scholarly journals Revision Stapedectomy in a Female Patient with Inner Ear Malformation

2016 ◽  
Vol 2016 ◽  
pp. 1-3
Author(s):  
Tirth R. Patel ◽  
Aaron C. Moberly
Keyword(s):  
Hearing Loss ◽  
Inner Ear ◽  
Female Patient ◽  
Stapes Surgery ◽  
Mixed Hearing Loss ◽  
Inner Ear Malformation ◽  
Ear Malformations ◽  
Surgical History ◽  
History Of ◽  
Cochlear Malformation

Objectives. We describe an unusual case of surgical management of congenital mixed hearing loss in a female patient with inner ear malformation. This report outlines the role of temporal bone imaging and previous surgical history in evaluating a patient’s risk of perilymph gusher during stapes surgery.Methods. A 68-year-old female patient with a history of profound bilateral mixed hearing loss due to ossicular and cochlear malformation presented to our otology clinic. She had undergone multiple unsuccessful previous ear surgeries. Computed tomography revealed bilateral inner ear malformations. She elected to proceed with revision stapedectomy.Results. The patient received modest benefit to hearing, and no operative complications occurred.Conclusions. Although stapedectomy has been shown to improve hearing in patients with stapes fixation, there is risk of perilymph gusher in patients with inner ear abnormalities. Evaluation and counseling of the risk of gusher during stapes surgery should be done on a case-by-case basis.

scholarly journals Pneumolabyrinth: a rare complication of stapes surgery

2019 ◽  
Vol 12 (11) ◽  
pp. e232190
Author(s):  
Patricia S Gomes ◽  
Sergio Caselhos ◽  
Ana Teresa Vide ◽  
Rui Fonseca
Keyword(s):  
Hearing Loss ◽  
High Resolution ◽  
Physical Examination ◽  
Inner Ear ◽  
Vein Graft ◽  
Rare Complication ◽  
Stapes Surgery ◽  
Mixed Hearing Loss ◽  
Hearing Thresholds ◽  
Vestibular Symptoms

Pneumolabyrinth is the entrapment of air within the inner ear and is a rare complication of stapes surgery. We report the case of a patient submitted to stapedectomy who, 4 weeks later, suddenly developed right hearing loss, ipsilateral tinnitus and vertigo. On the physical examination, the patient showed no signs of vestibular deficits. Audiometry was compatible with right profound mixed hearing loss and high-resolution CT of the temporal bone revealed the presence of pneumolabyrinth. During exploratory tympanotomy, the prosthesis was found dislodged; the communication between the middle and inner ear was closed with vein graft and a new prosthesis was placed. Following surgery, vestibular symptoms was abolished and the patient experienced great improvement of the hearing thresholds.

scholarly journals Study on the relationship between the pathogenic mutations of SLC26A4 and CT phenotypes of inner ear in patient with sensorineural hearing loss

2019 ◽  
Vol 39 (3) ◽  
Author(s):  
Lihua Wu ◽  
Yunliang Liu ◽  
Jianman Wu ◽  
Sheng Chen ◽  
Shupin Tang ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Ct Scan ◽  
Inner Ear ◽  
Gene Mutations ◽  
Pathogenic Mutation ◽  
Vestibular Aqueduct ◽  
Slc26a4 Gene ◽  
Pathogenic Mutations ◽  
Inner Ear Malformation ◽  
Single Allele

Abstract To investigate the possible association of pathogenic mutations of SLC26A4 and computerized tomography (CT) phenotypes of inner ear, and explore the feasibility of using the method of gene sequence analysis. A total of 155 patients with bilateral hearing loss carrying SLC26A4 gene mutations were further subjected to high-resolution temporal bone CT and thyroid B ultrasound tests. The potential relationship between the pathogenic mutations of gene and the CT phenotypes were analyzed. As a result, 65 patients (41.9%, 65/155) carried SLC26A4 gene mutations, and 27 cases were detected with pathogenic mutations of SLC26A4 where IVS7-2A>G (55.6%, 15/27) was the most common pathogenic mutation. Amongst them, 19 patients carrying bi-allelic SLC26A4 mutations were all confirmed to have inner ear malformation by CT scan including four cases of enlarged vestibular aqueduct (EVA) and 15 cases of Mondini dysplasia (MD). However, there was only one in eight cases of single allele pathogenic mutation who was confirmed to have EVA by CT scan. Further, only one patient with EVA was confirmed to be slightly higher of total T3 than normal by thyroid ultrasound scan and thyroid hormone assays. These findings suggested that CT detection and SLC26A4 gene detection are efficient methods to diagnose EVA, which can complement each other. Also, the bi-allelic pathogenic mutations of SLC26A4 are more likely to induce inner ear malformation than single allele pathogenic mutation.

scholarly journals Inner Ear Active Hearing Device in Non-Otosclerotic, Severe, Mixed Hearing Loss

2016 ◽  
Vol 37 (5) ◽  
pp. 520-523 ◽  
Author(s):  
Maurizio Barbara ◽  
Luigi Volpini ◽  
Edoardo Covelli ◽  
Chiara Filippi ◽  
Simonetta Monini
Keyword(s):  
Hearing Loss ◽  
Inner Ear ◽  
Mixed Hearing Loss ◽  
Hearing Device

scholarly journals Hearing Aid Treatment in Patients with Mixed Hearing Loss. Part I: Expected Benefit and Limitations after Stapes Surgery

2020 ◽  
Vol 25 (3) ◽  
pp. 125-132
Author(s):  
Nina Wardenga ◽  
Victoria Diedrich ◽  
Bernd Waldmann ◽  
Thomas Lenarz ◽  
Hannes Maier
Keyword(s):  
Hearing Loss ◽  
Middle Ear ◽  
Hearing Aids ◽  
State Of The Art ◽  
Treatment Success ◽  
Stapes Surgery ◽  
Pure Tone Average ◽  
Mixed Hearing Loss ◽  
Hearing Devices ◽  
Middle Ear Implants

Objective: The purpose of the present study was to determine the fraction of patients with mixed hearing loss who can or cannot expect benefit from power hearing aids (HAs) after stapes surgery. Design: The audiological outcome of 374 stapes surgeries was used to calculate the patients’ individual postoperative requirements in terms of gain and output of HAs. These requirements were compared to the available gain and output provided by state-of-the-art power HAs at 0.5, 1.0, 2.0, and 4.0 kHz. According to these comparisons, ears were divided into three groups. For G0, required gain and output lay within the corresponding technical limits of the HAs at all frequencies. In G1, one or both requirements could not be fulfilled at 1 frequency. G2 combined all ears where the requirements lay beyond the HA’s technical limitations at 2 or more frequencies. Results: Stapes surgery resulted in an improvement of air-bone gap (ABG) in 84.5% of the cases by 15.7 dB on average. Based on pure-tone average (0.5, 1.0, 2.0, 4.0 kHz), 40.6% of all cases showed an ABG ≤10 dB. 44.9% of all cases did no longer need a HA after stapes surgery. A power HA would fulfill both audiological criteria at all 4 frequencies in 81.6% of cases that needed a HA postoperatively. However, 18.4% would not be sufficiently treatable at 1 or more frequencies (15.0% in G1, 3.4% in G2). Conclusions: The present study identified a subset of patients with mixed hearing loss after stapes surgery that cannot be treated sufficiently with available power HAs. As the residual ABG is an important reason for this lack of treatment success, the advancement of alternative hearing devices that circumvent the middle ear, such as powerful active middle ear implants, is indicated.

Inner-ear malformations as a cause of single-sided deafness

2020 ◽  
Vol 134 (6) ◽  
pp. 509-518
Author(s):  
E Tahir ◽  
M D Bajin ◽  
S Jafarov ◽  
M Ö Yıldırım ◽  
B Ç Çınar ◽  
...  
Keyword(s):  
Inner Ear ◽  
Internal Auditory Canal ◽  
Cochlear Nerve ◽  
Resonance Imaging ◽  
Radiological Findings ◽  
Inner Ear Malformations ◽  
Disease Prognosis ◽  
Inner Ear Malformation ◽  
Ear Malformations ◽  
Single Sided Deafness

AbstractObjectiveTo determine the prevalence and distribution of inner-ear malformations in congenital single-sided deafness cases, as details of malformation type are crucial for disease prognosis and management.MethodsA retrospective study was conducted of 90 patients aged under 16 years with congenital single-sided deafness. Radiological findings were evaluated using computed tomography and magnetic resonance imaging. Inner-ear malformations were identified and cochlear nerve status was determined in affected ears.ResultsOut of 90 ears, 42 (46.7 per cent) were found to have inner-ear malformation. Isolated cochlear aperture stenosis was the most common anomaly (n = 18, 20 per cent), followed by isolated cochlear aperture atresia (n = 11, 12.2 per cent) and cochlear hypoplasia (n = 7, 7.8 per cent). Cochlear nerve deficiency was encountered in 41 ears (45.6 per cent). The internal auditory canal was also stenotic in 49 ears (54.4 per cent).ConclusionInner-ear malformations, especially cochlear aperture anomalies, are involved in the aetiology of single-sided deafness more than expected. The cause of single-sided deafness differs greatly between congenital and adult-onset cases. All children with single-sided deafness should undergo radiological evaluation, as the prognosis and management, as well as the aetiology, may be significantly influenced by inner-ear malformation type.

Sneeze-induced pneumolabyrinth 15 years after stapedotomy

2021 ◽  
Vol 14 (7) ◽  
pp. e243575
Author(s):  
Ya Fang Amanda Cheang ◽  
Seng Beng Yeo
Keyword(s):  
Hearing Loss ◽  
Pure Tone Audiometry ◽  
Semicircular Canals ◽  
Acute Onset ◽  
Sensorineural Hearing ◽  
Upper Respiratory Tract ◽  
Mixed Hearing Loss ◽  
Hearing Thresholds ◽  
History Of ◽  
Vestibular Symptoms

Pneumolabyrinth refers to the presence of air within the inner ear and is a fairly common occurrence immediately after stapes surgery, but rarely occurs in a delayed manner years after the initial operation. We present a case of a patient with a history of left stapedotomy 15 years prior, who presented with acute onset vertigo, tinnitus and hearing loss in her operated ear. Her symptoms were preceded by an upper respiratory tract infection associated with bouts of sneezing. Examination revealed a spontaneous right beating nystagmus and positive head thrust to the left. Pure tone audiometry demonstrated a left mixed hearing loss which subsequently deteriorated to a profound sensorineural hearing loss. CT showed the presence of air within the left vestibule and semicircular canals. The patient underwent an exploratory tympanotomy and repair of perilymphatic leak with resolution of vestibular symptoms but no improvement in sensorineural hearing thresholds.

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