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Cronkhite-Canada syndrome (CCS) is a very rare gastrointestinal disorder with ectodermal abnormalities. Taste abnormalities appear in more than 80% of cases. Our objective was to investigate the characteristics of CCS. Ten patients with taste abnormalities who were diagnosed with CCS were included. A medical interview, examination of the tongue findings, and blood tests were performed, and taste functions were assessed using an electrogustometry (EGM) and a filter paper disc (FPD) before and after treatment. There was nail atrophy in all cases, weight loss in 8 cases, hair loss in 6 cases, skin hyperpigmentation in 5 cases, gastrointestinal symptoms in 4 cases, and atrophy of the lingual papillae in at least 8 cases. Zinc therapy for taste disorders by the previous physicians was ineffective in all cases. The results of the FPD at the first examination showed a severe decrease in taste function of the anterior tongue, whereas taste function tended to be preserved in the posterior tongue (P < 0.01, Wilcoxon). In all cases, subjective symptoms improved within 3 mo after treatment of CCS. Taste function improved significantly after treatment (FPD in anterior tongue, P < 0.05, EGM in posterior tongue, P < 0.01, Wilcoxon). Taste disorder in CCS tended to be severe in the anterior tongue with findings of tongue papillary atrophy, which appears to be an ectodermal abnormality. Their taste function improved along with symptoms after treatment. The taste tests were useful for determining the effect of treatment for CCS., (© The Author(s) 2024. Published by Oxford University Press. All rights reserved. For commercial re-use, please contact reprints@oup.com for reprints and translation rights for reprints. All other permissions can be obtained through our RightsLink service via the Permissions link on the article page on our site—for further information please contact journals.permissions@oup.com.)

Though 270,000 patients with complaints of taste abnormalities visited medical institutions annually in 2019 survey, there are no drugs for the treatment of taste disorders that are covered by health insurance in Japan. In the survey, the number of patients with taste disorders was correlated with age, and the need for medical treatment for taste disorders is expected to increase in the future because of the super-aging society. The pathophysiology of taste disorders varies widely. There is an obvious need to decide the site and the causes of the disorder and understand the mechanism, by performing various examinations. It needs to first adjust the causative systemic diseases and medications in the treatment for taste disorder. Damage of taste cells due to zinc deficiency is the main pathophysiological mechanism of taste disorders, and zinc supplementation is a standard treatment in Japan. Oral zinc therapy is the treatment for taste disorders due to zinc deficiency or idiopathic taste disorder; though a double-blind study was conducted, it was considered low-level evidence in a clinical review. In Japan, the off-label use of polaprezinc for taste disorders was approved in 2011, and zinc acetate hydrate was approved for hypozincemia in March 2017, making it easier to use oral zinc therapy in general. In some cases, psychotherapy or herbal medicine therapy has been used with remarkable success, although its effectiveness has not been clearly tested. It might be expected to offer some help to patients. In the treatment of elderly patients with taste disorders, physicians need to consider the difference between "age-related changes in taste in healthy people" and "taste disorders in elderly persons", and they should separate them. Aggressive treatment is desirable regardless of age, because no significant difference in the efficacy of various treatments was found between patients older and younger than 65 years., Competing Interests: Declaration of Competing Interest The authors declare no conflicts of interest., (Copyright © 2023 Japanese Society of Otorhinolaryngology-Head and Neck Surgery, Inc. Published by Elsevier B.V. All rights reserved.)

Conclusion: Most of the clinical cases experienced taste disturbance after stapes surgery, and in a few cases this disturbance persisted for a long time. The animal experiment suggested the role of geniculate ganglion (GG) cells in nerve generation., Objectives: To clinically examine taste disorder and its recovery after stapes surgery and experimentally demonstrate a role of GG., Patients and Methods: Taste function after preservation of chorda tympani nerve (CTN) in stapes surgery was prospectively investigated with a questionnaire and electrogustometry (EGM). Further, expression of neurotrophic factors in GG after injury of CTN was examined by in situ hybridization histochemistry (ISSH) and RT-PCR., Results: Among the cases, 15/18 (83.3%) were associated with taste disturbance and 6/18 (33.3%) were associated with tongue numbness 2 weeks after surgery; however, the symptoms ceased in 14/18 cases (77.8%). Two weeks after surgery, the EGM threshold was found to be elevated in 15/18 cases (83.3%), while in 10/18 cases (55.6%), it did not decrease until 1 year after surgery. Expression of ISSH and amplified bands of BDNF and GFR increased at 7 and 14 days after nerve injury in ipsilateral GGs and also increased at 7 days on the contralateral side.

Objectives: Chorda tympani nerve (CTN) function may be damaged more by two‐stage than by one‐stage surgery for middle ear cholesteatoma. However, few studies have reported the relationship between two‐stage cholesteatoma surgery and CTN function. This study aimed to investigate CTN function after two‐stage surgery for cholesteatoma. Methods: In this prospective study, 35 patients underwent two‐stage canal wall up tympanoplasty (CWUT). Perioperative CTN function was assessed using questionnaires and electrogustometry (EGM). Participants were categorized into minor, major, and section groups, based on the degree of CTN manipulation during surgery. Results: In the first‐stage surgery, posterior tympanotomy with an intact canal wall reduced the degree of CTN manipulation. The incidence of taste disorder after the first‐stage surgery was 71.4%. Postoperative taste disorder and the EGM threshold improved early in the minor manipulation group. In the second‐stage surgery, no new CTN damage occurred, even if this surgery involved removal of residual cholesteatoma. The incidence of taste disorder after second‐stage surgery was less than that after first‐stage surgery, independent of CTN preservation. However, the recovery rate of the EGM threshold after second‐stage surgery was significantly lower in the section group than in those with CTN preservation. Conclusion: CTN function, including symptoms and EGM threshold, can be preserved during two‐stage cholesteatoma surgery if care is taken to preserve the CTN in both the first‐ and second‐stage surgeries. A two‐stage CWUT, ensuring an intact bony annulus, may be effective to facilitate CTN preservation. Level of Evidence: 2b. [ABSTRACT FROM AUTHOR]

Conclusion: Most of the clinical cases experienced taste disturbance after stapes surgery, and in a few cases this disturbance persisted for a long time. The animal experiment suggested the role of geniculate ganglion (GG) cells in nerve generation. Objectives: To clinically examine taste disorder and its recovery after stapes surgery and experimentally demonstrate a role of GG. Patients and methods: Taste function after preservation of chorda tympani nerve (CTN) in stapes surgery was prospectively investigated with a questionnaire and electrogustometry (EGM). Further, expression of neurotrophic factors in GG after injury of CTN was examined by in situ hybridization histochemistry (ISSH) and RT-PCR. Results: Among the cases, 15/18 (83.3%) were associated with taste disturbance and 6/18 (33.3%) were associated with tongue numbness 2 weeks after surgery; however, the symptoms ceased in 14/18 cases (77.8%). Two weeks after surgery, the EGM threshold was found to be elevated in 15/18 cases (83.3%), while in 10/18 cases (55.6%), it did not decrease until 1 year after surgery. Expression of ISSH and amplified bands of BDNF and GFR increased at 7 and 14 days after nerve injury in ipsilateral GGs and also increased at 7 days on the contralateral side. [ABSTRACT FROM AUTHOR]