Endoscopic-Assisted Cochlear Implantation in Children with Malformed Ears

2019 ◽  
Vol 161 (4) ◽  
pp. 688-693 ◽  
Author(s):  
Marco Carner ◽  
Andrea Sacchetto ◽  
Luca Bianconi ◽  
Davide Soloperto ◽  
Luca Sacchetto ◽  
...  
Keyword(s):  
Inner Ear ◽  
Cochlear Implantation ◽  
Round Window ◽  
Case Series ◽  
University Hospital ◽  
Profound Hearing Loss ◽  
Ear Malformations ◽  
Auditory Performance ◽  
Window Area ◽  
Microscopic Techniques

Objective Complex middle and inner ear malformations are considered an important limitation for cochlear implant (CI) with traditional microscopic techniques. The aim of the present study is to describe the results of the endoscopic-assisted CI procedure in children with malformed ears. Study Design Case series with chart review of consecutive patients. Setting Two tertiary referral centers: University Hospital of Verona and University Hospital of Modena, Italy. Subjects and Methods In total, 25 children underwent endoscopic-assisted cochlear implantation between January 2013 and January 2018. The audiologic and neuroradiologic assessment showed profound hearing loss and malformation of the middle and inner ear in all children. A complete review of anatomic features, surgical results, and audiologic outcomes was performed. The surgical technique is described step-by-step, and the outcomes are detailed. Results All patients (mean age, 3.6 years; range, 2.8-9 years) underwent a transattical/endoscopic-assisted CI procedure. All children showed varying degrees of auditory benefit, as measured by routine audiometry, speech perception tests, and Categories of Auditory Performance scores (mean, 6). No immediate or late postoperative complications were noted. Conclusion The endoscopic-assisted approach proved to be successful in cochlear implantation. The direct visualization and magnification allow (1) exploration of the tympanic cavity; (2) confirmation of all anatomic features, with strict control of the course of the facial nerve, round window area, and inner ear; and (3) performance of the cochleostomy with adequate insertion of the array.

scholarly journals A gyógyszeres kezelésre nem javuló hirtelen halláscsökkenés lépcsőzetes sebészi terápiája

2021 ◽  
Vol 162 (51) ◽  
pp. 2055-2060
Keyword(s):  
Hearing Loss ◽  
Inner Ear ◽  
Hearing Aids ◽  
Cochlear Implantation ◽  
Early Stage ◽  
Round Window ◽  
Complete Recovery ◽  
Round Window Membrane ◽  
Systemic Steroid ◽  
Profound Hearing Loss

Összefoglaló. A hirtelen halláscsökkenés patofiziológiája még nagyrészt tisztázatlan, így oki terápia nem lehetséges. Az elsődleges kezelést a helyileg vagy szisztémásan adott kortikoszteroid jelenti, egységes protokoll azonban nem áll rendelkezésre. Nagy vagy súlyos fokú hirtelen halláscsökkenés esetén kóroki tényezőként felmerül a perilymphafistula lehetősége még azoknál a betegeknél is, akiknél nem szerepel trauma az anamnézisben. A kórkép műtéti kezelése a dobüreg feltárását követően a belső fül ablakainak obliterálása. Amennyiben ez a megoldás nem eredményez megfelelő hallásjavulást, hagyományos vagy implantálható hallókészülékek alkalmazása javasolt. A közleményben részletezett esetünkben teljes siketséggel járó, jobb oldali hirtelen halláscsökkenés alakult ki, melynek hátterében egyértelmű okot azonosítani nem sikerült. Az eredménytelen kombinált, intratympanalis és szisztémás szteroidkezelést követően exploratív tympanotomiát végeztünk, melynek során a belső fül ablakait obliteráltuk. Hallásjavulást ezt követően sem sikerült kimutatni, így cochlearis implantáció elvégzése mellett döntöttünk. Az implantációt a kerek ablakon keresztül végeztük, mely alapján kijelenthetjük, hogy az előzetes kerekablak-obliteráció nem zárja ki a későbbi cochlearis implantációt. Orv Hetil. 2021; 162(51): 2055–2060. Summary. The pathophysiology of sudden sensorineural hearing loss is mainly unknown, therefore no causative treatment exists. Systemic and local administration of corticosteroids serves as first line therapy although protocols vary. In cases of severe or profound hearing loss with no improvement for medical therapy, perilymphatic fistulae can be assumed even without any history of trauma. Therefore, inner ear window obliteration as a primary surgical option in the early stage can be considered. For patients without complete recovery, conventional hearing aids or implantable hearing devices can be offered. In our case report, we present a patient with right sided idiopathic sudden deafness. After failure of conservative combined intratympanic and systemic steroid therapy, explorative tympanotomy and obliteration of the inner ear windows were performed. As no hearing improvement was witnessed, successful cochlear implantation via round window insertion was performed. Our case justifies that obliterating the round window membrane does not rule out further successful cochlear implantation. Orv Hetil. 2021; 162(51): 2055–2060.

scholarly journals Cystic Cochleovestibular Malformation (Incomplete Partition Type 1)

2010 ◽  
Vol 25 (1) ◽  
pp. 41-42 ◽  
Author(s):  
Nathaniel W. Yang
Keyword(s):  
Hearing Loss ◽  
Inner Ear ◽  
Temporal Bone ◽  
Cochlear Implantation ◽  
Type I ◽  
Profound Hearing Loss ◽  
Increased Risk ◽  
Ear Malformations ◽  
Temporal Bone Imaging ◽  
Incomplete Partition

A 5-year old female with bilateral profound hearing loss underwent computerized tomographic imaging of the temporal bone as part of the work-up to determine the etiology of her deafness, and to delineate middle and inner ear anatomy prior to cochlear implantation. The examination revealed an inner ear malformation which, based on the newest classification of cochleovestibular malformations by Sennaroglu and Saatci, is called an incomplete partition type I (IP-1) or cystic cochleovestibular malformation. This condition is characterized by (1) a cochlea that is lacking the entire modiolus and cribriform area, resulting in a cystic appearance, and (2) a large cystic vestibule.1   Temporal bone imaging is among the most useful examinations in the etiological investigation of idiopathic sensorineural hearing loss in children, with up to 30%2 of the imaging studies showing an abnormality. The detection of inner ear malformations is important, as some abnormalities are associated with an increased risk of meningitis or progressive hearing loss following head trauma.3 Likewise, the approach to cochlear implantation may be influenced by the type of malformation. In this particular patient, the use of a cochlear implant with a full-band electrode design may be more appropriate, as the location of the neural elements within the cystic cochlea is not definitely known.

Cochlear Implant Outcomes in Patients with Neurosarcoidosis

2021 ◽  
pp. 1-7
Author(s):  
Dario Ebode ◽  
Fleur Cohen-Aubart ◽  
Stéphanie Trunet ◽  
Evelyne Ferrary ◽  
Ghizlène Lahlou ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Cochlear Implantation ◽  
Case Series ◽  
Corticosteroid Treatment ◽  
Auditory Neuropathy ◽  
Disease Exacerbation ◽  
Profound Hearing Loss ◽  
Hearing Recovery ◽  
Speech Performance

<b><i>Introduction:</i></b> Audiovestibular symptoms are rare in sarcoidosis, but they may also be the first manifestation of the disease. Sudden or progressive bilateral hearing loss is usually associated with vestibular impairment. The mechanism of hearing loss remains unclear, but clinical presentation and magnetic resonance imaging suggest a retrocochlear site for the lesion in most patients. Several cases of hearing recovery after corticosteroid treatment have been reported. In patients with severe or profound hearing loss, the benefit of cochlear implantation is challenging to predict in the case of auditory neuropathy and is rarely described. We present a case series of cochlear implantation in patients with documented neurosarcoidosis. <b><i>Results:</i></b> Seven cases of cochlear implantation in 4 patients with neurosarcoidosis are reported. All of the patients showed a great improvement very quickly in both quiet and noise. Speech performance remained stable over time with a follow-up ranging from 4 to 11 years, even in patients who had disease exacerbation. <b><i>Conclusion:</i></b> Cochlear implantation is possible in deaf patients with neurosarcoidosis. The excellent benefit obtained in our patients suggests a particular type of neuropathy, but endocochlear involvement cannot be entirely ruled out.

Cochlear implantation in patients afflicted with inner ear malformations – the Pécs experience

2018 ◽  
Vol 16 (4) ◽  
pp. 232-237
Author(s):  
Peter Bako ◽  
Adrienn Nemeth ◽  
Krisztian Molnar ◽  
Tamas Toth ◽  
Kinga Harmat ◽  
...  
Keyword(s):  
Inner Ear ◽  
Cochlear Implantation ◽  
Inner Ear Malformations ◽  
Ear Malformations

Outcome of Cochlear Implantation in Postmeningitis Profound Sensorineural Hearing Loss at Rajavithi Hospital

2021 ◽  
Vol 104 (2) ◽  
pp. 260-263
Keyword(s):  
Hearing Loss ◽  
Sensorineural Hearing Loss ◽  
Cochlear Implantation ◽  
Sensorineural Hearing ◽  
Preoperative Imaging ◽  
Profound Hearing Loss ◽  
Language Status ◽  
Auditory Performance ◽  
Electrode Insertion ◽  
One Year

Background: Bacterial meningitis is one of the major factors in the etiology of acquired sensorineural hearing loss in children and adults. Cochlear implantation in these patients is challenging because of inner ear ossification and fibrosis, and this procedure sometimes achieves poorer outcomes in this scenario than with other causes of sensorineural hearing loss. There has been little research into the factors affecting the outcomes of this procedure. Objective: To evaluate the outcomes of cochlear implantation in patients with postmeningitis profound sensorineural hearing loss and to evaluate the factors that affect the results. Materials and Methods: A retrospective review was conducted of thirty patients who were diagnosed with post meningitis profound hearing loss and underwent cochlear implantation at Rajavithi Hospital between 2001 and 2016. Preoperative language status, duration of deafness, preoperative imaging, and degree of electrode insertion were recorded. Categories of auditory performance-II test (CAP-II) was evaluated in all cases, one year postoperative. Results: Thirty postmeningitis deafness patients underwent cochlear implantation. The median age at diagnosis of meningitis and age at implantation were 41 years (range 1 to 75) and 49.50 years (range 3 to 75), respectively. The median duration of deafness was 12 months (range 4 to 300), and the overall mean CAP-II at one year after surgery was 5.47±2.21. The postlinguistic group had a significantly higher CAP-II score than the prelinguistic one (p=0.006). Electrodes were successfully totally inserted in 19 patients (63.3%) and partially inserted in 11 (36.7%). The average CAP-II score in the group with fully-inserted electrodes was significantly higher than in the group with partially-inserted electrodes (p=0.045). There was no correlation between CAP-II score and age at meningitis diagnosis (p=0.069), age at time of surgery (p=0.105), duration of deafness (p=0.506), or preoperative CT (p=0.228) or MRI abnormality (p=0.078). Conclusion: Cochlear implantation in patients with postmeningitis profound hearing loss had high success rates and favorable outcomes. Preoperative language status and degree of electrode insertion were factors that affected auditory performance results. Keywords: Cochlear implantation, Postmeningitis hearing loss, Sensorineural hearing loss, Meningitis, Rajavithi Hospital

B111 Cochlear implantation in congenital middle and inner ear malformations in children

2011 ◽  
Vol 75 ◽  
pp. 30
Author(s):  
H. Skarzynski ◽  
M. Porowski ◽  
M. Mrówka ◽  
P. Mlotkowska-Klimek ◽  
A. Lorens ◽  
...  
Keyword(s):  
Inner Ear ◽  
Cochlear Implantation ◽  
Inner Ear Malformations ◽  
Ear Malformations

Role of Electrically Evoked Auditory Brainstem Response in Cochlear Implantation of Children With Inner Ear Malformations

2008 ◽  
Vol 29 (5) ◽  
pp. 626-634 ◽  
Author(s):  
Ana H. Kim ◽  
Paul R. Kileny ◽  
H. Alexander Arts ◽  
Hussam K. El-Kashlan ◽  
Steven A. Telian ◽  
...  
Keyword(s):  
Inner Ear ◽  
Cochlear Implantation ◽  
Auditory Brainstem Response ◽  
Auditory Brainstem ◽  
Inner Ear Malformations ◽  
Ear Malformations ◽  
Brainstem Response

scholarly journals Cochlear Implantation after Bacterial Meningitis in Infants Younger Than 9 Months

2011 ◽  
Vol 2011 ◽  
pp. 1-9 ◽  
Author(s):  
B. Y. Roukema ◽  
M. C. Van Loon ◽  
C. Smits ◽  
C. F. Smit ◽  
S. T. Goverts ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Bacterial Meningitis ◽  
Cochlear Implants ◽  
Cochlear Implantation ◽  
Speech Intelligibility ◽  
Pneumococcal Meningitis ◽  
Profound Hearing Loss ◽  
Young Infants ◽  
Key Points ◽  
Auditory Performance

Objective. To describe the audiological, anesthesiological, and surgical key points of cochlear implantation after bacterial meningitis in very young infants.Material and Methods. Between 2005 and 2010, 4 patients received 7 cochlear implants before the age of 9 months (range 4–8 months) because of profound hearing loss after pneumococcal meningitis.Results. Full electrode insertions were achieved in all operated ears. The audiological and linguistic outcome varied considerably, with categories of auditory performance (CAP) scores between 3 and 6, and speech intelligibility rating (SIR) scores between 0 and 5. The audiological, anesthesiological, and surgical issues that apply in this patient group are discussed.Conclusion. Cochlear implantation in very young postmeningitic infants is challenging due to their young age, sequelae of meningitis, and the risk of cochlear obliteration. A swift diagnostic workup is essential, specific audiological, anesthesiological, and surgical considerations apply, and the outcome is variable even in successful implantations.

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