Multiple endocrine neoplasia (MEN) type 2 syndrome is an autosomal dominant inherited disease caused by mutations of the RET proto-oncogene, and is clinically divided into three phenotypes: MEN2A, MEN2B, and familial medullary thyroid carcinoma. Although multiple mucosal neuromas are commonly observed in patients with MEN2B, there are only a few reports of laryngeal neuroma. We present here a rare case of laryngeal mucosal neuromas with MEN2B.
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http://dx.doi.org/10.1016/j.anl.2013.12.007 | DOI Listing |
Auris Nasus Larynx
December 2024
Department of Neurosurgery, Fukushima Medical University School of Medicine, Japan.
Objective: NF2-related schwannomatosis (NF2) is characterized by bilateral vestibular schwannomas (VS), often causing severe damage to the bilateral auditory function. Auditory brainstem implantation (ABI) provides hearing-impaired patients with an opportunity to reacquire auditory sensation through electrical stimulation of auditory neurons in the cochlear nucleus. However, ABI is not covered by public health insurance in Japan, leading to a large difference in financial burden compared to cochlear implantation (CI).
View Article and Find Full Text PDFEar Nose Throat J
September 2024
Department of Otolaryngology and Head and Neck Surgery, American University of Beirut Medical Center, Beirut, Lebanon.
Multiple endocrine neoplasia type 2B (MEN2B) syndrome is a rare genetic disorder primarily driven by mutations in the RET proto-oncogene. MEN2B exhibits diverse symptoms affecting multiple organ systems, with mucosal neuromas being a prominent feature. These benign tumors contribute to the syndrome's clinical variability, alongside medullary thyroid carcinoma and pheochromocytoma.
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October 2024
Department of Otolaryngology-Head and Neck Surgery, Sapporo Medical University School of Medicine, Minami 1-jo Nishi 16-chome, Chuo-ku, Sapporo, Hokkaido, 060-8543, Japan.
Objective: The objective of this study was to assess the utility of narrow-band imaging (NBI) for improving intraneural dissection during gross total resection of head and neck schwannoma. Specifically, we aimed to quantitatively evaluate whether NBI can enhance the identification of pseudocapsule and true capsule within the tumor.
Methods: Nine schwannoma surgery cases conducted between February 2018 and October 2022 were retrospectively analyzed.
BMJ Case Rep
July 2024
All India Institute of Medical Sciences - Mangalagiri, Guntur, Andhra Pradesh, India.
Laryngeal schwannoma is a rare type of neurogenous tumour with a non-specific presentation. We present a case of a middle-aged man with issues of hoarseness, globus sensation and intermittent episodes of throat pain. The Hopkins examination showed right vocal fold irregularity with fullness in the right ventricle.
View Article and Find Full Text PDFAuris Nasus Larynx
August 2024
Department of Hearing implant Sciences, Shinshu University School of Medicine, Matsumoto, Japan. Electronic address:
Objective: Acute sensorineural hearing loss represents a spectrum of conditions characterized by sudden onset hearing loss. The "Clinical Practice Guidelines for the Diagnosis and Management of Acute Sensorineural Hearing Loss" were issued as the first clinical practice guidelines in Japan outlining the standard diagnosis and treatment. The purpose of this article is to strengthen the guidelines by adding the scientific evidence including a systematic review of the latest publications, and to widely introduce the current treatment options based on the scientific evidence.
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