What is the likely diagnosis and recommended management for a patient with bilateral high‑frequency sensorineural hearing loss, low‑frequency asymmetry at 250‑500 Hz, normal brain MRI, and tinnitus for over two months that started bilaterally and is now left‑only?

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雙側高頻聽力下降伴低頻不對稱及單側耳鳴的診斷與處理

最可能診斷

呢個病例最可能係特發性突發性感音神經性聽力損失（SSNHL），但低頻不對稱（250-500 Hz）同埋由雙耳鳴轉為單側左耳鳴嘅表現需要排除自身免疫性內耳疾病或美尼爾氏病。 1, 2

診斷考慮要點

  • 雙側突發性聽力損失係罕見嘅，只佔所有SSNHL病例嘅1.7%，必須積極排除嚴重系統性疾病 2, 3
  • 低頻不對稱（250-500 Hz）係美尼爾氏病嘅典型特徵，特別係伴有耳鳴同耳脹感 1
  • 耳鳴由雙側轉為單側左耳可能提示病情演變或一側恢復 4
  • 正常MRI已排除前庭神經鞘瘤、腦膜瘤等後耳蝸病變 1

緊急處理方案

即時治療（如果仍在發病2週內）

必須立即開始全身性皮質類固醇治療，因為早期治療（2週內）預後較好 1, 5

  • 口服高劑量類固醇係首選治療 1, 6
  • 如果口服類固醇有禁忌症，可考慮鼓室內注射類固醇 1
  • 如果初始治療後恢復不完全，應提供補救治療（通常係鼓室內類固醇） 1

必須完成嘅檢查

全面實驗室檢查係必須嘅，因為雙側SSNHL唔應該假設係特發性 2, 5, 3

自身免疫/炎症檢查

  • 抗核抗體（ANA）滴度 2, 5
  • 紅血球沉降率（ESR）同C反應蛋白（CRP） 5, 7
  • 血管緊張素轉換酶水平 5
  • 抗磷脂抗體 5

感染性病因篩查

  • 梅毒血清學（FTA-abs） 8, 5
  • HIV檢測 8, 5
  • 萊姆病檢測 5
  • 病毒血清學 5

其他必要檢查

  • 全血細胞計數 5
  • 甲狀腺功能測試 5
  • 血清蛋白電泳 5

專科轉介時機

如果有以下特徵，必須在1-2週內轉介風濕科 2：

  • 雙側表現伴有或不伴有波動性聽力損失 2, 7
  • 復發性突發性聽力損失 2
  • 實驗室檢查提示自身免疫疾病 2, 7

預後評估

聽力恢復可能性

  • 約三分之一至三分之二嘅SSNHL患者可能在2週內自行恢復部分聽力 1
  • 在最初2週內恢復一半聽力嘅患者預後較好 1
  • 最初2週內變化極少嘅患者唔太可能有顯著恢復 1
  • 雙側SSNHL嘅預後比單側差，聽力恢復較差，死亡率達35% 3

耳鳴預後

  • 輕度至中度聽力損失嘅患者，耳鳴響度會迅速下降，三個月後三分之二患者耳鳴完全消失 4
  • 聽力恢復先於耳鳴消失 4
  • 嚴重至極重度聽力損失相關嘅耳鳴改善明顯較少 4

後續監測

必須在初次診斷後6個月內進行隨訪聽力測試 1, 8

需要警惕嘅情況

  • 如果聽力繼續惡化或出現新嘅神經系統症狀，需要重新評估 1
  • 如果出現眩暈，預後較差 1
  • 如果言語識別分數（WRS）比純音平均值（PTA）預期嘅差，需要評估後耳蝸病變可能 1

關鍵注意事項

  • 絕對唔好單純用類固醇治療雙側SSNHL而唔做全面檢查，因為可能會延誤診斷危及生命嘅系統性疾病 2, 5, 3
  • 唔好常規訂購頭部CT，MRI先係標準影像學檢查 2, 5
  • 低頻不對稱提示可能係美尼爾氏病，需要長期隨訪評估係咪有波動性聽力損失同復發性眩暈 1
  • 如果係自身免疫性內耳疾病，及時免疫抑制治療可能避免聽力後遺症 7

References

1
Guideline

Guideline Directed Topic Overview

Dr.Oracle Medical Advisory Board & Editors, 2025

2
Guideline

Sudden Sensorineural Hearing Loss: Diagnosis and Management

Praxis Medical Insights: Practical Summaries of Clinical Guidelines, 2026

3
Research

Bilateral sudden sensorineural hearing loss: review.

The Journal of laryngology and otology, 2014

4
Research

Characteristics and Spontaneous Recovery of Tinnitus Related to Idiopathic Sudden Sensorineural Hearing Loss.

Otology & neurotology : official publication of the American Otological Society, American Neurotology Society [and] European Academy of Otology and Neurotology, 2016

5
Guideline

Investigations for Bilateral Sudden Sensorineural Hearing Loss

Praxis Medical Insights: Practical Summaries of Clinical Guidelines, 2026

6
Research

Sudden sensorineural hearing loss.

Lancet (London, England), 2010

8
Guideline

Management of Sudden Bilateral Hearing Loss in HIV Patients

Praxis Medical Insights: Practical Summaries of Clinical Guidelines, 2026

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Professional Medical Disclaimer

This information is intended for healthcare professionals. Any medical decision-making should rely on clinical judgment and independently verified information. The content provided herein does not replace professional discretion and should be considered supplementary to established clinical guidelines. Healthcare providers should verify all information against primary literature and current practice standards before application in patient care. Dr.Oracle assumes no liability for clinical decisions based on this content.

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