrigation tests was obtained once the symptoms subsided. RESULTS: The observed incidence of 12.5% exceeds the figures reported in the literature. No anatomic abnormalities were identified in these patients, nor were any intraoperative or postoperative complications reported. PPV developed 1-12 months after CI surgery in the posterior canal and relapsed within 3 months. ENG showed a normal reflectivity in two patients, while the other two had a significant prevalence of the implanted side. The outcomes of the speech perception tests after CI in all the four patients matched those of their corresponding categories.


Assuntos
Implante Coclear , Complicações Pós-Operatórias/etiologia , Vertigem/etiologia , Adolescente , Audiometria de Tons Puros , Testes Calóricos , Criança , Pré-Escolar , Eletronistagmografia , Feminino , Seguimentos , Lateralidade Funcional/fisiologia , Humanos , Masculino , Membrana dos Otólitos/fisiopatologia , Complicações Pós-Operatórias/fisiopatologia , Complicações Pós-Operatórias/terapia , Recidiva , Percepção da Fala/fisiologia , Vertigem/fisiopatologia , Vertigem/terapia
19.
Otol Neurotol ; 27(6): 815-23, 2006 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-16936567

RESUMO

OBJECTIVE: To report on the outcomes of cochlear implantation (CI) in a child with cochleovestibular nerves (CVN) hypoplasia. STUDY DESIGN: Retrospective case review. SETTING: Tertiary referral center, University hospital. PATIENTS: An 18-month-old child with profound bilateral congenital hearing loss and bilateral hypoplasia of the CVN at imaging. INTERVENTION: Left CI at age 29 months with a Nucleus Contour device (Cochlear Ltd., Lane Cove, New South Wales, Australia) after unsatisfactory results of hearing aid use for 10 months. MAIN OUTCOME MEASURES: Speech perception tests, behavioral observation, electrophysiologic tests, and cognitive evaluation. RESULTS: Although the child scores poorly in every perceptive category with the CI alone, the device greatly enhances his speech understanding with the hearing aid in the opposite ear. In the bimodal condition, his words and sentences identification, recognition, and comprehension far exceed the monaural figures. The Meaningful Auditory Integration Scale (MAIS) tests reaches a score of 26/40, and the MacArthur's questionnaires confirm the improvement of language production and comprehension. These results became noticeable after 5 to 6 months and continued to improve up to the 10th month. The child's cognitive scores and overall performance competences greatly benefit from the CI, with the mental age overcoming the chronological age. CONCLUSION: We can confirm the chance of achieving satisfactory results by CI even when the imaging of CVN is doubtful and the electrophysiological tests are disappointing. In our experience, a CI in Type IIb dysplasia of the CVN is a feasible option, provided that the candidate shows some responses at aided audiogram and at least minimal signs of language development. Adequate counseling is necessary for these children because the expected outcome is somewhat lower than that of their deaf peers with normal appearance of the nerves.


Assuntos
Implantes Cocleares , Perda Auditiva/terapia , Nervo Vestibulococlear/anormalidades , Audiometria de Tons Puros , Limiar Auditivo , Implante Coclear/métodos , Feminino , Auxiliares de Audição , Perda Auditiva/etiologia , Perda Auditiva/cirurgia , Humanos , Lactente , Imageamento por Ressonância Magnética , Masculino , Estudos Retrospectivos , Testes de Discriminação da Fala , Percepção da Fala , Tomografia Computadorizada por Raios X , Resultado do Tratamento
20.
Int J Pediatr Otorhinolaryngol ; 70(7): 1175-82, 2006 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-16413617

RESUMO

OBJECTIVE: To review the clinical charts of 45 paediatric patients treated for acute otomastoiditis at the ORL Department of the University of Brescia (Italy) between January 1994 and March 2005 and to discuss the diagnostic workup and the outcome of treatment. METHODS: Twenty-six males and 19 females were admitted with acute mastoiditis and subperiosteal abscess. Thirteen of them (28.9%) presented an intracranial complication. Only three of them were not operated upon; one received a ventilation tube (VT); all the others underwent a mastoidectomy within 48-72 h. Twenty out of 32 uncomplicated mastoiditis were treated conservatively and the remaining 12 underwent myringotomy+/-VT, associated with a mastoidectomy in 9 cases. RESULTS: Antibiotics alone or with VTs achieved a full recovery in 28 out of 32 uncomplicated cases. Mastoidectomy resolved the disease in 13 patients (9 with complications). In severe complications, a canal wall down (CWD) (n=2) or an intact canal wall (ICW) mastoidectomy (n=7) were preferred, based on the extent of the lesions and the degree of hearing loss. All children recovered completely at 1 year follow-up. In the uncomplicated cases that were operated upon, the mean hospital stay was 7.8 days (versus 4.3 days for the conservative group). In children with intracranial complications the mean hospital stay was 12.8 days, significantly less than the four non-surgical patients, who remained hospitalized for an average of 18 days. CONCLUSION: Acute mastoiditis can fully recover with conservative treatment or myringotomy+VTs. Immediate surgical treatment is indicated for intracranial complications, if the neurological conditions are not critical. A simple mastoidectomy+/-tympanoplasty is warranted in: (1) exteriorization, if the child is older than 30 months or >15 kg of weight, (2) intracranial complications (combined with a neurosurgical procedure as needed) and (3) cholesteatoma or granulation tissue.


Assuntos
Trombose Intracraniana/etiologia , Mastoidite/complicações , Mastoidite/cirurgia , Abscesso/complicações , Abscesso/diagnóstico por imagem , Abscesso/tratamento farmacológico , Abscesso/cirurgia , Doença Aguda , Adolescente , Antibacterianos/uso terapêutico , Criança , Pré-Escolar , Feminino , Seguimentos , Humanos , Lactente , Trombose Intracraniana/diagnóstico por imagem , Veias Jugulares/diagnóstico por imagem , Masculino , Processo Mastoide/diagnóstico por imagem , Processo Mastoide/cirurgia , Mastoidite/diagnóstico por imagem , Mastoidite/tratamento farmacológico , Periósteo/diagnóstico por imagem , Osso Temporal/diagnóstico por imagem , Tomografia Computadorizada por Raios X , Resultado do Tratamento , Ultrassonografia
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