今日の臨床サポート

先天性外耳道閉鎖症

著者: 武市紀人1) しのろ耳鼻咽喉科クリニック

著者: 福田諭2) 北海道医療大学病院 耳鼻咽喉科

監修: 森山寛 東京慈恵会医科大学附属病院

著者校正/監修レビュー済:2021/02/10
患者向け説明資料
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、林太祐、渡邉裕次、井ノ口岳洋、梅田将光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。
※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、実際の保険適用の査定において保険適用及び保険適用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用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:
武市紀人 : 特に申告事項無し[2021年]
福田諭 : 特に申告事項無し[2021年]
監修:森山寛 : 特に申告事項無し[2021年]

改訂のポイント:
  1. 定期レビューを行った。

病態・疫学・診察

疾患(疫学・病態)  
  1. 胎生期における第1鰓溝の発生異常により外耳道閉鎖が生じる疾患である。
  1. 発症率は出生1~2万人に1人である。また、全体の1/3~1/4が両側性で、残りが片側性である[1]
  1. 大部分は軟骨部と骨部外耳道の全長が骨性に閉鎖している。一部に膜性の外耳道閉鎖がある。
  1. 聴力は中等度から重度の伝音難聴であることが多い。
  1. 合併症として小耳症(耳介低形成)が最も多く、ほかに顔面神経麻痺、小顎症、口蓋裂、頰骨低形成、咀嚼・嚥下障害などがある。
  1. 両側例は乳幼児期より積極的な補聴および言語指導が必要となる。また、小耳症合併例は耳介形成術を含めた手術適応の検討が必要になる。一方、片側例は必ずしも積極的な治療を要さないことが多い。
問診・診察のポイント  
問診:
  1. 診断は視診で容易であり、出生直後に確定診断されている場合が多い。

今なら12か月分の料金で14ヶ月利用できます(個人契約、期間限定キャンペーン)

11月30日(火)までにお申込みいただくと、
通常12ヵ月の使用期間が2ヶ月延長となり、14ヵ月ご利用いただけるようになります。

詳しくはクリック
本サイトの知的財産権は全てエルゼビアまたはコンテンツのライセンサーに帰属します。私的利用及び別途規定されている場合を除き、本サイトの利用はいかなる許諾を与えるものでもありません。 本サイト、そのコンテンツ、製品およびサービスのご利用は、お客様ご自身の責任において行ってください。本サイトの利用に基づくいかなる損害についても、エルゼビアは一切の責任及び賠償義務を負いません。 また、本サイトの利用を以て、本サイト利用者は、本サイトの利用に基づき第三者に生じるいかなる損害についても、エルゼビアを免責することに合意したことになります。  本サイトを利用される医学・医療提供者は、独自の臨床的判断を行使するべきです。本サイト利用者の判断においてリスクを正当なものとして受け入れる用意がない限り、コンテンツにおいて提案されている検査または処置がなされるべきではありません。 医学の急速な進歩に鑑み、エルゼビアは、本サイト利用者が診断方法および投与量について、独自に検証を行うことを推奨いたします。

文献 

著者: R A Jahrsdoerfer, J W Yeakley, E A Aguilar, R R Cole, L C Gray
雑誌名: 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  Am J Otol. 1992 Jan;13(1):6-12.
著者: Antonio De la Cruz, Karen Borne Teufert
雑誌名: Otolaryngol Head Neck Surg. 2003 Jul;129(1):121-7.
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  Otolaryngol Head Neck Surg. 2003 Jul;129(1):121-7.
著者: Eric R Oliver, Brian B Hughley, David C Shonka, Bradley W Kesser
雑誌名: 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  Otol Neurotol. 2011 Feb;32(2):252-8. doi: 10.1097/MAO.0・・・
著者: G Paul Digoy, Roberto A Cueva
雑誌名: 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  Otol Neurotol. 2007 Jan;28(1):54-60. doi: 10.1097/01.ma・・・
著者: H F Schuknecht
雑誌名: 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  Laryngoscope. 1989 Sep;99(9):908-17. doi: 10.1288/00005・・・

ページ上部に戻る

戻る

さらなるご利用にはご登録が必要です。

こちらよりご契約または優待日間無料トライアルお申込みをお願いします。

(※トライアルご登録は1名様につき、一度となります)


ご契約の場合はご招待された方だけのご優待特典があります。

以下の優待コードを入力いただくと、

契約期間が通常12ヵ月のところ、14ヵ月ご利用いただけます。

優待コード: (利用期限:まで)

ご契約はこちらから