Otosclerosis - Otosclerosis

Japanese: 耳硬化症 - じこうかしょう(英語表記)Otosclerosis
Otosclerosis - Otosclerosis

What is the disease?

Otosclerosis is Ossicles ( Knowledge ) This is a disease in which the stapes, which is located deepest in the ear and transmits vibrations to the inner ear, becomes less mobile. The space between the footplate of the stapes and the surrounding bones becomes less mobile. Conductive hearing loss ( Electricity ) As the condition progresses, Sensorineural hearing loss ( Kanonancho ) or Mixed hearing loss ( President of the ) It will be.

The disease is caused by genetic factors and is more common in Caucasians than Asians. It is slightly more common in women, and hearing loss can worsen during pregnancy.

How symptoms manifest

In many cases, the main symptom is bilateral progressive conductive hearing loss. In Japan, the age at which people become aware of hearing loss is around 30 years old, and the age at which people visit a hospital and receive a diagnosis is around 40 years old. eardrum ( Komaku ) If your hearing is normal, you may have otosclerosis.

Testing and diagnosis

Hearing tests usually reveal bilateral conductive hearing loss with greater loss in low frequencies.

In addition, tympanometry is used to measure the movement of the eardrum, and diagnosis is based on test findings such as the loss of the stapedius reflex. Although otosclerosis lesions are often not very clear on temporal bone targeted CT, this test is always performed because hearing loss can also be caused by fixation or underdevelopment of other ossicles.

Treatment methods

Hearing loss caused by otosclerosis is one of the few types of hearing loss where surgery can dramatically improve hearing. Because medical treatments are still under research, people who do not want to undergo surgery use hearing aids.

If the hearing loss is mild, surgery is not necessary. Air-bone gap ( Transportation ) Surgery is performed when the hearing loss exceeds 20-30 decibels. In cases of mixed hearing loss where sensorineural hearing loss has also occurred and the hearing loss is severe, surgery is performed to reduce the air-bone gap, and further treatment is performed using hearing aids. The effectiveness of wearing hearing aids can be significantly improved by surgery.

Stapes surgery is usually performed under general anesthesia. Various techniques have been developed to restore mobility to the fixed stapes footplate. These include removing the entire or part of the fixed footplate after removing the stapes superstructure, or drilling a small hole of 0.6 mm in the footplate and inserting a piston into it.

Recently, specialized microdrills and lasers have been developed, making surgery safer and more reliable.

Key points for examination

For patients with otosclerosis, stapes surgery can often improve hearing, but there is also the possibility of hearing loss due to inner ear disorders, so be sure to see a doctor who specializes in ear surgery.

Tetsuro Ikezono

Source: Houken “Sixth Edition Family Medicine Encyclopedia” Information about the Sixth Edition Family Medicine Encyclopedia

Japanese:

どんな病気か

 耳硬化症は、耳小骨(じしょうこつ)のなかで最も深部にあって、内耳に振動を伝えているアブミ骨が動きにくくなる病気です。アブミ骨底板と周囲の骨との間が動きにくくなり、伝音難聴(でんおんなんちょう)を起こします。進行すると感音難聴(かんおんなんちょう)混合難聴(こんごうなんちょう)になります。

 発症には遺伝的要因があり、東洋人より白人に多い病気です。女性にやや多く、妊娠によって難聴が悪化することもあります。

症状の現れ方

 両側進行性の伝音難聴が主症状となる場合が多く、日本人では難聴自覚年齢が30歳ころ、病院を受診し診断がつく年齢は40歳ころにピークがあります。思春期以降に発症する進行性伝音難聴で鼓膜(こまく)が正常であれば、耳硬化症の可能性が高くなります。

検査と診断

 聴力検査では通常両側性で、低音部により難聴がある伝音難聴です。

 そのほか、ティンパノメトリーで鼓膜の動きを測り、アブミ骨筋反射の消失などの検査所見を参考に診断します。側頭骨ターゲットCTでは、耳硬化症の病変はあまりはっきりしないことが多いですが、他の耳小骨の固着や発育不全が原因の難聴もあるため、必ずこの検査を行います。

治療の方法

 耳硬化症による難聴は、手術で劇的な聴力改善が得られる数少ない難聴のひとつです。薬による治療はまだ研究段階なので、手術を希望しない人は補聴器を使います。

 難聴が軽い場合は手術の必要はなく、気導骨導差(きどうこつどうさ)が20~30㏈(デシベル)以上になったら手術を行います。感音難聴も起きてしまっている混合難聴で、難聴が高度の場合は手術して気導骨導差を縮小させ、さらに補聴器を使った治療を行います。補聴器の装用効果は手術でかなり改善します。

 通常、全身麻酔でアブミ骨手術を行います。固着したアブミ骨底板の可動性を回復する技術がいろいろと開発されています。アブミ骨上部構造を摘出後に固着した底板全体または一部を摘出する方法、底板に0.6㎜の小孔をあけて、そこにピストンを入れる方法などがあります。

 最近は専用のマイクロドリルやレーザーが開発され、より安全、確実な手術が可能になってきました。

受診のポイント

 耳硬化症はアブミ骨手術により高い率で聴力の改善が得られますが、逆に内耳障害により聴覚を喪失する可能性もあるので、耳の手術を専門としている医師による診察を受けてください。

池園 哲郎

出典 法研「六訂版 家庭医学大全科」六訂版 家庭医学大全科について 情報

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