今日の臨床サポート 今日の臨床サポート

著者: 西村忠己 奈良県立医科大学 耳鼻咽喉・頭頸部外科学

監修: 森山寛1) 東京慈恵会医科大学附属病院

監修: 小島博己2) 東京慈恵会医科大学 耳鼻咽喉科

著者校正/監修レビュー済:2025/03/12
参考ガイドライン:
  1. 日本耳科学会:Baha適応基準(2023)
  1. 日本耳科学会:骨導インプラントBONEBRIDGE®の適応基準(2020)
  1. 日本耳科学会:人工中耳VSB適応基準(2019)
患者向け説明資料

改訂のポイント:
  1. 定期レビューを行い、以下について加筆・修正した。
  1. 外耳道真珠腫について加筆した。
  1. 治療方針について新しく加わった選択肢を加筆する形で修正した。
  1. 軟骨伝導補聴器、ADHEARについて加筆した。
  1. Baha適応基準が更新されたため、内容を修正した。
  1. 骨導インプラント、人工中耳VSBの適応基準について加筆した。

概要・推奨   

  1. 詳細な耳介・外耳の観察(推奨度1)
  1. 年齢に応じた聴力検査を施行し、両側の聴力の程度を確認する(推奨度1)
  1. 側頭骨CTにより外耳/中耳/内耳および周囲組織の形態異常の有無、外耳道真珠種の有無の確認(推奨度1)
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病態・疫学・診察 

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最新のエビデンスに基づいた二次文献データベース「今日の臨床サポート」。
常時アップデートされており、最新のエビデンスを各分野のエキスパートが豊富な図表や処方・検査例を交えて分かりやすく解説。日常臨床で遭遇するほぼ全ての症状・疾患から薬剤・検査情報まで瞬時に検索可能です。

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文献 

Flint: Cummings Otolaryngology: Head & Neck Surgery, 5th ed.
Jahrsdoerfer RA, Yeakley JW, Aguilar EA, Cole RR, Gray LC.
Grading system for the selection of patients with congenital aural atresia.
Am J Otol. 1992 Jan;13(1):6-12.
Abstract/Text It is generally recognized that surgery for congenital aural atresia is difficult. In an effort to select those patients who have the greatest chance of success, we have developed a grading scheme based on the preoperative temporal bone CT scan and the appearance of the external ear. Patients are graded on a possible best score of 10. The stapes is assigned the highest rating (2 points), while all other entrees on the scale are 1 point. The grade assigned preoperatively has been shown to correlate well with the patient's chance of success, herein defined as a postoperative speech reception threshold of 15 to 25 dB. A patient with a preoperative grade of 8/10 would, therefore, have a 80 percent chance of achieving this threshold. Patients with scores of 5/10, or less, are not considered surgical candidates, because the risk of the operation would outweigh the potential benefits. We have found that the grading system allows us to avoid impossible surgical cases while allowing for a reasonable prediction of the hearing outcome.

PMID 1598988
De la Cruz A, Teufert KB.
Congenital aural atresia surgery: long-term results.
Otolaryngol Head Neck Surg. 2003 Jul;129(1):121-7. doi: 10.1016/S0194-59980300531-X.
Abstract/Text OBJECTIVES: The study goal was to evaluate the stability of hearing results from short- to long-term follow-up in patients who underwent surgery for congenital aural atresia. We also reviewed complications such as external auditory canal stenosis, lateralization and perforation of the tympanic membrane, sensorineural hearing loss, and facial palsy.
METHODS: We conducted a retrospective chart review of 116 patients who underwent congenital aural atresiaplasty between 1985 and 2002 at the House Ear Clinic. There were 116 atretic ears. Complication rates and short- and long-term (up to 13.6 years) hearing results were evaluated for primary and revision cases.
RESULTS: Closure of the air-bone gap (ABG) to 30 dB or less at short-term follow-up occurred in 58.5% of primary surgeries and 56% of revisions. The long-term postoperative ABG was 30 dB or less in 50.8% of the primary cases and 39.1% of the revisions. Paired comparison analysis found no significant change in ABG from short- to long-term follow-up for either primary or revision cases. Soft tissue stenosis was seen in 8% of primary surgeries and 3.4% of revisions. Ossicular chain refixation was seen in 11.5% and 6.9% of primary and revision surgeries, respectively. There were no dead ears.
CONCLUSION: Atresiaplasty surgery in individuals with congenital aural atresia can yield reliable, lasting hearing results with a low incidence of complications.

PMID 12869927
Oliver ER, Hughley BB, Shonka DC, Kesser BW.
Revision aural atresia surgery: indications and outcomes.
Otol Neurotol. 2011 Feb;32(2):252-8. doi: 10.1097/MAO.0b013e3182015f27.
Abstract/Text OBJECTIVE: To determine the most common indications for revision congenital aural atresia (CAA) surgery and the postoperative healing and hearing outcomes of revision surgery.
STUDY DESIGN: Retrospective case review.
SETTING: Tertiary care academic otologic practice.
PATIENTS: Patients undergoing revision surgery for CAA.
INTERVENTION: Revision surgery for CAA.
MAIN OUTCOME MEASURES: Indications for revision atresiaplasty, time to revision surgery, postoperative external auditory canal (EAC) patency, incidence of chronic drainage and/or infection, and postoperative speech reception thresholds (SRTs), and air-bone gaps.
RESULTS: Indications for 75 ears (69 patients) undergoing 107 revision operations for CAA included 58% for EAC stenosis, 19% for chronic drainage and/or infection, and 20% for conductive hearing loss (CHL) alone. Fifty ears (67%) required a single revision. Twenty-five ears (33%) required more than 1 revision. With follow-up longer than 3 months (mean, 41 mo), 69% of ears revised for EAC stenosis achieved a patent canal (29% required >1 revision); 75% of ears revised for chronic drainage and/or infection (mean follow-up, 53 mo) realized a dry canal (22% required >1 revision). For all revision surgeries with adequate follow-up (n = 80), the mean postoperative short-term SRT of 24 dB HL was a significant improvement from the mean preoperative SRT of 39 dB HL (p < 0.01, paired t test).
CONCLUSION: EAC stenosis is the most common indication for revision atresiaplasty. Despite the challenges of revision surgery, improvement in canal patency, epithelialization, and hearing utcomes can be achieved.

PMID 21178807
Digoy GP, Cueva RA.
Congenital aural atresia: review of short- and long-term surgical results.
Otol Neurotol. 2007 Jan;28(1):54-60. doi: 10.1097/01.mao.0000227897.73032.95.
Abstract/Text OBJECTIVES/HYPOTHESIS: To review the short- (<1 yr) and long-term (>1 yr) surgical and hearing outcomes for congenital aural atresia and to analyze the stability of surgical results over time.
STUDY DESIGN: Retrospective chart review of 45 patients (54 ears) who underwent surgery for congenital aural atresia during a 5-year period at a tertiary care institution.
METHODS: Preoperative and postoperative speech reception thresholds, air-bone gaps (ABGs), and pure-tone averages were compared and further analyzed for outcome stability over time. The complication rate was reviewed and compared with results from similar studies.
RESULTS: Approximately 50% of patients achieved a speech reception threshold of 30 dB or better both in the short and long term. The average improvement in ABG was 22 dB, resulting in a postoperative ABG of 30 dB or less in three of four patients. Short- and long-term outcomes were not significantly different. Patients with an intact ossicular chain did not seem to have a significant advantage in hearing when compared with patients with a prosthetic reconstruction prosthesis. We report a low incidence of meatal stenosis compared with other similar reviews and describe surgical modifications that may attribute to this outcome.
CONCLUSION: Overall, the mean hearing outcome for this group collectively did not significantly degrade over time and compared favorably with other series. However, there was significant variability among individual patients. The safety of this procedure and the demonstrated hearing improvement makes it a reasonable option in patients with congenital aural atresia with favorable anatomy.

PMID 17195747
Schuknecht HF.
Congenital aural atresia.
Laryngoscope. 1989 Sep;99(9):908-17. doi: 10.1288/00005537-198909000-00004.
Abstract/Text This report is based on 25 years' experience in the surgical management of 69 ears with congenital aural atresia. There were 7 ears with meatal atresia, 11 with partial atresia, 50 with total atresia, and 1 with hypopneumatic total atresia. Meatoplasty was performed in 7 ears, canaloplasty was performed in 22 ears, canaloplasties with strust in 8 ears, and mastoidectomy with stapediopexy was performed in 26 ears. Of the 62 ears that had partial or total atresia, five (8%) had hidden cholesteatomas that were destined to create future complications by expansion and erosion had they not been removed. In five of the 62 ears, temporary facial palsy resulted from exposing the facial nerve in the atresia plate, and in another five ears, attempts to improve hearing were abandoned intraoperatively because of surgically insurmountable anomalies. A reasonable criterion for success in an operation for bilateral atresia is that the need for a hearing aid is obviated; whereas, for unilateral atresia, a successful procedure should largely eliminate the disadvantages of unilateral hearing loss. These criteria seem to be satisfied if the threshold of hearing can be brought to a level of 20 dB or better. This criterion was met in 30% of the group that underwent canaloplasty and in 8% of the group that underwent mastoidectomy with stapediopexy.

PMID 2770382
薬剤監修について:
オーダー内の薬剤用量は日本医科大学付属病院 薬剤部 部長 伊勢雄也 以下、渡邉裕次、井ノ口岳洋、梅田将光および日本医科大学多摩永山病院 副薬剤部長 林太祐による疑義照会のプロセスを実施、疑義照会の対象については著者の方による再確認を実施しております。
※薬剤中分類、用法、同効薬、診療報酬は、エルゼビアが独自に作成した薬剤情報であり、 著者により作成された情報ではありません。
尚、用法は添付文書より、同効薬は、薬剤師監修のもとで作成しております。
※同効薬・小児・妊娠および授乳中の注意事項等は、海外の情報も掲載しており、日本の医療事情に適応しない場合があります。
※薬剤情報の(適外/適内/⽤量内/⽤量外/㊜)等の表記は、エルゼビアジャパン編集部によって記載日時にレセプトチェックソフトなどで確認し作成しております。ただし、これらの記載は、実際の保険適応の査定において保険適応及び保険適応外と判断されることを保証するものではありません。また、検査薬、輸液、血液製剤、全身麻酔薬、抗癌剤等の薬剤は保険適応の記載の一部を割愛させていただいています。
(詳細はこちらを参照)
著者のCOI(Conflicts of Interest)開示:
監修:森山寛 : 特に申告事項無し[2025年]
監修:小島博己 : 特に申告事項無し[2025年]

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