It has been reported that delayed facial nerve palsy could sometimes occur after otologic surgery. We encountered a case of delayed onset of Ramsay Hunt syndrome after cochlear implantation.

The patient was a 71-year-old woman who began wearing hearing aids at the age of 69 for bilateral hearing loss. She then visited our department for cochlear implantation. Cochlear implantation was performed in a routine manner, and was completed without exposure of the facial nerve or chorda tympani. The insertion of the electrodes was smooth. After left cochlear implantation, the patient had good hearing. However, 12 days after the surgery, she presented with dizziness, auricular herpes zoster, and facial nerve palsy. The serum titer of varicella zoster virus (VZV) IgG was significantly high and the patient was diagnosed as showing VZV reactivation. She did not improve with the usual medications or to ENoG, and facial nerve decompression was performed; we used the TEES approach for the facial decompression in consideration of the effect on the inserted electrodes. Facial nerve decompression via the TEES approach is considered to be useful for decompressing at the horizontal portion of the facial nerve. The facial nerve palsy is slowly improving, and we are continuing to monitor the patient’s progress.

We think that careful follow-up after otologic surgery is necessary, keeping in mind the possibility of development of delayed facial nerve palsy and Ramsay Hunt syndrome.

The efficacy of transmastoid facial nerve decompression surgery for severe peripheral facial nerve palsy has been under debate for a long time, and continues to remain controversial. Therefore, we conducted a retrospective review of the patients’ backgrounds, timing of surgery, intraoperative findings, and prognosis, in particular, 1) the effect of the period from the onset to surgery on the 1-year prognosis after surgery, and 2) the correlation between the intraoperative facial nerve monitor reaction and the prognosis in 30 cases of delayed recovery from peripheral facial nerve palsy treated by transmastoid facial nerve decompression surgery. The results showed that there was no significant difference in the Yanagihara score at six months after surgery between the patients operated within and over 50 days after the onset, however, in those operated over 60 days after the onset, the Yanagihara score was significantly lower than those operated within 60 days after the onset. There was also a significant correlation between the presence/absence of response to intraoperative NIM and the Yanagihara score at six months after surgery. In other words, patients who responded to intraoperative NIM fared significantly better than those who did not. On the other hand, the prognosis was significantly worse in patients who underwent surgery at more than 2 months after the onset than in patients who underwent surgery earlier, and it is important to make patients aware of this information. From now on, we will continue to do surgery only cases who show no satisfactory response to conservative treatment for severe paralysis and for whom there are no other effective treatment options, and only in cases where the family, including the patient, strongly desires to receive the treatment.

Acyclovir and valacyclovir are effective therapeutic agents for viral infections caused by viruses such as herpes simplex virus type 1 (HSV-1) and varicella-zoster virus (VZV). These viruses are also known to be the causative viruses of peripheral facial palsies, such as Bell’s palsy and Ramsay Hunt syndrome. While acyclovir and valacyclovir are commonly used in the field of otolaryngology, they may cause renal dysfunction or encephalopathy as a side effect in rare cases. The patient reported herein was an 83-year-old man who developed left peripheral facial palsy and was prescribed a steroid and valacyclovir (3000 mg/day) to be taken at home. The patient was uncomfortable drinking fluids due to spillage from the mouth as a result of the paralysis, and reduced his fluid intake. Four days after visiting our department, he developed consciousness disorder and visited our hospital again. Dialysis and fluid management were initiated to treat the dehydration, drug-induced acute renal injury, and acyclovir encephalopathy. In the elderly, even if renal function appears to be normal in pre-medication tests, it may deteriorate during treatment with acyclovir or valacyclovir, due to environmental changes, decreased reserve capacity, dehydration, etc., and furthermore, may be complicated by acyclovir encephalopathy. Therefore, when administering acyclovir or valacyclovir to the elderly, it is necessary to provide sufficient guidance to the patients regarding adequate fluid intake; it may also be advisable to consider the use of alternative drugs.

We report a rare case of Ramsay Hunt syndrome in a patient with herpes zoster affecting the contralateral trigeminal nerve. A 69-year-old woman presented with a history of having become aware of a painful rash on the left forehead since 4 days prior to her first visit to our otolaryngology department. Three days later, she was referred to our dermatology department and was diagnosed as having ophthalmic zoster. In the evening on the same day, she noticed that she could not drink liquids through a straw. At her first visit, examination revealed painful redness in the right external ear and external auditory canal. Right facial palsy was also seen, with a score of 12 on the Yanagihara facial nerve grading system. In the audiogram, while there was no significant difference between the two sides, the hearing threshold of 8 kHz on the right side was 15 dB worse than that on the left side. Stapedial reflexes were positive on both sides. In the electric taste tests, the thresholds in the right chorda tympani nerve and greater petrosal nerve regions were outside the scale, whereas those in the right and left glossopharyngeal nerve regions were within the normal range. From her first visit, she was begun on intravenous hydrocortisone treatment, with gradual tapering of the dose from 500 mg daily, for 14 days. Antiviral drug was also administered. ENoG performed on day 10 after the initial visit was 65%. The prognosis was anticipated to be good. By day 22 after the initial visit, the redness of the right auricle disappeared, and by day 27, the facial nerve paralysis also resolved. The test for virus antibody (EIA) performed on day 13 after the initial visit revealed a significant increase in the VZV IgG titer. As the mechanism of onset in this case, transmission through the cerebrospinal fluid or simultaneous reactivation of latent VZV in the ganglion of the opposite side was speculated.

再発性多発軟骨炎は全身の軟骨組織，特に耳，鼻，喉頭，気管軟骨などの繰り返す炎症と破壊に加え，眼炎症，蝸牛・前庭機能障害，関節炎などの多彩な症状を来す稀な全身性の疾患である．神経障害はそのうち3％程度しか生じないとされている．症例は84歳女性．左顔面神経麻痺，左耳介腫脹と発熱で前医を受診した．左Ramsay Hunt症候群と考えられ，抗ウイルス薬が投与されたが改善はみられず，両側難聴や吸気性喘鳴も出現したため，当科へ転院搬送となった．入院後，両側感音難聴，声門下腫脹，強膜炎の所見に加え，耳介腫脹も両側で認めたため，診断基準より再発性多発軟骨炎と診断した．顔面神経麻痺も一症候として説明可能であった．プレドニゾロン40 mg/日の投与を開始したところ症状の著明な改善を得た．稀ではあるが顔面神経麻痺と両側の耳介炎症をみた場合，再発性多発軟骨炎も鑑別の一つである．

Relapsing polychondritis (RP), which is a multisystem, inflammatory, autoimmune disease affecting the cartilage, is relatively rare. RP is characterized by swelling of the auricles, audiovestibular disorders, and airway chondritis. Symptoms commonly appear in the ears, neck, and throat. Here in, we describe the case of a patient with RP who developed complete, bilateral hearing loss over the course of one to two months.

A 69-year-old, woman presented with a history of left auricular swelling, vertigo, and bilateral hearing impairment. She had previously been diagnosed as having incomplete Ramsay Hunt syndrome and treated with steroids, but her hearing had failed to improve. Over the course of time, the bilateral hearing impairment progressed to complete deafness. She was referred to our hospital for further management of the deafness and subglottic swelling and was admitted for treatment.

An auricular cartilage biopsy revealed no lymphocytic infiltration, but showed cartilage degeneration. Based on these findings, the patient was diagnosed as having auricular chondritis.

RP was later diagnosed based on the presence of bilateral auricular swelling, airway chondritis, episcleritis, and audiovestibular disorders, which fulfilled the diagnostic criteria for the disease. Pulse methylprednisolone therapy improved the subglottic swelling, auricular swelling, and episcleritis. Although the patient also received intravenous cyclophosphamide therapy (IVCY), the bilateral hearing loss failed to improve.

Inner ear disorders in RP are often resistant to steroid therapy and have a poor, long-term prognosis. The patient received steroid treatment from an early stage, but her hearing failed to improve. Based on a previous study demonstrating the efficacy of cochlear implants in cases of RP-related hearing loss, cochlear implants were recommended to the patient, but she declined the surgery.

Histopathological diagnosis of RP is often made during steroid and/or immunosuppressant therapy administration, as in this case, or afterwards, so that the typical features of the disease may not be evident. Therefore, when making a histopathological diagnosis, it is necessary to consider the possibility that the findings may have been affected by previous treatments.

2016年4月から2019年5月に当院で診断された外国人労働者の成人水痘5例について報告する．また同時期に診断した日本人の成人水痘5例，すべての水痘および帯状疱疹患者の年代別分布，発症月別分布も疫学的に検討した．外国人水痘患者5例はすべてベトナム人であり，平均年齢は22.6±0.8歳であった．日本人成人水痘患者5例は，平均年齢が64.6±21.2歳と年代にばらつきがあった．成人水痘患者を含む水痘患者の総数は54例であり，その年代別分布において10歳未満の小児が41例（75％）と多かった．帯状疱疹患者総数は73例であり日本人のみであった．その年代別分布において発症は70歳代が最も多く，21例（28.8％）であった．水痘と帯状疱疹患者の発症月別分布を比較すると，帯状疱疹患者は2月と8月に多く1年を通して発症していたが，水痘患者は夏にはおらず，3～5月と11～1月に二峰性のピークを示した．外国人水痘患者が発症した時期は1月，5月，11月であり，日本人の水痘患者が多い時期と一致していた．さらに外国人水痘5例のうち4例は，日本に入国して3週間以上経過後に発症しており，入国してから初感染の成人水痘を発症した可能性が高いと考えた．来日する水痘に免疫のない外国人労働者に対して，水痘ワクチン接種の必要性を勧奨することが重要である．

　三叉神経第1枝（以下，V1）領域の帯状疱疹に合併する眼球運動障害は半年以内に改善することが多いとされているが，今回我々は，80歳女性の帯状疱疹に伴う左眼の外転不全が1年半以上遷延した症例について報告する．10日前より皮膚症状が出現し，近医皮膚科で帯状疱疹と診断され治療するも改善せず，当院皮膚科に紹介となり，同日に当科コンサルトとなった．初診時，強い左眼瞼腫脹と左V1領域に水疱と紅斑を認め，前眼部や網膜硝子体に炎症所見は認めないが，左眼外転不全と内斜視を認めた．複視の強い訴えはなく，明らかな頭蓋内病変や外眼筋の肥厚・炎症所見は認められなかった．経過観察を行い，徐々に眼位は改善したが，左眼の外転不全は発症1年半が経過しても残存した．V1領域の帯状疱疹が外眼筋麻痺を合併した症例は各国から報告されているが，1年以上遷延した症例は稀である．V1領域の帯状疱疹においては，角膜や眼内炎症所見だけでなく，眼位や眼球運動にも注意深い観察が必要であるとともに，眼瞼腫脹が強く患者が複視を訴えない例もあるが，遷延する外眼筋麻痺を伴っていることもあるため，初診時や経過中も必ず評価することが必要であると考える．

63歳女性例，右耳介部・左肩の複発性帯状疱疹（herpes zoster，以下HZと略記）に伴い右Ramsay Hunt症候群と左C5～6神経根性疼痛・左上肢運動麻痺を呈し，頸椎MRI 3D nerve viewにおいてC5～C8神経根が椎間孔外側で高信号，造影T₁WI脂肪抑制像にて増強を示した．上肢正中・尺骨運動感覚神経伝導速度・F波には異常を認めなかった．神経障害を伴う対側複発性HZは極めて稀であり，本例を類似症例と比較し，HZに伴う末梢神経合併症における多発根神経炎の存在と免疫グロブリン大量静注療法の有用性について考察を加えた．

採血時に皮膚病変に気付き，血液検査で大型の顆粒リンパ球を確認し，骨髄浸潤を伴う悪性リンパ腫を推測し得た皮膚ENKTLの一例を経験したので報告した。症例は26才，女性。当院受診1か月前に右上腕内側皮膚に結節を自覚した。初期は紫斑様で，疼痛があり，次第に増大・肥厚して鶏卵大となり，潰瘍形成を示した。結節は腹部，腰部，下腿にも出現した。その後，当院受診時の採血の際，患者上腕に結節を認め，「全身に結節が生じている」という患者の訴えから，悪性リンパ腫を疑った。その末梢血液像でAzur顆粒を有する大型のリンパ球を多数認めたため，直ちに血液内科医に報告した。後日，皮膚生検の病理組織学的検索と骨髄の検索から骨髄浸潤を伴う皮膚ENKTLと診断された。採血中，注意深く患者を観察し，会話から情報を得ることで疾患をいち早く推測し得た症例であった。

In the treatment of intratemporal bone facial nerve schwannoma, those with a mild paralysis score are often considered for observation, while those with a score of 20 or less are often considered for total excision, including nerve reconstruction. Also, if the paralysis progresses during follow-up, facial nerve decompression surgery is recommended. At our institution, we have obtained good results with transmastoid and transmidcranial fossa facial nerve decompression surgeries. Nerve decompression surgery is associated with a low risk of functional impairment and is less likely to interfere with any additional treatment that may be required afterwards, so that it seems appropriate to widen the range of surgical indications.