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.

scholarly journals Prevalence & features of inner ear malformations among children with congenital sensorineural hearing loss: A Public Cochlear Implant Centre Experience

2020 ◽  
Vol 36 (7) ◽  
Author(s):  
Jawwad Ahmed ◽  
Ghulam Saqulain ◽  
Muhammad Iqbal javed Khan ◽  
Mobeen Kausar
Keyword(s):  
Hearing Loss ◽  
Cochlear Implant ◽  
Sensorineural Hearing Loss ◽  
Inner Ear ◽  
Demographic Data ◽  
Sensorineural Hearing ◽  
Profound Hearing Loss ◽  
Creative Commons ◽  
Inner Ear Malformations ◽  
Ear Malformations

Objective: To determine the prevalence and features of inner ear anomalies in children with congenital profound hearing loss who presented at our cochlear implant center based on imaging studies. Methods: This retrospective study reviewed charts of children with congenital SNHL, who presented to Department of Otolaryngology & Auditory Implant Centre, Capital Hospital Islamabad over a period of 2 years from 1st May 2017 to 30th April 2019. These included 481 cases of both genders aged between 1 to 12 years. After gathering demographic data, audiological data, computed tomography findings of the temporal bone were analyzed. Data was analyzed using SPSS 22. Results: The Inner Ear Malformations were identified in 48(10%) children including 28 (58.33%) males and 20 (41.67%) female. Most 20(41.67%) presented at >3-5 years of age followed by 19(39.58%) at 2-3 years. However, no significant association of gender (p=0.57, p=0.076) and age of presentation (p=0.344, p=0.697) for right and left ears was noted with inner ear malformations. The most common anomaly noted were CLA, CH-III and CH-II in decreasing order of frequency in both ears. Conclusion: The prevalence of IEM’s was found to be 48(10%). Commonest anomalies noted were CLA, CH-III and CH-II. No significant association of gender and age of presentation was noted with type of anomaly in both ears. doi: https://doi.org/10.12669/pjms.36.7.3134 How to cite this:Ahmed J, Saqulain G, Khan MIJ, Kausar M. Prevalence & features of inner ear malformations among children with congenital sensorineural hearing loss: A Public Cochlear Implant Centre Experience . Pak J Med Sci. 2020;36(7):---------.  doi: https://doi.org/10.12669/pjms.36.7.3134 This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/3.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

scholarly journals Unilateral Horizontal Semicircular Canal Malformation Causing Recurrent Vertigo

2018 ◽  
Vol 32 (2) ◽  
pp. 58-59
Author(s):  
Nathaniel W. Yang
Keyword(s):  
Hearing Loss ◽  
Inner Ear ◽  
Temporal Bone ◽  
Semicircular Canal ◽  
Lateral Semicircular Canal ◽  
Vestibular Loss ◽  
Vestibular Neurectomy ◽  
Horizontal Semicircular Canal ◽  
Ear Malformations ◽  
Vestibular Symptoms

A 62-year-old man consulted for recurrent episodes of vertigo lasting from seconds to several minutes. The vertigo was variably described as spinning, lateral swaying, and a feeling of being “unsure of his position in space.” These episodes were noted to have begun when the patient was still in his 20’s. Standard pure tone audiometry revealed a mild-to-moderate downsloping mixed hearing loss in the left ear. Bithermal caloric testing indicated the presence of a significant left-sided peripheral vestibular loss. Due to the fact that the vertigo episodes presented relatively early in life, the possibility of a congenital inner ear malformation was considered as a cause for his symptoms.  Computerized tomographic (CT) imaging of the temporal bone was performed. This clearly showed the left horizontal semicircular canal lacking a central bony island. (Figure 1 and 2) The cochlea, superior and posterior semicircular canals, vestibular and cochlear aqueducts, and ossicular chain were grossly normal. A malformation of the horizontal or lateral semicircular canal is one of the most common inner ear malformations, as it is the last vestibular structure to be formed during inner ear embryogenesis. As such, it may occur in isolation or may be associated with other vestibular, cochlear, or middle ear malformations.1,2  Although vertigo and dizziness are symptoms to be expected in such a condition, existing data indicates that it may be totally asymptomatic, or it may also present as a sensorineural, conductive, or mixed type of hearing loss.1,3   Radiologic imaging is of prime importance in diagnosing such conditions, especially when auditory and/or vestibular symptoms manifest early in life. This case perfectly illustrates the need for such studies, as the patient went undiagnosed for more than forty years!             No definitive statements can be gleaned from existing medical literature with respect to treatment. However, in patients with debilitating vestibular symptoms, management with modalities that selectively target the vestibular system, but spare the auditory system, such as vestibular neurectomy and trans-tympanic aminoglycoside therapy appear to be reasonable options.   References   Johnson J, Lalwani AK. Sensorineural and conductive hearing loss associated with lateral semicircular canal malformation. Laryngoscope 2000 Oct;110(10):1673–1679. DOI:10.1097/00005537-200010000-00019 PMID: 11037823   Casselman JW, Delanote J, Kuhweide R, van Dinther J, De Foer B, Offeciers EF. Congenital malformations of the temporal bone. In: Lemmerling M, De Foer B, editors. Temporal bone imaging. Berlin Heidelberg: Springer-Verlag; 2015, pp. 120-154.   Kim CH, Shin JE, Lee YJ, Park HJ. Clinical characteristics of 7 patients with lateral semicircular canal dysplasia. Res Vestib Sci 2012;11(2):64-68.

scholarly journals Incomplete Partition type I: Radiological Evaluation of the Temporal Bone

Acta Medica ◽  
2021 ◽  
pp. 1-9
Author(s):  
Safak Parlak ◽  
Ayca Akgoz Karaosmanoglu ◽  
Sevtap Arslan ◽  
Levent Sennaroglu
Keyword(s):  
Inner Ear ◽  
Temporal Bone ◽  
Correct Diagnosis ◽  
Semicircular Canals ◽  
Type I ◽  
Vestibular Aqueduct ◽  
Endolymphatic Duct ◽  
Incomplete Partition ◽  
Internal Acoustic Canal ◽  
Large Vestibular Aqueduct

Objective: Incomplete partition type I is an uncommon congenital anomaly of the inner ear, characterized by typical cystic cochleovestibular appearance. Incomplete partition type I was firstly defined as cystic cochlea and vestibule without large vestibular aqueduct; however, large vestibular aqueduct and/or enlarged endolymphatic duct could rarely be seen in incomplete partition type I anomaly. Correct diagnosis of the type of cochlear malformation and differentiation of incomplete partition type I is necessary for patient management and surgical approach. Our aim was to document the temporal bone imaging findings in a series of patients with incomplete partition type I. Materials and Methods: CT (n=85) and/or MRI (n=80) examinations of 99 ears in 59 incomplete partition type I patients were retrospectively evaluated. All structures of the otic capsule were retrospectively assessed. The appearances of cochlea and vestibule, vestibular aqueduct/endolymphatic duct, semicircular canals were qualitatively evaluated by an experienced neuroradiologist. The vertical dimension of vestibular aqueduct and/or endolymphatic duct (from the point where the duct arises from the vestibule) was measured on CT/MRI. Anterior-posterior diameter of the internal acoustic canal and the diameter of cochlear aperture were measured on CT. The cochleovestibular nerves were evaluated on sagittal-oblique high T2-weighted imaging. Results: All 99 ears had defective partition with unpartitioned cochlear basal turn and absent interscalar septae, separated but cystic cochlea. The vestibule was enlarged in all ears except one. Semicircular canals were usually dysplastic (92.9%). A total of 35 incomplete partition type I ears (35.3%) had large vestibular aqueduct and/or enlarged endolymphatic duct. Internal acoustic canal was wide in 21% of ears. Cochlear aperture was wide in 5.9% of ears. Cochlear nerve was either hypoplastic or aplastic in about a quarter of incomplete partition type I ears. Conclusion: In up to one-third of incomplete partition type I patients, an associated large vestibular aqueduct /endolymphatic duct could be seen accompanying typical inner ear findings. Although the cochlear nerves are normal in the majority of cases, auditory brainstem implantation may be necessary in certain cases of incomplete partition type I anomaly.

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.

Cochlear Nerve Hypoplasia: Audiological Characteristics in Children and Adults

2019 ◽  
Vol 24 (3) ◽  
pp. 147-153
Author(s):  
Betul Cicek Cinar ◽  
Emel Tahir ◽  
Merve Ozbal Batuk ◽  
Mehmet Yarali ◽  
Gonca Sennaroglu ◽  
...  
Keyword(s):  
Hearing Loss ◽  
General Term ◽  
Cochlear Nerve ◽  
Type I ◽  
Radiological Findings ◽  
Ear Malformations ◽  
Cochlear Nerve Aplasia ◽  
Incomplete Partition ◽  
Cochlear Malformation ◽  
Cochlear Nerve Hypoplasia

Background: Cochlear nerve deficiency is a general term used to describe both cochlear nerve hypoplasia (CNH) and cochlear nerve aplasia. Although these two conditions can have similar results on audiological evaluation, CNH yields more variation in audiological tests. Objectives: To describe the audiological characteristics of the CNH cases in our series in relation to radiological findings. Methods: We reviewed the medical charts, audiological findings, and radiological findings on cases with CNH. We included cases with CNH in one ear or both ears. Out of 90 subjects with CNH, we included a total of 40 individuals (21 women and 19 men; 49 ears) in the current study. We reviewed and analyzed the participants’ audiological test results according to the radiological findings. Results: Cases with CNH showed variations according to the cochlear structure. There were 13 normal cochleae, 4 with incomplete partition type I, and 32 with cochlear hypoplasia. The accompanying cochlear apertures also showed variation: 17 were normal, 28 stenotic, and 4 aplastic cochlear apertures. The subjects displayed hearing loss ranging from moderate to profound; furthermore, 4 subjects had no response to sound whatsoever. The degree of hearing loss was not statistically significantly different with regard to the presence or absence of cochlear malformation with CNH (p > 0.005). We observed both sensorineural hearing loss and mixed-type hearing loss among the CNH cases. Conclusions: CNH is the presence of a cochlear nerve that is smaller in diameter than the facial nerve. It can be accompanied with other associated inner ear malformations of different degrees of severity. We observed degrees of hearing loss ranging from moderate to profound.

A Revision Surgery for Cochlear Implantation in a Case of Incomplete Partition Type I

2016 ◽  
Vol 27 (10) ◽  
pp. 846-850 ◽  
Author(s):  
Habib G. Rizk ◽  
Francesca Hagood ◽  
Meredith A. Holcomb ◽  
Ted A. Meyer
Keyword(s):  
Cochlear Implant ◽  
Inner Ear ◽  
Revision Surgery ◽  
Cochlear Implantation ◽  
Type I ◽  
Facial Recess ◽  
Long Term Follow Up ◽  
Incomplete Partition

Background: Patients with cochlear malformations were long considered poor candidates for cochlear implantation (CI), and surgical approaches different than the standard facial recess approach were used to access the inner ear. There is no previous long-term follow-up of a patient with significantly malformed inner ear operated through an untraditional route and requiring a revision surgery. Purpose: This case provides a long-term follow-up from the initial surgery, a short-term follow-up from the revision surgery, and it illustrates the evolving classification of inner ear malformations as well as the potential problems associated with nonstandard approaches to the cochlea. Research Design: A case report. Intervention: Herein, we report a case of revision CI in a patient with incomplete partition type I, through the round window via a facial recess approach, 18 yr after an initial implantation via a transmastoid labyrinthotomy approach. Results: The patient had an uncomplicated surgery, and after activation, she noted auditory perception on all electrodes without facial stimulation. A sound field sound awareness threshold was obtained at 15 dB HL. Conclusions: As the prior generation of cochlear implant recipients ages, the probability of a revision surgery for various causes increases. Cochlear implant surgeons should be aware of the potential pitfalls associated in these often challenging cases.

Auditory and vestibular manifestations of Vogt–Koyanagi–Harada disease

2010 ◽  
Vol 125 (2) ◽  
pp. 138-141 ◽  
Author(s):  
S Al Dousary
Keyword(s):  
Hearing Loss ◽  
Inner Ear ◽  
Cochlear Implantation ◽  
Organ Involvement ◽  
Disease Outcome ◽  
Profound Hearing Loss ◽  
Systemic Steroids ◽  
Chronic Disorder ◽  
High Incidence ◽  
Post Implantation

AbstractIntroduction and aims:Vogt–Koyanagi–Harada disease is a chronic disorder involving the eye and the central nervous, auditory, vestibular and integumentary systems. This study aimed to determine the auditory and vestibular manifestations of this disease.Methods:Twenty-four patients diagnosed with Vogt–Koyanagi–Harada disease were assessed for auditory and vestibular dysfunction.Results:Uveitis presents in all cases. Sensory hearing loss was present in 50 per cent of cases, tinnitus in 42 per cent, vertigo in 17 per cent and headache in 17 per cent. Nine patients received systemic steroids. Six patients who were treated early regained their hearing, but three patients whose treatment was delayed did not. One patient with bilateral profound hearing loss underwent cochlear implantation, and achieved excellent post-implantation hearing.Conclusion:There is a high incidence of cochlear and vestibular end-organ involvement in patients with Vogt–Koyanagi–Harada disease. The adequacy and timing of treatment has a significant effect on the disease outcome. Vogt–Koyanagi–Harada disease appears to affect the inner ear end-organ. Patients who develop bilateral profound sensory hearing loss are suitable candidates for cochlear implantation.

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.

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.

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