Benign positional vertigo after cochlear implantation

2005 ◽  
Vol 132 (5) ◽  
pp. 741-745 ◽  
Author(s):  
C. J. Limb ◽  
H. F. Francis ◽  
L. R. Lustig ◽  
J. K. Niparko ◽  
H. Jammal
Keyword(s):  
Hearing Loss ◽  
Cochlear Implantation ◽  
Medical Records ◽  
Contributing Factors ◽  
Tertiary Referral Center ◽  
Vestibular Hypofunction ◽  
Positional Vertigo ◽  
Inner Ear Disease ◽  
Autoimmune Inner Ear Disease ◽  
Head Neck

OBJECTIVE: To identify patients who underwent cochlear implantation (CI) and who subsequently developed benign positional vertigo (BPV) after the procedure and to identify any contributing factors. STUDY DESIGN AND SETTING: Academic tertiary referral center. Cochlear implant recipients’ medical records were retrospectively reviewed to identify patients with both vertigo and, more specifically, BPV. Preoperative, intraoperative, and postoperative factors were studied vis-à-vis the development of BPV. RESULTS: BPV was newly diagnosed in 12 patients after CI. The etiology of hearing loss included presbycusis (16.6%), autoimmune inner ear disease (16.6%), congenital hearing loss (41.6%), Meniere's disease (8.3%), prematurity (8.3%), and idiopathic factors (8.3%). The onset of BPV varied after the procedure (mean ± SD, 292 ± 309 days). BPV symptoms did not affect implant performance. All patients were treated for BPV by Epley's maneuver and vestibular exercises. Symptoms disappeared in 11 patients and persisted in 1. CONCLUSIONS: BPV is an uncommon development after CI, although it occurs more frequently than in the general population. Two theories are proposed: the introduction of bone dust into the labyrinth and the dislodging of otoconia during surgery. The diagnosis, treatment, and prognosis of BPV after CI do not differ from those for non-CI-associated BPV. SIGNIFICANCE: Dizziness after CI usually develops as a result of vestibular hypofunction. BPV, which is a hyperfunctioning form of vestibular dysfunction, should be recognized as a possible sequelae of CI. (Otolaryngol Head Neck Surg 2005;132:741-5.)

Cochlear Implantation in Autoimmune Inner Ear Disease

2008 ◽  
Vol 139 (2_suppl) ◽  
pp. P53-P53 ◽  
Author(s):  
Katrina R Stidham ◽  
Joseph B Roberson
Keyword(s):  
Hearing Loss ◽  
Inner Ear ◽  
Immunosuppressive Therapy ◽  
Cochlear Implantation ◽  
Hearing Aid ◽  
Speech Discrimination ◽  
Inner Ear Disease ◽  
Potential Risks ◽  
The Impact ◽  
Autoimmune Inner Ear Disease

Objective 1) Evaluate the success of cochlear implantation (CI) in patients with autoimmune inner ear disease (AIED). 2) Discuss the impact of CI on consideration of immunosuppressive therapy for patients with fluctuating hearing loss due to AIED. Methods Retrospective chart review was conducted of patients undergoing CI with diagnosis of AIED between 6/02 and 6/07. Patients’ autoimmune and immunotherapy treatment history was explored. Non-operative ear hearing thresholds and speech discrimination scores (SDS) were documented pre- and postoperatively. Objective and subjective implant success in setting of fluctuating hearing in non-implant ear was evaluated. Results Four patients with AIED, aged 5–55, underwent CI. Two had isolated otologic symptoms and 2 had other systemic autoimmune diagnoses. All had been previously treated with steroids. Three had received other immunosuppressive therapy. One declined other treatment due to potential risks. At the time of implantation, 3 patients had functional hearing in the non-implant ear and received benefit from a hearing aid. Each continued to have usable hearing, with fluctuating improvements post-implant at times to much better SDS than would normally meet candidacy requirements for CI. All patients found the implant provided better hearing than the hearing-aid ear. Conclusions CI is a viable option for patients with AIED. Implanting a profoundly deafened ear where opposite ear has fluctuating but usable hearing does not negatively impact outcomes. With the success of CI for AIED, the potential risks of long-term immunosuppressive therapy may not warrant its use in patients with isolated hearing loss symptoms.

Azathioprine in Combination with Steroids in the Treatment of Autoimmune Inner-Ear Disease

1993 ◽  
Vol 21 (4) ◽  
pp. 192-196 ◽  
Author(s):  
Aytac Saraçaydin ◽  
Sedat Katircioğlu ◽  
Sami Katircioğlu ◽  
M Can Karatay
Keyword(s):  
Hearing Loss ◽  
Sensorineural Hearing Loss ◽  
Inner Ear ◽  
Sensorineural Deafness ◽  
Sensorineural Hearing ◽  
Once Daily ◽  
Uncommon Disease ◽  
Inner Ear Disease ◽  
Pure Tones ◽  
Autoimmune Inner Ear Disease

A total of twelve patients with a relatively uncommon form of progressive sensorineural deafness (autoimmune innerear disease) were treated orally with 1 mg/kg azathioprine, once daily, and with 30 mg prednisolone, every other day, for 4 weeks. Statistically significant increases in the ability to hear pure tones or in discrimination on audiometry took place in 10/12 patients. This condition was initially described as ‘sensorineural hearing loss', but it is now clear that the term ‘autoimmune inner-ear disease’ is more appropriate since the vestibular compartment as well as the cochlear compartment is involved. This relatively uncommon disease is one of the few forms of sensorineural deafness that can be successfully treated.

Cochlear Implantation Outcomes in Children With CDH23 Mutations–Associated Hearing Loss

2021 ◽  
pp. 019459982110574
Author(s):  
Kaitian Chen ◽  
Bixue Huang ◽  
Jincangjian Sun ◽  
Yue Liang ◽  
Guanxia Xiong
Keyword(s):  
Hearing Loss ◽  
Cochlear Implantation ◽  
Outer Hair Cell ◽  
Cell Function ◽  
Hearing Level ◽  
Case Review ◽  
Retrospective Case ◽  
Profound Hearing Loss ◽  
Tertiary Referral Center ◽  
Cadherin 23

Objective Mutations in the cadherin 23 gene ( CDH23) have been reported to cause cochlear damage, but few studies have investigated the auditory and speech outcome of patients after cochlear implantation. Here, we describe the genetic, auditory, and postoperative outcomes of patients with CDH23 mutations who received cochlear implants. Study Design Retrospective case review. Setting Tertiary referral center. Methods Targeted deafness-related gene panels were sequenced in Chinese families with profound sensorineural hearing loss. The clinical features of subjects carrying potentially pathogenic CDH23 mutations were analyzed. Results Between 2017 and 2019, we identified 5 children with prelinguistically profound hearing loss at our center who harbored 6 variants of CDH23 that segregated with the disease. Of these, 4 variants were novel (c.2591G>T, c.4785G>C, c.5765A>G, and c.9280_9281insTT). All affected individuals had a loss of outer hair cell function, with an average residual hearing level of 3 to 10 dB SPL. Cochlear implantations were arranged for the patients at 11 to 36 months of age. All children made gains in their hearing, language, and speech performances 14 to 120 months after surgery. Their auditory outcomes improved during follow-up intervals. Conclusion This study revealed that children with congenital cochlear defects caused by CDH23 variants can acquire an acceptable auditory and speech outcome after cochlear implantation. Early genetic detection and prenatal counseling for rare deafness genes such as CDH23 remain a priority for the future.

Cochlear implantation in patients with autoimmune inner ear disease including cogan syndrome: A comparison with age- and sex-matched controls

2010 ◽  
Vol 120 (12) ◽  
pp. 2478-2483 ◽  
Author(s):  
Jenny R. Wang ◽  
Heng W. Yuen ◽  
David B. Shipp ◽  
Suzanne Stewart ◽  
Vincent Y. W. Lin ◽  
...  
Keyword(s):  
Inner Ear ◽  
Cochlear Implantation ◽  
Cogan Syndrome ◽  
Inner Ear Disease ◽  
Autoimmune Inner Ear Disease ◽  
Age And Sex

Autoimmune inner ear disease: A treatment update

1984 ◽  
Vol 98 (S9) ◽  
pp. 220-227 ◽  
Author(s):  
T. J. Yoo ◽  
J. J. Shea ◽  
J. Per Lee ◽  
A. Shulman ◽  
Y. Yazawa ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Middle Ear ◽  
Sensorineural Deafness ◽  
High Dose ◽  
Low Grade ◽  
Tissue Destruction ◽  
Laboratory Features ◽  
Inner Ear Disease ◽  
Vestibular Symptoms ◽  
Autoimmune Inner Ear Disease

McCabe (1979) described 18 cases of sensorineural deafness having clinical and laboratory features suggestive of an auto-immune etiology. This condition was characterized by a prolonged period of pro-gressive deafness over weeks or months, rather than hours, days or years. It is usually bilateral, asymmetrical and the cochlear is always involved. Other features of this disorder are facial paralysis and vestibular dysfunction. Tissue destruction of the tympanic membrane and middle ear and mastoid may also occur. These responded well to a high dose of steroid therapy and cyclophosphamide. A total of 42 cases of this disorder were diagnosed in McCabe's series as of January, 1982 (McCabe and Harker, 1982). Two-thirds of the patients had vestibular symptoms of low grade without spells and the reduction in caloric responses paralleled the hearing loss.

Autoimmune Inner Ear Disease Preliminary Case Report: Audiometric Findings Following Steroid Treatments

2013 ◽  
Vol 22 (1) ◽  
pp. 120-124 ◽  
Author(s):  
Dave A. Harris ◽  
Anthony A. Mikulec ◽  
Sarah L. Carls
Keyword(s):  
Hearing Loss ◽  
Inner Ear ◽  
Steroid Treatment ◽  
Special Focus ◽  
Saint Louis ◽  
Saint Louis University ◽  
Oral Steroids ◽  
Inner Ear Disease ◽  
Autoimmune Inner Ear Disease ◽  
Intratympanic Steroids

Purpose This study examined the audiometric findings following steroid treatment for autoimmune inner ear disease (AIED), which is characterized by bilateral subacute hearing loss, using oral and intratympanic treatment. Method The charts of 9 patients with AIED who had been seen in the Saint Louis University School of Medicine's clinic between 2004 and 2011 were reviewed, with special focus on audiometric results. Results The majority of patients benefited from treatment for AIED. Four patients (6 ears) received intratympanic therapy as part of their treatment. Conclusion Patients with AIED appeared to benefit from a clinical pathway using a prolonged course of oral steroids, followed by a trial of intratympanic steroids and/or immunosuppresants for refractory cases.

scholarly journals Unilateral autoimmune inner ear disease in a patient with lung cancer treated with nivolumab

2020 ◽  
Vol 2020 (9) ◽  
Author(s):  
Aleksandra Rajapakse ◽  
Connor O’Leary ◽  
Raefe Gundelach ◽  
Rajeev Deva ◽  
Ken O’Byrne
Keyword(s):  
Lung Cancer ◽  
Hearing Loss ◽  
Inner Ear ◽  
Metabolic Response ◽  
Early Stage ◽  
Oral Prednisolone ◽  
Gradual Improvement ◽  
Inner Ear Disease ◽  
Bilateral Sensorineural Hearing Loss ◽  
Autoimmune Inner Ear Disease

ABSTRACT A 69-year-old male presented with early stage non-small cell lung cancer in 2016. The tumor was resected; however, the patient experienced recurrence 2 years later and subsequently received paclitaxel/carboplatin concurrently with radiotherapy. Within weeks of completing this treatment, he developed a symptomatic pancoast tumor secondary to disease progression and commenced second line nivolumab. Following the second dose of nivolumab, he developed acute unilateral right hearing loss. He commenced intravenous methylprednisolone followed by a slow taper of oral prednisolone. With steroids, he noted a gradual improvement in hearing, confirmed by audiology. Restaging imaging post-nivolumab demonstrated a complete metabolic response. Two prior cases have reported bilateral sensorineural hearing loss post-immune checkpoint inhibitor (ICI). We postulate the hearing impairment relates to the development of autoimmune inner ear disease. To our knowledge, this is the only case of a patient experiencing unilateral loss of hearing following an ICI.

Complications in Pediatric Cochlear Implants

2008 ◽  
Vol 139 (2_suppl) ◽  
pp. P53-P54
Author(s):  
Anita Jeyakumar ◽  
Randall A Clary
Keyword(s):  
Hearing Loss ◽  
Cochlear Implants ◽  
Cochlear Implantation ◽  
Pediatric Patients ◽  
Demographic Information ◽  
Tertiary Referral Center ◽  
Common Etiology ◽  
Cerebrospinal Fluid Leaks ◽  
Common Device

Objective Be able to retrospectively review the complications of pediatric patients undergoing cochlear implantation at a tertiary referral center. Methods IRB permission was obtained. Retrospective analysis of all children who underwent cochlear implantation from January 2001 to December 2005 at a major academic cochlear implantation center. The patients were reviewed for demographic information, type of hearing loss, cochlear implant device, and complications, including implant failure, meningitis, hematomas (intracranial and extracranial), implant extrusion, cerebrospinal fluid leaks, facial palsy, and wound infections. Results A total of 165 patients records were reviewed. 53.5% of the patients were male. The most common device used was the Freedom NucleusTM (60%). The most common etiology of hearing loss was non-syndromic, non-genetic, congenital sensorineural hearing loss (60.6%) All patients had a follow-up of at least 1 year. The most common complication seen was device failure (3.0%), followed by flap infections (2.6%), and immediate postoperative hematomas (1.9%). There was no difference between the kind of device which failed, statistically. There was only one case of Ps. aeruginosa meningitis in a child with a Mondini deformity, who had the implant removed. Conclusions Cochlear implantation in children continues to be reliable and safe, with a low percentage of severe complications, in experienced hands. The patients should have lifetime follow-up.

scholarly journals Sudden Bilateral Sensorineural Hearing Loss Associated with HLA A1-B8-DR3 Haplotype

2013 ◽  
Vol 2013 ◽  
pp. 1-4
Author(s):  
G. Psillas ◽  
M. Daniilidis ◽  
A. Gerofotis ◽  
K. Veros ◽  
A. Vasilaki ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Sensorineural Hearing Loss ◽  
Inner Ear ◽  
Cyclosporine A ◽  
Immunosuppressive Treatment ◽  
Clinical Manifestations ◽  
Sensorineural Hearing ◽  
High Dose ◽  
Inner Ear Disease ◽  
Autoimmune Inner Ear Disease

Sudden sensorineural hearing loss may be present as a symptom in systemic autoimmune diseases or may occur as a primary disorder without another organ involvement (autoimmune inner ear disease). The diagnosis of autoimmune inner ear disease is still predicated on clinical features, and to date specific diagnostic tests are not available. We report a case of bilateral sudden hearing loss, tinnitus, intense rotatory vertigo, and nausea in a female patient in which the clinical manifestations, in addition to raised levels of circulating immune complexes, antithyroglobulin antibodies, and the presence of the HLA A1-B8-DR3 haplotype, allowed us to hypothesize an autoimmune inner ear disease. Cyclosporine-A immunosuppressive treatment in addition to steroids helped in hearing recovery that occurred progressively with normalization of the hearing function after a five-month treatment. Cyclosporine-A could be proposed as a therapeutic option in case of autoimmune inner ear disease allowing the suspension of corticosteroids that, at high dose, expose patients to potentially serious adverse events.

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