scholarly journals A Case of Perilymphatic Fistula with Inner Ear Anomaly Diagnosed Preoperatively by the Cochlin-Tomoprotein Detection Test

2020 ◽  
Vol 2020 ◽  
pp. 1-4
Author(s):  
Kana Lee ◽  
Naoki Ochi ◽  
Kohei Yamahara ◽  
Kunihiko Makino ◽  
Tetsuo Ikezono
Keyword(s):  
Hearing Loss ◽  
Sensorineural Hearing Loss ◽  
Inner Ear ◽  
Clinical Course ◽  
Sudden Hearing Loss ◽  
Sensorineural Hearing ◽  
Fistula Repair ◽  
Perilymphatic Fistula ◽  
Ear Anomalies ◽  
Inner Ear Anomaly

We present a case of perilymphatic fistula (PLF) with inner ear anomalies having sudden, progressive sensorineural hearing loss and describe the fistula repair surgeries. We focus on the diagnosis methods of PLF and clinical course of PLF with inner ear anomaly. The cochlin-tomoprotein (CTP) detection test is very useful for the surgeons to encourage the earlier operation to sudden hearing loss cases. It is also helpful to define the diagnosis of PLF after operation. We could not get the good result as to hearing from the fistula repair surgery mainly because surgery was held 1 month after the onset. The results of the case, as well as recommendations of other reports, suggest that patients with sudden sensorineural hearing loss and PLF may need repair surgery within at most 2 weeks from the onset. We describe how to diagnose PLF more accurately using CTP detection combined with intraoperative findings.

Noise-Induced Autoimmune Sensorineural Hearing Loss

2003 ◽  
Vol 112 (7) ◽  
pp. 569-573 ◽  
Author(s):  
Reena Gupta ◽  
Robert T. Sataloff
Keyword(s):  
Hearing Loss ◽  
Sensorineural Hearing Loss ◽  
Inner Ear ◽  
Environmental Noise ◽  
Sudden Hearing Loss ◽  
Sensorineural Hearing ◽  
Autoimmune Conditions ◽  
Inner Ear Disease ◽  
Asymmetric Hearing Loss ◽  
Autoimmune Inner Ear Disease

Typically, autoimmune sensorineural hearing loss has been described as a slowly progressive, asymmetric hearing loss that is responsive to medications traditionally used in the treatment of other autoimmune conditions. These medications include steroids and cytotoxic drugs. Inciting factors in autoimmune inner ear disease are rarely cited. We describe a case of episodic sudden hearing loss triggered consistently by environmental noise. The hearing loss was responsive to steroids at the time of each occurrence and was determined to be autoimmune. This case raises questions about the relationship between autoimmune inner ear disease and sensitivity to environmental noise that warrant further research.

Sudden Sensorineural Hearing Loss Associated with Inner Ear Anomaly

2005 ◽  
Vol 26 (2) ◽  
pp. 241-246 ◽  
Author(s):  
Makoto Sugiura ◽  
Tsutomu Nakashima ◽  
Shinji Naganawa ◽  
Yasutaka Otake ◽  
Toru Mukaida ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Sensorineural Hearing Loss ◽  
Inner Ear ◽  
Sudden Sensorineural Hearing Loss ◽  
Sensorineural Hearing ◽  
Inner Ear Anomaly

scholarly journals Genetics of Inner Ear Malformations: A Review

2021 ◽  
Vol 11 (4) ◽  
pp. 524-536
Author(s):  
Davide Brotto ◽  
Flavia Sorrentino ◽  
Roberta Cenedese ◽  
Irene Avato ◽  
Roberto Bovo ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Sensorineural Hearing Loss ◽  
Inner Ear ◽  
18Th Century ◽  
Sensorineural Hearing ◽  
Genetic Mutations ◽  
Inner Ear Malformations ◽  
Ear Malformations ◽  
Inner Ear Anomaly ◽  
First Descriptions

Inner ear malformations are present in 20% of patients with sensorineural hearing loss. Although the first descriptions date to the 18th century, in recent years the knowledge about these conditions has experienced terrific improvement. Currently, most of these conditions have a rehabilitative option. Much less is known about the etiology of these anomalies. In particular, the evolution of genetics has provided new data about the possible relationship between inner ear malformations and genetic anomalies. In addition, in syndromic condition, the well-known presence of sensorineural hearing loss can now be attributed to the presence of an inner ear anomaly. In some cases, the presence of these abnormalities should be considered as a characteristic feature of the syndrome. The present paper aims to summarize the available knowledge about the possible relationships between inner ear malformations and genetic mutations.

Inner Ear Anomalies Causing Congenital Sensorineural Hearing Loss: CT and MR Imaging Findings

2005 ◽  
Vol 52 (3) ◽  
pp. 165
Author(s):  
Hyun Sook Hong ◽  
Sang Hyun Paik ◽  
Jang Gyu Cha ◽  
Seong Jin Park ◽  
Joon Hee Joh ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Mr Imaging ◽  
Sensorineural Hearing Loss ◽  
Inner Ear ◽  
Sensorineural Hearing ◽  
Imaging Findings ◽  
Ear Anomalies

Characteristics of sensorineural hearing loss in children with inner ear anomalies

2006 ◽  
Vol 27 (1) ◽  
pp. 33-38 ◽  
Author(s):  
James M. Coticchia ◽  
Abhay Gokhale ◽  
Josh Waltonen ◽  
Baran Sumer
Keyword(s):  
Hearing Loss ◽  
Sensorineural Hearing Loss ◽  
Inner Ear ◽  
Sensorineural Hearing ◽  
Ear Anomalies

Drug Delivery to the Inner Ear: Strategies and Their Therapeutic Implications for Sensorineural Hearing Loss

2012 ◽  
Vol 9 (3) ◽  
pp. 231-242 ◽  
Author(s):  
Teresa Rivera ◽  
Lorena Sanz ◽  
Guadalupe Camarero ◽  
Isabel Varela-Nieto
Keyword(s):  
Drug Delivery ◽  
Hearing Loss ◽  
Sensorineural Hearing Loss ◽  
Inner Ear ◽  
Sensorineural Hearing ◽  
Therapeutic Implications

Sudden bilateral sensorineural hearing loss associated with urticarial vasculitis

2013 ◽  
Vol 127 (7) ◽  
pp. 708-711 ◽  
Author(s):  
A C Hall ◽  
A C Leong ◽  
D Jiang ◽  
A Fitzgerald-O'Connor
Keyword(s):  
Hearing Loss ◽  
Sensorineural Hearing Loss ◽  
Cochlear Implantation ◽  
Skin Lesions ◽  
Sudden Hearing Loss ◽  
Sudden Onset ◽  
Sensorineural Hearing ◽  
Urticarial Vasculitis ◽  
Wells Syndrome ◽  
Bilateral Sensorineural Hearing Loss

AbstractBackground:Bilateral sensorineural hearing loss associated with recurrent urticarial skin lesions may be signs of underlying Muckle–Wells syndrome. Previous reports have described the hearing loss to be progressive in nature.Method:To our knowledge, this paper presents the first published case of sudden onset, bilateral sensorineural hearing loss associated with urticarial vasculitis due to underlying Muckle–Wells syndrome.Results:The patient underwent a cochlear implantation with a modest outcome.Conclusion:Cochlear implantation may help to rehabilitate sudden hearing loss associated with this condition, but early diagnosis may allow treatment with interleukin-1β inhibitors such as anakinra.

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